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Sample records for acute systemic vasculitis

  1. Acute necrotizing retinal vasculitis as onset of systemic lupus erythematosus

    PubMed Central

    Monov, Simeon; Hristova, Ruska; Dacheva, Rositza; Toncheva, Reni; Shumnalieva, Russka; Shoumnalieva-Ivanova, Viara; Monova, Daniela

    2017-01-01

    Abstract Rationale: Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterized by autoantibody production, complement activation, and deposition of immune complexes in tissues and organs. SLE can involve any region of the visual system. Although ocular manifestations are not part of the classification criteria for SLE, they can be observed in up to one-third of the patients with SLE. They are rarely reported at the time of disease onset. Retinal vasculitis is usually associated with active generalized disease. Due to its low frequency, we report a case of acute necrotizing retinal vasculitis as onset of SLE. Patient concerns and diagnosis: A 25-year-old white female was referred to the rheumatology clinic with gradually and rapid deterioration of the vision due to abnormal vessel permeability in the right fundus with edema along the vessels, occlusion of arterial branches in the middle periphery with leakage of the dye in these areas and indentical but less prominent changes with cotton wool spots in the papillomacular area and extensive hemorrhages in the left eye. The onset of malar rash, arthralgias and positive antinuclear, anti-double stranded DNA, anti-ribosomal P and anti-β2 glycoprotein I antibodies with decreased C4 complement levels, as well as the positive lupus-band test confirmed the diagnosis of SLE. Interventions: Aggressive immunomodulating therapy with high-dose methylprednisolone, intravenous immunoglobulin, and cyclophosphamide was used for suppression of the disease activity followed by azathioprine as maintaince therapy. Outcomes: Substantial improvement and partial resorption of the vasculitic changes, including central retinal artery and vein, was achieved prominently in the left eye. The study was conducted in accordance with the Declaration of Helsinki and written informed consent was obtained from the patient. Because of this, there is no need to conduct special ethic review and the ethical approval is not necessary

  2. Systemic vasculitis.

    PubMed

    Sharma, Poonam; Sharma, Sanjeev; Baltaro, Richard; Hurley, John

    2011-03-01

    The systemic vasculitides are characterized by inflammation of blood vessel walls. Vessels of any type, in any organ can be affected, resulting in a broad spectrum of signs and symptoms. The heterogenous nature of vasculitides presents a diagnostic challenge. The American College of Rheumatology classification criteria and the Chapel Hill Consensus Conference nomenclature are the most widely used to distinguish different forms of vasculitis. The Chapel Hill Consensus Conference nomenclature defines 10 primary vasculitides based on vessel size (large, medium, and small). The diagnosis relies on the recognition of a compatible clinical presentation supported by specific laboratory or imaging tests and confirmatory histology. Antineutrophilic cytoplasmic antibody testing has been of particular benefit in defining a subgroup of small vessel vasculitides. Treatment is based on clinical presentation and the pattern of organ involvement. Glucocorticoids are the primary treatment for many forms of vasculitis. Additional immunosuppressive agents, including methotrexate and cyclophosphamide, are sometimes required. Newer approaches, such as the use of anti-tumor necrosis factor or B cell therapies, are being tried in resistant cases. Patients can experience considerable treatment-related toxicity, especially infection from immunosuppressive therapy and adverse effects from steroids (e.g., osteoporosis, diabetes mellitus, cataract). Vitamin D and calcium prophylaxis are recommended in patients on long-term steroid therapy.

  3. Damage assessment in systemic vasculitis.

    PubMed

    Silveira, Luis H

    2008-12-01

    Systemic vasculitides were initially reported as acute, progressive, severe, and life-threatening diseases. The introduction of glucocorticoids and cyclophosphamide for the treatment of vasculitis improved survival dramatically, but morbidity has remained high. Damage develops as a consequence of recurrent or persistent active vasculitis or its treatment. It is defined as the accumulation of nonhealing scars that are unlikely to respond to immunosuppressive therapy. Damage assessment is essential in systemic vasculitis because it may facilitate patient stratification in clinical trials and possibly in clinical practice. Moreover, it may avoid unnecessary use of immunosuppressive therapy. The Vasculitis Damage Index, developed and validated in 1997, has been very useful in solving many matters in systemic vasculitis and is currently the only validated damage-assessment tool available. However, the vasculitis community has recognized that there is a growing need to improve the evaluation of damage in vasculitis. The development of a Combined Damage Assessment index, which would permit a more appropriate and standardized approach to disease assessment applicable to systemic vasculitis, has been proposed.

  4. Central Nervous System Vasculitis

    MedlinePlus

    ... Alert Bracelet More information on CNS Vasculitis Patient Information Book Vasculitis Terms A – Z VF Medical Consultants YouTube Education ... Mission The Vasculitis Foundation supports and empowers ...

  5. Silica exposure and systemic vasculitis.

    PubMed Central

    Mulloy, Karen B

    2003-01-01

    Work in Department of Energy (DOE) facilities has exposed workers to multiple toxic agents leading to acute and chronic diseases. Many exposures were common to numerous work sites. Exposure to crystalline silica was primarily restricted to a few facilities. I present the case of a 63-year-old male who worked in DOE facilities for 30 years as a weapons testing technician. In addition to silica, other workplace exposures included beryllium, various solvents and heavy metals, depleted uranium, and ionizing radiation. In 1989 a painful macular skin lesion was biopsied and diagnosed as leukocytoclastic vasculitis. By 1992 he developed gross hematuria and dyspnea. Blood laboratory results revealed a serum creatinine concentration of 2.1 mg/dL, ethrythrocyte sedimentation rate of 61 mm/hr, negative cANCA (antineutrophil cytoplasmic antibody cytoplasmic pattern), positive pANCA (ANCA perinuclear pattern), and antiglomerular basement membrane negative. Renal biopsy showed proliferative (crescentric) and necrotizing glomerulonephritis. The patient's diagnoses included microscopic polyangiitis, systemic necrotizing vasculitis, leukocytoclastic vasculitis, and glomerulonephritis. Environmental triggers are thought to play a role in the development of an idiopathic expression of systemic autoimmune disease. Crystalline silica exposure has been linked to rheumatoid arthritis, scleroderma, systemic lupus erythematosus, rapidly progressive glomerulonephritis and some of the small vessel vasculitides. DOE workers are currently able to apply for compensation under the federal Energy Employees Occupational Illness Compensation Program (EEOICP). However, the only diseases covered by EEOICP are cancers related to radiation exposure, chronic beryllium disease, and chronic silicosis. PMID:14644669

  6. Educational Needs of Patients With Systemic Vasculitis

    ClinicalTrials.gov

    2014-07-11

    Behcet's Disease; Churg-Strauss Syndrome; Vasculitis, Central Nervous System; Giant Cell Arteritis; Wegener Granulomatosis; Henoch-Schoenlein Purpura; Microscopic Polyangiitis; Polyarteritis Nodosa; Takayasu's Arteritis

  7. New insights on biomarkers in systemic vasculitis.

    PubMed

    Perez-Alamino, Rodolfo; Maldonado-Ficco, Hernán

    2015-03-01

    The systemic vasculitis is a heterogeneous group of diseases characterized by the inflammation of blood vessels. The development of advanced diagnostic tests and genetic studies have resulted in greater improvement in our understanding of vasculitis pathogenesis and thus in the development of newer therapies. However, there is still an unmet need in the management of systemic vasculitis, focused on developing of new biomarkers that would enable distinction between active disease from damage or infection and predict treatment response and prognosis.

  8. Clinical Transcriptomics in Systemic Vasculitis (CUTIS)

    ClinicalTrials.gov

    2017-02-21

    Cryoglobulinemic Vasculitis (CV); Drug-induced Vasculitis; Eosinophilic Granulomatosis With Polyangiitis (EGPA); IgA Vasculitis; Isolated Cutaneous Vasculitis; Granulomatosis With Polyangiitis (GPA); Microscopic Polyangiitis (MPA); Polyarteritis Nodosa (PAN); Urticarial Vasculitis; Vasculitis

  9. Challenging mimickers of primary systemic vasculitis.

    PubMed

    Miloslavsky, Eli M; Stone, John H; Unizony, Sebastian H

    2015-01-01

    The need to distinguish true primary systemic vasculitis from its multiple potential mimickers is one of the most challenging diagnostic conundrums in clinical medicine. This article reviews 9 challenging vasculitis mimickers: fibromuscular dysplasia, calciphylaxis, segmental arterial mediolysis, antiphospholipid syndrome, hypereosinophilic syndrome, lymphomatoid granulomatosis, malignant atrophic papulosis, livedoid vasculopathy, and immunoglobulin G4-related disease.

  10. Systemic vasculitis associated with vemurafenib treatment

    PubMed Central

    Mirouse, Adrien; Savey, Léa; Domont, Fanny; Comarmond, Cloé; Barete, Stéphane; Plaisier, Emmanuelle; Rouvier, Philippe; Cacoub, Patrice; Saadoun, David

    2016-01-01

    Abstract Rationale: Vemurafenib, an inhibitor of mutated B-rapidly accelerated fibrosarcoma, is frequently used in the treatment of melanoma and Erdheim–Chester disease (ECD) patients. Inflammatory adverse effects have been increasingly reported after vemurafenib treatment. Patient concerns and diagnose: We report 6 cases of vemurafenib-associated vasculitis, of whom a personal case of a 75-year-old man with history of ECD who developed purpura and rapidly progressive pauci-immune glomerulonephritis during treatment with vemurafenib. Intervention: In the 5 others cases from the literature, all patients presented skin vasculitis, and with joint involvement in 60% of them. Vemurafenib treatment was stopped (n = 3), continued at reduced doses (n = 1), or continued at the same dose (n = 2). Outcomes: Three patients (50%) received corticosteroids combined with cyclophosphamide (n = 1), and all achieved remission of vasculitis. One patient experienced vasculitis relapse after vemurafenib therapy was restarted. Lessons: Systemic vasculitis is a rare vemurafenib-associated adverse event that may be life-threatening. PMID:27861332

  11. Systemic Vasculitis During the Course of Systemic Sclerosis

    PubMed Central

    Quéméneur, Thomas; Mouthon, Luc; Cacoub, Patrice; Meyer, Olivier; Michon-Pasturel, Ulrique; Vanhille, Philippe; Hatron, Pierre-Yves; Guillevin, Loïc; Hachulla, Eric

    2013-01-01

    Abstract Although the presence of antineutrophil cytoplasmic antibodies (ANCA) has been reported in patients with systemic sclerosis (SSc), the association of SSc and systemic vasculitis has rarely been described. We obtained information on cases of systemic vasculitis associated with SSc in France from the French Vasculitis Study Group and all members of the French Research Group on Systemic Sclerosis. We identified 12 patients with systemic vasculitis associated with SSc: 9 with ANCA-associated systemic vasculitis (AASV) and 3 with mixed cryoglobulinemia vasculitis (MCV). In all AASV patients, SSc was of the limited type. The main complication of SSc was pulmonary fibrosis. Only 2 patients underwent a D-penicillamine regimen before the occurrence of AASV. The characteristics of AASV were microscopic polyangiitis (n = 7) and renal limited vasculitis (n = 2). Anti-myeloperoxidase antibodies were found in 8 of the 9 patients. The Five Factor Score was above 1 in 3 of the 9 patients. Of the 3 patients with MCV, Sjögren syndrome was confirmed in 2. We compared our findings with the results of a literature review (42 previously reported cases of AASV with SSc). Although rare, vasculitis is a complication of SSc. AASV is the most frequent type, and its diagnosis can be challenging when the kidney is injured. Better awareness of this rare association could facilitate earlier diagnosis and appropriate management to reduce damage. PMID:23263715

  12. Therapeutic immunomodulation in systemic vasculitis: taking stock.

    PubMed

    Puéchal, Xavier; Guillevin, Loïc

    2013-07-01

    Current data on therapeutic immunomodulation used to treat systemic vasculitides are reviewed in this paper, which also discusses ongoing and future developments in the field. In vasculitides associated with anti-neutrophil cytoplasmic antibodies, rituximab is a validated induction treatment that can serve as an alternative to cyclophosphamide and must be followed by maintenance treatment. In addition, the usefulness of rituximab as maintenance treatment was established recently. Immunoglobulins can be helpful adjuncts, most notably in patients with severe immunodepression. Plasmapheresis is indicated in patients with severe renal failure and may have a role in the treatment of alveolar hemorrhage syndromes. Mepolizumab has produced encouraging preliminary results in eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Rituximab can be used in cryoglobulinemic vasculitis associated with hepatitis C virus infection when antiviral therapy fails or the disease is severe. Very low doses of interleukin-2 may be helpful in refractory forms. Rituximab is also an option in essential mixed cryoglobulinemia with uncontrolled vasculitis despite glucocorticoid and/or immunosuppressive treatment. In polyarteritis nodosa associated with the hepatitis B virus, a combination of short-course glucocorticoids, plasmapheresis, and antiviral therapy produces excellent outcomes. Intravenous immunoglobulins are used to treat Kawasaki disease, in which they diminish the incidence of coronary artery aneurysms. Several prospective controlled trials are currently assessing tocilizumab in giant-cell arteritis. Rituximab has useful effects in systemic vasculitis associated with rheumatoid arthritis. In Goodpasture's syndrome, plasmapheresis is indicated to clear the antibodies to glomerular membrane antigen, which can induce glomerulonephritis.

  13. Impact of Vasculitis on Employment and Income

    ClinicalTrials.gov

    2016-01-26

    Vasculitis; Systemic Vasculitis; Behcet's Disease; CNS Vasculitis; Cryoglobulinemic Vasculitis; Eosinophilic Granulomatosis; Temporal Arteritis; Wegener Granulomatosis; Henoch-Schoenlein Purpura; Microscopic Polyangiitis; Polyarteritis Nodosa (PAN); Takayasu's Arteritis; Urticarial Vasculitis

  14. Segmental arterial mediolysis mimics systemic vasculitis.

    PubMed

    Kalfa, Melike; Kocanaoğulları, Hayriye; Karabulut, Gonca; Emmungil, Hakan; Çınar, Celal; Yılmaz, Zevcet; Gücenmez, Sercan; Kabasakal, Yasemin

    2016-09-01

    Segmental arterial mediolysis (SAM) is a rare, nonarteriosclerotic, noninflammatory vascular disease and mostly affects medium-to-large sized abdominal arteries as well as presents with hemorrhages in the abdominal cavity. We report the case of a patient with SAM of the celiac, right renal, jejunal branch of the superior mesenteric, left gastric, and splenic arteries who was diagnosed by excluding other causes and in whom transcatheter embolization was performed in two different sessions, but he died because of an undefined reason. SAM mimics systemic vasculitis and causes abdominal pain; it should be considered because abdominal hemorrhage or arterial infarction can result in death.

  15. Segmental arterial mediolysis mimics systemic vasculitis

    PubMed Central

    Kalfa, Melike; Kocanaoğulları, Hayriye; Karabulut, Gonca; Emmungil, Hakan; Çınar, Celal; Yılmaz, Zevcet; Gücenmez, Sercan; Kabasakal, Yasemin

    2016-01-01

    Segmental arterial mediolysis (SAM) is a rare, nonarteriosclerotic, noninflammatory vascular disease and mostly affects medium-to-large sized abdominal arteries as well as presents with hemorrhages in the abdominal cavity. We report the case of a patient with SAM of the celiac, right renal, jejunal branch of the superior mesenteric, left gastric, and splenic arteries who was diagnosed by excluding other causes and in whom transcatheter embolization was performed in two different sessions, but he died because of an undefined reason. SAM mimics systemic vasculitis and causes abdominal pain; it should be considered because abdominal hemorrhage or arterial infarction can result in death. PMID:27733945

  16. Cutaneous necrotizing vasculitis. Relation to systemic disease.

    PubMed

    Lotti, T M; Comacchi, C; Ghersetich, I

    1999-01-01

    Cutaneous necrotizing vasculitis (CNV) is a complex multisystem disease generally involving the skin and mucous membranes, often accompanied by renal, gastrointestinal, pericardial, neurological, and articular signs and symptoms. CNV may be idiopatical or occur in association with a drug, infection, or underlying disease. CNV has been shown in patients with chronic infections (viral, bacterial, protozoa, helminthic), serum sickness, a variety of collagen vascular diseases (systemic lupus erythematous, Sjögren's syndrome, rheumatoid arthritis, Behçet's disease) hyperglobulinemic states, cryoglobulinemia, bowel bypass syndrome, ulcerative colitis, cystic fibrosis, primary biliary cirrhosis and HIV infection. Association with malignancies is not frequent. Lymphoproliferative disorders (Hodgkin's disease, mycosis fungoides, lymphosarcoma, adult T-cell leukemia, multiple mieloma) and solid tumors (lung cancer, colon carcinoma, renal, prostate, head and neck cancer and breast cancer) may be associated with CNV. Whenever possible, treatment is directed at the elimination of the cause. In other cases after adequate laboratory screening local and systemic therapy are recommended.

  17. [Central nervous system vasculitis according to the 2012 revise international Chapel Hill consensus conference nomenclature of vasculitides].

    PubMed

    Koike, Haruki; Sobue, Gen

    2015-03-01

    Vasculitis can be primarily or secondarily to various underlying diseases. It frequently affects the nervous system, and neurological deficits may remain even after disease remission. Because the progression of vasculitides is usually acute to subacute, early initiation of treatment is important from the viewpoint of patients' functional status. However, early diagnosis may be difficult, particularly in patients with central nervous system vasculitis. Hence, it is important to understand the wide-ranging clinical manifestations of vasculitides. Here, we summarize the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides from the standpoint of central nervous system vasculitis.

  18. Vasculitis Pregnancy Registry

    ClinicalTrials.gov

    2016-09-08

    Vasculitis; Behcet's Disease; CNS Vasculitis; Cryoglobulinemic Vasculitis; Eosinophilic Granulomatosis With Polyangiitis (EGPA); Churg-Strauss Syndrome (CSS); Granulomatosis With Polyangiitis (GPA); Wegener's Granulomatosis; IgA Vasculitis; Henoch-Schoenlein Purpura (HSP); Microscopic Polyangiitis (MPA); Polyarteritis Nodosa (PAN); Takayasu Arteritis (TAK); Urticarial Vasculitis; Systemic Vasculitis

  19. A Unique Case of Systemic Lupus Erythematosus Pelvic Vasculitis

    PubMed Central

    Basnyat, Shristi; Eid, Hala; Kuzyshyn, Halyna; Feinstein, David

    2016-01-01

    The clinical presentation of Systemic Lupus Erythematosus (SLE) is diverse and vasculitis can be a potential manifestation. Cutaneous lesions involving small vessels are the most frequent presentation. However, medium and large vessel vasculitis may present with life-threatening visceral manifestations. We present a unique case of pelvic vasculitis mimicking a pelvic mass as an initial presentation of SLE. There are case reports of systemic vasculitis involving the female genital tract with giant cell arteritis (GCA), polyarteritis nodosa (PAN), and granulomatous with polyangiitis and microscopic polyangiitis (GPA/MPA), among others, but only a few cases attributed to SLE. Awareness of this condition and a prompt diagnosis are warranted as this is a severe and potentially life-threatening condition. PMID:28127489

  20. An unexpected cause of acute kidney injury in a patient with ANCA associated vasculitis.

    PubMed

    Choudhry, Wajid M; Nori, Uday S; Nadasdy, Tibor; Satoskar, Anjali A

    2016-05-01

    Diagnostic kidney biopsies sometimes yield clinically unsuspected diagnoses. We present a case of a 69-year-old woman with established ANCA-associated vasculitis (AAV) of 4 years duration who was in clinical remission following cytotoxic therapy and was on maintenance immunosuppression. She presented to the hospital with acute kidney injury (AKI), symptoms suggestive of a systemic vasculitis, and in addition had hypercalcemia, metabolic alkalosis. A relapse in the AAV was suspected but a diagnostic kidney biopsy showed acute tubular necrosis, patchy interstitial inflammation, and calcium phosphate deposits. It was found that the patient recently started consuming large doses of over-the-counter calcium-containing antacids and vitamin Dcontaining multivitamin supplements. Cessation of these drugs led to improvement of renal function to baseline. This case highlights several teaching points: (1) the kidney biopsy can prove to be critically important even in cases where there appears to be a more obvious clinical diagnosis, (2) AK due to calcium-alkali syndrome has characteristic histopathological changes, and (3) that the triad of hypercalcemia, metabolic alkalosis, and AKI is exclusively associated with the ingestion of excessive quantities of calcium-containing antacids. The physician should keep this in mind, and pro-actively seek pertinent medication history from the patient. A brief review of calcium-alkali syndrome is given.

  1. Acute brucellosis associated with leukocytoclastic vasculitis and splenic infarct.

    PubMed

    Uçmak, Feyzullah; Uçmak, Derya; Beştaş, Remzi; Anli, Ruken Azizoğlu; Adanir, Haydar

    2014-12-01

    Brucellosis is globally the most prevalent multisystem infection of zoonotic origin, while it is still one of the most important public health problems in Turkey as non-pasteurised milk and dairy products are consumed. Early diagnosis is vital to prevent the possibly lethal complications caused by the disease. However, diagnosis might be delayed as the disease does not have a single and typical manifestation and presents with various symptoms of different systems. Brucellosis and associated splenic infarct have rarely been studied, there being few cases in the literature. One of the rare involvements in this disease is dermatological involvement, which has been found in less than 10 percent of brucellosis cases. In this study, we discuss a 17 year old male patient who was admitted to our hospital due to fever, abdominal pain, arthralgia and rash on legs, diagnosed with brucellosis through brucellosis tube agglutination test and found to have splenic infarct upon examination and leukocytoclastic vasculitis according to the skin biopsies in the light of the present literature.

  2. Vasculitis and rheumatologic diseases may play role in the pathogenesis of acute disseminated encephalomyelitis (ADEM).

    PubMed

    Sabayan, B; Zolghadrasli, Abdolali

    2007-01-01

    Acute disseminated encephalomyelitis (ADEM) is defined as a multifocal, monophasic, demyelinating, and inflammatory disease involving the central nervous system. It typically begins within 6 weeks of an antigenic challenge such as infection or immunization. Perivenous inflammation, edema and demyelination are the pathological hallmarks of ADEM. Reactivity of T-cells against myelin components such as myelin basic protein has been found in children with ADEM. The triggers for immune responses in ADEM are not known, but the two most widely accepted hypotheses are molecular mimicry and self-sensitization secondary to CNS infection. Inflammatory cytokines including tumor necrosis factor alpha (TNFalpha), interleukin 2 (IL2) and interferon gamma (INFgamma) are thought to be important in lesion formation in ADEM. Due to the active role of inflammatory cytokines in the pathogenesis of ADEM, any disease contributing to systemic formation of inflammatory cytokines can potentially be an etiologic factor for the initiation of ADEM. In vasculitis and rheumatologic diseases the number of T-cells, T helper type 1 cytokines and other inflammatory cytokines such as TNFalpha increase substantially. We present this hypothesis that in such setting of inflammation, adhesion molecules are up-regulated on the brain capillary endothelium by cytokines and other inflammatory mediators, altering the permeability of the brain blood barrier and so allowing for inflammatory cell migration. The migratory cells attack the basic myelin protein and the final result is the demyelination seen in ADEM. So we propose that vasculitis and rheumatologic diseases may play role in the pathogenesis of ADEM.

  3. Fulminant systemic vasculitis in systemic lupus erythematosus. Case report and review of the literature.

    PubMed

    Medina, G; González-Pérez, D; Vázquez-Juárez, C; Sánchez-Uribe, M; Saavedra, M A; Jara, L J

    2014-11-01

    Vasculitis in systemic lupus erythematosus (SLE) has a broad spectrum of clinical manifestations from cutaneous to visceral involvement and its prognosis ranges from mild to life-threatening. We report the case of a previously healthy 17-year-old woman with eight months' history of arthralgias and myalgias. Subsequently, she developed facial and lower limbs edema, and hair loss. Two weeks before admission to a secondary level hospital, she developed fever up to 40°C followed by abdominal pain, rectal bleeding, hematemesis and blisters on both legs, reason for which she was hospitalized. With active bullous SLE with rapidly progressive glomerulonephritis suspected, she was treated with methylprednisolone pulses without response. After one week of treatment, she was transferred to a tertiary level hospital. On admission she presented acute arterial insufficiency of the lower extremities, respiratory failure with apnea, metabolic acidosis and shock; six hours later she died. Autopsy findings showed active diffuse lupus nephritis and diffuse systemic vasculitis that involved vessels from the skin, brain, myocardium, spleen, iliac and renal arteries. In addition, serositis of the small intestine and colon, acute and chronic pericarditis, pericardial effusion and myocarditis were found. Immunologic tests confirmed SLE diagnosis. In this case the fulminant course was the result of SLE high disease activity, visceral vasculitis of several organs and late diagnosis, referral and treatment. Early diagnosis, and opportune referral to the rheumatologist for intensive treatment can improve the outlook in these patients.

  4. Epigenomic functional characterization of genetic susceptibility variants in systemic vasculitis.

    PubMed

    Sawalha, Amr H; Dozmorov, Mikhail G

    2016-02-01

    Systemic vasculitides are poorly understood inflammatory diseases of the blood vessels that are frequently associated with significant organ damage. Genetic risk variants contribute to the susceptibility of vasculitis, but functional consequences of these genetic variants are largely unknown. Most genetic risk variants in immune-mediated diseases, including systemic vasculitis, are localized to non-coding genetic regions suggesting they might increase disease risk by influencing regulatory elements within the genome. Long range regulatory interactions pose an additional obstacle in localizing functional consequences associated with risk variants to specific genes or cell types. We used cell-type specific enrichment patterns of histone changes that mark poised, primed, and active enhancers, and DNase hypersensitivity to identify specific immune cells mediating genetic risk in vasculitis. Our data suggest that genetic risk variants in ANCA-associated vasculitis are significantly enriched in enhancer elements in Th17 cells, supporting a role for Th17 cells in this disease. Primed and active enhancer elements in B cells can be potentially affected by genetic risk variants associated with Kawasaki disease. Genetic risk in Behçet's disease and Takayasu arteritis might affect enhancer elements in multiple cell types, possibly explained by influencing enhancers in hematopoietic stem cells. Interestingly, our analyses indicate a role for B cells in Kawasaki disease, Behçet's disease, and Takayasu arteritis, and suggest that further work to characterize the involvement of B cells in these diseases is warranted.

  5. [Treatment of vasculitis].

    PubMed

    Novak, Srdan

    2013-01-01

    Treatment of vasculitis depends on etiology and type of vasculitis. Gluccocorticoids are drug of choice in treatment of systemic vasculitis. While in vasculitis of large vessels treatment with gluccocorticoids is often sufficient, in ANCA associated vasculitis almost always intial combination of gluccocorticoids and immunosupresive drugs (cyclofosfamide in severe forms; azatioprin and metotrrexate in moderate disease) is needed. Maintance therapy of ANCA associated vasculitis is methotrexate or azatioprin. From biologic therapy, in gigantocellular vasculitis treatment with tocilizumb has a great expectation, while in ANCA associated vasculitis recently rituximab was approved by regulatory agencies, and it is nontinferior to cyclofosfamide in induction of remission and prefarable in relapsing disease.

  6. Acute dilated cardiomyopathy in a patient with beriberi and cryoglobulinaemic vasculitis: an unusual potential complication of two rare disorders.

    PubMed

    Tejedor, Ana; Solé, Manel; Prieto-González, Sergio; Alba, Marco Antonio; Grau, Josep Maria; Cid, Maria Cinta; Hernández-Rodríguez, José

    2014-01-01

    We report the case of a 45-year-old patient who presented with acute dilated cardiomyopathy. During admission the patient was consecutively diagnosed with cryoglobulinaemic vasculitis and beriberi. In both diseases, cardiac involvement may occur as dilated cardiomyopathy. Thiamin deficiency was the final cause for the severe cardiac manifestations (cardiac acute beriberi or Shoshin syndrome), which returned to normal after thiamin supplementation.

  7. Vasculitis and gastrointestinal involvement.

    PubMed

    Casella, G; Bronzino, B; Cutrino, L; Montani, N; Somma, A; Baldini, V

    2006-06-01

    The incidence of gastrointestinal involvement is relatively observed in patients with vasculitis processes. Vasculitis can be primary (necrotising or hypersensitivity) or secondary to another primary disease. Gastrointestinal involvement is present in up to 50% of the various forms of systemic vasculitis. Primary or secondary vasculitic process, according to the classification in necrotizing and hypersensitivity vasculitis, are described in this paper. A review of the literature on the the subject is also presented.

  8. Cerebral angiography as a guide for therapy in isolated central nervous system vasculitis

    SciTech Connect

    Stein, R.L.; Martino, C.R.; Weinert, D.M.; Hueftle, M.; Kammer, G.M.

    1987-04-24

    The authors present a case of isolated central nervous system vasculitis documented by cerebral arteriography in which remission, using a treatment regimen of prednisone and cyclophosphamide, was guided by serial arteriography during a 15-month period.

  9. Vasculitis in antiphospholipid syndrome.

    PubMed

    Lally, Lindsay; Sammaritano, Lisa R

    2015-01-01

    The major manifestations of antiphospholipid syndrome (APS) are caused by thrombosis within the venous or arterial vasculature, whereas the vascular lesions in systemic vasculitis result from an inflammatory infiltrate in the vessel wall. There is an association between vascular thrombosis and inflammation, however, as vasculitis can occur in APS and thromboembolic complications are seen in systemic vasculitis. Although differentiating between vasculitis and antiphospholipid-associated thrombosis can be difficult, it may be crucial to do so given the different therapeutic implications for immunosuppression or anticoagulation. This article explores the relationship between thrombosis and inflammation as it relates to APS and systemic vasculitis.

  10. ANCA-Associated Systemic Vasculitis Presenting With Hypertrophic Spinal Pachymeningitis

    PubMed Central

    Li, Xia; Zhao, Jiuliang; Wang, Qian; Fei, Yunyun; Zhao, Yan

    2015-01-01

    Abstract Reports of hypertrophic pachymeningitis associated with myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) localized exclusively in the spine were quite rare. Two cases of ANCA-associated systemic vasculitis (AASV) presenting with hypertrophic spinal pachymeningitis (HSP) causing low back pain and numbness are described. Two patients showed prominent systemic and local inflammatory reactions manifested as fever, elevated levels of erythrocyte sedimentation rate and C-reactive protein, and markedly increased levels of total protein of cerebrospinal fluid. The gadolinium (Gd)-enhanced T1-weighted magnetic resonance imaging scan of spinal cord demonstrated diffuse spinal dura matter thickening. Additionally, simple microscopic hematuria was found in 1 case suggestive of renal involvement and the other 1 complicated with interstitial lung disease. Then, a diagnosis of HSP secondary to AASV was made. Combination therapy of corticosteroids and cyclophosphamide produced a rapid improvement in the clinical symptoms and laboratory parameters. Followed up for 6 months, 1 case relapsed when the dosage of prednisone was tapered to 10 mg daily. Since the patient refused rituximab-based regimen, an immunosuppressive triple-therapy (corticosteroid, cyclophosphamide, and azathioprine) was initiated and brought control of the disease during the subsequent 6 months of follow-up. HSP is a relatively rare form of central nervous system involvement of AASV. Early recognition and intervention are of great significance since the pathogenesis of HSP starts with an inflammatory and fibrosing process. PMID:26579814

  11. The prognostic significance of the Birmingham Vasculitis Activity Score (BVAS) with systemic vasculitis patients transferred to the intensive care unit (ICU)

    PubMed Central

    Biscetti, Federico; Carbonella, Angela; Parisi, Federico; Bosello, Silvia Laura; Schiavon, Franco; Padoan, Roberto; Gremese, Elisa; Ferraccioli, Gianfranco

    2016-01-01

    Abstract Systemic vasculitides represent a heterogeneous group of diseases that share clinical features including respiratory distress, renal dysfunction, and neurologic disorders. These diseases may often cause life-threatening complications requiring admission to an intensive care unit (ICU). The aim of the study was to evaluate the validity and responsiveness of Birmingham Vasculitis Activity Score (BVAS) score to predict survival in patients with systemic vasculitides admitted to ICU. A retrospective study was carried out from 2004 to 2014 in 18 patients with systemic vasculitis admitted to 2 different Rheumatology divisions and transferred to ICU due to clinical worsening, with a length of stay beyond 24 hours. We found that ICU mortality was significantly associated with higher BVAS scores performed in the ward (P = 0.01) and at the admission in ICU (P = 0.01), regardless of the value of Acute Physiology And Chronic Health Evaluation (APACHE II) scores (P = 0.50). We used receiver-operator characteristic (ROC) curve analysis to evaluate the possible cutoff value for the BVAS in the ward and in ICU and we found that a BVAS > 8 in the ward and that a BVAS > 10 in ICU might be a useful tool to predict in-ICU mortality. BVAS appears to be an excellent tool for assessing ICU mortality risk of systemic vasculitides patients admitted to specialty departments. Our experience has shown that performing the assessment at admission to the ward is more important than determining the evaluation before the clinical aggravation causing the transfer to ICU. PMID:27902615

  12. Necrotizing arteritis occurring in an intralobar pulmonary sequestration of a patient without systemic vasculitis syndrome.

    PubMed

    Hashimoto, Hirotsugu; Hara, Kei; Matsumoto, Jun; Nashiro, Tamaki; Nagano, Masaaki; Kusakabe, Masashi; Kurata, Atsushi; Kuroda, Masahiko; Suzuki, Yoshio; Horiuchi, Hajime

    2016-01-01

    Necrotizing arteritis is a complex lesion of pulmonary hypertension, as are plexiform lesions, and is classically recognized as grade 6 in the Heath and Edwards grading scheme for hypertensive pulmonary vascular disease. The vascular changes observed in intralobar pulmonary sequestration have been reported to be similar to those observed in pulmonary hypertension, such as plexiform lesions. However, necrotizing arteritis occurring in an intralobar sequestration of a patient without systemic vasculitis syndrome has never been reported to our knowledge. Here, we report a case of a 38-year-old woman with pulmonary sequestration detected on a medical checkup. She was treated with surgery, and subsequent pathological analyses revealed necrotizing vasculitis in her sequestrated lung. We suspected systemic vasculitis syndromes, such as Takayasu arteritis, polyarteritis nodosa, and antineutrophil cytoplasmic antibody-associated vasculitis. However, physical and blood examination did not show any other abnormalities, and hence, she did not have systemic vasculitis syndrome. Immunohistochemical analyses of the resected specimen showed that inflammatory cells of the arteries were mainly composed of T lymphocytes. T-lymphocytic inflammation with little neutrophil and histiocyte infiltration may be a pathological feature of necrotizing arteritis observed in pulmonary sequestration. This is the first case to our knowledge of necrotizing arteritis in an intralobar pulmonary sequestration of a patient without systemic vasculitis syndrome.

  13. Urticarial Vasculitis

    MedlinePlus

    ... IgA Vasculitis (Henoch-Schönlein Purpura) Hypersensitivity Vasculitis (Leukocytoclastic) Kawasaki Disease Microscopic Polyangiitis Polyarteritis Nodosa Polymyalgia Rheumatica Takayasu’s Arteritis ...

  14. Intravascular immunity as a key to systemic vasculitis: a work in progress, gaining momentum

    PubMed Central

    Ramirez, G A; Maugeri, N; Sabbadini, M G; Rovere-Querini, P; Manfredi, A A

    2014-01-01

    Vascular inflammation contributes to the defence against invading microbes and to the repair of injured tissues. In most cases it resolves before becoming apparent. Vasculitis comprises heterogeneous clinical entities that are characterized by the persistence of vascular inflammation after it has served its homeostatic function. Most underlying mechanisms have so far remained elusive. Intravascular immunity refers to the surveillance of the vasculature by leucocytes that sense microbial or sterile threats to vessel integrity and initiate protective responses that entail most events that determine the clinical manifestations of vasculitis, such as end-organ ischaemia, neutrophil extracellular traps generation and thrombosis, leucocyte extravasation and degranulation. Understanding how the resolution of vascular inflammation goes awry in patients with systemic vasculitis will facilitate the identification of novel pharmacological targets and bring us a step closer in each patient to the selection of more effective and less toxic treatments. PMID:24128276

  15. Systemic lupus erythematosus and antiphospholipid syndrome related retinal vasculitis mimicking ocular cysticercosis: a case report.

    PubMed

    Wu, Chan; Dong, Fang-tian; Chen, You-xin; Wang, Qian; Dai, Rong-ping; Zhang, Hua

    2015-03-01

    Making accurate and timely diagnosis is often challenging when patients with a systemic disease first present with ocular manifestations. The possibility that vasculitis associated with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) can be misdiagnosed as cysticercosis has not been discussed in the literatures.

  16. Allergic vasculitis

    MedlinePlus

    ... damage to blood vessels, primarily in the skin. Causes Hypersensitivity vasculitis is caused by an allergic reaction to ... affects people older than age 15. Often, the cause of the problem cannot be found even with ... vasculitis may look like necrotizing vasculitis , which can ...

  17. Identification of haptoglobin as an angiogenic factor in sera from patients with systemic vasculitis.

    PubMed Central

    Cid, M C; Grant, D S; Hoffman, G S; Auerbach, R; Fauci, A S; Kleinman, H K

    1993-01-01

    Angiogenesis is an important process in chronic inflammatory diseases. We observed that sera from patients with systemic vasculitis stimulated angiogenesis in an in vitro model using human umbilical vein endothelial cells cultured on a basement membrane (Matrigel) substrate. After 40% ammonium sulfate precipitation, angiogenic activity remained in the low molecular weight fraction and could be inactivated by heat. SDS-page of serum FPLC fractions exhibiting maximal angiogenic activity demonstrated two prominent species of 45 and 16-20 kD in patients' sera. These bands were much less apparent in sera obtained from control subjects. Amino-terminal sequencing of the 45-kD protein demonstrated that it was haptoglobin. Purified haptoglobin stimulated angiogenesis in a dose-dependent manner. The angiogenic activity of vasculitis patients' sera was partially inhibited by an antihaptoglobin antibody. Furthermore, serum haptoglobin levels in vasculitis patients correlated both with disease and angiogenic activity. Haptoglobin angiogenic activity was confirmed in two in vivo models using an implanted disc and a subcutaneous injection of basement membrane. Stimulation of angiogenesis is a newly recognized biological function of haptoglobin. The increased levels of haptoglobin found in chronic inflammatory conditions may play an important role in tissue repair. In systemic vasculitis, haptoglobin might also compensate for ischemia by promoting development of collateral vessels. Images PMID:7680672

  18. CNS Vasculitis Associated with Waldenström Macroglobulinemia.

    PubMed

    Riangwiwat, Tanawan; Wu, Chris Y; Santos-Ocampo, Alberto S; Liu, Randal J; McMurtray, Aaron M; Nakamoto, Beau K

    2016-01-01

    Waldenström macroglobulinemia (WM) is an indolent B cell lymphoproliferative disorder with monoclonal IgM secretion. We present a patient with WM who presented with multifocal acute cortical ischemic strokes and was found to have central nervous system (CNS) vasculitis. Workup was negative for cryoglobulins and hyperviscosity syndrome. Immunosuppression with intravenous steroids and cyclophosphamide stabilized the patient's mental status and neurologic deficits. On followup over 7 years, patient gained independence from walking aids and experienced no recurrences of CNS vasculitis. To our knowledge, CNS vasculitis in a WM patient, in the absence of cryoglobulins, has not been reported. Immunosuppression is the preferred treatment.

  19. CNS Vasculitis Associated with Waldenström Macroglobulinemia

    PubMed Central

    Riangwiwat, Tanawan; Wu, Chris Y.; Santos-Ocampo, Alberto S.; Liu, Randal J.

    2016-01-01

    Waldenström macroglobulinemia (WM) is an indolent B cell lymphoproliferative disorder with monoclonal IgM secretion. We present a patient with WM who presented with multifocal acute cortical ischemic strokes and was found to have central nervous system (CNS) vasculitis. Workup was negative for cryoglobulins and hyperviscosity syndrome. Immunosuppression with intravenous steroids and cyclophosphamide stabilized the patient's mental status and neurologic deficits. On followup over 7 years, patient gained independence from walking aids and experienced no recurrences of CNS vasculitis. To our knowledge, CNS vasculitis in a WM patient, in the absence of cryoglobulins, has not been reported. Immunosuppression is the preferred treatment. PMID:27818812

  20. Marfan syndrome with antineutrophil cytoplasmic antibody-associated systemic vasculitis presenting as severe anaemia and haematuria after the Bentall procedure.

    PubMed

    Sijia, Li; Shuangxin, Liu; Wei, Shi; Yanhai, Cui

    2013-08-01

    One month previously, a 28-year old male underwent an emergency modified Bentall procedure because of Marfan syndrome with acute aortic dissection Stanford Class A. Computed tomography of the chest did not reveal severe graft stenosis of the anastomosis. To explore the cause of anaemia, renal dysfunction and macroscopic haematuria, the patient was tested for antineutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis (AASV). Antimyeloperoxidase antibodies (MPO)-ANCA and antiproteinase 3 antibodies (PR3)-ANCA were strongly positive. Corticosteroid therapy was applied, followed by cyclophosphamide and azathioprine. In response to treatment, the MPO-ANCA and PR3-ANCA levels gradually decreased, proteinuria was alleviated and haemoglobin levels returned to normal after 6 months. This is the first report to highlight haemolytic anaemia and AASV with Marfan syndrome after surgery for aortic dissection.

  1. Refractory rheumatoid vasculitis

    PubMed Central

    Kumar, Ashok; Goel, Anshul; Lapsiwala, Mehul; Singhal, Suman

    2016-01-01

    Systemic rheumatoid vasculitis (SRV) can develop in rheumatoid arthritis of long duration and high disease activity. It most commonly manifests as cutaneous vasculitis and mononeuritis multiplex. This can involve any organ of the body and carries very high mortality. We report a case of a young male who had rheumatoid arthritis for the past 15 years and became refractory to standard drugs and anti-TNF agents. He subsequently developed SRV, which started as mononeuritis multiplex. Disease progressed to result in gangrene of hands and feet despite receiving intravenous cyclophosphamide. Intravenous immunoglobulin and rituximab also could not provide any response. Prolonged ICU stay resulted in critical care neuromyopathy. Central nervous system vasculitis developed even after repeated infusions of intravenous immunoglobulins and at last he died of complications. In this case report, we have presented rare and chronic protracted presentation of rheumatoid vasculitis involving skin, nerves, brain and testis, which was refractory to the recommended therapies. PMID:28031844

  2. Pediatric vasculitis.

    PubMed

    Weiss, Pamela F

    2012-04-01

    Childhood vasculitis is a challenging and complex group of conditions that are multisystem in nature and often require integrated care from multiple subspecialties, including rheumatology, dermatology, cardiology, nephrology, neurology, and gastroenterology. Vasculitis is defined as the presence of inflammation in the blood vessel wall. The site of vessel involvement, size of the affected vessels, extent of vascular injury, and underlying pathology determine the disease phenotype and severity. This article explores the classification and general features of pediatric vasculitis, as well as the clinical presentation, diagnostic evaluation, and therapeutic options for the most common vasculitides.

  3. Rheumatoid Vasculitis

    MedlinePlus

    ... RV) is an unusual complication of longstanding, severe rheumatoid arthritis. The active vasculitis associated with rheumatoid disease occurs ... a manifestation of “extra-articular” (beyond the joint)rheumatoid arthritis and involves the small and medium-sized arteries ...

  4. Pulmonary Vasculitis

    PubMed Central

    Brown, Kevin K.

    2006-01-01

    Pulmonary vasculitis describes a number of distinct disorders that are pathologically characterized by the destruction of blood vessels. The clinical manifestations of each disorder are defined by the size, type, and location of the affected vasculature. The clinical approach to these disorders rests upon an astute clinician considering the diagnosis and identifying the specific patterns of clinical, radiologic, laboratory, and pathologic abnormalities. Lung involvement is most commonly seen with the primary, idiopathic, small-vessel, or antineutrophil cytoplasmic antibody–associated vasculitides; Wegener's granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome. However, primary, idiopathic medium and large-vessel vasculitis, primary immune complex–mediated vasculitis, and secondary vasculitis are all capable of presenting with lung involvement. In this article, we focus on the more common, antineutrophil cytoplasmic antibody–associated disorder, vasculitides. PMID:16493151

  5. Vasculitis Syndromes of the Central and Peripheral Nervous Systems

    MedlinePlus

    ... the Central and Peripheral Nervous Systems Fact Sheet Table of Contents (click to jump to sections) What ... Information Page NINDS Epilepsy Information Page NINDS Familial Periodic Paralyses Information Page NINDS Farber's Disease Information Page ...

  6. Systemic Lupus Erythematosus Presenting with a Fatal Intestinal Vasculitis: a Case Report

    PubMed Central

    Nozari, Neda; Divsalar, Parisa

    2014-01-01

    This case report demonstrates fatal gastrointestinal vasculitis as a rare presentation of systemic lupus erythematosus. A 34-year-old woman presented with abdominal pain and diarrhea. Anti nuclear antibody was positive and high titre of anti-ds DNA antibody was also reported. Treatment with corticosteroid and supportive cares were started; however, her condition worsened. Eventually, she was considered as a candidate for diagnostic laparoscopy. Immediately after laparoscopy, she developed respiratory distress along with upper gastrointestinal bleeding. Soon after, the patient died because of disseminated intravascular coagulation . PMID:25093065

  7. Cocaine/levamisole-induced systemic vasculitis with retiform purpura and pauci-immune glomerulonephritis

    PubMed Central

    Veronese, F.V.; Dode, R.S.O.; Friderichs, M.; Thomé, G.G.; da Silva, D.R.; Schaefer, P.G.; Sebben, V.C.; Nicolella, A.R.; Barros, E.J.G.

    2016-01-01

    Levamisole has been increasingly used as an adulterant of cocaine in recent years, emerging as a public health challenge worldwide. Levamisole-associated toxicity manifests clinically as a systemic vasculitis, consisting of cutaneous, hematological, and renal lesions, among others. Purpura retiform, cutaneous necrosis, intravascular thrombosis, neutropenia, and less commonly crescentic nephritis have been described in association with anti-neutrophil cytoplasmic antibodies (ANCAs) and other autoantibodies. Here we report the case of a 49-year-old male who was a chronic cocaine user, and who presented spontaneous weight loss, arthralgia, and 3 weeks before admission purpuric skin lesions in the earlobes and in the anterior thighs. His laboratory tests on admission showed serum creatinine of 4.56 mg/dL, white blood count 3,800/μL, hemoglobin 7.3 g/dL, urinalysis with 51 white blood cells/μL and 960 red blood cells/μL, and urine protein-to-creatinine ratio 1.20. Serum ANCA testing was positive (>1:320), as well as serum anti-myeloperoxidase and anti-proteinase 3 antibodies. Urine toxicology screen was positive for cocaine and levamisole, with 62.8% of cocaine, 32.2% of levamisole, and 5% of an unidentified substance. Skin and renal biopsies were diagnostic for leukocytoclastic vasculitis and pauci-immune crescentic glomerulonephritis, respectively. The patient showed a good clinical response to cocaine abstinence, and use of corticosteroids and intravenous cyclophosphamide. Last serum creatinine was 1.97 mg/dL, white blood cell count 7,420/μL, and hemoglobin level 10.8 g/dL. In levamisole-induced systemic vasculitis, the early institution of cocaine abstinence, concomitant with the use of immunosuppressive drugs in severe cases, may prevent permanent end organ damage and associate with better clinical outcomes. PMID:27119429

  8. Acute effect of substance P in immunologic vasculitis in the rat colon.

    PubMed

    Murthy, S N; DePace, D M; Shah, R S; Podell, R

    1991-01-01

    Substance P has been implicated as a neuronal mediator of inflammation in various inflammatory conditions. However, the exact role played by substance P in inflammatory bowel diseases or in experimental colonic vasculitis has not been clearly understood. In this study, we examined the effect of close superior mesenteric artery injection of substance P under prevailing inflammatory conditions induced by intravenous human albumin antialbumin immune complex followed by intracolonic perfusion of 2.5% formaldehyde in rats or intracolonic perfusion of 5% alcohol alone. The immune complex- and formaldehyde-treated rats showed severe microvascular changes such as microvascular plugging by red blood cells, endothelial breakage and extravasation of plasma proteins and red blood cells. The bolus injection of 10(-8) M substance P reduced extravasation of Evans blue dye by 50% and the tissue wet to dry ratio by 20% in immune complex- and formaldehyde-perfused rats. Myeloperoxidase activity was not changed. Substance P also significantly inhibited (44%) the extravasation in alcohol-perfused rats. Pretreatment of immune complex- and formaldehyde-treated rats with substance P antagonist reversed the effect of substance P. These findings suggest that the most immediate effect of substance P may be vasodilation and clearing of vascular plugs induced by immune complex and formaldehyde. This effect of substance P differs from its chronic effect, which causes vasodilation and extravasation.

  9. Crohns disease with central nervous system vasculitis causing subarachnoid hemorrhage due to aneurysm and cerebral ischemic stroke

    PubMed Central

    Garge, Shaileshkumar S.; Vyas, Pooja D.; Modi, Pranav D.; Ghatge, Sharad

    2014-01-01

    Cerebral vasculitis secondary to Crohn's disease (CD) seems to be a very rare phenomenon. We report a 39-year-old male who presented with headache, vomiting, and left-sided weakness in the known case of CD. Cross-sectional imaging (computed tomography and magnetic resonance imaging,) showed right gangliocapsular acute infarct with supraclinoid cistern subarachnoid hemorrhage (SAH). Cerebral digital substraction angiography (DSA) showed dilatation and narrowing of right distal internal carotid artery (ICA). Left ICA was chronically occluded. His inflammatory markers were significantly raised. Imaging features are suggestive of cerebral vasculitis. Arterial and venous infarcts due to thrombosis are known in CD. Our case presented with acute subarachnoid hemorrhage in supraclinoid cistern due to rupture of tiny aneurysm of perforator arteries causing SAH and infarction in right basal ganglia. Patient was treated conservatively with immunosuppression along with medical management of SAH. PMID:25506170

  10. Recombinant protein to analyze autoantibodies to proteinase 3 in systemic vasculitis.

    PubMed

    Rarok, Agnieszka A; Huitema, Minke G; van der Leij, Marcel J; van der Geld, Ymke M; Berthold, Heike; Schmitt, Jacky; Stegeman, Coen A; Limburg, Pieter C; Kallenberg, Cees G

    2003-10-01

    The presence of antineutrophil cytoplasmic autoantibodies with specificity for proteinase 3 (PR3-ANCA) usually is detected by enzyme-linked immunosorbent assay (ELISA) with purified PR3 as a substrate. We studied the technical performance of direct and capture ELISA using a recombinant proteolytically inactive form of PR3 produced in the baculovirus expression system for the detection of PR3-ANCA in 114 patients with systemic vasculitis at diagnosis. We found that ELISA using recombinant PR3 produced in insect cells is a promising alternative for ELISA with native PR3. We found a correlation between tests using recombinant or native PR3, as well as correlation of the ELISA results with ANCA titers measured by the indirect immunofluorescence technique. However, the specificity for ANCA-associated vasculitis of ELISA with recombinant PR3 was lower than ELISA using native PR3. Compared with the direct assay, capture ELISA is a more sensitive method for PR3-ANCA detection, with both native and recombinant PR3, and its results depend on the monoclonal antibody used to capture the antigen.

  11. [Systemic necrotizing vasculitis presenting as gangrene combined with diabetes insipidus: a case report].

    PubMed

    Huang, Qing; Liu, Yu-lan

    2015-12-18

    The male patient reported here presented as gangrene and central diabetes insipidus (CDI), who had characteristics of vasculitis. The patient complained about polydipsia and polyuria half a year ago, and then developed tingling, pain and blackish discoloration of some fingers and toes 3 month ago. He also had Raynaud's phenomenon. After admission, his laboratory examination showed the rise of erythrocyte sedimentation rate, C-reactive protein, immunoglobulin, β2-glycoprotein I and the activity of rheumatoid factors, lupus anticoagulant test. his pituitary gland showed loss of posterior signal on magnetic resonance imaging. In addition, his vasopressin test was active. However, there was no sufficient evidence to diagnose any specific disease; as a consequence the patient was diagnosed as idiopathic systemic necrotizing vasculitis (SNV). For SNV, the patient was treated with glucocorticoid 40 mg/d and impact therapy of cyclophosphamide 0.4 g every 2 weeks. He also received symptomatic treatment for gangrene and CDI. Cutaneous involvement leading to gangrene was widely reported in SNV, however pituitary involvement in SNV leading to CDI was rare. The prognosis of this patient was poor.

  12. Antibodies to endothelial cells in systemic lupus erythematosus: a potential marker for nephritis and vasculitis.

    PubMed

    D'Cruz, D P; Houssiau, F A; Ramirez, G; Baguley, E; McCutcheon, J; Vianna, J; Haga, H J; Swana, G T; Khamashta, M A; Taylor, J C

    1991-08-01

    Using an ELISA, anti-endothelial cell antibodies (AECA) have been found in sera obtained at the time of renal biopsy in 46 out of 57 patients (81%) with systemic lupus erythematosus (SLE) and nephritis (mean binding index (BI) = 84% +/- 52.8) compared with 22 out of 50 SLE patients (44%) without nephritis (mean BI = 45% +/- 35.9). Seventy normal human sera had a mean BI of 10% +/- 9.8. The highest levels were seen in patients with diffuse proliferative glomerulonephritis (WHO grade IV) and in patients with proteinuria and nephrotic syndrome. When the biopsies were assessed for activity and chronicity scores, AECA were associated with active renal lesions (P less than 0.001). AECA levels correlated with low complement levels but not with anti-DNA antibodies to extractable nuclear antigens (ENA), anti-cardiolipin or anti-neutrophil cytoplasmic antibodies. The presence of AECA conferred a positive predictive value of 0.68 for the presence of nephritis. Twenty-five patients had active vasculitis at the time of assay and the highest AECA values were seen in patients with both nephritis and vasculitis. No correlation was seen with serum immunoglobulin levels and immune complexes did not bind significantly to the endothelial surface. The possible role of these antibodies as a marker in lupus nephritis is discussed.

  13. T cell receptor (TCR) V gene usage in patients with systemic necrotizing vasculitis.

    PubMed Central

    Giscombe, R; Grunewald, J; Nityanand, S; Lefvert, A K

    1995-01-01

    Wegener's granulomatosis (WG) and polyarteritis nodosa (PAN) are systemic necrotizing vasculitides of unknown etiology. These disorders run a fatal course if untreated. T lymphocytes are implicated in the pathogenesis of WG, since they have been found to infiltrate affected organs, and sIL-2R correlates with disease activity. To elucidate further the role of T cells in necrotizing vasculitis, we have used a panel of 12 TCR V-specific MoAbs to investigate the number of cells expressing certain V alpha and V beta gene segments in the CD4+ and CD8+ subsets of altogether 11 patients with WG or PAN. In the group of patients, we found abnormal expansions of T cells using particular TCR V alpha or V beta gene products. These T cell expansions were more numerous, of a dramatically higher magnitude, and frequently more often found in the CD4 subset, compared with T cell expansions identified in healthy individuals. In long-term studies of the T cell expansions for up to 18 months, a heterogeneous pattern was revealed, with no obvious correlation to clinical features such as disease activity or treatment. Studies of TCR V gene usage in this group of patients may help in understanding the pathogenesis of necrotizing vasculitis, and in the identification of unknown antigens, and may open the possibility to a highly selective immunotherapy by targeting disease-mediating T cells. PMID:7648706

  14. Cutaneous vasculitis: diagnosis and management.

    PubMed

    Carlson, J Andrew; Cavaliere, L Frank; Grant-Kels, Jane M

    2006-01-01

    Vasculitis is histologically defined as inflammatory cell infiltration and destruction of blood vessels. Vasculitis is classified as primary (idiopathic, eg, cutaneous leukocytoclastic angiitis, Wegener's granulomatosis) or secondary, a manifestation of connective tissue diseases, infections, adverse drug eruptions, or a paraneoplastic phenomenon. Cutaneous vasculitis, manifested as urticaria, purpura, hemorrhagic vesicles, ulcers, nodules, livedo, infarcts, or digital gangrene, is a frequent and often significant component of many systemic vasculitic syndromes such as lupus or rheumatoid vasculitis and antineutrophil cytoplasmic antibody-associated primary vasculitic syndromes such as Churg-Strauss syndrome. In most instances, cutaneous vasculitis represents a self-limited, single-episode phenomenon, the treatment of which consists of general measures such as leg elevation, warming, avoidance of standing, cold temperatures and tight fitting clothing, and therapy with antihistamines, aspirin, or nonsteroidal anti-inflammatory drugs. More extensive therapy is indicated for symptomatic, recurrent, extensive, and persistent skin disease or coexistence of systemic disease. For mild recurrent or persistent disease, colchicine and dapsone are first-choice agents. Severe cutaneous and systemic disease requires more potent immunosuppression (prednisone plus azathioprine, methotrexate, cyclophosphamide, cyclosporine, or mycophenolate mofetil). In cases of refractory vasculitis, plasmapheresis and intravenous immunoglobulin are viable considerations. The new biologic therapies that work via cytokine blockade or lymphocyte depletion such as tumor alpha inhibitor infliximab and the anti-B-cell antibody rituximab, respectively, are showing benefit in certain settings such as Wegener's granulomatosis, antineutrophil cytoplasmic antibody-associated vasculitis, Behçet's disease, and cryoglobulinemic vasculitis.

  15. Systemic vasculitis: how little we know about their societal and economic burden.

    PubMed

    Trieste, Leopoldo; Palla, Ilaria; Baldini, Chiara; Talarico, Rosaria; D'Angiolella, Lucia; Mosca, Marta; Turchetti, Giuseppe

    2012-01-01

    This article attempts to perform an evaluation of the state of the art of the economic and societal burden of systemic vasculitis (VAs). Due to the rarity of these diseases and their variable clinical picture, few data are available in the literature on their health economic issues, and only some papers have been published that marginally examine the problem. Since VAs are severe conditions with a high medical and societal impact and determine high healthcare resource consumption, studies able to define societal, quality of life and economic burden of these pathologies are needed. Policy makers, private and public organisations involved in the care of VAs need data to programme future investment or make cost-effectiveness analysis for introducing new drugs or protocols.

  16. Vasculitis as a paraneoplastic syndrome. Report of 11 cases and review of the literature.

    PubMed

    Sánchez-Guerrero, J; Gutiérrez-Ureña, S; Vidaller, A; Reyes, E; Iglesias, A; Alarcón-Segovia, D

    1990-11-01

    In a study of 222 patients with vasculitis, we identified 11 who had an associated neoplasia. Seven had hematological neoplasia and 4 had solid malignant tumors. In 4 patients vasculitis gave the first evidence of the neoplasia or of its recurrence. Nine of our patients had cutaneous vasculitis. The other 2 had vasculitis involving the intestine and resulted in acute abdomens. These 2 patients needed prednisone treatment for the vasculitis. Neoplasia should be considered in patients with vasculitis without an apparent cause.

  17. Pediatric Vasculitis Initiative

    ClinicalTrials.gov

    2016-08-19

    Wegeners Granulomatosis (Granulomatosis With Polyangiitis); Microscopic Polyangiitis; Churg Strauss Syndrome (Eosinophilic Granulomatosis With Polyangiitis); Polyarteritis Nodosa; Takayasu Arteritis; Primary CNS Vasculitis; Unclassified Vasculitis

  18. The Role of Catheter Angiography in the Diagnosis of Central Nervous System Vasculitis

    PubMed Central

    Edgell, Randall C.; Sarhan, Ahmed E.; Soomro, Jazba; Einertson, Collin; Kemp, Joanna; Shirani, Peyman; Malmstrom, Theodore K.; Coppens, Jeroen

    2016-01-01

    Background Central nervous system vasculitis (CNSV) is a rare disorder, the pathophysiology of which is not fully understood. It involves a combination of inflammation and thrombosis. CNSV is most commonly associated with headache, gradual changes in mental status, and focal neurological symptoms. Diagnosis requires the effective use of history, laboratory testing, imaging, and biopsy. Catheter angiography can be a powerful tool in the diagnosis when common and low-frequency angiographic manifestations of CNSV are considered. We review these manifestations and their place in the diagnostic algorithm of CNSV. Summary We reviewed the PubMed database for case series of CNSV that included 5 or more patients. Demographic and angiographic findings were collected. Angiographic findings were dichotomized between common and low-frequency findings. A system for incorporating these findings into clinical decision-making is proposed. Key Message CNSV is a diagnostic challenge due to the absence of a true gold standard test. In the absence of such a test, catheter angiography remains a central piece of the diagnostic puzzle when appropriately employed and interpreted. PMID:27781050

  19. Leukocytoclastic Vasculitis with Systemic Involvement Associated with Ciprofloxacin Therapy: Case Report and Review of the Literature

    PubMed Central

    Madeira, Catarina; Pinto, Joana; Pestana, Joana

    2016-01-01

    A 71-year-old woman presented with constitutional signs and lower extremity palpable purpura after being prescribed a four-day course of 500 mg of ciprofloxacin two times daily for a gastrointestinal infection. She was admitted for inpatient treatment. During the third hospital day, she presented with an episode of abundant hematemesis while her skin lesions remained unchanged. Upper endoscopy revealed multiple lesions consistent with vasculitis and histological examination of the skin biopsy disclosed a leukocytoclastic vasculitis. The patient was successfully treated with prednisone following ciprofloxacin discontinuation. Complete resolution of the lesions on drug withdrawal strongly suggested drug toxicity, which was further supported by a score of 8 in the Naranjo Adverse Drug Reaction Probability Scale. Awareness that the development of skin and gastrointestinal lesions following administration of ciprofloxacin may be a manifestation of ciprofloxacin-induced vasculitis can help early detection, treatment, and lead to an overall good prognosis. PMID:28070469

  20. Retinal Vasculitis in Anti-Synthetase Syndrome.

    PubMed

    Donovan, Christopher P; Pecen, Paula E; Baynes, Kimberly; Ehlers, Justis P; Srivastava, Sunil K

    2016-09-01

    A 31-year-old woman with a history of anti-synthetase syndrome-related myositis and interstitial lung disease presented with acute-onset blurry vision and rash on her hands and feet. Visual acuity was hand motion in her right eye and 20/40 in her left eye. Dilated fundus exam showed extensive retinal vasculitis, diffuse intraretinal hemorrhages, and subretinal fluid. Optical coherence tomography revealed significant macular thickening, and fluorescein angiography revealed vascular leakage with peripheral nonperfusion. Aggressive systemic immunosuppression was initiated, with gradual resolution of her disease during 8 months of follow-up. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:874-879.].

  1. Reproductive Health in Men and Women With Vasculitis

    ClinicalTrials.gov

    2014-06-25

    Giant Cell Arteritis; Takayasu's Arteritis; Polyarteritis Nodosa; Wegener's Granulomatosis; Microscopic Polyangiitis; Churg-Strauss Syndrome; Behcet's Disease; Kawasaki Disease; Henoch-schoenlein Purpura; Vasculitis, Central Nervous System; Drug-induced Necrotizing Vasculitis

  2. Systemic vasculitis and aneurysm formation in the Wiskott-Aldrich syndrome.

    PubMed Central

    McCluggage, W G; Armstrong, D J; Maxwell, R J; Ellis, P K; McCluskey, D R

    1999-01-01

    A 24 year old male who suffered from the Wiskott-Aldrich syndrome developed intra-abdominal bleeding on two occasions. Radiological investigations showed aneurysmal dilatation of branches of the hepatic and superior mesenteric arteries. The second abdominal bleed necessitated laparotomy and the bleeding was localised to the kidneys. Right nephrectomy was performed and histological examination showed a necrotising vasculitis, mainly involving medium and small sized renal blood vessels. Steroids, immunosuppressive treatment, and control of blood pressure resulted in resolution of the vasculitic process and prevented further haemorrhage. Vasculitis and aneurysm formation are rarely described complications of Wiskott-Aldrich syndrome and may account for the life threatening haemorrhage which occurs in this condition. Images PMID:10560364

  3. Types of Vasculitis

    MedlinePlus

    ... other signs develop that point to arteritis. Mostly Medium Vessel Vasculitis These types of vasculitis usually, but not always, affect the body’s medium-sized blood vessels. Buerger's Disease Buerger's disease , also ...

  4. An Update of the Mayo Clinic Cohort of Patients With Adult Primary Central Nervous System Vasculitis

    PubMed Central

    Salvarani, Carlo; Brown, Robert D.; Christianson, Teresa; Miller, Dylan V.; Giannini, Caterina; Huston, John; Hunder, Gene G.

    2015-01-01

    Abstract Primary central nervous system vasculitis (PCNSV) is an uncommon condition in which lesions are limited to vessels of the brain and spinal cord. Because the clinical manifestations are not specific, the diagnosis is often difficult, and permanent disability and death are frequent outcomes. This study is based on a cohort of 163 consecutive patients with PCNSV who were examined at the Mayo Clinic over a 29-year period from 1983 to 2011. The aim of the study was to define the characteristics of these patients, which represents the largest series in adults reported to date. A total of 105 patients were diagnosed by angiographic findings and 58 by biopsy results. The patients diagnosed by biopsy more frequently had at presentation cognitive dysfunction, greater cerebrospinal fluid total protein concentrations, less frequent cerebral infarcts, and more frequent leptomeningeal gadolinium-enhanced lesions on magnetic resonance imaging (MRI), along with less mortality and disability at last follow-up. The patients diagnosed by angiograms more frequently had at presentation hemiparesis or a persistent neurologic deficit or stroke, more frequent infarcts on MRI and an increased mortality. These differences were mainly related to the different size of the vessels involved in the 2 groups. Although most patients responded to therapy with glucocorticoids alone or in conjunction with cyclophosphamide and tended to improve during the follow-up period, an overall increased mortality rate was observed. Relapses occurred in one-quarter of the patients and were less frequent in patients treated with prednisone and cyclophosphamide compared with those treated with prednisone alone. The mortality rate and degree of disability at last follow-up were greater in those with increasing age, cerebral infarctions on MRI, angiographic large vessel involvement, and diagnosis made by angiography alone, but were lower in those with gadolinium-enhanced lesions on MRI and in those with

  5. The histological assessment of cutaneous vasculitis.

    PubMed

    Carlson, J Andrew

    2010-01-01

    Vasculitis is defined as inflammation directed at vessels, which compromises or destroys the vessel wall leading to haemorrhagic and/or ischaemic events. Skin biopsy is the gold standard for the diagnosis of cutaneous vasculitis, whose manifestations include urticaria, infiltrative erythema, petechiae, purpura, purpuric papules, haemorrhagic vesicles and bullae, nodules, livedo racemosa, deep (punched out) ulcers and digital gangrene. These varied morphologies are a direct reflection of size of the vessels and extent of the vascular bed affected, ranging from a vasculitis affecting few superficial, small vessels in petechial eruptions to extensive pan-dermal small vessel vasculitis in haemorrhagic bullae to muscular vessel vasculitis in lower extremity nodules with livedo racemosa. Skin biopsy, extending to subcutis and taken from the earliest, most symptomatic, reddish or purpuric lesion is crucial for obtaining a high-yielding diagnostic sample. Based on histology, vasculitis can be classified on the size of vessels affected and the dominant immune cell mediating the inflammation (e.g. neutrophilic, granulomatous, lymphocytic, or eosinophilic). Disruption of small vessels by inflammatory cells, deposition of fibrin within the lumen and/or vessel wall coupled with nuclear debris allows for the confident recognition of small vessel, mostly neutrophilic vasculitis (also known as leukocytoclastic vasculitis). In contrast, muscular vessel vasculitis can be identified solely by infiltration of its wall by inflammatory cells. Extravasation of red blood cells (purpura) and necrosis are supportive, but not diagnostic of vasculitis as they are also seen in haemorrhagic and/or vaso-occlusive disorders (pseudovasculitis). Vasculitic foci associated with extravascular granulomas (palisaded neutrophilic and granulomatous dermatitis), tissue eosinophilia, or tissue neutrophilia signal the risk for, or co-existence of systemic disease. This essential histological information

  6. Vasculitis associated with malignancy.

    PubMed

    Mertz, L E; Conn, D L

    1992-02-01

    A large variety of vasculopathic syndromes are uncommonly associated with malignancies. Vasculitis is usually manifested by skin lesions and is generally associated with hematologic malignancies rather than solid tumors. Evidence of autoantibodies, immune complexes, and complement consumption is typically absent. Myelodysplastic syndromes can be confidently linked to vasculitis on the basis of recent literature. The temporal relationship of malignancy to vasculitis development is variable except that vasculitis generally follows the discovery of hairy cell leukemia and splenectomy. Vasculitis may occasionally be a complication of chemotherapy, radiation therapy, and bone marrow transplantation. Occasionally, malignant disorders may mimic vasculitic syndromes. The etiopathogenesis of vasculitis in patients with malignant disorders is unknown. The recent literature on vasculitis and malignancy addresses predominantly case reports and small patient cohorts and identifies clinical characteristics rather than pathogenic mechanisms.

  7. [Efficacy of rituximab combined with cyclophosphamide in a patient with systemic lupus erythematosus and peritoneal vasculitis refractory to conventional inmunosupressive therapy].

    PubMed

    Garrido Rasco, Rocío; García Hernández, Francisco José; González León, Rocío; Castillo Palma, María Jesús; Ocaña Medina, Celia; Sánchez Román, Julio

    2009-02-01

    Peritoneal vasculitis is a rare and severe clinical manifestation of systemic lupus erythematosus. We report a patient who presented with ascites due to peritoneal vasculitis and cutaneous, articular, hematological and renal inflammatory activity. Treatment with glucocorticoids and immunosuppressive drugs was ineffective. In view of the resistance to different therapies, 4 weekly infusions of 375mg/m2 of rituximab (RTX) were started, in association with cyclophosphamide pulses during the first and the third weeks. With this treatment strategy, the patient reached a complete response which was achieved in later flares of inflammatory activity (the second and third flares were multisystemic and with ascites again, and the fourth flare with nephritis).

  8. A Case of Leukocytoclastic Vasculitis Caused by Listeria monocytogenes Bacteremia

    PubMed Central

    2016-01-01

    Importance. Infections can cause leukocytoclastic vasculitis. Observations. We report the case of a patient with a left ventricular assist device who presented with acute kidney injury and biopsy proven leukocytoclastic vasculitis. Blood cultures grew Listeria monocytogenes. The patient's rash improved with treatment of the underlying Listeria infection. Conclusion. Clinicians should be aware that there are a number of broad categories of disease associated with the histologic finding of vasculitis, including infection. It is important to keep in mind the risk factors of a particular patient when formulating a differential diagnosis. This is the first reported case of Listeria bacteremia causing leukocytoclastic vasculitis. PMID:27313916

  9. True vasculitis in lupus nephritis.

    PubMed

    Abdellatif, A A; Waris, S; Lakhani, A; Kadikoy, H; Haque, W; Truong, L D

    2010-08-01

    Vascular lesions are encountered frequently in renal biopsy specimens of patients with systemic lupus erythematosus (SLE) and can present in a variety of morphologic forms. True renal lupus vasculitis (TRLV) is one of the rare vascular lesions associated with lupus nephritis that has been infrequently reported in the medical literature. The primary focus on glomerular pathology and collective classification of the vascular lesions under lupus vasculopathy is one of the reasons why this form of inflammatory vasculitis has been under-recognized as a separate disease entity. Here we have comprehensively reviewed the literature on renal vascular involvement in SLE for a better understanding of the epidemiology, morphologic features, pathogenesis, clinical course and treatment of TRLV. It can be morphologically differentiated from other forms of renal vascular lesions in lupus nephritis, i.e. arteriosclerosis, uncomplicated vascular immune deposits, non-inflammatory necrotizing vasculopathy, and thrombotic microangiopathy. Despite close similarities with antineutrophil cytoplasmic autoantibody associated vasculitis (AASV), there are certain morphological differences that warrant a thorough investigation of the possible pauci-immune mechanism of pathogenesis. The vasculitis follows a severe clinical course in general with rapid progression to renal failure, although favorable outcomes have been reported in certain cases. The standard use of steroids and cytotoxic drugs has yielded variable results in the treatment of TRLV. Current treatment modalities being used in lupus nephritis and AASV have been compared in this article with focus on drugs acting on the inflammatory cells implicated in TRLV pathogenesis.

  10. [Vasculitis and vasculopathy].

    PubMed

    Marković, Asjastipić

    2012-10-01

    Many pathophysiological process components are known to be implicated in lower limb ulcerations, among which vascular lesions have a major role. Vasculitis denotes a heterogeneous group of clinical entities which all are characterized by the inflammatory process of arterial and venous walls of any size and in any organ, quite frequently in the skin. Vasculopathy, on the other hand, refers to vascular and capillary lesions caused by, for example, some medications. The classification of vasculitides according to the size of the blood vessels involved serves for proper understanding the issue among clinicians and researchers, and not as a diagnostic tool. According to histologic finding obtained by examination of blood vessel biopsy specimen, vasculitides are divided into three groups: lymphocytic, leukocytoclastic and granulomatous. Livedoid vasculitis (livedo reticularis) most commonly affects women and is generally localized on lower extremities. The etiology oflivedoid vasculitis may imply autoimmune diseases, capillary obstruction with cryoglobulins, or antiphospholipid syndrome. Livedoid vasculopathy is a hyalinization disease of the vasculature, with thromboses and ulcerations on lower extremities, and of unknown etiology. Livedoid vasculopathy has been singled out as a separate disease that usually does not occur consequentially to other primary diseases. Livedoid vasculopathy typically affects women (71%) at a mean age of 45 (range 10-85) years; bilateral involvement of both lower limbs is present in 80.8%, disease manifested with ulcerations in 68.9%, ulcerations followed by development of atrophie blanche in 71.1%, transcutaneous oximetry reduction is found in 74.1%, factor V mutation (Leiden heterozygotes) in 22.2%, reduced protein C activity in 13.3%, prothrombin gene mutation (G20210A) in 8.3%, positive lupus anticoagulant in 17.9%, positive anticardiolipin antibodies in 28.6%, and elevated homocysteine level in 14.3% cases; blood vessel histology shows

  11. How Is Vasculitis Treated?

    MedlinePlus

    ... Clinical Trials Links Related Topics Aneurysm Blood Tests Kawasaki Disease Raynaud's Smoking and Your Heart Send a link ... of vasculitis. For example, the standard treatment for Kawasaki disease is high-dose aspirin and immune globulin. Immune ...

  12. Tuberculosis versus vasculitis.

    PubMed

    Baig, Zahid Farooq; Raja, Khalid Mahmood; Abbas, Fahad

    2014-01-01

    Vasculitis (Wegeners Granulomatosis and Microscopic Polyangiitis) and Tuberculosis share many features including constitutional symptoms and respiratory tract involvement. The presence of kidney involvement with new onset azotaemia and active urine sediment support the diagnosis of vasculitis. We describe two cases that were diagnosed to be suffering from tuberculosis and placed on anti-tuberculosis therapy. On further workup they were found to be suffering from pauci- immune glomerulonephritis and recovered well with treatment.

  13. Hypocomplementemic urticarial vasculitis in mixed connective tissue disease.

    PubMed

    Calistru, Ana Maria; Lisboa, Carmen; Cruz, Maria João; Delgado, Luis; Poças, Licínio; Azevedo, Filomena

    2010-12-15

    Urticarial vasculitis is characterized clinically by urticaria-like skin lesions and histologically by leukocytoclastic vasculitis. It may be idiopathic or associated with various conditions such as infections, hematologic disorders, drugs, and connective tissue diseases, primarily systemic lupus erythematosus; an association with mixed connective tissue disease (MCTD) has rarely been reported. We present a case of hypocomplementemic urticarial vasculitis in a patient with MCTD that responded to hydroxychloroquine after a period of corticosteroid dependence.

  14. Post-transplant immune complex nephritis in a patient with systemic lupus erythematosus associated with ANCA vasculitis.

    PubMed

    Sanchez, Carlos; Rebolledo, Alejandra; Gahona, Junior; Rojas, Mauricio; Jiménez, Raquel; Bojórquez, Aurora

    2017-01-29

    Nearly 20% of SLE corresponds to the pediatric population, and 75% of them have kidney involvement representing an important etiology of chronic kidney disease. A correlation between SLE and ANCA-associated vasculitis has been identified as an overlapping syndrome. Kidney allograft recurrence is rare in SLE when disease control is achieved and with nowadays immunosuppression treatment. Histologic transformation is unusual, especially when there are negative serologic markers and no immune complex deposition reported in native kidneys. A 17-year-old female with crescentic glomerulonephritis, p-ANCA-positive antibodies with pauci-immune pattern in kidney biopsy develops end-stage renal disease requiring hemodialysis. Deceased donor kidney transplant was performed receiving triple immunosuppression thereafter. Thirteen months later serum creatinine rises without evidence of infection, urinary obstruction, or clinical and serologic disease relapse. Allograft biopsy reports mesangial proliferation and "full-house" immunofluorescence. The role of ANCA in SLE physiopathology is controversial, and its relation with lupus nephritis is also discordant. ANCA could represent an important factor in the heterogeneity of systemic lupus erythematosus and lupus nephritis.

  15. Rheumatoid Vasculitis: Vanishing Menace or Target for New Treatments?

    PubMed Central

    Bartels, C. M.; Bridges, A. J.

    2010-01-01

    Rheumatoid vasculitis is a rare but serious complication of rheumatoid arthritis. Herein we examine the pathophysiology, epidemiology, clinical diagnosis, and treatment of rheumatoid vasculitis. Seropositivity, specific HLA variations, and tobacco use are among the genetic and environmental predictors of rheumatoid vasculitis. Fortunately, recent reports have noted declines in the prevalence of rheumatoid vasculitis. Nevertheless, proper recognition of systemic manifestations may assist in pathologically confirming the diagnosis, determining the extent of disease, and guiding treatment. Contemporary treatment reports are discussed in the context of the ongoing debate regarding whether new agents may trigger, treat, or even prevent rheumatoid vasculitis. Evolving genetic, histopathologic, and immunologic studies partnered with ongoing clinical experience with biologics offer promise to inform future prevention and treatment of rheumatoid vasculitis. PMID:20842467

  16. [Vasculitis and viral infection].

    PubMed

    Martínez Aguilar, N E; Guido Bayardo, R; Vargas Camaño, M E; Compañ González, D; Miranda Feria, A J

    1997-01-01

    Viruses have been implicated in vasculitis. To determine activity of viral infection associated with vasculitis. 17 patients with vasculitis had been in immunological and antiviral antibodies evaluation. Twenty five healthy controls sex and age matched with hematic biometry (BH) and AA. All subjects were negative to HIV and HBV. Viral activity was demonstrated in eight patients; vascular purpura (5), Takayasu disease (1), polyarteritis nodosa (1), erythema nodosum (1). None subject of control group had IgM activity. Antibodies response of IgG in patients were of lesser intensity than in control group. 14 abnormalities in BH were found in patients and 4 in control group. Immune response in patients, measured by lymphocyte subpopulations and circulating immune complexes was abnormal. In conclusion 47% showed viral activity, but the dominant feature was abnormal immune response in 82%.

  17. [Allergic vasculitis in brucellosis].

    PubMed

    Boudghène-Stambouli, O; Mérad-Boudia, A; Ghernaout-Benchouk, S

    1994-01-01

    Brucellosis is an anthropozoonosis caused by a Gram negative bacillus of the Brucella gender. Skin manifestations have been reported in 1.5 to 11 p. 100 of the cases. Allergic vasculitis is rare. Recently a 24-year-old man was hospitalized for signs of infection. He had been treated with tetracycline. The clinical picture was suggestive of brucellosis and the Wright test was positive at 1/1,280. There were violet and purpuric papulae on the limbs, arthritis of the knee and ankle joints and renal involvement (haematuria, proteinuria). Histology revealed fibrinoid and leukocytoclastic vasculitis of the small veinules of the subpapillary plexus. Outcome was favourable with rifampicin, doxycycline and adjuvant dapsone, together with bed rest. Several types of skin manifestations have been reported in brucellosis although cases of allergic vasculitis are rare.

  18. Cocaine-Induced Vasculitis

    PubMed Central

    Berman, Mark; Paran, Daphna; Elkayam, Ori

    2016-01-01

    The use of cocaine continues to grow worldwide. One of the possible side-effects of cocaine is vasculitis. Two distinct vasculitic syndromes have been described due to cocaine. One is cocaine-induced midline destructive lesion, secondary to a direct vasoconstrictor effect of cocaine, inducing ischemic necrosis of the septal cartilage and perforation of the nasal septum, mimicking findings of granulomatosis with polyangiitis in the upper airways. The other is ANCA-associated vasculitis, attributed to the levamisole component that contaminates about 70% of the cocaine. This type of vasculitis may be myeloperoxidase (MPO) and proteinase 3 (PR3) positive, and its main manifestations are typical cutaneous findings, arthralgia, otolaryngologic involvement, and agranulocytosis. A high degree of suspicion and awareness is needed in order properly to diagnose and treat these patients. PMID:27824551

  19. Systemic Lupus Erythematosus and Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Overlap Syndrome in Patients With Biopsy-Proven Glomerulonephritis

    PubMed Central

    Jarrot, Pierre-Andre; Chiche, Laurent; Hervier, Baptiste; Daniel, Laurent; Vuiblet, Vincent; Bardin, Nathalie; Bertin, Daniel; Terrier, Benjamin; Amoura, Zahir; Andrés, Emmanuel; Rondeau, Eric; Hamidou, Mohamed; Pennaforte, Jean-Loup; Halfon, Philippe; Daugas, Eric; Dussol, Bertrand; Puéchal, Xavier; Kaplanski, Gilles; Jourde-Chiche, Noemie

    2016-01-01

    Abstract The aim of the study was to report the clinical, biological, and pathological characteristics of patients with glomerulonephritis (GN) secondary to systemic lupus erythematosus (SLE)/antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) overlap syndrome. A nationwide survey was conducted to identify cases of SLE/AAV overlap syndrome. Data were collected from SLE and AAV French research groups. Inclusion criteria were diagnosis of both SLE and AAV according to international classification criteria and biopsy-proven GN between 1995 and 2014. Additional cases were identified through a systematic literature review. A cohort of consecutive biopsy-proven GN was used to study the prevalence of overlapping antibodies and/or overlap syndrome. The national survey identified 8 cases of SLE/AAV overlap syndrome. All patients were female; median age was 40 years. AAV occurred before SLE (n = 3), after (n = 3), or concomitantly (n = 2). Six patients had rapidly progressive GN and 3/8 had alveolar hemorrhage. All patients had antinuclear antibodies (ANA); 7/8 had p-ANCA antimyeloperoxidase (MPO) antibodies. Renal biopsies showed lupus nephritis (LN) or pauci-immune GN. Remission was obtained in 4/8 patients. A literature review identified 31 additional cases with a similarly severe presentation. In the GN cohort, ANCA positivity was found in 30% of LN, ANA positivity in 52% of pauci-immune GN, with no correlation with pathological findings. The estimated prevalence for SLE/AAV overlap syndrome was 2/101 (2%). In patients with GN, SLE/AAV overlap syndrome may occur but with a low prevalence. Most patients have an aggressive renal presentation, with usually both ANA and anti-MPO antibodies. Further studies are needed to assess shared pathogenesis and therapeutic options. PMID:27258503

  20. Primary CNS vasculitis presenting as intraventricular bleeding

    PubMed Central

    Pillai, Sreeja Hareendranathan; Sreedharan, Sapna Erat; Menon, Girish; Kannoth, Santhosh; PN, Sylaja

    2016-01-01

    Primary angiitis of the central nervous system (PACNS) is a rare disorder affecting both medium- and small-sized vessels. Intracranial haemorrhages though less reported are in the form of parenchymal haemorrhage and subarachnoid haemorrhage. We report a case of PACNS with intraventricular haemorrhage due to aneurysms secondary to progression of vasculitis. PMID:27570401

  1. What Are the Signs and Symptoms of Vasculitis?

    MedlinePlus

    ... Weight loss Fatigue (tiredness) General aches and pains Organ- or Body System-Specific Signs and Symptoms Vasculitis can affect specific organs and body systems, causing a range of signs and symptoms. Skin ...

  2. T Cell–Macrophage Interactions and Granuloma Formation in Vasculitis

    PubMed Central

    Hilhorst, Marc; Shirai, Tsuyoshi; Berry, Gerald; Goronzy, Jörg J.; Weyand, Cornelia M.

    2014-01-01

    Granuloma formation, bringing into close proximity highly activated macrophages and T cells, is a typical event in inflammatory blood vessel diseases, and is noted in the name of several of the vasculitides. It is not known whether specific properties of the microenvironment in the blood vessel wall or the immediate surroundings of blood vessels contribute to granuloma formation and, in some cases, generation of multinucleated giant cells. Granulomas provide a specialized niche to optimize macrophage–T cell interactions, strongly activating both cell types. This is mirrored by the intensity of the systemic inflammation encountered in patients with vasculitis, often presenting with malaise, weight loss, fever, and strongly upregulated acute phase responses. As a sophisticated and highly organized structure, granulomas can serve as an ideal site to induce differentiation and maturation of T cells. The granulomas possibly seed aberrant Th1 and Th17 cells into the circulation, which are known to be the main pathogenic cells in vasculitis. Through the induction of memory T cells, aberrant innate immune responses can imprint the host immune system for decades to come and promote chronicity of the disease process. Improved understanding of T cell–macrophage interactions will redefine pathogenic models in the vasculitides and provide new avenues for immunomodulatory therapy. PMID:25309534

  3. Anti-neutrophil cytoplasmic antibody-associated paraneoplastic vasculitis.

    PubMed Central

    Navarro, J. F.; Quereda, C.; Rivera, M.; Navarro, F. J.; Ortuño, J.

    1994-01-01

    A 68 year old man presented with a systemic necrotizing vasculitis and elevated levels of anti-neutrophil cytoplasmic antibody (ANCA) which responded to treatment with steroids and cyclophosphamide, with a decrease in the titre of ANCA until its disappearance. Four months later he presented with weakness, loss of weight, aphonia and dysphagia. A computerized tomography scan showed a solid mass in the anterior mediastinum, and histological studies revealed an undifferentiated adenocarcinoma. Vasculitis improved although the malignancy progressed and ANCA was persistently negative. Our case demonstrates an association between ANCA and paraneoplastic vasculitis. Images Figure 2 PMID:8016013

  4. Imaging findings in pulmonary vasculitis.

    PubMed

    Castañer, Eva; Alguersuari, Anna; Andreu, Marta; Gallardo, Xavier; Spinu, Cristina; Mata, Josep M

    2012-12-01

    Vasculitis is a destructive inflammatory process affecting blood vessels. Pulmonary vasculitis may develop secondary to other conditions or constitute a primary idiopathic disorder. Thoracic involvement is most common in primary idiopathic large-vessel vasculitides (Takayasu arteritis, giant cell arteritis, Behçet disease) and primary antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitides (Wegener granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome). Primary pulmonary vasculitides are rare, and their signs and symptoms are nonspecific, overlapping with those of infections, connective tissue diseases, and malignancies. The radiologic findings in primary pulmonary vasculitis vary widely and can include vessel wall thickening, nodular or cavitary lesions, ground-glass opacities, and consolidations, among others. Diffuse alveolar hemorrhage usually results from primary small-vessel vasculitis in the lungs. To diagnose vasculitis, medical teams must recognize characteristic combinations of clinical, radiologic, laboratory, and histopathologic features.

  5. The infectious etiology of vasculitis.

    PubMed

    Lidar, Merav; Lipschitz, Noga; Langevitz, Pnina; Shoenfeld, Yehuda

    2009-08-01

    Infectious agents have been implicated in the etiopathogenesis of various vasculitides via numerous and overlapping mechanisms including direct microbial invasion of endothelial cells, immune complex mediated vessel wall damage and stimulation of autoreactive B and/or T cells through molecular mimicry and superantigens. While the causative role of hepatitis B virus in polyarteritis nodosa and hepatitis C virus in mixed cryoglobulinemia is clearly established, evidence for the association of other infectious agents with vasculitis, including human immunodeficiency virus, parvovirus B19, cytomegalovirus, varicella zoster virus, Staphylococcus aureus, rickettsiaceae, Treponema pallidum and Borrelia burgdorferi, among numerous others, is accumulating. The spectrum of association of infectious agents; bacteria, viruses and parasites, with systemic vasculitides, will be reviewed herewith.

  6. The activation of the neutrophil respiratory burst by anti-neutrophil cytoplasm autoantibody (ANCA) from patients with systemic vasculitis requires tyrosine kinases and protein kinase C activation

    PubMed Central

    Radford, D J; Lord, J M; Savage, C O S

    1999-01-01

    The ability of antineutrophil cytoplasm autoantibodies (ANCA) from patients with systemic vasculitis to stimulate protein kinase C (PKC) and tyrosine kinases was examined in human neutrophils. Using the superoxide dismutase-inhibitable reduction of ferricytochrome C, the kinetics of ANCA-induced superoxide (O2−) production were characterized and subsequently manipulated by specific inhibitors of PKC and tyrosine kinases. With this approach, ANCA IgG, but not normal IgG or ANCA F(ab′)2 fragments caused a time and dose dependent release of O2− from TNF-α primed neutrophils. The kinetics of ANCA-induced O2− production showed an initial 10–15 min lag phase compared to the N-formyl-l-methionyl-l-leucyl-l-phenylalanine response, suggesting differences in the signalling pathways recruited by these two stimuli. Inhibitor studies revealed that ANCA-activation involved members of both the Ca2+-dependent and -independent PKC isoforms and also tyrosine kinases. ANCA IgG resulted in the translocation of the βII isoform of PKC at a time corresponding to the end of the lag phase of O2− production, suggesting that PKC activity may be instrumental in processes regulating the activity of the NADPH oxidase in response to ANCA. Tyrosine phosphorylation of numerous proteins also peaked 10–15 min after stimulation with ANCA but not normal IgG. These data suggest that PKC and tyrosine kinases regulate O2− production from neutrophils stimulated with autoantibodies from patients with systemic vasculitis. PMID:10540175

  7. Mannose-binding lectin (MBL) codon 54 (rs1800450) polymorphism predisposes towards medium vessel vasculitis in patients with systemic lupus erythematosus.

    PubMed

    Negi, Vir Singh; Devaraju, Panneer; Misra, Durga Prasanna; Jain, Vikramraj K; Usdadiya, Jignesh Babulal; Antony, Paul T; Gulati, Reena

    2017-04-01

    Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with multiple etiological factors. Mannose-binding lectin (MBL) plays a key role in innate immunity by activating antibody-independent lectin complement pathway, opsonisation, phagocytosis, and immune complex (IC) clearance. Genetic polymorphisms in the promoter and coding regions of MBL gene affect the circulatory levels and biological activity of MBL. Defects in MBL can lead to defective opsonisation and, hence, hamper clearance of apoptotic debris, the persistence of which can drive autoantibody formation in lupus. The exon1 variants at codon 52, 54, and 57 have been reported to augment the risk of SLE in different ethnic populations. Three hundred South Indian Tamil patients with SLE and 460 age-, sex-, and ethnicity-matched controls were genotyped for three polymorphisms at codon 52, 54, and 57 in exon1 of MBL gene by Taqman real-time PCR. The three polymorphisms in exon1 of MBL were observed not to confer risk of developing SLE. However, MBL codon 54 rs1800450 polymorphism was associated with the development of medium vessel vasculitis and gangrene (OR-2.29, CI 95% 1.08-4.83, p = 0.02), whereas, the ancestral allele G conferred protection (OR-0.44, CI 95% 0.21-0.93, p = 0.02). Genetic variants in the exon1 of MBL gene per se are not risk factors for SLE in South Indian Tamils. However, the association of codon 54 (rs1800450) with medium vessel vasculitis suggests that it may be a genetic modifier of clinical phenotype in SLE.

  8. Can rheumatoid vasculitis predate a diagnosis of rheumatoid arthritis?

    PubMed Central

    Sacks, Sarah; Steuer, Alan

    2017-01-01

    We report regarding a male patient who presented with a systemic vasculitis that was consistent with a diagnosis of polyarteritis nodosa. At presentation, he had no features of inflammatory arthritis but had a high rheumatoid factor titer and low C4 level. Withdrawal of immunosuppression after 6 years resulted in the development of classical rheumatoid arthritis (RA). This case supports previous reports that revealed that vasculitis may predate the development or occur very early in the course of articular RA. PMID:28293454

  9. Renal vascular lesions in systemic lupus erythematosus.

    PubMed

    Katz, S M; Korn, S; Umlas, S L; DeHoratius, R J

    1990-01-01

    In the past, necrotizing vasculitis has been considered to be one of the dominant intrarenal vascular abnormalities in systemic lupus erythematosus (SLE). To test the validity of this statement, 70 consecutive renal biopsies from patients with SLE were reviewed. Light microscopy (LM) and immunofluorescence (IF) studies documented abnormalities, including thrombosis and nephrosclerosis, in 30 patients (43 percent), but no cellular infiltration of the vessel walls or other evidence of acute necrotizing vasculitis was seen. It is concluded that while intrarenal vasculopathy with thrombosis and nephrosclerosis is a common finding in SLE, our data and recently published studies suggest that acute necrotizing vasculitis occurs rarely, if at all, in SLE nephritis.

  10. Changes in urinary metabolomic profile during relapsing renal vasculitis

    PubMed Central

    Al-Ani, Bahjat; Fitzpatrick, Martin; Al-Nuaimi, Hamad; Coughlan, Alice M.; Hickey, Fionnuala B.; Pusey, Charles D.; Savage, Caroline; Benton, Christopher M.; O’Brien, Eóin C.; O’Toole, Declan; Mok, Ken H.; Young, Stephen P.; Little, Mark A.

    2016-01-01

    Current biomarkers of renal disease in systemic vasculitis lack predictive value and are insensitive to early damage. To identify novel biomarkers of renal vasculitis flare, we analysed the longitudinal urinary metabolomic profile of a rat model of anti-neutrophil cytoplasmic antibody (ANCA) vasculitis. Wistar-Kyoto (WKY) rats were immunised with human myeloperoxidase (MPO). Urine was obtained at regular intervals for 181 days, after which relapse was induced by re-challenge with MPO. Urinary metabolites were assessed in an unbiased fashion using nuclear magnetic resonance (NMR) spectroscopy, and analysed using partial least squares discriminant analysis (PLS-DA) and partial least squares regression (PLS-R). At 56 days post-immunisation, we found that rats with vasculitis had a significantly different urinary metabolite profile than control animals; the observed PLS-DA clusters dissipated between 56 and 181 days, and re-emerged with relapse. The metabolites most altered in rats with active or relapsing vasculitis were trimethylamine N-oxide (TMAO), citrate and 2-oxoglutarate. Myo-inositol was also moderately predictive. The key urine metabolites identified in rats were confirmed in a large cohort of patients using liquid chromatography–mass spectrometry (LC-MS). Hypocitraturia and elevated urinary myo-inositol remained associated with active disease, with the urine myo-inositol:citrate ratio being tightly correlated with active renal vasculitis. PMID:27905491

  11. Circulating and tissue-bound immune complexes in allergic vasculitis: relationship between immunoglobulin class and clinical features.

    PubMed Central

    Kauffmann, R H; Herrmann, W A; Meijer, C J; Daha, M R; Vanes, L A

    1980-01-01

    Sixty-two patients with allergic vasculitis and histological evidence of leukocytoclastic vasculitis were examined to determine whether clinical features, such as course and degree of systemic involvement, could be related to the class of the immunoglobulins in immune complexes (IC) in both the circulation and the vessel wall. Circulating IC were detected with the 125I-C1q-binding assay (C1q-BA), an anti-IgA inhibition binding assay (a-IgA-Inh BA) and the conglutinin-binding assay (Con-BA). Stabilized heat-aggregated IgG, IgA and IgM were used to determine the immunoglobulin class specificity of these assays. With the C1q-BA only aggregated IgG were detected, with the a-IgA-Inh Ba only aggregates containing IgA. With the Con-BA IgG, IgA or IgM in reactive aggregates were identified with class-specific antibodies. For patients with acute cutaneous vasculitis all assays were negative. The a-IgA-Inh BA was frequently positive in sera of patients with chronic cutaneous and acute systemic vasculitis; in the latter group conglutinin-binding IC of the IgG, IgA and IgM class were also detected. Levels in the C1q-BA were high for patients with chronic systemic vasculitis. Comparison of the results of the IC assays with the immunofluorescence studies of the cutaneous vessel walls of the same patients showed agreement between the results of the C1q-BA and deposition of IgG and the results of the a-IgA-Inh BA and IgA deposition. The class of immunoglobulin in conglutinin-binding IC did not correspond as well with the immunoglobulins in vessel walls. This study shows that certain clinical features of allergic vasculitis are related to the composition of the IC in the circulation and in the vessel wall. PMID:7438561

  12. The treatment of disseminated vasculitis with methotrexate.

    PubMed

    Mitchell, M S; Gifford, R H; Bertino, J R; Kenney, J D; Malawista, S E

    1976-06-01

    With the great interest in recent years in the use of immunosuppressive/antiinflammatory drugs for the treatment of severe systemic inflammatory disorders, it is surprising that, except in Wegener's granulomatosis, the use of methotrexate (MTX) in disseminated vasculitides has been neglected. We have treated three patients with MTX, each with a different form of severe, corticosteroid-resistant, life-threatening, disseminated vasculitis. One had a nonspecific vasculitis characterized by excruciating muscle pain, weakness, and atrophy; the second had rheumatoid vasculitis with extensive skin ulceration and four-limb mononeuritis multiplex; the third had polyarteritis nodosa with hypertension, eventually fatal renal disease, and severe peripheral neuropathy. Treatment in each case was associated with a dramatic, favorable change in a course previously marked by relentless deterioration; two patients are alive 4 and 5 years later, without MTX. The purpose of this preliminary report is to indicate the possible efficacy of MTX in a wider group of disorders than those to which it is generally limited at present, and to stimulate its further evaluation.

  13. The ANCA Vasculitis Questionnaire (AAV-PRO©)

    ClinicalTrials.gov

    2016-05-10

    Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss) (EGPA); Churg-Strauss Syndrome (CSS); Granulomatosis With Polyangiitis (Wegener's) (GPA); Wegener Granulomatosis (WG); Microscopic Polyangiitis (MPA); ANCA-Associated Vasculitis (AAV); Vasculitis

  14. A Rare Case of Hepatitis C-Associated Cryoglobulinemic Duodenal Vasculitis

    PubMed Central

    Gomez, Alexandra; Dholaria, Kevin; Arosemena, Leopoldo R.; Ladino-Avellaneda, Marco A.; Barisoni, Laura; Bhamidimarri, Kalyan R.

    2016-01-01

    Intestinal involvement of cryoglobulinemia is an uncommon manifestation and marker of severe vasculitis. We describe the case of a woman admitted to our service for management of acute renal failure and progressive gastrointestinal symptoms after initiating hepatitis C virus treatment with ribavirin and sofosbuvir 4 weeks prior. With an undetectable hepatitis C viral load and persistent symptoms despite hepatitis C virus therapy cessation, an upper endoscopy revealed duodenal sloughing, erythema, and bleeding, sparking suspicion for recurrence of cryoglobulinemic vasculitis. PMID:27807586

  15. Cerebral vasculitis associated with cocaine abuse

    SciTech Connect

    Kaye, B.R.; Fainstat, M.

    1987-10-16

    A case of cerebral vasculitis in a previously healthy 22-year-old man with a history of cocaine abuse is described. Cerebral angiograms showed evidence of vasculitis. A search for possible causes other than cocaine produced no results. The authors include cocaine with methamphetamines, heroin, and ephedrine as illicit drugs that can cause cerebral vasculitis.

  16. Exercise-Induced Vasculitis: A Review with Illustrated Cases.

    PubMed

    Espitia, Olivier; Dréno, Brigitte; Cassagnau, Elisabeth; Didier, Quentin; Quillard, Thibaut; Nicol, Christelle; Le Bouch, Yann; Planchon, Bernard; Pistorius, Marc-Antoine

    2016-12-01

    Although exercise-induced vasculitis (EIV) is usually misdiagnosed, it is not uncommon. Occurring mostly after prolonged exercise, especially in hot weather, EIV is an isolated cutaneous vasculitis with stereotypical presentation. This article reviews the clinical characteristics, treatments, and outcomes of EIV based on the published literature. We report 99 patients who developed EIV after walking, dancing, swimming, or hiking especially during hot weather, including the records of 16 patients with EIV treated at our hospital from 2007 to 2015. Erythematous or purpuric plaques arise on the lower legs, without the involvement of compression socks or stockings. Symptoms include itch, pain, and a burning sensation. EIV is an isolated cutaneous vasculitis. Lesions resolve spontaneously after 10 days. When triggering conditions persist, relapses are frequent (77.5 %). Histopathology demonstrates leukocytoclastic vasculitis in 95 % of cases with C3 or immunoglobulin M deposits in 88 and 46 % of cases, respectively. Blood investigations are negative. EIV appears to be a consequence of venous stasis induced by an acute failure of the muscle pump of the calf and thermoregulation decompensation. Both appear after prolonged and unusual exercise in hot weather. Treatment is not codified; topical corticosteroids may reduce symptoms and wearing light clothes might limit lesion occurrence.

  17. Mycoplasmal Upper Respiratory Infection Presenting as Leukocytoclastic Vasculitis

    PubMed Central

    Rao, Mana; Agrawal, Abhinav; Parikh, Manan; Banayat, Rikka; Thomas, Maria Joana; Guo, Tianhua; Lee, Andrew

    2015-01-01

    Mycoplasma is a virulent organism that is known to primarily infect the respiratory tract; however, affection of the skin, nervous system, kidneys, heart and bloodstream has been observed in various forms, which include Stevens Johnson syndrome, erythema multiforme, toxic epidermal necrolysis, encephalitis, renal failure, conduction system abnormalities and hemolytic anemia. Small vessel vasculitis is a lesser-known complication of mycoplasma pneumonia infection. We report a case of mycoplasmal upper respiratory tract infection with striking cutaneous lesions as the presenting symptom. Mycoplasmal infection was confirmed by serology testing, skin biopsy was suggestive of leukocytoclastic vasculitis. This case brings forth an uncommon manifestation of mycoplasmal infection with extra-pulmonary affection, namely small vessel vasculitis. PMID:25874067

  18. Interstitial Lung Disease with ANCA-associated Vasculitis

    PubMed Central

    Katsumata, Yasuhiro; Kawaguchi, Yasushi; Yamanaka, Hisashi

    2015-01-01

    The association between interstitial lung disease (ILD) and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), particularly microscopic polyangiitis (MPA), has been described in a number of case reports and case series reports in the last 2 decades. In addition, patients with pulmonary fibrosis and ANCA positivity but without other manifestations of systemic vasculitis have also been reported. Pulmonary fibrosis was clinically manifested at the time of diagnosis in the majority of AAV patients that developed this condition. Moreover, ANCA-positive conversion occurs in patients initially diagnosed with idiopathic pulmonary fibrosis, and as a result, other manifestations of systemic vasculitis develop in some of these patients. There is significant predominance of myeloperoxidase (MPO)-ANCA and MPA in patients with AAV and ILD. Radiological and pathological findings generally demonstrate usual interstitial pneumonia (pattern) in the lungs of these patients. In most studies, AAV patients with ILD have a worse prognosis than those without it. PMID:26448696

  19. The natural history of vasculitis. What the histology tells us about pathogenesis.

    PubMed

    Smoller, B R; McNutt, N S; Contreras, F

    1990-01-01

    While histopathologic analysis may offer some clues as to the pathogenesis of vasculitis, observations must be interpreted with caution, as there is considerable overlap in the histologic pattern. In most cases, a predominantly neutrophilic vasculitis affecting small dermal venules suggests a relatively acute, immune complex-mediated reaction. Less commonly, this histologic pattern may be seen in non-immunologically mediated processes, such as in the presence of bacterial toxins or malignant hypertension, or in more chronic disease states, such as granuloma faciale or erythema elevatum diutinum. A predominantly lymphocytic vasculitis may represent several pathogenetic mechanisms. In lesions more than 24 to 48 hours old, a lymphocytic vasculitis may represent a resolving phase of an immune complex-mediated neutrophilic vasculitis. Alternatively, this histologic pattern may be seen de novo in conditions with a presumed cell-mediated immunologic pathogenesis. Lymphocytic vasculitis may also be seen in rickettsial infections such as Rocky Mountain spotted fever. The pathogenesis of granulomatous vasculitis remains poorly understood and is thought to be induced by a combination of circulating immune complexes and a cell-mediated immune response.

  20. A Case of Brucellosis with Recurrent Attacks of Vasculitis

    PubMed Central

    Korkmaz, Pınar; Kıdır, Mehtap; Namdar, Nazlı Dizen; Özmen, Ahmet; Uyar, Cemile; Değer, Ayşe Nur

    2016-01-01

    Brucellosis is a zoonosis that affects several organs or systems. Skin involvement is nonspecific and it is reported to range between 0,4 and 17% of the patients with brucellosis. Here, we defined a 36-year-old female patient presented to our clinic with a clinical picture of recurrent attacks of vasculitis due to brucellosis for the first time. Skin involvement and vasculitic lesions as a finding of skin involvement are nonspecific in brucellosis. Therefore, in the regions like Turkey where brucellosis is endemic, brucellosis should be kept in mind necessarily in the differential diagnosis of vasculitis. PMID:27042369

  1. A Case of Brucellosis with Recurrent Attacks of Vasculitis.

    PubMed

    Korkmaz, Pınar; Kıdır, Mehtap; Namdar, Nazlı Dizen; Özmen, Ahmet; Uyar, Cemile; Değer, Ayşe Nur

    2016-01-01

    Brucellosis is a zoonosis that affects several organs or systems. Skin involvement is nonspecific and it is reported to range between 0,4 and 17% of the patients with brucellosis. Here, we defined a 36-year-old female patient presented to our clinic with a clinical picture of recurrent attacks of vasculitis due to brucellosis for the first time. Skin involvement and vasculitic lesions as a finding of skin involvement are nonspecific in brucellosis. Therefore, in the regions like Turkey where brucellosis is endemic, brucellosis should be kept in mind necessarily in the differential diagnosis of vasculitis.

  2. Behçet's disease diagnosed after acute HIV infection: viral replication activating underlying autoimmunity?

    PubMed

    Roscoe, Clay; Kinney, Rebecca; Gilles, Ryan; Blue, Sky

    2015-05-01

    Behçet's disease is an autoimmune systemic vasculitis that can occur after exposure to infectious agents. Behçet's disease also has been associated with HIV infection, including de novo development of this condition during chronic HIV infection and resolution of Behçet's disease symptoms following initiation of antiretroviral therapy. We describe a patient who presented with systemic vasculitis with skin and mucous membrane ulcerations in the setting of acute HIV infection, who was eventually diagnosed with Behçet's disease, demonstrating a possible link between acute HIV infection, immune activation and development of autoimmunity.

  3. [Uptodate in the management and treatment of ANCA-associated vasculitis].

    PubMed

    Belaconi, Ionela Nicoleta; Toma, Claudia Lucia; Bogdan, Miron Alexandru

    2014-01-01

    The antineutrophil cytoplasm antibody (ANCA)-associated vasculitis are heterogeneous, multisystem, autoimmune diseases characterized by necrotizing small and medium vessel vasculitis and the association with ANCA. The diagnosis and management of these patients may be challenging due to the variability of clinical features, the possibility of life-threatening events (acute renal failure or pulmonary hemorrhage) and the relative rarity of these syndromes. ANCA-associated vasculitis include granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. The treatment requires significant immunosuppression and there are frequent treatment related side effects. Although the standard protocol with cytotoxic agents and glucocorticoids has dramatically improved patient outcome, its toxic profile remains a major problem. Recent progress in evidence base and consensus in understanding the pathogenic mechanism and the quantification of disease activity further improved patient's life. Special attention was paid in refining immunosuppressive treatment to minimize his toxicity. This review will focus on evidence based treatment of ANCA-associated vasculitis.

  4. Proteinase 3-ANCA Vasculitis versus Myeloperoxidase-ANCA Vasculitis

    PubMed Central

    Hilhorst, Marc; van Paassen, Pieter

    2015-01-01

    In patients with GN or vasculitis, ANCAs are directed against proteinase 3 (PR3) or myeloperoxidase (MPO). The differences between PR3-ANCA-associated vasculitis (AAV) and MPO-AAV described in the past have been supplemented during the last decade. In this review, we discuss the differences between these two small-vessel vasculitides, focusing especially on possible etiologic and pathophysiologic differences. PR3-AAV is more common in northern parts of the world, whereas MPO-AAV is more common in southern regions of Europe, Asia, and the Pacific, with the exception of New Zealand and Australia. A genetic contribution has been extensively studied, and there is a high prevalence of the HLA-DPB1*04:01 allele in patients with PR3-AAV as opposed to patients with MPO-AAV and/or healthy controls. Histologically, MPO-AAV and PR3-AAV are similar but show qualitative differences when analyzed carefully. Clinically, both serotypes are difficult to distinguish, but quantitative differences are present. More organs are affected in PR3-AAV, whereas renal limited vasculitis occurs more often in patients with MPO-AAV. For future clinical trials, we advocate classifying patients by ANCA serotype as opposed to the traditional disease type classification. PMID:25956510

  5. Chronic iritis associated with cutaneous leukocytoclastic vasculitis.

    PubMed

    Lambert, Jessica; Rodriguez, Alexis; Pearcy-Baluyot, Mischelle; Shahi, Sanjeet K

    2015-05-01

    Cutaneous leukocytoclastic vasculitis (LCV) is a systemic condition that can be associated with iritis. LCV is characterized as a small-vessel vasculitis of the cutaneous area. The disease demonstrates purple lesions on the skin due to the destruction of small cutaneous blood vessels. These lesions are palpable and most often coalesce forming larger patches on the surface of the skin. During early stages of LCV, the disease can be undetected due to the infrequency and small size of the skin lesions. As such, the patient might go undiagnosed for years while having symptoms of LCV or iritis of unknown etiology. This article discusses the correlation seen with LCV and iritis. We report a case on a patient that presented to our clinic with a history of bilateral chronic iritis. After extensive laboratory testing, we concluded that the chronicity of her iritis was due to her LCV. The correlation between LCV and iritis was not evident for several years in our patient. We also discuss the correlation with systemic Sjogren's syndrome and LVC and how these two separate diseases are linked in many patients. We will illustrate the importance of serological testing, imaging, and skin lesion biopsy for the diagnosis of LCV.

  6. Cardiovascular magnetic resonance for evaluation of heart involvement in ANCA-associated vasculitis. A luxury or a valuable diagnostic tool?

    PubMed

    Mavrogeni, Sophie; Markousis-Mavrogenis, George; Kolovou, Genovefa

    2014-01-01

    Antineutrophil cytoplasmic antibody (ANCA)-related vasculitis is a systemic small-vessel vasculitis, including 3 clinical syndromes: granulomatosis with polyangiitis, known as Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and the Churg-Strauss syndrome (CSS). ANCA-related vasculitis usually presents with severe kidney or pulmonary disease, has a mortality of 28% at 5 years, and also contributes to increased morbidity in vasculitis patients. Cardiac involvement in this entity may have different forms, including coronary vessels, pericarditis, myocarditis, endocarditis, myocardial infarction and subendocardial vasculitis that can contribute to reduced life expectancy. Cardiovascular magnetic resonance using oedema and fibrosis imaging can early reveal, noninvasively and without radiation, heart involvement during vasculitis, undetected by other imaging techniques and guide further risk stratification and treatment of these patients.

  7. Ischemic Neuropathy Associated with Livedoid Vasculitis

    PubMed Central

    Kim, Jee-Eun; Park, Su-Yeon; Sinn, Dong In; Kim, Sung-Min; Hong, Yoon-Ho; Park, Kyung Seok; Lee, Kwang-Woo

    2011-01-01

    Background Livedoid vasculitis is a chronic dermatological problem with an unclear etiology. Clinical findings are petechiae with painful ulcers in both lower extremities, which heal to become hyperpigmented and porcelain-white satellite lesions. There are only a few reported cases of livedoid vasculitis presenting in combination with peripheral neuropathy. Case Report We report the first case of a Korean patient presenting with mononeuritis multiplex combined with livedoid vasculitis, which was confirmed by electrophysiological and pathological studies. Conclusions Our report supports the possible vaso-occlusive etiology of livedoid vasculitis in multifocal ischemic neuropathy. PMID:22259622

  8. Focal cerebral vasculitis associated with circulating immune complexes and brain irradiation

    SciTech Connect

    Groothuis, D.R.; Mikhael, M.A.

    1986-06-01

    In this report we describe a patient with a benign glioma treated with surgery and radiation. After a period of stability he developed subacute bacterial endocarditis, and deteriorated neurologically. Computed tomographic scans did not show recurrent tumor. An angiogram showed vasculitis restricted to the previously irradiated area. Secondary to subacute bacterial endocarditis was the presence of high levels of circulating immune complexes. His neurological status was unchanged after antibiotics, but improved after treatment with dexamethasone. We interpret the clinical course as an immune-complex-mediated vasculitis superimposed on a subclinical radiation vasculitis. This case supports the hypothesis that immune mechanisms may be involved in delayed radiation injury to the nervous system.

  9. Testicular Vasculitis: A Sonographic and Pathologic Diagnosis

    PubMed Central

    Hague, Cameron; Bicknell, Simon

    2017-01-01

    Very little has been published about single-organ vasculitis of the testicle in the radiological literature. Consequently, it is a diagnosis that is unfamiliar to most radiologists. This case report describes the sonographic, pathologic, and laboratory findings of testicular vasculitis and reviews the available literature with regard to this subject. PMID:28246567

  10. Cerebral vasculitis in adults: what are the steps in order to establish the diagnosis? Red flags and pitfalls

    PubMed Central

    Berlit, P; Kraemer, M

    2014-01-01

    Cerebral vasculitis is a rare cause of juvenile stroke. It may occur as primary angiitis of the central nervous system (PACNS) or as CNS manifestation in the setting of systemic vasculitis. Clinical hints for vasculitis are headache, stroke, seizures, encephalopathy and signs of a systemic inflammatory disorder. Diagnostic work-up includes anamnesis, whole body examination, laboratory and cerebral spinal fluid (CSF) studies, magnetic resonance imaging (MRI), angiography and brain biopsy. Due to the rarity of the disease, exclusion of more frequent differential diagnoses is a key element of diagnostic work-up. This review summarizes the steps that lead to the diagnosis of cerebral vasculitis and describes the red flags and pitfalls. Despite considering the dilemma of angiography-negative vasculitis and false-negative brain biopsy in some cases, it is important to protect patients from ‘blind’ immunosuppressive therapy in unrecognized non-inflammatory differential diagnosis. PMID:24117125

  11. Vasculitis

    MedlinePlus

    ... topics/vas/. Accessed July 16, 2014. Longo DL, et al. Harrison's Online. 18th ed. New York, N. ... resourceID=4. Accessed July 16, 2014. Gwathmey KG, et al. Vasculitic neuropathies. Lancet Neurology. 2014;13:67. ...

  12. Vasculitis

    MedlinePlus

    ... Fellows Evidence-Based Practice for Academic Researchers Responsible Data Management in Research Career Planning Treatments Patient and Caregiver ... Fellows Evidence-Based Practice for Academic Researchers Responsible Data Management in Research Career Planning Treatments Patient and Caregiver ...

  13. Acute transverse myelopathy complicating systemic lupus erythematosus.

    PubMed Central

    Propper, D J; Bucknall, R C

    1989-01-01

    A sixteen year old girl with systemic lupus erythematosus developed acute transverse myelopathy. She was treated with high dose steroids, cyclophosphamide, and plasma exchange and regained partial neurological function. Previous descriptions of transverse myelopathy complicating systemic lupus erythematosus are reviewed, with particular reference to the efficacy of high dose steroid treatment. PMID:2662918

  14. [Rituximab for the treatment of ANCA associated vasculitis: the future today?].

    PubMed

    Alba, Marco A; Flores-Suárez, Luis F

    2011-12-01

    Since cyclophosphamide was introduced for the treatment of ANCA-associated vasculitis, the mortality of these diseases has decreased considerably. However, such treatment is related to acute and chronic serious adverse effects, which contribute to the morbidity and mortality of such diseases. Therefore, one of the main challenges in the treatment of such conditions is to find newer and effective therapies with a safer profile. Rituximab (RTX), an anti-CD20 monoclonal antibody stands at the top of new options for the treatment of ANCA-associated vasculitis, and is the strongest candidate to establish itself as a first choice therapeutic agent. Here, we review the rationale of RTX treatment in ANCA-associated small vessel vasculitis, and the current evidence of both its efficacy and toxicity.

  15. What matters for patients with vasculitis?

    PubMed

    Novakovich, Elaine; Grayson, Peter C

    2015-06-01

    Advances in clinical care for patients with vasculitis have improved survival rates and created new challenges related to the ongoing management of chronic disease. Lack of curative therapies, burden of disease, treatment-related side effects, and fear of relapse contribute to patient-perceived reduction in quality of life. Patient-held beliefs about disease and priorities may differ substantially from the beliefs of their health care providers, and research paradigms are shifting to reflect more emphasis on understanding vasculitis from the patient's perspective. Efforts are ongoing to develop disease outcome measures in vasculitis that better represent the patient experience. Health care providers who care for patients with vasculitis should be sensitive to the substantial burdens of disease commonly experienced by patients living with the disease and should strive to provide comprehensive care directed towards the medical and biopsychological needs of these patients.

  16. Translational Mini-Review Series on Immunology of Vascular Disease: Accelerated atherosclerosis in vasculitis

    PubMed Central

    Tervaert, J W Cohen

    2009-01-01

    Premature atherosclerosis has been observed during the course of different systemic inflammatory diseases such as rheumatoid arthritis and sytemic lupus erythematosus. Remarkably, relatively few studies have been published on the occurrence of accelerated atherosclerosis in patients with vasculitis. In giant cell arteritis (GCA), mortality because of ischaemic heart disease is not increased. In addition, intima media thickness (IMT) is lower in patients with GCA than in age-matched controls. In contrast, IMT is increased significantly in Takayasu arteritis, another form of large vessel vasculitis occurring in younger patients. In Takayasu arteritis and in Kawasaki disease, a form of medium-sized vessel vasculitis, accelerated atherosclerosis has been well documented. In small vessel vasculitis because of anti-neutrophil cytoplasmic autoantibodies-associated vasculitis, cardiovascular diseases are a major cause of mortality. IMT measurements reveal conflicting results. During active disease these patients experience acceleration of the atherosclerotic process. However, when inflammation is controlled, these patients have atherosclerotic development as in healthy subjects. Several risk factors, such as diabetes and hypertension, are present more often in patients with vasculitis compared with healthy controls. In addition, steroids may be pro-atherogenic. Most importantly, many patients have impaired renal function, persistent proteinuria and increased levels of C-reactive protein, well-known risk factors for acceleration of atherosclerosis. Enhanced oxidation processes, persistently activated T cells and reduced numbers of regulatory T cells are among the many pathophysiological factors that play a role during acceleration of atherogenesis. Finally, autoantibodies that may be relevant for acceleration of atherosclerosis are found frequently in elevated titres in patients with vasculitis. Because patients have an increased risk for cardiovascular events, vasculitis

  17. Therapy of livedo vasculitis with pentoxifylline.

    PubMed

    Ely, H; Bard, J W

    1988-11-01

    Two patients with idiopathic livedo vasculitis responded favorably to therapy with pentoxifylline. One patient had responded to fibrinolytic therapy with phenformin and ethylestrenol before phenformin was taken off the market. Pentoxifylline (Trental) has multiple mechanisms of action, including stimulation of prostacyclin synthesis, decreased aggregation of platelets, increased deformability of red blood cells, increased mobility of neutrophils, and increased fibrinolysis. All of these factors may contribute to its successful use in the treatment of idiopathic livedo vasculitis.

  18. D-penicillamine-induced ANA (+) ANCA (+) vasculitis in pediatric patients with Wilson's disease.

    PubMed

    Lee, Yeonhee; Lee, Sang Taek; Cho, Heeyeon

    2016-05-01

    Anti-neutrophil cytoplasmic antibodies (ANCA) are associated with systemic vasculitis. The pathophysiology of ANCA-associated vasculitis (AAV) has not been clearly proven, and drug-induced ANCA-associated vasculitis has been reported. Wilson's disease is an inborn error of copper metabolism caused by a mutation in the copper transporting gene ATP7B, and traditional treatment is based on copper chelation with agents such as D-penicillamine. There have been rare reports that prolonged D-penicillamine therapy might cause adverse renal events such as membranous nephropathy and minimal change disease, but it is questionable if D-penicillamine induces ANCA-associated vasculitis. We describe 2 patients with Wilson's disease treated with D-penicillamine who presented with ANCA (+) vasculitis and renal involvement. The 2 patients also showed positive results for antinuclear antibody (ANA). Their kidney biopsy findings were compatible with crescentic/necrotizing glomerulonephritis, pauci-immune type. After diagnosis of AAV, D-penicillamine was stopped. Patients were then treated with plasmapheresis and immunosuppressants, including methylprednisolone pulse therapy and intravenous cyclophosphamide. One patient progressed to end-stage renal disease and the other showed persistent proteinuria. These cases suggest that D-penicillamine may induce ANA (+) ANCA (+) vasculitis with severe renal involvement in pediatric patients, and plasmapheresis combined with immunosuppressant should be considered.

  19. History of primary vasculitis in Latin America.

    PubMed

    Iglesias Gammara, Antonio; Coral, Paola; Quintana, Gerardo; Toro, Carlos E; Flores, Luis Felipe; Matteson, Eric L; Restrepo, José Félix

    2010-03-01

    A literature review utilizing Fepafem, Bireme, LiLacs, Scielo Colombia, Scielo Internacional, former MedLine, Pubmed, and BVS Colombia as well as manual searches in the libraries of major Latin American universities was performed to study vasculitis in Latin America. Since 1945, a total of 752 articles have been published by Latin American authors. However, only a minority are devoted to primary vasculitides, and even fewer have been published in indexed journals. Approximately 126 are in OLD, Medline, Pubmed, Bireme, and Scielo. Most publications are from Mexico, followed by Brazil and Colombia. Systematic studies of the epidemiology of primary idiopathic vasculitis are available for a few countries, i.e. Brazil, Mexico, Colombia, Chile, and Peru. Takayasu arteritis and ANCA-associated vasculitis are the best studied forms of vasculitis in Latin America. Interest and expertise in vasculitis is growing in Latin America, as reflected in the increased number of published articles from this region of the world in the last decade. Racial and environmental factors are possibly responsible for the differential expression of various types of primary vasculitis observed in Latin America. With time, the unique features, epidemiology, and better treatment strategies for idiopathic vasculitides in Latin America will emerge.

  20. [Treatment of cryoglobulinemic vasculitis associated with hepatitis C virus infection].

    PubMed

    Retamozo, Soledad; Brito-Zerón, Pilar; Ramos-Casals, Manuel

    2015-05-08

    Cryoglobulinemia is a heterogeneous systemic autoimmune disease with a wide variety of causes, symptoms and outcomes, and different etiopathogenic pathways involved in the vasculitic organ damage. The discovery of the hepatitis C virus (HCV) in 1989 changed radically the focus of research of the so-called "essential" cryoglobulinemia. Cryoglobulins can be detected in 25-30% of patients with HCV, overwhelmingly representing mixed cryoglobulins. However, only 10-15% of patients present with cryoglobulinemic vasculitis, with a broad spectrum of symptoms including mild or life-threatening manifestations. Consequently, not all patients can be uniformly treated. The key therapeutic points in HCV+ patients with cryoglobulinemic vasculitis cover different aspects. The first is to treat the underlying cause of cryoglobulinemia whenever possible, hence the use of antiviral therapies must always be considered in these patients. An individualized diagnostic approach to assess the number of organs involved and the severity of organ involvement is also essential in the therapeutic planning. This complex clinical scenario leads to an equally complex therapeutic scenario. There are three main treatment strategies for HCV-associated cryoglobulinemic vasculitis: conventional immunosuppression, antiviral treatment and biological therapies. The most recent studies are suggesting a change from the classical therapeutic approach (monotherapeutic regimens) to combination/sequential regimens, including treatments targeting the virus and those directed against the induced autoimmune disease, with the aim of blocking the various etiopathogenic pathways involved.

  1. Lung vasculitis and alveolar hemorrhage: pathology.

    PubMed

    Fishbein, Gregory A; Fishbein, Michael C

    2011-06-01

    Pulmonary vasculitides are a diverse group of limited and systemic disorders associated with inflammation of pulmonary vessels and parenchyma. These diseases often have distinctive clinical, serological, and histopathological features-extrapulmonary sites of involvement, circulating autoantibodies, predispositions for small or large vessels, and others. Some have characteristic inflammatory lesions; others are characterized by the absence of such lesions. Frequently pathological findings overlap, rendering classification, and diagnosis a challenge. The anti-neutrophil cytoplasmic antibody (ANCA)-associated small-vessel diseases constitute the major pulmonary vasculitides. These include Wegener granulomatosis (WG), Churg Strauss syndrome (CSS), and microscopic polyangiitis (MPA). Less frequently, diseases such as polyarteritis nodosa, Takayasu arteritis, Behçet syndrome, and connective tissue diseases may involve pulmonary vessels, but these entities are better associated with extrapulmonary disease. Diffuse alveolar hemorrhage (DAH) is a severe manifestation of pulmonary vasculitis. DAH is most commonly seen in small-vessel vasculitides, specifically MPA and WG. Other syndromes associated with DAH include Goodpasture syndrome, Henoch-Schönlein purpura, and systemic lupus erythematosus. Less commonly, DAH may be secondary to infection or drugs/toxins. Furthermore, in the absence of discernable systemic disease, DAH may be idiopathic-referred to as isolated pulmonary capillaritis (IPC) or idiopathic pulmonary hemosiderosis (IPH), depending on the presence of capillaritis.

  2. [Neuromuscular biopsy and diagnosis of vasculitis].

    PubMed

    Vital, Anne; Vital, Claude

    2006-09-01

    One characteristic histological lesion on biopsy specimens is mandatory to establish the diagnosis of vasculitis. Combined nerve and muscle biopsies, by the same cutaneous incision, improve significantly the percentage of positive results. Nerve fragments should be taken in every patient presenting sensory manifestations. Such vasculitic lesions are present in medium-sized arterioles and/or small vessels, and correspond mainly to 4 necrotizing vasculitis: panarteritis nodosa (PAN), microscopic polyangiitis (MPA), Churg and Strauss syndrome and Wegener granulomatosis. Microvasculitis should be added to these classical entities, because it corresponds to small vessel wall infiltration by inflammatory cells, as observed in PAN and MPA, but without any necrosis. Microvasculitis has to be differentiated from the inflammatory cell infiltrates surrounding small vessels. However, such perivascular inflammatory cell infiltrates enable the diagnosis of probable vasculitis when associated with clusters of neo-vessels, hemosiderin deposits, or a focal damage of nerve fibers. Grossly, one third of vasculitis diagnosis is confirmed on muscle fragments, a second third on nerve fragments, and the last third on both nerve and muscle fragments. Moreover, in the search for vasculitis, an unpredicted diagnosis of lymphoma or amyloidosis is occasionally established on the neuro-muscular biopsy.

  3. ANCA-associated vasculitis in Hispanic Americans: an unrecognized severity.

    PubMed

    Sreih, Antoine G; Mandhadi, Ranadeep; Aldaghlawi, Fadi; Khan, Asad; Irshad, Vajiha; Finn, Katherine; Block, Joel A

    2015-05-01

    This study aims to compare the severity and outcomes of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) between Hispanics and Caucasians living in the same geographical area. All patients diagnosed with AAV at two academic institutions in Chicago from January 2006 to December 2012 were retrospectively and prospectively identified. Disease activity was measured with the Birmingham Vasculitis Activity Score (BVAS), and disease damage was measured with the Vasculitis Damage Index (VDI). Student's t test and chi-square tests were employed; p ≤ 0.05 was considered significant. Seventy patients with AAV were identified; 15 patients were excluded. Fifty-five patients were included in the study: 23 Hispanics and 32 Caucasians, 35 patients with granulomatosis with polyangiitis (Wegener's), 12 with microscopic polyangiitis, 7 with eosinophilic granulomatosis with polyangiitis, and 1 with renal-limited vasculitis. Compared to Caucasians, Hispanics had a higher BVAS at presentation (16.3 ± 7.6 versus 10.7 ± 7.5, p = 0.006), a higher VDI at presentation (2.90 ± 1.50 versus 2.06 ± 1.30, p = 0.030), and a cumulative VDI (3.90 ± 1.70 versus 2.50 ± 1.90, p = 0.010). Renal involvement was more common among Hispanics (85 % of Hispanics versus 48 % of Caucasians, p = 0.01). Seventy percent of Hispanics had acute renal failure (mean creatinine = 3.37 ± 4.4 mg/dl) of whom seven (50 %) required dialysis, versus 25 % of Caucasians (mean creatinine = 1.78 ± 1.57 mg/dl, p = 0.03) and only two requiring dialysis. Compared to Caucasians, Hispanics with AAV present with more severe disease and higher damage indices. Larger studies are required to confirm these findings and delineate the respective roles of environment and genetics in the pathogenesis of the disease.

  4. Propylthiouracil-Associated Leukocytoclastic Necrotizing Cutaneous Vasculitis: A Case Report and Review of the Literature.

    PubMed

    Wall, Anji E; Weaver, Sheena M; Litt, Jeffrey S; Rae, Lisa

    2016-11-11

    The purpose of this case report and review of the literature is to provide an exploration of the clinical symptoms, diagnosis, prevention, and management of propylthiouracil (PTU)-associated vasculitis in the intensive care setting. A PubMed search of the available literature was conducted using the MeSH search terms "propylthiouracil" and "vasculitis." The literature search returned 121 articles. Twenty-five were excluded because they were not in English. Fifty-nine case reports or case studies describing PTU-associated vasculitis were included. Data extracted from each case study included patient age, sex, autoimmune markers, laboratory tests, length of time on PTU, treatment for vasculitis, and patient outcomes. The authors reviewed 128 cases of PTU-associated vasculitis. The majority were women (8.8:1 F:M ratio), and the most common presenting symptoms were rash (51.6%), fever (46.9%), and arthralgia (43.8%). In addition to discontinuing PTU, the most common treatment was steroids (71.9%). Eight patients (6.3%) progressed to end-stage renal disease; two (1.6%) required intubation for respiratory failure; and five (3.9%) died of various organ systems failure related to vasculitis development. A high index of suspicion for vasculitis should be maintained, especially when presented with skin manifestations in the presence of PTU therapy. Screening with myeloperoxidase-antinuclear cytoplasmic antibodies is most sensitive. Positive screening should prompt a thorough clinical investigation. In cases of severe skin manifestations, the focus should be on aggressive wound care. Our case report is unique, not only in the size and extent of cutaneous involvement, but also as the first description of mortality secondary to cutaneous manifestations.

  5. Therapeutic advances in the treatment of vasculitis.

    PubMed

    Eleftheriou, Despina; Brogan, Paul A

    2016-04-26

    Considerable therapeutic advances for the treatment of vasculitis of the young have been made in the past 10 years, including the development of outcome measures that facilitate clinical trial design. Notably, these include: a recognition that some patients with Kawasaki Disease require corticosteroids as primary treatment combined with IVIG; implementation of rare disease trial design for polyarteritis nodosa to deliver the first randomised controlled trial for children; first clinical trials involving children for anti-neutrophil cytoplasmic antibody (ANCA) vasculitis; and identification of monogenic forms of vasculitis that provide an understanding of pathogenesis, thus facilitating more targeted treatment. Robust randomised controlled trials for Henoch Schönlein Purpura nephritis and Takayasu arteritis are needed; there is also an over-arching need for trials examining new agents that facilitate corticosteroid sparing, of particular importance in the paediatric population since glucocorticoid toxicity is a major concern.

  6. Impaired activation of the fibrinolytic system in children with Henoch-Schönlein purpura: beneficial effect of hydrocortisone plus Sigma-aminocaproic acid therapy on disappearance rate of cutaneous vasculitis and fibrinolysis.

    PubMed

    Prandota, J; Pankow-Prandota, L; Kotecki, L

    2001-01-01

    Systemic vasculitis is a predominant clinical symptom in Henoch-Schönlein purpura (HSP), and some studies suggested that decreased blood fibrinolytic activity, as well as blood platelets, is of importance in the development of cutaneous vasculitis. Although patients with HSP have normal blood coagulation, little is known about the fibrinolytic system. On the other hand, it is known that the focus of Sigma-aminocaproic acid (EACA) activity in vivo is probably the blood platelet-vessel wall interaction or a vascular component alone. The aim of this study was, therefore, to investigate blood coagulation and fibrinolytic system as well as the effect of hydrocortisone (H) plus EACA therapy (Group I) on plasma antithrombin-III (AT-III), alpha1-proteinase inhibitor (alpha1-PI), alpha2-antiplasmin (alpha2-A), alpha2-macroglobulin (alpha2-M) activity, fibrinogen and plasminogen concentrations in plasma, euglobulin clot lysis time (ELT), and disappearance rate of cutaneous vasculitis in 14 children with HSP aged 7.6 +/- 3.1 (SD) years. Ten patients (8.6 +/- 2.5 years old) were treated with H alone (Group II), and 8 healthy, age-matched children served as controls. Plasma proteinase inhibitor activity was estimated with the kinetic method using Boehringer chromozyme tests before administration of H (9.2 +/- 3.3 mg/kg/d, i.v.) plus EACA (140 +/- 52 mg/kg/d, p.o.) for 5.93 +/- 2.05 days, and 24 hours after the last dose of EACA, as well as before and after treatment with H alone (8.25 +/- 1.74 mg/kg/24 h, i.v.) for 7.1 +/- 1.2 days. It was found that patients with HSP had the initial fibrinogen and plasminogen plasma concentrations significantly increased compared with the controls (Group I: 3.93 +/- 1.3 g/L and 124 +/- 38%; Group II: 4.24 +/- 0.89 g/L and 134 +/- 42% vs. 2.96 +/- 0.34 g/L, and 90 +/- 14%, respectively). Also, there was a marked decrease of the initial plasma alpha2-A activity in Group II compared with the controls (0.69 +/- 0.29 vs. 0.94 +/- 0.11 IU

  7. Leukocytoclastic vasculitis drug reaction to certolizumab pegol

    PubMed Central

    Warren, Donald; Speck, Laura; Jackson, Julie

    2017-01-01

    Tumor necrosis factor (TNF)-alpha antagonists are a common treatment modality for autoimmune disorders, but their use can be associated with many side effects, including various dermatologic conditions. Certolizumab pegol, a newer TNF antagonist that lacks the Fc portion of the IgG antibody, has recently been approved to treat psoriatic arthritis, rheumatoid arthritis, and Crohn's disease. Though other TNF antagonists have been associated with leukocytoclastic vasculitis, this finding has not yet been reported with certolizumab pegol. We present a case report of leukocytoclastic vasculitis caused by certolizumab pegol.

  8. Rheumatoid vasculitis: early presentation of rheumatoid arthritis.

    PubMed

    Abdulqader, Yasir; Al-Ani, Muhsen; Parperis, Konstantinos

    2016-11-08

    Rheumatoid vasculitis is a rare and late complication of rheumatoid arthritis and may affect small-to-medium-sized vessels. Here, we report a case of a 49-year-old man who presented with amaurosis fugax in the left eye, symmetric polyarthritis, Raynaud's symptoms and paraesthesia in both lower extremities. The patient subsequently experienced right foot drop, nail fold infracts and gangrene of his right second toe. He was found to have a high titre of rheumatoid factor and treatment with rituximab and high dose of corticosteroids led to significant improvement of his symptoms. This is rare case describing the early onset of rheumatoid vasculitis in a patient with rheumatoid arthritis.

  9. Acute pneumonia and the cardiovascular system.

    PubMed

    Corrales-Medina, Vicente F; Musher, Daniel M; Shachkina, Svetlana; Chirinos, Julio A

    2013-02-09

    Although traditionally regarded as a disease confined to the lungs, acute pneumonia has important effects on the cardiovascular system at all severities of infection. Pneumonia tends to affect individuals who are also at high cardiovascular risk. Results of recent studies show that about a quarter of adults admitted to hospital with pneumonia develop a major acute cardiac complication during their hospital stay, which is associated with a 60% increase in short-term mortality. These findings suggest that outcomes of patients with pneumonia can be improved by prevention of the development and progression of associated cardiac complications. Before this hypothesis can be tested, however, an adequate mechanistic understanding of the cardiovascular changes that occur during pneumonia, and their role in the trigger of various cardiac complications, is needed. In this Review, we summarise knowledge about the burden of cardiac complications in adults with acute pneumonia, the cardiovascular response to this infection, the potential effects of commonly used cardiovascular and anti-infective drugs on these associations, and possible directions for future research.

  10. Muscle restricted vasculitis causing dropped head syndrome: a case report and review of the literature.

    PubMed

    Nielsen, Amy Almaraz; Smith, Benn E; Engel, Andrew G; Bosch, Erich Peter

    2012-03-01

    A 52-year-old man presented with a severe head drop and proximal extremity weakness. Magnetic resonance imaging of the cervical spine showed T2 hyperintensity in cervical paraspinal muscles. Electrodiagnostic studies revealed an axial myopathy isolated to paraspinal muscles. A splenius capitis muscle biopsy confirmed an acute myopathy associated with nonsystemic vasculitis. The patient improved on steroids, intravenous immunoglobulin, and monthly pulse doses of cyclophosphamide. Our case emphasizes that a subgroup of patients with dropped head syndrome have treatable conditions.

  11. Dense Deposit Disease Mimicking a Renal Small Vessel Vasculitis.

    PubMed

    Singh, Lavleen; Singh, Geetika; Bhardwaj, Swati; Sinha, Aditi; Bagga, Arvind; Dinda, Amit

    2016-01-01

    Dense deposit disease is caused by fluid-phase dysregulation of the alternative complement pathway and frequently deviates from the classic membranoproliferative pattern of injury on light microscopy. Other patterns of injury described for dense deposit disease include mesangioproliferative, acute proliferative/exudative, and crescentic GN. Regardless of the histologic pattern, C3 glomerulopathy, which includes dense deposit disease and C3 GN, is defined by immunofluorescence intensity of C3c two or more orders of magnitude greater than any other immune reactant (on a 0-3 scale). Ultrastructural appearances distinguish dense deposit disease and C3 GN. Focal and segmental necrotizing glomerular lesions with crescents, mimicking a small vessel vasculitis such as ANCA-associated GN, are a very rare manifestation of dense deposit disease. We describe our experience with this unusual histologic presentation and distinct clinical course of dense deposit disease, discuss the pitfalls in diagnosis, examine differential diagnoses, and review the relevant literature.

  12. Dense Deposit Disease Mimicking a Renal Small Vessel Vasculitis

    PubMed Central

    Singh, Lavleen; Bhardwaj, Swati; Sinha, Aditi; Bagga, Arvind; Dinda, Amit

    2016-01-01

    Dense deposit disease is caused by fluid-phase dysregulation of the alternative complement pathway and frequently deviates from the classic membranoproliferative pattern of injury on light microscopy. Other patterns of injury described for dense deposit disease include mesangioproliferative, acute proliferative/exudative, and crescentic GN. Regardless of the histologic pattern, C3 glomerulopathy, which includes dense deposit disease and C3 GN, is defined by immunofluorescence intensity of C3c two or more orders of magnitude greater than any other immune reactant (on a 0–3 scale). Ultrastructural appearances distinguish dense deposit disease and C3 GN. Focal and segmental necrotizing glomerular lesions with crescents, mimicking a small vessel vasculitis such as ANCA-associated GN, are a very rare manifestation of dense deposit disease. We describe our experience with this unusual histologic presentation and distinct clinical course of dense deposit disease, discuss the pitfalls in diagnosis, examine differential diagnoses, and review the relevant literature. PMID:26361799

  13. Fatal pulmonary complication during induction therapy in a patient with ANCA-associated vasculitis

    PubMed Central

    Brigita, Jonaitytė; Rūta, Kibarskytė; Edvardas, Danila; Marius, Miglinas; Dmitrij, Šeinin; Rokas, Stulpinas; Jurgita, Mitrikevičienė; Vygantas, Gruslys; Virginija, Šileikienė; Rolandas, Zablockis

    2016-01-01

    ANCA-associated vasculitis (AAV) is an inflammatory systemic disorder affecting small to medium sized vessels and likely leading to any organ dysfunction. Adequate treatment is important to avoid mortality or severe organ damage. In most cases initial treatment (induction therapy) allows to achieve remission. Induction therapy leads to immunosuppression and may cause severe infections. However, in vasculitis patients even an intensive immunosuppressive therapy is rarely complicated by an invasive fungal infection. We present a case in a 29-year old male patient with newly diagnosed AAV. He suffered a fatal pulmonary complication of the induction immunosuppressive treatment. Pathological (infectious) changes in the lungs were misinterpreted as progression of the vasculitis and he died due to disseminated angioinvasive aspergillosis. A clinical course, imaging and histopathology of this case are described and discussed. PMID:28356801

  14. Therapy for acute retinal necrosis.

    PubMed

    Kawaguchi, Tatsushi; Spencer, Doran B; Mochizuki, Manabu

    2008-01-01

    Acute retinal necrosis is a progressive necrotizing retinopathy caused by herpes simplex virus (HSV) or varicella zoster virus (VZV). The mainstay of its treatment is antiviral therapy against these pathogenic organisms, such as intravenous acyclovir or oral valacyclovir. Systemic and topical corticosteroids together with antiviral therapy are used as an anti-inflammatory treatment to minimize damages to the optic nerve and retinal blood vessels. Because the majority of severe cases of the disease show occlusive retinal vasculitis, a low dosage of aspirin is used as anti-thrombotic treatment. Vitreo-retinal surgery is useful to repair rhegmatogenous retinal detachment, one of the main late-stage complications. Moreover, recent articles have reported some encouraging results of prophylactic vitrectomy before rhegmatogenous retinal detachment occurs. The efficacy of laser photocoagulation to prevent the development or extension of rhegmatogenous retinal detachment is controversial. Despite these treatments, the visual prognosis of acute retinal necrosis is still poor, in particular VZV-induced acute retinal necrosis.

  15. Etiologies and prognostic factors of leukocytoclastic vasculitis with skin involvement

    PubMed Central

    Bouiller, Kévin; Audia, Sylvain; Devilliers, Hervé; Collet, Evelyne; Aubriot, Marie Hélène; Leguy-Seguin, Vanessa; Berthier, Sabine; Bonniaud, Philippe; Chavanet, Pascal; Besancenot, Jean-François; Vabres, Pierre; Martin, Laurent; Samson, Maxime; Bonnotte, Bernard

    2016-01-01

    Abstract In this study, outcomes of patients with leukocytoclastic vasculitis (LCV) were analyzed focusing on clinical, histopathology and laboratory findings, relapses, and survival. Data from patients with cutaneous vasculitis diagnosed between January 1, 2000, and December 31, 2010, at Dijon University Hospital (France) were retrospectively reviewed. LCV was defined as perivascular neutrophilic infiltrate, endothelial cell nuclear swelling, extravasation of red blood cells, and/or fibrin deposition in vessels. Patients were classified according to the 2012 Chapel Hill Consensus Conference. Relapses were defined as the recurrence of vasculitis symptoms after a period of remission >1 month. Time to relapse and/or death was calculated from the date of diagnosis. Univariate and multivariate (Cox model) analyses were performed. A total of 112 patients (57 males and 55 females), with a mean age of 60 ± 19 (18–98) years, were analyzed. Overall follow-up was 61 ± 38 months. At diagnosis, all patients had skin lesions, purpura being the most common (n = 83). Lesions were associated with systemic involvement in 55 (51%) patients. Only 41 (36.6%) patients received specific treatment: glucocorticoids in 29 of 41 (70.7%) and immunosuppressants in 9 of 41 (22%). Sixty-two patients (55%) had LCV due to underlying causes, 29 (25.9%) had single-organ cutaneous small vessel vasculitis (SoCSVV), and 21 (18.8%) had unclassifiable LCV. Twenty patients of the cohort (18%) experienced relapse, 14 ± 13 (1–40) months after the diagnosis of LCV. None of the 29 patients with SoCSVV relapsed. Independent risk factors for relapse were vascular thrombosis in the biopsy [hazard ratio (HR) = 4.9; P = 0.017], peripheral neuropathy (HR = 9.8; P = 0.001), hepatitis (HR = 3.1; P = 0.004), and positive antineutrophil cytoplasm antibodies (ANCA, HR = 5.9 P = 0.005). In contrast, SoCSVV was a protective factor for relapse (HR = 0.12; P = 0.043). The 1-, 3-, and 6-year overall

  16. Hypernephroma Presenting with Cutaneous Leukocytoclastic Vasculitis and Lupus Anticoagulant: Resolution after Nephrectomy

    PubMed Central

    Murray, Nigel P.; Ruíz, Amparo; Reyes, Eduardo

    2012-01-01

    Hypernephroma can present as a variety of paraneoplastic, nonmetastatic conditions, including vasculitis, and rarely a lupus-type anticoagulant. Nephrectomy leads to the resolution of the systemic complaints. Malignancy, in this case hypernephroma, can present as an immune-mediated paraneoplastic syndrome which resolves after removal of the underlying tumor. PMID:22919534

  17. Animal models of ANCA associated vasculitis

    PubMed Central

    Salama, Alan D.; Little, Mark A

    2012-01-01

    This review seeks to provide an update on the experimental models that have been developed recapitulating clinical ANCA associated vasculitis. The recent insights regarding the application of the models in the study of pathogenesis, and the therapeutic implications of this, are covered in the article by van Timmeren and Heeringa in this issue. Recent findings Rodent models of both MPO- and PR3 ANCA associated vasculitis have been developed, which have provided important insights into the pathogenesis of ANCA associated pulmonary and renal disease. The vast majority of in vivo work in this field has concerned MPO-ANCA associated disease, although the last year has seen some advances in modelling of anti-PR3 disease. As with all experimental animal models they are flawed in one way or another, by virtue of the means by which they are induced, but they have already provided novel directions for future intervention in these complex diseases. To date there are no good models that replicate the granulomatous lesions found in granulomatosis with polyangiitis (GPA, formerly Wegener’s), or the development of vasculitis lesions in organs other than the lungs or kidneys. However, use of a combination of the available models should allow greater understanding of the critical requirements for disease and how these may be potentially monitored and modified in patients. Summary ANCA associated vasculitis can be induced in various forms in susceptible rodents. Further refinements are required for the full spectrum of disease phenotype to be replicated in animals, but critical new targets have been proposed based on use of molecular blocking agents and transgenic animals to elucidate disease pathways. PMID:22089094

  18. Warfarin-induced leukocytoclastic vasculitis and proteinuria

    PubMed Central

    Jumean, Khalid; Arqoub, Ahmad Abu; Hawatmeh, Amer; Qaqa, Firas; Bataineh, Ayham; Shaaban, Hamid

    2016-01-01

    Warfarin is typically prescribed for patients with thromboembolic diseases and atrial fibrillation. In addition to the complications of bleeding, allergic skin reaction is one of its rare adverse effects. We herein report a case of a 79 year old male patient with leukocytoclastic vasculitis and proteinuria secondary to warfarin. The warfarin was discontinued and oral prednisone therapy was initiated. The cutaneous lesions and the proteinuria resolved thereafter. PMID:27453863

  19. Cutaneous Necrotizing Vasculitis and Leukopenia in a Cocaine User: Is Levamisole the Culprit?

    PubMed Central

    Zeineddine, Nabil; Felix, Richard; Goldstein, Mark

    2016-01-01

    Levamisole is an antihelminthic drug banned by the US Food and Drug Administration (FDA) in 2000 because of its dangerous side effects. Over the past few years, it has been identified as an adulterant in cocaine and reported to cause cutaneous vasculitis in cocaine users. The health burden of levamisole is serious since it is estimated that over 5 million Americans use cocaine and that 70% of the cocaine used in the USA contains levamisole. In this paper we report the case of a 23-year-old female cocaine user that presented with purpuric rash and skin necrosis, found to have positive c-ANCA and anti-proteinase 3 antibodies. Her skin biopsy showed fibroconnective tissue with signs of necrosis, acute and chronic inflammation, and thrombus formation. She was diagnosed with levamisole-induced vasculitis and successfully treated with withdrawal of cocaine use and local wound care. PMID:27579207

  20. The "other" vasculitis syndromes and kidney involvement.

    PubMed

    Ozen, Seza

    2010-09-01

    There are a number of vasculitides that are not confined to a specific vessel size, do not have characteristic features, and/or are not secondary to another disease. Most of these vasculitides are rare in childhood. Behçet disease is representative of this group as it involves vessels of any size on both the arterial and venous side. In addition to renal vascular involvement, Behçet disease may involve the kidney through glomerulonephritis, secondary amyloidosis and, rarely, tubulointerstital involvement. Vasculitis secondary to infections, malignancy, and drugs are not common among children. However, vasculitis may be associated with a number of rheumatic diseases in childhood and the auto-inflammatory syndromes (periodic fever syndromes). Auto-inflammatory syndromes are diseases characterized by periodic attacks of clinical and laboratory inflammation. Studies carried out during the past decade have provided valuable information on the mechanism of inflammation and innate immunity in general. This group of vasculitides is associated with secondary amyloidosis of the kidney if not treated. Hypocomplementemic urticarial vasculitis is an interesting vasculitic disease with frequent kidney involvement. Here, we introduce the reader to the wide scope of these diseases; although rare, such diseases represent a challenge to the nephrologist.

  1. Acute cyanide intoxication and central transmitter systems.

    PubMed

    Persson, S A; Cassel, G; Sellström, A

    1985-12-01

    In rats treated with sodium cyanide (5-20 mg/kg, ip) dopamine was dose dependently decreased in the striatum within 60 sec. One of the main metabolites of dopamine in the central nervous system, 3-methoxy-4-hydroxyphenylacetic acid (HVA), was decreased in striatum, olfactory tubercle, and hippocampus. However, the oxidatively deaminated metabolite, 3,4-dihydroxyphenylacetic acid (DOPAC), was not significantly altered in any of the brain regions studied. Naturally occurring levels of 3,4-dihydroxy-L-phenylalanine (L-dopa), as well as L-dopa accumulated after inhibition of the neuronal L-aromatic amino acid decarboxylase, increased in cyanide-treated rats. The dopamine receptor antagonist spiperone (0.05 mg/kg, ip) slightly increased the survival in acute cyanide intoxication. Sodium cyanide increased the levels of glutamine in frontal cortex and striatum at all doses studied. Glutamic acid was increased in the cerebellum, striatum, and hippocampus after sodium cyanide (5-10 mg/kg, ip). Higher doses decreased glutamic acid in the cerebellum, the frontal cortex, and the striatum. gamma-Aminobutyric acid (GABA) concentrations were diminished at high doses in all regions studied. Cyanide increased the levels of cyclic GMP in the cerebellum. In the striatum cyclic GMP was decreased after sodium cyanide (10 and 20 mg/kg). No significant alterations in the concentrations of acetylcholine or choline were seen in the striatum of cyanide-treated rats. The acetylcholinesterase inhibitor physostigmine and the muscarinic receptor antagonist atropine decreased the survival of mice given sodium cyanide. Acute cyanide intoxication thus produces rapid and fairly specific changes in central dopaminergic and GABA-ergic pathways.

  2. Endoscopic Findings of Upper Gastrointestinal Involvement in Primary Vasculitis

    PubMed Central

    Gong, Eun Jeong; Kim, Do Hoon; Chun, Joo Hyun; Ahn, Ji Yong; Choi, Kwi-Sook; Jung, Kee Wook; Lee, Jeong Hoon; Choi, Kee Don; Song, Ho June; Lee, Gin Hyug; Jung, Hwoon-Yong; Kim, Jin Ho; Song, In Hye; Kim, Yong-Gil

    2016-01-01

    Background/Aims Gastrointestinal involvement in vasculitis may result in life-threatening complications. However, its variable clinical presentations and endoscopic features, and the rarity of the disease, often result in delayed diagnosis. Methods Clinical characteristics, endoscopic features, and histopathological findings were reviewed from medical records. Results Of 6,477 patients with vasculitis, 148 were diagnosed as primary vasculitis with upper gastrointestinal involvement. Of these, 21 cases (14.2%) were classified as large-vessel vasculitis, 17 cases (11.5%) as medium-vessel vasculitis, and 110 cases (74.3%) as small-vessel vasculitis. According to the specific diagnosis, IgA vasculitis (Henoch-Schönlein purpura) was the most common diagnosis (56.8%), followed by Takayasu arteritis (14.1%), microscopic polyangiitis (10.1%), and polyarteritis nodosa (6.8%). Gastrointestinal symptoms were present in 113 subjects (76.4%), with abdominal pain (78.8%) the most common symptom. Erosion and ulcers were striking endoscopic features, and the second portion of the duodenum was the most frequently involved site. Biopsy specimens were obtained from 124 patients, and only eight (5.4%) presented histopathological signs of vasculitis. Conclusions Diagnosis of vasculitis involving the upper gastrointestinal tract is difficult. Because of the widespread use of endoscopy, combining clinical features with endoscopic findings may facilitate making appropriate diagnoses; however, the diagnostic yield of endoscopic biopsy is low. PMID:27226428

  3. A Case Report Describing a Rare Presentation of Simultaneous Occurrence of MPO-ANCA-Associated Vasculitis and Rheumatoid Arthritis

    PubMed Central

    Papireddy, Muralidhar; Gao, John

    2016-01-01

    Background. Renal-limited myeloperoxidase vasculitis with simultaneous rheumatoid arthritis is reported as a rare occurrence. Review of literature suggests that most patients had a diagnosis of rheumatoid arthritis for several years prior to presenting with renal failure from myeloperoxidase vasculitis. Case Presentation. A 58-year-old Caucasian male presented to the hospital experiencing malaise, fevers, decreased oral intake, nausea, and vomiting for one week duration. His past medical history consisted of newly diagnosed but untreated rheumatoid arthritis, hypertension, and non-insulin-dependent diabetes mellitus. He was found to have acute renal failure, proteinuria, and hypoglycemia. Standard therapy, including intravenous fluids, did not improve his acute renal failure. A vasculitis workup resulted in a positive myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA). Renal biopsy revealed crescentic glomerulonephritis (GN) pauci-immune type, suggestive of MPO-ANCA-associated vasculitis (MPO-AAV). Treatment consisted of prednisone, cyclophosphamide, and seven cycles of plasmapheresis, in addition to hemodialysis for uremia. Upon discharge, he received hemodialysis for another week and continued treatment with cyclophosphamide and prednisone. Conclusion. Patients with longstanding rheumatoid arthritis may develop renal failure due to nonsteroidal anti-inflammatory medication use and AA type amyloidosis; however, necrotizing glomerulonephritis with crescent formation has been rarely reported. This stresses the importance of early recognition and swift initiation of treatment. PMID:27891268

  4. Eosinophilic Granulomatosis with Polyangiitis Presenting with Skin Rashes, Eosinophilic Cholecystitis, and Retinal Vasculitis

    PubMed Central

    Zeng, Mingbing; Liu, Xialin; Liu, Yizhi

    2016-01-01

    Patient: Female, 20 Final Diagnosis: Eosinophilic granulomatosis with polyangiitis Symptoms: Fever • skin rashes • eosinophilic cholecystitis • retinal vasculitis Medication: — Clinical Procedure: — Specialty: Ophthalmology Objective: Rare co-existance of disease or pathology Background: Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome (CSS), is a rare vasculitis of unknown etiology. Most of the patients have a long history of asthma and then develop autoimmune inflammation of small and medium-sized blood vessels, with consequent reduction of blood flow to various organs and tissues. EGPA can cause disorders in multiple systems; the most seriously affected organs are the retina, kidney, brain, cardiovascular system, and skin. Case Report: The patient was hospitalized for high fever and skin rashes and then developed right upper abdominal pain, decreased visual acuity, coma, and convulsions. Laboratory investigations showed marked eosinophilia (9412/mm3). Following cholecystectomy, histopathological examination revealed a marked inflammatory cell infiltrate composed mainly of eosinophils. Retinal vasculitis and medium and peripheral vascular closure were confirmed by fundus fluorescence angiography (FFA). The coma and convulsions were controlled successfully by high-dose methylprednisolone. After gradual tapering of the methylprednisolone, the patient’s blood count recovered to a normal level, and the other systematic disorders disappeared; however, she was left with irreversible blindness. Conclusions: EGPA can cause eosinophilic cholecystitis, retinal vasculitis, and neuropathy in the short term and calls for effective treatments in order to avoid binocular blindness. PMID:27857032

  5. Higher Serum Angiotensinogen Is an Indicator of IgA Vasculitis with Nephritis Revealed by Comparative Proteomes Analysis

    PubMed Central

    Luan, Jiangwei; Zhao, Peiwei; Yue, Xin; Yu, Chunhua; Laing, Xiaohui; Zhao, YuLan

    2015-01-01

    IgA vasculitis (IgAV), previously named as Henoch–Schönlein purpura, is the most common systematic vasculitis with unknown etiology. Lack of appropriate study system and/or animal model limits the understanding of its molecular pathogenesis and hinders the identification of targets for rational therapy, especially for its long-term complication, IgAV nephritis (IgAVN). In this study, we applied comparative analysis of serum proteomes to obtain an insight about disease pathogenesis. This study has utilized high sensitivity nanoscale ultra performance liquid chromatography-mass spectrometry (nanoLC-MS/MS) to investigate the alterations in serum proteomic profiles in patients with IgAV (n=6), IgAVN (n=6) and healthy subjects (n=7). The differentially expressed proteins were subjected to functional pathway analysis by PANTHER and DAVID software. We identified 107 differentially expressed proteins among three different groups, and functional analysis suggested that, in addition to earlier reported pathways, such as acute phase response, immune response, complement and blood coagulation pathways, hemostasis and Wnt signaling pathway were probably involved in pathogenesis of IgAV. A few differentially abundant proteins identified, such as C4a, serum amyloid A, angiotensinogen, and kininogen 1, were further validated by ELISA. More importantly, we found that angiotensinogen concentration is correlated with IgAVN and could be used as a potential marker for the progression of IgAV. This is the first report of analyzing the proteomic alterations in IgAV patients and the differentially proteins identified in this study may enhance understanding of the pathology of IgAV and a few of them may be used to monitor disease progression. PMID:26098644

  6. Acute amygdaloid response to systemic inflammation.

    PubMed

    Engler, Harald; Doenlen, Raphael; Engler, Andrea; Riether, Carsten; Prager, Geraldine; Niemi, Maj-Britt; Pacheco-López, Gustavo; Krügel, Ute; Schedlowski, Manfred

    2011-10-01

    The amygdala, a group of nuclei located in the medial temporal lobe, is a key limbic structure involved in mood regulation, associative learning, and modulation of cognitive functions. Functional neuroanatomical studies suggest that this brain region plays also an important role in the central integration of afferent signals from the peripheral immune system. In the present study, intracerebral electroencephalography and microdialysis were employed to investigate the electrophysiological and neurochemical consequences of systemic immune activation in the amygdala of freely moving rats. Intraperitoneal administration of bacterial lipopolysaccharide (100 μg/kg) induced with a latency of about 2 h a significant increase in amygdaloid neuronal activity and a substantial rise in extracellular noradrenaline levels. Activated neurons in the amygdaloid complex, identified by c-Fos immunohistochemistry, were mainly located in the central nucleus and, to a lesser extent, in the basolateral nucleus of the amygdala. Gene expression analysis in micropunches of the amygdala revealed that endotoxin administration induced a strong time-dependent increase in IL-1β, IL-6, and TNF-α mRNA levels indicating that these cytokines are de novo synthesized in the amygdala in response to peripheral immune activation. The changes in amygdaloid activity were timely related to an increase in anxiety-like behavior and decreased locomotor activity and exploration in the open-field. Taken together, these data give novel insights into different features of the acute amygdaloid response during experimental inflammation and provides further evidence that the amygdala integrates immune-derived information to coordinate behavioral and autonomic responses.

  7. The impact of vasculitis on patients’ social participation and friendships

    PubMed Central

    Carpenter, Delesha M.; Meador, Amy E.; Elstad, Emily A.; Hogan, Susan L.; DeVellis, Robert F.

    2013-01-01

    Objectives Our objective is to explore how vasculitis, affects patients’ friendships and social participation. Methods Vasculitis patients (n=221) completed an online questionnaire that asked if, and how, relationships with friends have changed since receiving a vasculitis diagnosis. Participants’ written responses were imported into Atlas.ti, and two independent researchers used both structured and unstructured coding to identify themes. After reaching 100% consensus on the themes present in each participant’s responses, the coders determined how themes were interrelated across participants. Results Over half of patients (52%) expressed that vasculitis negatively impacted their friendships and 25% noted a negative impact on their social participation. At limes, this negative impact was related to structural changes in patients’ social networks due to loss of friendships. Reduced social participation was also associated with friends’ inability to understand vasculitis and its effects, vasculitis-related fatigue, and lifestyle changes such as not being able to drink alcohol and avoiding infection-prone events. Additionally, patients withdrew from social engagements due to fatigue or because of physical symptoms and side effects. Conclusion The unique circumstances associated with a rare chronic illness like vasculitis can create significant barriers to friendships, including loss of these relationships. Interventions designed to help patients cope with the social impact of vasculitis are implicated, especially if they increase patients’ ability to engage in dialogue about their illness with their friends. PMID:22325346

  8. Persistent cryoglobulinemic vasculitis following successful treatment of hepatitis C virus.

    PubMed

    Levine, James W; Gota, Carmen; Fessler, Barri J; Calabrese, Leonard H; Cooper, Sheldon M

    2005-06-01

    There is a well established link between type II mixed cryoglobulinemia (MC) and hepatitis C virus (HCV) infection, and HCV is believed to be the cause of cryoprotein formation and tissue deposition. Successful treatment of HCV infection has resulted in resolution of cryoglobulinemia and vasculitis. We describe 4 patients who had persistent MC and vasculitis despite successful eradication of HCV with antiviral therapy.

  9. Anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis and vasculitis.

    PubMed Central

    Jennette, J. C.; Wilkman, A. S.; Falk, R. J.

    1989-01-01

    Anti-neutrophil cytoplasmic autoantibodies (ANCA) react with constituents of neutrophil primary granules and monocyte lysosomes. Indirect immunofluorescence microscopy using alcohol-fixed neutrophils demonstrates two ANCA types: one causing cytoplasmic staining (C-ANCA), and a second causing artifactual perinuclear staining (P-ANCA) that frequently has specificity for myeloperoxidase. Using indirect immunofluorescence microscopy (IIFM) and enzyme immunoassays (EIA), sera from over 300 patients with renal disease, with and without systemic vasculitis, were analyzed. Of 76 patients with pauci-immune glomerulonephritis with crescents or necrosis, 87% had ANCA by IIFM (38% of C-ANCA type, 49% of P-ANCA type), and 78% had ANCA by EIA. Of 55 patients with nonlupus immune complex-mediated glomerulonephritis, only 11% had ANCA by IIFM and 5% had ANCA by EIA. Of 24 patients with anti-GBM antibody-mediated glomerulonephritis, none had ANCA. Renal and extrarenal lesions were studied in 81 patients with ANCA-associated glomerulonephritis. These patients formed a pathologic continuum ranging from renal-limited to widespread systemic vascular injury, including patients with primary crescentic glomerulonephritis, Wegener's granulomatosis, and polyarteritis nodosa. In ANCA-positive patients the frequency of C-ANCA and P-ANCA correlated with disease distribution. P-ANCA was most frequent with renal-limited disease and C-ANCA was most frequent when there was lung and sinus involvement. It is proposed that ANCA are not only useful diagnostic markers, but may also be directly involved in a novel pathogenetic mechanism that is a frequent cause of crescentic glomerulonephritis and systemic necrotizing vasculitis. Images Figure 1 Figure 4 Figure 5 Figure 6 Figure 7 PMID:2683800

  10. Scleroderma associated with ANCA-associated vasculitis.

    PubMed

    Rho, Young Hee; Choi, Seong Jae; Lee, Young Ho; Ji, Jong Dae; Song, Gwan Gyu

    2006-03-01

    We have recently reported on two cases of scleroderma patients with ANCA-associated vasculitis for the first time in Korea. In order to explore the nature of this disease combination, we pooled together all the previously known cases and statistically analyzed them. Out of the 50 selected cases, survival analysis was done for comparison of the scleroderma disease period and the clinical factors associated with ANCA-associated vasculitis (AAV). Kaplan-Meier analysis revealed that patients having anti-topoisomerase antibody (anti-Scl-70) and, probably, PR-3 ANCA are at a higher risk for developing AAV than patients without both anti-topoisomerase antibody and anti-centromere antibody (ACA), and patients with MPO-ANCA. Multivariate Cox regression analysis revealed having anti-topoisomerase antibody as a risk factor for developing AAV [OR 3.1 (95% CI 1.11-8.55), P=0.031]. We suggest that having anti-topoisomerase antibodies may play a role among scleroderma patients in developing AAV.

  11. Complement blockade in ANCA-associated vasculitis: an index case, current concepts and future perspectives.

    PubMed

    Manenti, Lucio; Urban, Maria Letizia; Maritati, Federica; Galetti, Maricla; Vaglio, Augusto

    2017-02-13

    Complement alternative pathway (cAP) hyperactivation seems to be involved in ANCA-associated vasculitis (AAV). We here describe a case of AAV with severe activation of cAP that developed acute renal failure. No mutation predisposing to cAP dysregulation was identified. We treated our patient with the standard immunosuppressive therapy, but disease progression was only reversed after the addition of eculizumab, a monoclonal antibody against C5; the patient eventually achieved an almost complete renal function recovery. A review of the available literature about the role of complement targeted therapies in the treatment of AAV is discussed.

  12. Histopathology of the Inner Ear in a Case With Recent Onset of Cogan's Syndrome: Evidence for Vasculitis.

    PubMed

    Jung, David H; Nadol, Joseph B; Folkerth, Rebecca D; Merola, Joseph F

    2016-01-01

    The association of sensorineural hearing loss and vertigo with inflammatory eye disease, usually interstitial keratitis, has been called Cogan's syndrome. The pathogenesis of Cogan's syndrome is unknown, but it has been assumed to be an immune mediated disorder with vasculitis. The histopathology of the inner ear in Cogan's syndrome has been described in 6 case reports. Although common pathologic findings in these reports include degeneration of the auditory and vestibular neuroepithelium, endolymphatic hydrops, fibrosis, and new bone formation, direct pathologic evidence of a vasculitis has not been published. A possible reason for this failure to identify vasculitis was a substantial delay (range, 4-40 years) between the onset of symptoms and examination of the otopathology. In the current case report, the patient had both auditory and vestibular symptoms and interstitial keratitis with a time delay of only 2 to 4 weeks between symptoms and death. Evidence of a vasculitis as a possible underlying etiology included H&E histopathology and anti-CD45 immunostaining of vessels both in the auditory and vestibular systems, supporting the hypothesis of a vasculitis as a mechanism in this disorder.

  13. The Spectrum of Paraneoplastic Cutaneous Vasculitis in a Defined Population

    PubMed Central

    Loricera, Javier; Calvo-Río, Vanesa; Ortiz-Sanjuán, Francisco; González-López, Marcos A.; Fernández-Llaca, Hector; Rueda-Gotor, Javier; Gonzalez-Vela, Maria C.; Alvarez, Lino; Mata, Cristina; González-Lamuño, Domingo; Martínez-Taboada, Victor M.; González-Gay, Miguel A.; Blanco, Ricardo

    2013-01-01

    Abstract Cutaneous vasculitis may be associated with malignancies, and may behave as a paraneoplastic syndrome. This association has been reported in a variable proportion of patients depending on population selection. We conducted the current study to assess the frequency, clinical features, treatment, and outcome of paraneoplastic vasculitis in a large unselected series of 766 patients with cutaneous vasculitis diagnosed at a single university hospital. Sixteen patients (10 men and 6 women; mean age ± standard deviation, 67.94 ± 14.20 yr; range, 40–85 yr) presenting with cutaneous vasculitis were ultimately diagnosed as having an underlying malignancy. They constituted 3.80% of the 421 adult patients. There were 9 hematologic and 7 solid underlying malignancies. Skin lesions were the initial clinical presentation in all of them, and the median interval from the onset of cutaneous vasculitis to the diagnosis of the malignancy was 17 days (range, 8–50 d). The most frequent skin lesions were palpable purpura (15 patients). Other clinical manifestations included constitutional syndrome (10 patients) and arthralgia and/or arthritis (4 cases). Hematologic cytopenias (11 cases) as well as immature peripheral blood cells (6 cases) were frequently observed in the full blood cell count, especially in those with vasculitis associated with hematologic malignancies. Specific treatment for vasculitis was prescribed in 10 patients; nonsteroidal antiinflammatory drugs (4 patients), corticosteroids (3 patients), chloroquine (1 patient), antihistamines (1 patient), and cyclophosphamide (1 patient). Ten patients died due to the malignancy and 6 patients recovered following malignancy therapy. Patients with paraneoplastic vasculitis were older, more frequently had constitutional syndrome, and less frequently had organ damage due to the vasculitis than the remaining patients with cutaneous vasculitis. In summary, cutaneous paraneoplastic vasculitis is an entity not uncommonly

  14. ANCA-associated vasculitis: report from Korea.

    PubMed

    Kim, Hye Won; Song, Yeong Wook

    2013-10-01

    We investigated the clinical features of Korean patients with anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) by reviewing the literature. The characteristics of AAV in Korean patients were as follows: (1) granulomatous and limited disease is prevalent in granulomatosis with polyangiitis (Wegener's) (GPA), (2) ANCA positivity is lower in GPA (56.6-68.9%) and eosinophilic granulomatosis with polyangiitis (EGPA) (5.9-8.3%), whereas it is higher in microscopic polyangiitis (MPA) (69-94%), (3) C-ANCA/proteinase 3 (PR3)-ANCA positivity is 71.5-100% in GPA and P-ANCA/myeloperoxidase (MPO)-ANCA positivity reached 94-100% in patients with MPA, (4) renal involvement or progression to end-stage renal disease was lower in Korean patients with GPA and EGPA than in Caucasians with GPA and EGPA (according to data provided in reports). The data provided here may need to be confirmed in large-scale studies.

  15. Churg-Strauss syndrome and hemorrhagic vasculitis

    PubMed Central

    Marques, Rui Moreira; Cabral, Ana Rita; Monteiro, Antonio; Henriques, Pedro

    2011-01-01

    Churg-Strauss syndrome (CSS) is a rare syndrome characterized by sinusitis, asthma and peripheral eosinophilia. This vasculitic syndrome affects medium and small-sized vessels, the lung being the most commonly affected organ, followed by the skin. The authors report a case of a 59-year-old male with a past history of asthma and allergic rhinitis. He presented necrohemorragic lesions in the distal phalanx of the 2nd, 3rd and 4th fingers of the left-hand and petechial lesions in the plant of both feet, accompanied by asthenia, anorexia and weight loss. The analytical study revealed leukocytosis with eosinophilia, elevated inflammatory parameters and p-ANCA positive antibodies. The diagnosis of CSS was established based on clinical and histopathological data. Cutaneous manifestations of hemorragic vasculitis are rare in CSS syndrome but can be the first manifestation of the disease. The recognition of this presentation is important for the early diagnosis and treatment of this syndrome. PMID:25386301

  16. Role of therapeutic plasmapheresis in ANCA-associated vasculitis.

    PubMed

    Walters, Giles

    2016-02-01

    Plasma exchange, or plasmapheresis, is a treatment method that developed over a period of two decades and involves the removal and replacement of a patient's circulating plasma. The aim of treatment is to remove disease-associated molecules and therefore interrupt disease progression. This article summarizes the developmental history of this treatment and then looks in more detail at data on the use of plasma exchange in treating antineutrophil antibody (ANCA)-associated vasculitis. The eight randomized trials and the Cochrane Systematic Review on treating renal vasculitis are summarized to show that plasma exchange may be effective in this disease, specifically in reducing the development of end-stage kidney disease (ESKD) by approximately 40%. The plasma exchange and glucocorticoid dosing in the treatment of anti-neutrophil cytoplasm antibody associated vasculitis (PEXIVAS) study is a currently enrolling study aiming to answer some of the outstanding questions relating to the use of this treatment in ANCA-associated vasculitis.

  17. Optimisation of vasculitis disease assessments in clinical trials, clinical care and long-term databases.

    PubMed

    Ponte, C; Sznajd, J; O'Neill, L; Luqmani, R A

    2014-01-01

    The systemic vasculitides are a group of rare, chronic, relapsing, but often progressive inflammatory conditions. They are associated with a significant burden of morbidity both due to scarring from the disease itself and as a consequence of treatment with glucocorticoids and other potent immunosuppressive agents. Careful assessment of disease activity is critical to guide appropriate use of these potentially toxic therapies. It is also important to differentiate features of active disease from those attributable to damage, which will not respond to immunosuppression. As these are chronic complex conditions, the impact on a patient's functional ability and quality of life are also important considerations. Given the lack of a reliable biomarker for assessment of disease activity or damage in systemic vasculitis, clinical tools developed and validated for use initially in clinically trials are key outcome measures in the evaluation of these patients. While the conduct of randomised clinical trials in vasculitis has been significantly enhanced by the development and use of validated outcome measures, regular use of validated disease activity and damage measurements as part of routine care offers a structured approach, which can serve as the basis of justifying treatment decisions. The authors review the concepts of clinical assessment tools used in the evaluation of patients with systemic vasculitis in the setting of clinical practice, clinical trials and long term databases with particular emphasis on disease activity, damage, prognosis and function.

  18. Indomethacin-related leukocytoclastic vasculitis: a case report and review of literature.

    PubMed

    Hussain, Nasir; Mustafa, Usman; Davis, James; Thakkar, Shivani; Ali, Alaa M; Mirrakhimov, Aibek E; Barbaryan, Aram; Rowley, Guy Anthony

    2013-01-01

    Leukocytoclastic vasculitis (LCV) is a small-vessel vasculitis with a reported incidence rate of 30 cases per million persons per year. It usually presents as a palpable purpuric skin rash on legs, though any part of the body can be affected. LCV rash may have an associated burning sensation or pain and in some cases may involve internal organs. In some cases, LCV rash may present as nodules, recurrent ulcerations or asymptomatic lesions. The diagnosis of LCV is usually made on skin biopsy. Etiological triggers may not be identified in as many as half of the cases. Treatment is usually conservative and includes identification and removal or treatment of the etiological trigger except in cases with internal organ involvement where systemic steroids and immunosuppressant may be necessary. In this article we present a case of indomethacin-associated LCV that improved with discontinuation of the offending agent.

  19. When to suspect pulmonary vasculitis: radiologic and clinical clues.

    PubMed

    Castañer, Eva; Alguersuari, Anna; Gallardo, Xavier; Andreu, Marta; Pallardó, Yolanda; Mata, Josep Maria; Ramírez, José

    2010-01-01

    Vasculitis is an inflammatory destructive process affecting blood vessels. Pulmonary vasculitis may be secondary to other conditions or constitute a primary, and in most cases idiopathic, disorder. Underlying conditions in the secondary vasculitides are infectious diseases, connective tissue diseases, malignancies, and hypersensitivity disorders. The most widely used approach to classifying the primary vasculitides is based on the size of the affected vessels (large, medium, small). Thoracic involvement is most commonly seen with primary idiopathic large-vessel vasculitides (Takayasu arteritis, giant cell arteritis, Behçet disease) and primary small-vessel antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (Wegener granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome). The radiologic manifestations of primary pulmonary vasculitis are extremely variable and include vessel wall thickening, nodular or cavitary lesions, ground-glass opacities, and consolidations. Diffuse alveolar hemorrhage is a clinical syndrome that usually results from primary small-vessel vasculitis in the lungs. Although chest radiography is often the first imaging study performed in patients with pulmonary involvement by vasculitis, chest radiographs often fail to show the exact pattern and extent of thoracic involvement and CT is more useful in assessment of the thoracic findings. The pulmonary primary vasculitides are rare disorders, and their diagnoses are among the most demanding challenges in medicine because their signs and symptoms are nonspecific and overlap with those of infections, connective tissue diseases, and malignancies; thus, diagnosis of vasculitis relies on recognition of characteristic combinations of particular clinical, radiologic, laboratory, and histopathologic features.

  20. B cell epitope specificity in ANCA-associated vasculitis: does it matter?

    PubMed

    van der Geld, Y M; Stegeman, C A; Kallenberg, C G M

    2004-09-01

    Pauci-immune idiopathic small-vessel vasculitis is strongly associated with the presence of antineutrophil cytoplasm autoantibodies (ANCA). Antibodies to PR3 predominate in patients with Wegener's granulomatosis; antibodies to myeloperoxidase (MPO) are found more frequently in patients with microscopic polyangiitis. There is increasing in vivo and in vitro evidence for a pathogenic role of ANCA in systemic vasculitis based on associations of ANCA with disease activity. If ANCA are pathogenic, why is the course of disease different from one patient to another? Antibodies can recognize different binding sites (epitopes) on their corresponding antigens. Differences in binding specificity may influence the pathogenic potential of the antibodies. Differences between epitope specificity of ANCA between patients or changes in epitope specificity of ANCA in time in an individual patient may, accordingly, result in differences in disease expression. This review will focus on epitope specificity of autoantibodies in systemic autoimmune diseases and especially on the epitope specificity of PR3- and MPO-ANCA. We will discuss whether PR3-ANCA or MPO-ANCA recognize different epitopes on PR3 and MPO, respectively, and whether the epitopes recognized by ANCA change in parallel with the disease activity of ANCA-associated vasculitis. Finally, we will speculate if the direct pathogenic role of ANCA can be ascribed to one relapse- or disease-inducing epitope. Characterization of relapse- or disease-inducing epitopes bound by PR3-ANCA and MPO-ANCA is significant for understanding initiation and reactivation of ANCA-associated vasculitis. Elucidating a disease-inducing epitope bound by ANCA may lead to the development of epitope-specific therapeutic strategies.

  1. Relapse of HCV Genotype 1b Infection After Sofosbuvir/Ledipasvir Treatment Presenting as De Novo Cryoglobulinemic Vasculitis

    PubMed Central

    Moreno, Alan D.; Joseph, Nora; Kim, George; Fimmel, Claus J.

    2017-01-01

    Relapse of hepatitis C virus (HCV) genotype 1 infection after combination therapy with sofosbuvir and ledipasvir is unusual. We report a treatment-naïve, non-cirrhotic patient in whom the relapse of genotype 1b HCV infection was accompanied by de novo cryoglobulinemic vasculitis and glomerulonephritis, requiring hemodialysis for acute renal failure. Sequence analysis revealed several resistance-associated variants in the HCV NS5a gene but not in NS3/4A. The patient’s vasculitis was successfully treated with immunosuppression and plasmapheresis, followed by retreatment of HCV with a combination of sofosbuvir, simeprevir, and ribavirin. The patient achieved sustained virological response, recovered his renal function, and remains in remission from cryoglobulinemia. PMID:28184378

  2. Clinical study on single-organ cutaneous small vessels vasculitis (SoCSVV)

    PubMed Central

    Pastuszczak, Maciej; Celińska-Löwenhoff, Magdalena; Sułowicz, Joanna; Wojas-Pelc, Anna; Musiał, Jacek

    2017-01-01

    Abstract Leukocytoclastic vasculitis (LCV) is a heterogenous group of disorders that may manifest as a mild disease isolated to the skin or be a part of life-threatening systemic vasculitis. According to the 2012 Chapel Hill Consensus Conference nomenclature, patients presenting symptoms of LCV confined only to the skin should be defined as suffering from a single-organ cutaneous small vessel vasculitis (SoCSVV). SoCSVV is a benign disease with a good clinical outcome but with a significant risk of relapse and skin ulcer formation. The aim of the current study was to characterize SoCSVV and to identify factors that may be associated with the risk of recurrence and skin ulcers. Medical records of patients with LCV hospitalized at the Department of Dermatology at University Hospital in Cracow in the years 2010 to 2015 were analyzed. A total of 24 patients fulfilled criteria of SoCSVV. Drugs and preceding infections were identified as precipitating factors in 40% and 20% of cases, respectively. Skin lesions other than palpable purpura (i.e., macules, urticarial vasculitis, or ulcers) were identified in almost half of the patients. Interestingly, the presence of macules independently increased the risk of skin ulcer formation (odds ratio = 16; 95% confidence interval: 1.5–176.6; P = 0.0075) in the multivariate logistic regression analysis. One-quarter of patients with SoCSVV experienced relapse during the 6-month follow-up. The greater number of affected skin areas was an independent risk factor of recurrence (odds ratio = 5; 95% confidence interval: 2–45; P = 0.02). SoCSVV was usually associated with drugs and preceding infections. The disease relapses in approximately one-quarter of the patients. The more severe the skin involvement in the course of SoCSVV, the higher is the risk of recurrence. PMID:28328827

  3. Long-term outcome of antineutrophil cytoplasmic antibody-associated small vessel vasculitis after renal transplantation.

    PubMed

    Marco, Helena; Mirapeix, Eduard; Arcos, Emma; Comas, Jordi; Ara, Jordi; Gil-Vernet, Salvador; Puig, Josep; Vinyas, Odette; Perello, Manel; Oppenheimer, Federico; Poveda, Rafael; Ibernón, Meritxell; Díaz, Montserrat; Ballarin, Jose

    2013-01-01

    The survival after renal transplantation of patients with antineutrophil cytoplasmic antibody (ANCA)-associated to systemic vasculitis is as good as in other diseases, although most of the reports are based on small numbers of patients. Furthermore, it is not known whether comorbidities (cardiovascular [CV] disease and cancer) are more frequent than in general population. We report our experience and the analysis of the published data on this topic. The outcome after transplantation in 49 patients with ANCA-associated small vessel vasculitis was compared with a control group. The relapse rate of vasculitis was 0.01 per patient per year. Comparison with the control patients revealed no difference in long-term outcome, CV mortality or incidence of malignancies. In the published literature, patients with ANCA at transplantation and with Wegener's granulomatosis are at greater risk of relapse. Taking our own results together with the review of the literature, we conclude that patient and graft survival rates compare favorably with those in control group that the recurrence rate is very low and that there is no increase in the incidence of cancer or in CV mortality. Patients with ANCA at transplantation and with Wegener's granulomatosis have a higher relapse rate.

  4. Percutaneous Management of Occlusive Arterial Disease Associated with Vasculitis: A Single Center Experience

    SciTech Connect

    Both, M.; Jahnke, T.; Reinhold-Keller, E.; Reuter, M.; Grimm, J.; Biederer, J.; Brossmann, J.; Gross, W.L.; Heller, M.; Mueller-Huelsbeck, S.

    2003-02-15

    The purpose of this study was to evaluate the safety and effectiveness of percutaneous transluminal angioplasty for occlusive arterial disease associated with vasculitis. Eleven patients(10 women, 1 man; ages 35-82 years) with the diagnosis of vasculitis of the large vessels underwent interventional treatment during intraarterial angiography. The causes included giant cell arteritis(n = 8) and Takayasu arteritis (n = 3).Thirty-three occlusive lesions (including brachiocephalic and renalarteries, and arteries of upper and lower extremities) were treated with balloon angioplasty and/or stent placement. Follow-up included clinical examination, angiography, and color duplex ultrasound.Technical success was 100% (25/25) for stenoses and 50% (4/8) for occlusive lesions, representing all lesions combined from different anatomic locations. Dissection (n = 3) and arterial rupture with retroperitoneal hematoma (n = 1) was found in three patients. During follow-up (mean 12 months), restenoses(n = 8) and re-restenoses (n = 1)occurred in 8 vascular areas. Three of these lesions were treated with repeated PTA (n = 4). The cumulative primary clinical success rate was 67.6%, cumulative secondary success rate 74.4%, and cumulative tertiary success rate 75.9%. Interventional therapy in systemic vasculitis provides promising results in technical success rates and followup. Angioplasty may result in arterial injury, but the rate of complications is low.

  5. Progressive outer retinal necrosis associated with occlusive vasculitis in acquired immunodeficiency syndrome.

    PubMed

    Tseng, Chien-Chi; Chen, San-Ni; Hwang, Jiunn-Feng; Lin, Chun-Ju; Chen, Huan-Sheng

    2015-05-01

    A 45-year-old man, a case of acquired immunodeficiency syndrome, received a highly active antiretroviral therapy at the outpatient service for 4 years without regular follow-up. He experienced progressively blurred vision for 6 months and a cutaneous zoster on his back 3 months ago. He was diagnosed with progressive outer retinal necrosis by polymerase chain reaction-restriction fragment length polymorphism using an aqueous humor sample, which revealed an existence of varicella zoster virus. He was given a combination of systemic, intravitreal antiviral and a highly active antiretroviral therapy. Occlusive vasculitis, an unusual finding for progressive outer retinal necrosis, developed in both eyes 1 week after the secondary intravitreal injection. Unfortunately, his vision deteriorated to no light perception in both eyes within 2 weeks. Progressive outer retinal necrosis is characterized clinically as showing minimal or no inflammation in the aqueous and vitreous humors, absence of retinal vasculitis, and patches of yellowish spots located deep in the retina. Physicians should pay attention to this rare case of progressive outer retinal necrosis associated occlusive vasculitis with very poor prognosis in spite of aggressive treatment.

  6. Extensive review of fish embryo acute toxicities for the prediction of GHS acute systemic toxicity categories.

    PubMed

    Scholz, Stefan; Ortmann, Julia; Klüver, Nils; Léonard, Marc

    2014-08-01

    Distribution and marketing of chemicals require appropriate labelling of health, physical and environmental hazards according to the United Nations global harmonisation system (GHS). Labelling for (human) acute toxicity categories is based on experimental findings usually obtained by oral, dermal or inhalative exposure of rodents. There is a strong societal demand for replacing animal experiments conducted for safety assessment of chemicals. Fish embryos are considered as alternative to animal testing and are proposed as predictive model both for environmental and human health effects. Therefore, we tested whether LC50s of the fish embryo acute toxicity test would allow effectively predicting of acute mammalian toxicity categories. A database of published fish embryo LC50 containing 641 compounds was established. For these compounds corresponding rat oral LD50 were identified resulting in 364 compounds for which both fish embryo LC50 and rat LD50 was available. Only a weak correlation of fish embryo LC50 and rat oral LD50 was obtained. Fish embryos were also not able to effectively predict GHS oral acute toxicity categories. We concluded that due to fundamental exposure protocol differences (single oral dose versus water-borne exposure) a reverse dosimetry approach is needed to explore the predictive capacity of fish embryos.

  7. Neutrophil Extracellular Traps in ANCA-Associated Vasculitis

    PubMed Central

    Söderberg, Daniel; Segelmark, Mårten

    2016-01-01

    A group of pauci-immune vasculitides, characterized by neutrophil-rich necrotizing inflammation of small vessels and the presence of antineutrophil cytoplasmic antibodies (ANCAs), is referred to as ANCA-associated vasculitis (AAV). ANCAs against proteinase 3 (PR3) (PR3-ANCA) or myeloperoxidase (MPO) (MPO-ANCA) are found in over 90% of patients with active disease, and these ANCAs are implicated in the pathogenesis of AAV. Dying neutrophils surrounding the walls of small vessels are a histological hallmark of AAV. Traditionally, it has been assumed that these neutrophils die by necrosis, but neutrophil extracellular traps (NETs) have recently been visualized at the sites of vasculitic lesions. AAV patients also possess elevated levels of NETs in the circulation. ANCAs are capable of inducing NETosis in neutrophils, and their potential to do so has been shown to be affinity dependent and to correlate with disease activity. Neutrophils from AAV patients are also more prone to release NETs spontaneously than neutrophils from healthy blood donors. NETs contain proinflammatory proteins and are thought to contribute to vessel inflammation directly by damaging endothelial cells and by activating the complement system and indirectly by acting as a link between the innate and adaptive immune system through the generation of PR3- and MPO-ANCA. Injection of NET-loaded myeloid dendritic cells into mice results in circulating PR3- and MPO-ANCA and the development of AAV-like disease. NETs have also been shown to be essential in a rodent model of drug-induced vasculitis. NETs induced by propylthiouracil could not be degraded by DNaseI, implying that disordered NETs might be important for the generation of ANCAs. NET degradation was also highlighted in another study showing that AAV patients have reduced DNaseI activity resulting in less NET degradation. With this in mind, it might be that prolonged exposure to proteins in the NETs due to the overproduction of NETs and/or reduced

  8. Rare Presentation of Lupus Myocarditis With Acute Heart Failure-A Case Report.

    PubMed

    Malhotra, Gurveen; Chua, Serafin; Kodumuri, Vamsi; Sivaraman, Sivashankar; Ramdass, Priya

    Systemic lupus erythematosus is an autoimmune disease with diffuse organ involvement. The cardiac complications include pericarditis, myocarditis, pulmonary hypertension, coronary vasculitis, and Libman-Sacks endocarditis. Symptomatic lupus myocarditis presenting with left ventricular dysfunction, acute heart failure, and pulmonary edema, although rare, is a life-threatening complication. We report the occurrence of acute lupus myocarditis in a 38-year-old postpartum female who had a cesarean section a week before presentation for preeclampsia. Initially she was managed for pneumonia but later found to have acute pericarditis and myocarditis related to systemic lupus erythematosus. She had a complicated hospital course including acute respiratory failure and cardiogenic shock. She was started on pulse dose steroids besides the treatment for heart failure and had a dramatic improvement within days.

  9. Normocomplementaemic Urticarial Vasculitis in a 19-Month-Old Girl

    PubMed Central

    2016-01-01

    Urticaria is common in children. Urticarial vasculitis (UV) is a potentially more serious, rare variant. The youngest reported case was 12 months of age. A systemically well, 19-month-old girl presented with her mother who was concerned about the development of a rash. On presentation, the child had normal vital signs, was alert, and was well and playing with toys. There was a widespread urticarial rash (raised, pruritic, and erythematous) that was most apparent on the trunk with minimal rash on the legs. Overlying this urticarial rash in a similar distribution was a blotchy, palpable purpuric rash and associated hyperpigmentation. Investigations revealed a normal level of haemoglobin, white cells, platelets, and electrolytes. Renal function, international normalised ratio, and activated partial thromboplastin time were all normal. There was no blood or protein in the urine. The erythrocyte sedimentation rate was mildly elevated at 19 mm/hour. Complement results (including C1q) obtained later were normal. This case is striking not only because of the rarity of UV in children but also due to the unique diagnostic and prognostic challenges that it raises. PMID:27818822

  10. Preliminary classification criteria for the cryoglobulinaemic vasculitis

    PubMed Central

    De Vita, S; Soldano, F; Isola, M; Monti, G; Gabrielli, A; Tzioufas, A; Ferri, C; Ferraccioli, G F; Quartuccio, L; Corazza, L; De Marchi, G; Casals, M Ramos; Voulgarelis, M; Lenzi, M; Saccardo, F; Fraticelli, P; Mascia, M T; Sansonno, D; Cacoub, P; Tomsic, M; Tavoni, A; Pietrogrande, M; Zignego, A L; Scarpato, S; Mazzaro, C; Pioltelli, P; Steinfeld, S; Lamprecht, P; Bombardieri, S; Galli, M

    2011-01-01

    Background To develop preliminary classification criteria for the cryoglobulinaemic syndrome or cryoglobulinaemic vasculitis (CV). Methods Study part I developed a questionnaire for CV to be included in the formal, second part (study part II). Positivity of serum cryoglobulins was defined by experts as an essential condition for CV classification. In study part II, a core set of classification items (questionnaire, clinical and laboratory items, as agreed) was tested in three groups of patients and controls—that is, group A (new patients with the CV), group B (controls with serum cryoglobulins but lacking CV) and group C (controls without serum cryoglobulins but with features which can be observed in CV). Results In study part I (188 cases, 284 controls), a positive response to at least two of three selected questions showed a sensitivity of 81.9% and a specificity of 83.5% for CV. This questionnaire was employed and validated in study part II, which included 272 patients in group A and 228 controls in group B. The final classification criteria for CV, by pooling data from group A and group B, required the positivity of questionnaire plus clinical, questionnaire plus laboratory, or clinical plus laboratory items, or all the three, providing a sensitivity of 88.5% and a specificity of 93.6% for CV. By comparing data in group A versus group C (425 controls), the same classification criteria showed a sensitivity 88.5% and a specificity 97.0% for CV. Conclusion Classification criteria for CV were developed, and now need validation. PMID:21571735

  11. Diagnosing ANCA-associated vasculitis in ANCA positive patients

    PubMed Central

    Houben, Eline; Bax, Willem A.; van Dam, Bastiaan; Slieker, Walentina A.T.; Verhave, Gideon; Frerichs, Fenneke C.P.; van Eijk, Izhar C.; Boersma, Wim G.; de Kuyper, Guido T.M.; Penne, Erik L.

    2016-01-01

    Abstract Currently no validated diagnostic system for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is available. Therefore, diagnosing AAV is often challenging. We aimed to identify factors that lead to a clinical diagnosis AAV in ANCA positive patients in a teaching hospital in The Netherlands. In this study, all patients that tested positive for ANCA proteinase 3 (PR3) and/or myeloperoxidase (MPO) between 2005 and 2015 were analysed. Patients with a clinical diagnosis of AAV were compared with patients without a clinical diagnosis of AAV. Clinical symptoms and laboratory variables at presentation, including the ANCA titre, were collected for both patients with and without AAV. Clinical and laboratory variables related with AAV were investigated, using multivariable logistic regression. Two hundred thirty seven consecutive patients with a positive ANCA were included, of whom 119 were clinically diagnosed with AAV. Of the 118 ANCA positive patients without AAV, 87 patients had an alternative diagnosis, including inflammatory bowel disease (n = 24), other rheumatic diseases (n = 23), infection (n = 11), malignancy (n = 4), and other diagnoses (n = 25). In a multivariable regression model, a high ANCA titre (odds ratio [OR] 14.16, 95% confidence interval [CI] 6.93–28.94) and a high number of affected organ systems (OR 7.67, 95% CI 3.69–15.94) were associated with AAV. MPO and PR3 ANCA can be positive in a variety of diseases that mimic AAV. A higher ANCA titre and multiple affected organ systems may help to discriminate between AAV and other systemic illnesses in anti-PR3 and anti-MPO positive patients. A diagnostic scoring system incorporating these factors should be considered. PMID:27749588

  12. Streptococcus Pneumoniae Intracranial Abscess and Post-Infectious Vasculitis

    PubMed Central

    Lucas, Alexandra; Maung, Ko Ko; Ratts, Ryan

    2016-01-01

    Intracranial abscesses are rare complications of Streptococcus pneumoniae infections, and to our knowledge, there have been no case reports of post-infectious vasculitis developing in such patients. Here we describe the case of a 48-year-old post-splenectomy male who developed post-infectious vasculitis following S. pneumoniae otitis media complicated by mastoiditis, osteomyelitis, meningitis, and intracranial abscess. Clinicians ought to be aware of the possible adverse outcomes of invasive S. pneumoniae and the limitations of current treatment options. PMID:28191299

  13. Acute Limb Ischemia and Coronary Artery Disease in a Case of Kimura’s Disease

    PubMed Central

    Heo, Woon; Jun, Hee Jae; Kang, Do Kyun; Min, Ho-Ki; Hwang, Youn-Ho; Kim, Ji Yong; Nam, Kyung Han

    2017-01-01

    Kimura disease (KD) is an immune-mediated chronic inflammatory disease of unknown etiology. KD has many complications associated with hypereosinophilia, including various forms of allergic reactions and eosinophilic lung disease. Additionally, hypereosinophilia is associated with hypercoagulability, which may lead to thromboembolic events. A 36-year-old man with KD presented with acute limb ischemia and coronary artery occlusion. He underwent thrombectomy, partial endarterectomy of both popliteal arteries, and coronary artery stent insertion. KD is a systemic disease that affects many organs and presents with thromboembolism and vasculitis. In a patient with KD, physicians should evaluate the vascular system, including the coronary arteries. PMID:28382271

  14. Acute closed lock in a patient with lupus erythematosus: case review.

    PubMed

    Bade, D M; Lovasko, J H; Montana, J; Waide, F L

    1992-01-01

    Collagen failure has been shown to result in synovitis, joint adhesions, and internal joint derangement. This case report illustrates the similarities between patients with systemic lupus erythematosus and an internally deranged temporomandibular joint and patients with internal derangement with no lupus erythematosus. If abnormalities in intra-articular collagen tissue lead to adhesion formation and restrict normal mobility during translatory movements, joint mechanics would be compromised. Arthritic changes, vasculitis, and synovitis of systemic lupus erythematosus appear to be contributory factors in this pathophysiologic process. Diagnostic and therapeutic arthroscopic surgery was performed. Acute and chronic signs of synovitis were observed during surgery, and tissue samples were obtained for histologic interpretation.

  15. Acute urinary retention secondary to urethral involvement of granulomatosis with polyangiitis

    PubMed Central

    Anderson, Patrick T.; Gottheil, Stephanie; Gabril, Manal; Barra, Lillian; Power, Nicholas

    2017-01-01

    Granulomatosis with polyangiitis (GPA, formerly Wegener’s granulomatosis) is a systemic necrotizing vasculitis of small- and medium-sized blood vessels, primarily affecting the upper and lower respiratory tracts, as well as the kidneys. Urogenital manifestations of GPA are exceedingly rare and usually respond well to systemic immunosuppressive therapy. Here, we present a case of a 36-year-old female presenting with acute urinary obstruction secondary to urethral GPA involvement in the immediate postpartum period. Special consideration should be given to ruling out malignancy in all patients with a history of GPA and urethral lesions, especially when there is a history of cyclophosphamide treatment. PMID:28163812

  16. Infiltration of central nervous system in adult acute myeloid leukaemia.

    PubMed Central

    Pippard, M J; Callender, S T; Sheldon, P W

    1979-01-01

    Out of 64 consecutive unselected patients with acute myeloid leukaemia studied during 1973-6, five developed clinical evidence of spread to the central nervous system (CNS). Neuroradiological examination showed cerebral deposits in three, in whom rapid symptomatic relief was obtained with radiotherapy. In two of these patients who developed solid intracranial deposits haematological remission could be reinduced or maintained; they were still alive 86 and 134 weeks later. When patients presented with spread to the CNS complicating generalised uncontrolled leukaemia they had short survivals. CNS infiltration may respond dramatically to appropriate treatment provided that it is not associated with generalised uncontrolled leukaemia, which has a poor prognosis. In view of this, routine "prophylaxis" of the CNS in adult acute myeloid leukaemia does not seem justified at present. Images FIG 1 FIG 2 FIG 3 PMID:283873

  17. Decision support systems for robotic surgery and acute care

    NASA Astrophysics Data System (ADS)

    Kazanzides, Peter

    2012-06-01

    Doctors must frequently make decisions during medical treatment, whether in an acute care facility, such as an Intensive Care Unit (ICU), or in an operating room. These decisions rely on a various information sources, such as the patient's medical history, preoperative images, and general medical knowledge. Decision support systems can assist by facilitating access to this information when and where it is needed. This paper presents some research eorts that address the integration of information with clinical practice. The example systems include a clinical decision support system (CDSS) for pediatric traumatic brain injury, an augmented reality head- mounted display for neurosurgery, and an augmented reality telerobotic system for minimally-invasive surgery. While these are dierent systems and applications, they share the common theme of providing information to support clinical decisions and actions, whether the actions are performed with the surgeon's own hands or with robotic assistance.

  18. Systemic sarcoidosis complicated of acute renal failure: about 12 cases.

    PubMed

    Mahfoudhi, Madiha; Mamlouk, Habiba; Turki, Sami; Kheder, Adel

    2015-01-01

    The sarcoidosis is a systemic granulomatosis affecting most frequently the lungs and the mediastinum. An acute renal failure reveals exceptionally this disease. It's a retrospective study implicating 12 cases of sarcoidosis complicated of acute renal failure. The aim of this study is to determine epidemiological, clinical, biological and histological profile in these cases and then to indicate the interest to consider the diagnosis of sarcoidosis in cases of unexplained renal failure. Extra-renal complications, therapeutic modalities and the outcome were determined in all patients. Our series involved 12 women with an average age of 40 years. Biological investigations showed an abnormal normocalcemia in 7 cases, a hypercalcemia in 5 cases, a hypercalciuria in 10 cases and polyclonal hypergammaglobulinemia in 7 cases. An acute renal failure was found in all patients with a median creatinin of 520 umol/L. For all patients, the renal echography was normal however, the kidney biopsy showed tubulo-interstitial nephritis. The extra-renal signs highlighting pulmonary interstitial syndrome in 5 cases, a sicca syndrome in 4 cases, mediastinal lymph nodes in 2 cases, a lymphocytic alveolitis in 3 cases, an anterior granulomatous uveitis in 2 cases and a polyarthritis in 5 cases. Five patients benefited of hemodialysis. The treatment consisted of corticosteroid in all cases. The follow up was marked by complete resolution of clinical and biological signs. The diagnosis of renal sarcoidosis must be done quickly to prevent renal failure.

  19. Acute systemic rapamycin induces neurobehavioral alterations in rats.

    PubMed

    Hadamitzky, Martin; Herring, Arne; Keyvani, Kathy; Doenlen, Raphael; Krügel, Ute; Bösche, Katharina; Orlowski, Kathrin; Engler, Harald; Schedlowski, Manfred

    2014-10-15

    Rapamycin is a drug with antiproliferative and immunosuppressive properties, widely used for prevention of acute graft rejection and cancer therapy. It specifically inhibits the activity of the mammalian target of rapamycin (mTOR), a protein kinase known to play an important role in cell growth, proliferation and antibody production. Clinical observations show that patients undergoing therapy with immunosuppressive drugs frequently suffer from affective disorders such as anxiety or depression. However, whether these symptoms are attributed to the action of the distinct compounds remains rather elusive. The present study investigated in rats neurobehavioral consequences of acute rapamycin treatment. Systemic administration of a single low dose rapamycin (3mg/kg) led to enhanced neuronal activity in the amygdala analyzed by intracerebral electroencephalography and FOS protein expression 90min after drug injection. Moreover, behavioral investigations revealed a rapamycin-induced increase in anxiety-related behaviors in the elevated plus-maze and in the open-field. The behavioral alterations correlated to enhanced amygdaloid expression of KLK8 and FKBP51, proteins that have been implicated in the development of anxiety and depression. Together, these results demonstrate that acute blockade of mTOR signaling by acute rapamycin administration not only causes changes in neuronal activity, but also leads to elevated protein expression in protein kinase pathways others than mTOR, contributing to the development of anxiety-like behavior. Given the pivotal role of the amygdala in mood regulation, associative learning, and modulation of cognitive functions, our findings raise the question whether therapy with rapamycin may induce alterations in patients neuropsychological functioning.

  20. Systemic blood coagulation activation in acute coronary syndromes

    PubMed Central

    Undas, Anetta; Szułdrzyński, Konstanty; Brummel-Ziedins, Kathleen E.; Tracz, Wiesława; Zmudka, Krzysztof

    2009-01-01

    We evaluated systemic alterations to the blood coagulation system that occur during a coronary thrombotic event. Peripheral blood coagulation in patients with acute coronary thrombosis was compared with that in people with stable coronary artery disease (CAD). Blood coagulation and platelet activation at the microvascular injury site were assessed using immunochemistry in 28 non-anticoagulated patients with acute myocardial infarction (AMI) versus 28 stable CAD patients matched for age, sex, risk factors, and medications. AMI was associated with increased maximum rates of thrombin-antithrombin complex generation (by 93.8%; P < .001), thrombin B-chain formation (by 57.1%; P < .001), prothrombin consumption (by 27.9%; P = .012), fibrinogen consumption (by 27.0%; P = .02), factor (f) Va light chain generation (by 44.2%; P = .003), and accelerated fVa inactivation (by 76.1%; P < .001), and with enhanced release of platelet-derived soluble CD40 ligand (by 44.4%; P < .001). FVa heavy chain availability was similar in both groups because of enhanced formation and activated protein C (APC)–mediated destruction. The velocity of coagulant reactions in AMI patients showed positive correlations with interleukin-6. Heparin treatment led to dampening of coagulant reactions with profiles similar to those for stable CAD. AMI-induced systemic activation of blood coagulation markedly modifies the pattern of coagulant reactions at the site of injury in peripheral vessels compared with that in stable CAD patients. PMID:18931343

  1. IL-1 signaling is critically required in stromal cells in Kawasaki Disease Vasculitis Mouse Model. Role of both IL-1α and IL-1β

    PubMed Central

    Lee, Youngho; Wakita, Daiko; Dagvadorj, Jargalsaikhan; Shimada, Kenichi; Chen, Shuang; Huang, Ganghua; Lehman, Thomas J.A.; Fishbein, Michael C.; Hoffman, Hal M.; Crother, Timothy R.; Arditi, Moshe

    2015-01-01

    Objective Kawasaki disease (KD) is the most common cause of acute vasculitis and acquired cardiac disease among US children. We have previously shown that both TLR2/MyD88 and IL-1β signaling are required for the Lactobacillus casei cell wall extract (LCWE)-induced KD vasculitis mouse model. The objectives of this study were to investigate the cellular origins of IL-1 production, the role of CD11c+ Dendritic Cells (DCs) and macrophages and the relative contribution of hematopoietic and stromal cells for IL-1 responsive cells, as well the MyD88 signaling in LCWE-induced KD mouse model of vasculitis. Approach and Results Using mouse knockout models as well as antibody depletion, we found that both IL-1α and IL-1β were required for LCWE-induced KD. Both DCs and macrophages were necessary and we found that MyD88 signaling was required in both hematopoietic and stromal cells. However, IL-1 response and signaling was critically required in non-endothelial stromal cells, but not hematopoietic cells. Conclusions Our results suggest that IL-1α and IL-1β as well as CD11c+ DCs and macrophages are essential for the development of KD vasculitis and coronary arteritis in this mouse model. Bone marrow chimera experiments suggest that MyD88 signaling is important in both hematopoietic and stromal cells, while IL-1 signaling and response is required only in stromal cells, but not in endothelial cells. Determining the role IL-1α and IL-1β and of specific cell types in the KD vasculitis mouse model may have important implications for the design of more targeted therapies and understanding of the molecular mechanisms of KD immunopathologies. PMID:26515418

  2. A Case of Polyarteritis Nodosa Associated with Vertebral Artery Vasculitis Treated Successfully with Tocilizumab and Cyclophosphamide

    PubMed Central

    Watanabe, Kae; Rajderkar, Dhanashree A.; Modica, Renee F.

    2016-01-01

    Pediatric polyarteritis nodosa is rare systemic necrotizing arteritis involving small- and medium-sized muscular arteries characterized by aneurysmal dilatations involving the vessel wall. Aneurysms associated with polyarteritis nodosa are common in visceral arteries; however intracranial aneurysms have also been reported and can be associated with central nervous system symptoms, significant morbidity, and mortality. To our knowledge extracranial involvement of the vertebral arteries has not been reported but has the potential to be deleterious due to fact that they supply the central nervous system vasculature. We present a case of a 3-year-old Haitian boy with polyarteritis nodosa that presented with extracranial vessel involvement of his vertebral arteries. After thorough diagnostic imaging, including a bone scan, ultrasound, Magnetic Resonance Imaging/Angiography, and Computed Tomography Angiography, he was noted to have vertebral artery vasculitis, periostitis, subacute epididymoorchitis, arthritis, and myositis. He met diagnostic criteria for polyarteritis nodosa and was treated with cyclophosphamide, methylprednisolone, and tocilizumab, which resulted in improvement of his inflammatory markers, radiographic findings, and physical symptoms after treatment. To the authors' knowledge, this is the first report of vertebral artery vasculitis in polyarteritis nodosa as well as successful treatment of the condition using the combination cyclophosphamide and tocilizumab for this condition. PMID:27018080

  3. [Mononeuritis multiplex due to thrombotic ischemia of primary antiphospholipid antibody syndrome without vasculitis: an autopsy case report].

    PubMed

    Takahashi, Masatoshi; Katada, Fumiaki; Sato, Susumu; Shibayama, Hidehiro; Fukutake, Toshio; Murayama, Shigeo

    2015-01-01

    The patient was a 78-year-old man. Three years before admission, he developed transient peripheral neuropathy and purpura, and at admission, he presented with livedo reticularis of both his lower extremities and with mononeuritis multiplex. Vasculitis was not observed, and antiphospholipid antibodies were detected. The nerve and skin biopsies revealed no inflammation; axonal degeneration accompanied by thrombi was found in his arterioles and venules. Based on these findings, he was diagnosed with ischemic peripheral neuropathy due to primary antiphospholipid syndrome. Administration of anticoagulant therapy resulted in an improvement in symptoms; however, two months later, a relapse occurred, and the patient contracted an infection while undergoing immunosuppressive therapy. The infection became fulminant, and the patient succumbed to multiple organ failure. The autopsy revealed a systemic arterial and venous embolism; however, no vasculitis was observed. Antiphospholipid syndrome, which is responsive to antithrombotic treatment, should be considered as a differential diagnosis of mononeuritis multiplex.

  4. Pathogenesis and diagnosis of otitis media with ANCA-associated vasculitis.

    PubMed

    Yoshida, Naohiro; Iino, Yukiko

    2014-12-01

    Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is histologically characterized by systemic necrotizing vasculitis and is clinically classified into two phases, systemic or localized. Recently, otological symptoms such as otitis media and hearing loss, not previously often associated with AAV, have been reported in AAV cases. In these cases we propose a diagnosis of otitis media with AAV (OMAAV). The ANCA titer is important for the diagnosis of OMAAV, and in most cases rapid progressive hearing loss is observed as localized AAV. Peripheral facial nerve palsy or hypertrophic pachymeningitis are coupled with 25% of cases and 18% of cases respectively. Proteinase 3-ANCA (PR3-ANCA) positive otitis media causes granulomatous formation or middle ear effusion in the middle ear, on the other hand myeloperoxidase-ANCA (MPO-ANCA) positive otitis media predominantly presents as otitis media with effusion. The early diagnosed case and the sensorineural hearing loss not progressed deaf could be recovered by the immunosuppressive therapy. Delayed diagnosis of AAV occasionally leads to progression to the irreversible phase; therefore, diagnosis at the early-localized stage is important for treating AAV. In this review, we discuss the current understanding of this newly proposed concept of OMAAV.

  5. Treatment of renal manifestations of ANCA-associated vasculitis

    PubMed Central

    Galesic, Kresimir; Ljubanovic, Danica; Horvatic, Ivica

    2013-01-01

    Context Vasculitis is a clinicopathological entity characterized by inflammation and necrosis of blood vessels. Evidence Acquisitions Directory of Open Access Journals (DOAJ), Google Scholar, Pubmed (NLM), LISTA (EBSCO) and Web of Science have been searched. Results Two major autoantigens for ANCA are myeloperoxidase (MPO) and proteinase 3 (PR3), which are proteins in the primary granules of neutrophils and in the lysosomes of monocytes. They are expressed in mature neutrophils of patients with ANCA, while absent in healthy subjects. Conclusions The kidney is the most commonly affected vital organ in ANCA-associated vasculitis, and patient outcomes are largely determined by the severity of renal disease at diagnosis and by its response to treatment. PMID:24475421

  6. Carbimazole-associated hypersensitivity vasculitis in a cat.

    PubMed

    Bowlt, K; Cattin, I; Stewart, J

    2014-12-01

    Feline hyperthyroidism can be treated medically, surgically or with radioactive iodine. Carbimazole inhibits both triiodothyronine and thyroxine synthesis in the thyroid gland and reported side effects include mild eosinophilia, leucopenia and lymphocytosis, thrombocytopenia, elevated liver enzyme activities, gastrointestinal signs and skin abnormalities. This case report describes a cat with carbimazole-associated apparent hypersensitivity vasculitis causing digital and tail necrosis, with multiple renal infarcts. Withdrawal of carbimazole resulted in stable disease.

  7. [Review article: Antineutrophil cytoplasmic antibody in small vessel vasculitis].

    PubMed

    Nakano, Hiromasa; Ozaki, Shoichi

    2010-05-01

    The vasculitides are defined by the presence of leukocytes in vessel walls with reactive damage to mural structures. Vasculitis may occur as a primary process or may be secondary to another underlying disease. In general, the affected vessels vary in size, type, and location in association with the specific vasculitic disorder. Classically, vasculitic syndromes have been categorized by the predominant sizes of the blood vessels and types of vessel most commonly affected among patients with the disorder. Currently, antineutrophil cytoplasmic antibody (ANCA) testing plays a critical role in the pathogenesis, diagnosis, and classification of vasculitides. Two types of ANCA assay (indirect immunofluorescence assay and enzyme-linked immunosorbent assay (ELISA)) are in wide use. Two major immunofluorescence patterns are observed, the C-ANCA and P-ANCA patterns. In vasculitis, the two relevant target antigens for ANCA are proteinase 3 (PR3) and myeloperoxidase (MPO). Antibodies with target specificities for PR3 and MPO are called PR3-ANCA and MPO-ANCA, respectively. Vasculitides associated with serum positivity for ANCA that affect small to medium-sized vessels are commonly known as ANCA-associated vasculitis and include Wegener's granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome. In this article, the pathogenesis of ANCA will be reviewed as well as the pitfalls regarding its clinical application.

  8. Disease patterns in vasculitis-still a mystery.

    PubMed

    Hoffman, Gary S

    2008-01-01

    In the field of autoimmunity, much has been learned from studying circulating and tissue bound immune-reactive cells, cytokines and antibodies. However, what has brought those cells to the site of injury, for most forms of vasculitis remains a mystery. Might the etiology of at least certain forms of vasculitis be related to generation of neoantigens in the native vessel, making that vessel the target of a pathogenic immune response? How might one explain organ targeting and patterns of disease that are so critical to the diagnostic process? Embryologists have demonstrated great diversity in the vasculature of different organs. Unique quantitative and qualitative features become apparent in vascular territories as early as the third week of gestation. These differences are later amplified by the effects of further development, aging, infection, spontaneous mutations and other co-morbidities. Based on data from these observations a testable hypothesis would be that many forms of vasculitis may begin with emergence of new antigens within affected vessel walls and the resulting immune response may in fact be a normal reaction to perceived foreign protein(s).

  9. 77 FR 34326 - Medicare Program; Hospital Inpatient Prospective Payment Systems for Acute Care Hospitals and the...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-06-11

    ... 0938-AR12 Medicare Program; Hospital Inpatient Prospective Payment Systems for Acute Care Hospitals and the Long-Term Care Hospital Prospective Payment System and Fiscal Year 2013 Rates; Hospitals' Resident... Program; Hospital Inpatient Prospective Payment Systems for Acute Care Hospitals and the Long-Term...

  10. 75 FR 60640 - Medicare Program; Hospital Inpatient Prospective Payment Systems for Acute Care Hospitals and the...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-10-01

    ... Systems for Acute Care Hospitals and the Long-Term Care Hospital Prospective Payment System Changes and FY... Rehabilitation and Respiratory Care Services; Medicaid Program: Accreditation for Providers of Inpatient... ``Medicare Program; Hospital Inpatient Prospective Payment Systems for Acute Care Hospitals and the...

  11. Serum proteins reflecting inflammation, injury and repair as biomarkers of disease activity in ANCA-associated vasculitis

    PubMed Central

    Monach, Paul A; Warner, Roscoe L; Tomasson, Gunnar; Specks, Ulrich; Stone, John H; Ding, Linna; Fervenza, Fernando C; Fessler, Barri J; Hoffman, Gary S; Iklé, David; Kallenberg, Cees GM; Krischer, Jeffrey; Langford, Carol A; Mueller, Mark; Seo, Philip; St. Clair, E William; Spiera, Robert; Tchao, Nadia; Ytterberg, Steven R; Johnson, Kent J; Merkel, Peter A

    2016-01-01

    Objective To identify circulating proteins that distinguish between active anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and remission in a manner complementary to markers of systemic inflammation. Methods Twenty-eight serum proteins representing diverse aspects of the biology of AAV were measured before and 6 months after treatment in a large clinical trial of AAV. Subjects (n=186) enrolled in the Rituximab in ANCA-Associated Vasculitis (RAVE) trial were studied. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels were available for comparison. The primary outcome was the ability of markers to distinguish severe AAV (Birmingham Vasculitis Activity Score for Wegener’s granulomatosis (BVAS/WG)≥3 at screening) from remission (BVAS/WG=0 at month 6), using areas under receiver operating characteristic (ROC) curve (AUC). Results All subjects had severe active vasculitis (median BVAS/WG=8) at screening. In the 137 subjects in remission at month 6, 24 of the 28 markers showed significant declines. ROC analysis indicated that levels of CXCL13 (BCA-1), matrix metalloproteinase-3 (MMP-3) and tissue inhibitor of metalloproteinases-1 (TIMP-1) best discriminated active AAV from remission (AUC>0.8) and from healthy controls (AUC>0.9). Correlations among these markers and with ESR or CRP were low. Conclusions Many markers are elevated in severe active AAV and decline with treatment, but CXCL13, MMP-3 and TIMP-1 distinguish active AAV from remission better than the other markers studied, including ESR and CRP. These proteins are particularly promising candidates for future studies to address unmet needs in the assessment of patients with AAV. PMID:22975753

  12. Static and fatigue tensile properties of cross-ply laminates containing vascules for self-healing applications

    NASA Astrophysics Data System (ADS)

    Luterbacher, R.; Trask, R. S.; Bond, I. P.

    2016-01-01

    The effect of including hollow channels (vascules) within cross-ply laminates on static tensile properties and fatigue performance is investigated. No change in mechanical properties or damage formation is observed when a single vascule is included in the 0/90 interface, representing 0.5% of the cross sectional area within the specimen. During tensile loading, matrix cracks develop in the 90° layers leading to a reduction of stiffness and strength (defined as the loss of linearity) and a healing agent is injected through the vascules in order to heal them and mitigate the caused degradation. Two different healing agents, a commercial low viscosity epoxy resin (RT151, Resintech) and a toughened epoxy blend (bespoke, in-house formulation) have been used to successfully recover stiffness under static loading conditions. The RT151 system recovered 75% of the initial failure strength, whereas the toughened epoxy blend achieved a recovery of 67%. Under fatigue conditions, post healing, a rapid decay of stiffness was observed as the healed damage re-opened within the first 2500 cycles. This was caused by the high fatigue loading intensity, which was near the static failure strength of the healing resin. However, the potential for ameliorating (via self-healing or autonomous repair) more diffuse transverse matrix damage via a vascular network has been shown.

  13. Acute macular neuroretinopathy associated with systemic lupus erythematosus.

    PubMed

    Lee, D H; Lee, S C; Kim, M

    2016-04-01

    Acute macular neuroretinopathy (AMN) is a rare disorder that presents with abrupt visual change with wedge-shaped or flower-like lesions pointing towards the fovea. Ischemic insults to the retinal capillary plexus may be important for development of this disease. While many case reports have been published on AMN, none have described AMN in association with systemic lupus erythematosus (SLE). Here, we report a case of AMN associated with newly-diagnosed SLE. We speculate that in patients with lupus flares, immune complex-mediated vascular injury and microvascular thrombosis may disrupt the deep retinal capillary network, causing ischemic damages to the outer retina and leading to the development of AMN. AMN can develop in patients with lupus flares, and must be considered as an SLE-associated ophthalmologic complication. To the best of our knowledge, this is the first case report of AMN associated with SLE.

  14. System of acute medical support to emergency during dental treatment.

    PubMed

    Kawahara, M; Takeshita, T; Akita, S

    1986-01-01

    The Resuscitation Committee of Hiroshima City Dental Association was established in 1983 in order to provide acute medical support in case of emergency during dental treatment at private dental clinics. This Committee is composed of representatives from the Hiroshima City Dental Association, Hiroshima University School of Dentistry, Hiroshima University School of Medicine, Hiroshima City Health Bureau, and Hiroshima City Fire and Ambulance Department. A portable ECG monitor with defibrillator and a resuscitation kit are held in readiness at the Hiroshima University Hospital. In case of emergency during dental treatment at a private dental clinic, we hurry to the clinic with the resuscitation set and give emergency treatment. We have been involved in two cases of emergency since this system started. Both of them recovered without any sequelae. Besides these activities, we give lectures annually to dentists and dental hygienists on the treatment of medical emergencies.

  15. Diagnostic Utility of Contrast-enhanced 3D T1-weighted Imaging in Acute Cerebral Infarction Associated with Graves Disease.

    PubMed

    Gon, Yasufumi; Sakaguchi, Manabu; Oyama, Naoki; Mochizuki, Hideki

    2017-02-01

    Graves disease is rarely complicated with cerebrovascular steno-occlusive diseases. Previous studies have suggested several hypotheses for this occurrence, including excess thyroid hormone, which stimulates the sympathetic nervous system, which in turn causes an abnormal hemodynamic response with consequent atherosclerotic changes, and antithyroid antibodies cause local vascular inflammation in patients with Graves disease. However, radiological findings of vasculitis in patients with Graves disease and cerebral infarction remain less known. We report the case of a 30-year-old Japanese woman with acute cerebral infarction due to vasculitis associated with Graves disease. She was admitted to our hospital with a 4-day history of intermittent transient dysarthria and limb shaking of the left leg when standing. Three weeks before admission, she went to a local hospital because of general malaise and was diagnosed with Graves disease. Neurological examination revealed paralytic dysarthria, left central facial nerve palsy, and left hemiparesis (manual muscle testing, 4 of 5). Blood examinations showed hyperthyroidism (thyroid-stimulating hormone ≤.010 µU/mL; free T3 ≥25.0 pg/mL; free T4 ≥8.0 ng/dL) and elevation of antithyroid antibody levels (thyroid peroxidase antibody, 87 IU/mL). The vessel wall of the right internal carotid artery was markedly enhanced on contrast-enhanced three-dimensional T1-weighted magnetic resonance imaging, suggesting vasculitis. Magnetic resonance angiography revealed right internal carotid artery occlusion after the branching ophthalmic artery. Arterial stenosis due to vasculitis was considered the cause of hemodynamic ischemic stroke. Vessel wall imaging such as high-resolution contrast-enhanced T1-weighted imaging seems useful for assessing the underlying mechanism of stroke in patients with Graves disease.

  16. Acute Chorioamnionitis and Funisitis: Definition, Pathologic Features, and Clinical Significance

    PubMed Central

    Kim, Chong Jai; Romero, Roberto; Chaemsaithong, Piya; Chaiyasit, Noppadol; Yoon, Bo Hyun; Kim, Yeon Mee

    2015-01-01

    Acute inflammatory lesions of the placenta consist of diffuse infiltration of neutrophils at different sites in the organ. These lesions include acute chorioamnionitis, funisitis, and chorionic vasculitis, and represent a host response (maternal or fetal) to a chemotactic gradient in the amniotic cavity. While acute chorioamnionitis is evidence of a maternal host response, funisitis and chorionic vasculitis represent fetal inflammatory responses. Intra-amniotic infection has been generally considered to be the cause of acute histologic chorioamnionitis and funisitis; however, recent evidence indicates that “sterile” intra-amniotic inflammation, which occurs in the absence of demonstrable microorganisms but can be induced by “danger signals”, is frequently associated with these lesions. In the context of intra-amniotic infection, chemokines (such as interleukin-8 and granulocyte chemotactic protein) establish a gradient favoring the migration of neutrophils from maternal or fetal circulation into the chorioamniotic membranes or umbilical cord, respectively. Danger signals released during the course of cellular stress or cell death can also induce the release of neutrophil chemokines. The prevalence of chorioamnionitis is a function of gestational age at birth, and is present in 3-5% of placentas delivered at term, but in 94% of placentas delivered between 21-24 weeks of gestation. The frequency is higher in patients with spontaneous labor, preterm labor, clinical chorioamnionitis (preterm or term), or ruptured membranes. Funisitis and chorionic vasculitis are the hallmarks for the fetal inflammatory response syndrome, a condition characterized by an elevation in fetal plasma concentrations of interleukin-6, associated with the impending onset of preterm labor, a higher rate of neonatal morbidity (after adjustment for gestational age), and multi-organ fetal involvement. This syndrome is the counterpart of the systemic inflammatory response syndrome in adults

  17. Multidrug-related leukocytoclastic vasculitis raising suspicion of sexual homicide-things are not always what they seem.

    PubMed

    Tattoli, Lucia; Krocker, Klaus; Sautter, Julia; Tsokos, Michael

    2016-01-01

    Ambiguous findings during external examination of a deceased in combination with dubious autopsy findings can raise doubts concerning the manner and cause of death. We report the case of a 35-year-old female deceased who had suffered from a borderline personality and depressive disorder with suicidal ideation. At the death scene, the body showed massive facial swelling accompanied by complete reddening of the skin of the face, with patchy skin abrasions on the forehead and neck, and purple bruise-like discolorations distributed symmetrically over both shoulders, elbows, hands, hips, knees, lower legs, and feet, raising the suspicion of underlying massive external blunt force injury. Police investigators strongly suspected sexual homicide. At autopsy, dissection in layers revealed massive subcutaneous hemorrhages as the cause of the reddish skin discolorations. Toxicological analyses showed fatal levels of lamotrigine with additional proof of zopiclone, zolpidem, diphenhydramine, O-desmethylvenlafaxine, pregabalin, tramadol, and modafinil in venous blood. Histologically, both the macroscopically impressive purple skin changes with underlying bleeding into the subcutaneous tissue and the skin abrasions were due to leukocytoclastic vasculitis, a form of acute hypersensitivity vasculitis that was a reaction to the multiple therapeutic drugs that the woman had taken shortly before death. The manner of death was classified as suicide, and sexual homicide was ruled out.

  18. Antineutrophil cytoplasmic antibodies (ANCA): diagnostic utility and potential role in the pathogenesis of vasculitis.

    PubMed

    Malenica, Branko; Rudolf, Marija; Kozmar, Ana

    2004-01-01

    Antineutrophil cytoplasmic antibodies (ANCA) are a heterogeneous group of circulating antibodies directed toward the cytoplasmic constituents of neutrophils and monocytes. ANCA have been described in various diseases including idiopathic systemic vasculitides, connective tissue diseases, inflammatory bowel diseases, autoimmune liver diseases, infectious diseases, and some drugs. ANCA recognize different target antigens such as proteinase 3 (PR3-ANCA), myeloperoxidase (MPO-ANCA), cathepsin G, lactoferrin, bactericidal/permeability-increasing protein (BPI), and some others. However, only PR3-ANCA and MPO-ANCA are closely associated with systemic vasculitides, in particular Wegener's granulomatosis, microscopic polyangiitis and its renal limited manifestation, and Churg-Strauss syndrome. Both in vitro and in vivo experimental data strongly support a pathogenic role for ANCA in vasculitis and glomerulonephritis.

  19. Risk of viral acute gastrointestinal illness from non-disinfected drinking water distribution systems

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Acute gastrointestinal illness (AGI) resulting from pathogens directly entering the piping of drinking water distribution systems is insufficiently understood. Here, we estimate AGI incidence attributable to virus intrusions into non-disinfecting municipal distribution systems. Viruses were enumerat...

  20. Acute acalculous cholecystitis in systemic lupus erythematosus: a rare initial manifestation.

    PubMed

    Manuel, Valdano; Pedro, Gertrudes Maria; Cordeiro, Lemuel Bornelli; de Miranda, Sandra Maria da Rocha Neto

    2016-01-01

    Acute acalculous cholecystitis is a very rare gastrointestinal manifestation in systemic lupus erythematosus and becomes rarer as an initial manifestation. There are only two cases reported. The authors report a 20-year-old black woman that presented acute acalculous cholecystitis revealed by abdominal computed tomography. During hospitalization, she was diagnosed systemic lupus erythematosus. Conservative treatment with antibiotics was performed with complete remission of the symptoms. Corticosteroid was started in ambulatory. Cholecystectomy has been the treatment of choice in acute acalculous cholecystitis as a complication of systemic lupus erythematosus. The patient responded well to conservative treatment, and surgery was not required. This case is unique in the way that corticosteroid was started in ambulatory care. We should not forget that the acute acalculous cholecystitis can be the initial presentation of systemic lupus erythematosus although its occurrence is very rare. Conservative treatment should be considered. Abdominal computed tomography was a determinant exam for better assessment of acute acalculous cholecystitis.

  1. The role of the immune system in central nervous system plasticity after acute injury.

    PubMed

    Peruzzotti-Jametti, L; Donegá, M; Giusto, E; Mallucci, G; Marchetti, B; Pluchino, S

    2014-12-26

    Acute brain injuries cause rapid cell death that activates bidirectional crosstalk between the injured brain and the immune system. In the acute phase, the damaged CNS activates resident and circulating immune cells via the local and systemic release of soluble mediators. This early immune activation is necessary to confine the injured tissue and foster the clearance of cellular debris, thus bringing the inflammatory reaction to a close. In the chronic phase, a sustained immune activation has been described in many CNS disorders, and the degree of this prolonged response has variable effects on spontaneous brain regenerative processes. The challenge for treating acute CNS damage is to understand how to optimally engage and modify these immune responses, thus providing new strategies that will compensate for tissue lost to injury. Herein we have reviewed the available information regarding the role and function of the innate and adaptive immune responses in influencing CNS plasticity during the acute and chronic phases of after injury. We have examined how CNS damage evolves along the activation of main cellular and molecular pathways that are associated with intrinsic repair, neuronal functional plasticity and facilitation of tissue reorganization.

  2. [Cutaneous vasculitis during bacteremia caused by Meningococcus serogroup B].

    PubMed

    García-Patos, V; Barnadas, M A; Domingo, P; Esquius, J; de Moragas, J M

    1992-04-01

    We report a patient with an upper respiratory tract infection who presented outbreaks of erythematosus-purpuric macules and papules (the pathological substrate of which was a leukocytoclastic vasculitis with numerous intravascular thrombi) coinciding with two autolimited febrile episodes. In serial hemocultures B meningococcus was identified oral antibiotic treatment was given achieving a good clinical evolution. Although both episodes could be considered as a meningococcemia without sepsis, they could also correspond to the initial phase of a chronic meningococcemia. The possible etiopathogenesis of the cutaneous lesions is discussed and the therapeutic and prognostic repercussion of an early identification of these forms of meningococcal disease, which are poorly expressed clinically, are highlighted.

  3. [The systemic inflammatory response syndrome correction in acute destructive pancreatitis].

    PubMed

    Agapov, M A; Khoreva, M V; Gorskiĭ, V A

    2011-01-01

    Acute pancreatitis is a disease of variable severity. In which some patients experience mild, self-limited attacks while others manifest a severe, highly morbid, and frequently lethal attack. The exact mechanisms by which diverse etiological factors induce an attack are still unclear. Recent studies have established the role played by inflammatory mediators in the pathogenesis of acute pancreatitis. In our research we have estimated influence of not steroid anti-inflammatory preparation on synthesis pro-and anti-inflammatory Cytokines at healthy donors and at patients with Acute pancreatitis.

  4. Acute Ozone-Induced Pulmonary and Systemic Metabolic ...

    EPA Pesticide Factsheets

    Acute ozone exposure increases circulating stress hormones and induces peripheral metabolic alterations in animals and humans. We hypothesized that the increase of adrenal-derived stress hormones is necessary for ozone-induced systemic metabolic effects and lung injury. Male Wistar-Kyoto rats (12 week-old) underwent total bilateral adrenalectomy (ADREX), adrenal demedullation (DEMED) or sham surgery (SHEM). After 4 day recovery, rats were exposed to air or ozone (1ppm), 4h/day for 1 or 2 days. Circulating adrenaline levels dropped to nearly zero in DEMED and ADREX rats relative to air-exposed SHAM. Corticosterone levels tended to be low in DEMED rats and dropped to nearly zero in ADREX rats. Adrenalectomy in air-exposed rats caused modest changes in metabolites and lung toxicity parameters. Ozone-induced hyperglycemia and glucose intolerance were markedly attenuated in DEMED with nearly complete reversal in ADREX rats. Ozone increased circulating epinephrine and corticosterone in SHAM but not in DEMED or ADREX rats. Free fatty acids and branched-chain amino acids tended to increase after ozone exposure in SHAM but not in DEMED or ADREX rats. Lung minute volume was not affected by surgery or ozone but ozone-induced labored breathing was less pronounced in ADREX rats. Ozone-induced increases in lung protein leakage and neutrophilic inflammation were markedly reduced in DEMED and ADREX rats (ADREX>DMED). Ozone-mediated decrease in circulating WBC in SHAM was not

  5. Role of Brain Perfusion SPECT with 99mTc HMPAO in the Assessment of Response to Drug Therapy in Patients with Autoimmune Vasculitis: A Prospective Study

    PubMed Central

    Mauro, Liberatore; Manuela, Morreale; Valentina, Megna; Sara, Collorone; Chondrogiannis, Sotirios; Maria, Drudi Francesco; Christos, Anagnostou; Liana, Civitelli; Ada, Francia; Maffione, Anna Margherita; Marzola, Maria Cristina; Rubello, Domenico

    2015-01-01

    Background: The diagnosis of vasculitis in the brain remains a quite difficult achievement. To the best of our knowledge, there is no imaging method reported in literature which is capable of reaching to a diagnosis of vasculitis with very high sensitivity. Aim: The aim of this study was to determine whether perfusion brain single photon emission computed tomography (SPECT) can be usefully employed in monitoring the treatment of vasculitis, allowing treating only potentially responder patients and avoiding the side effects on patients who do not respond. Materials and Methods: Twenty patients (two males and 18 females) suffering from systemic lupus erythematosus (SLE; n = 5), Behcet's disease (BD; n = 5), undifferentiated vasculitis (UV; n = 5), and Sjogren's syndrome (SS; n = 5) were included in the study. All patients underwent a wide neurological anamnestic investigation, a complete objective neurological examination and SPECT of the brain with 99mTc-hexamethyl-propylene-aminoxime (HMPAO). The brain SPECT was then repeated after appropriate medical treatment. The neurological and neuropsychiatric follow-up was performed at 6 months after the start of the treatment. Results: Overall, the differences between the scintigraphic results obtained after and before the medical treatment indicated a statistically significant increase of the cerebral perfusion (CP). In 19 out of 200 regions of interest (ROI) studied, the difference between pre- and post treatment percentages had negative sign, indicating a worsening of CP. This latter event has occurred six times (five in the same patients) in the UV, 10 times (eight in the same patients) in the SLE, never in BD, and three times (two in the same patient) in the SS. Conclusion: The reported results seem to indicate the possibility of identifying, by the means of a brain SPECT, responder and nonresponder (unchanged or worsened CP) patients, affected by autoimmune vasculitis, to the therapy. PMID:25973400

  6. [Cavitating lung lesions in the course of ANCA-associated vasculitis: differential diagnostic aspects].

    PubMed

    Kirchner, J; Raab, H P; Länger, F; Wigand, R; Mitrou, P; Jacobi, V

    1998-05-01

    Antineutrophil cytoplasmatic antibodies (ANCA)-associated vasculitides (Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome) show quite variable courses. Clinical features of the full blown generalized systemic vasculitis are usually found in the respiratory tract and the kidney. Pulmonary involvement of Wegener's granulomatosis shows commonly nodules and cavitations but also diffuse alveolar hemorrhage. We report the case of a 57 year-old man suffering from dyspnea, thoracal pain, arthralgia, purpura, scleritis and tinitus. Specimen of the kidney showed segmental glomerulosclerosis and tubulointerstitial nephritis. Because of the presence of cANCA Wegener's disease was assumed. Pulmonary infiltrates developed under immunosuppressive treatment with cyclophosphamid. As differential diagnosis of the pulmonary infiltrates, we considered invasive pulmonary aspergillosis as well as infiltrates due to Wegener's granulomatosis. In spite of maximal therapeutic management of patient died of respiratory and cardiovascular failure. The findings at autopsy showed distinct invasive pulmonary aspergillosis and perifocal hemorrhage.

  7. Recent advances in anti-neutrophil cytoplasmic antibody-associated vasculitis

    PubMed Central

    Lazarus, B.; John, G. T.; O’Callaghan, C.; Ranganathan, D.

    2016-01-01

    Anti-neutrophil cytoplasmic antibody-associated vasculitis is an uncommon inflammatory disease of small to medium-sized vessels that frequently presents with rapidly progressive glomerulonephritis and renal failure though it can affect any organ system. If untreated, the vast majority of patients will die within a year. Current treatments improve prognosis but affected patients remain at a substantially higher risk of death and adverse outcomes. We review the classification of the disease, our understanding of the pathogenesis and epidemiology, and propose future directions for research. We also evaluate the evidence supporting established treatment regimens and the progress of clinical trials for newer treatments to inform the design of future studies. PMID:27051131

  8. Leukocytoclastic Vasculitis in a Patient with Ankylosing Spondylitis

    PubMed Central

    Kobak, Senol; Yilmaz, Hatice; Karaarslan, Ahmet; Yalcin, Murat

    2014-01-01

    A 26-year-old male patient presented to our rheumatology clinic with pain, swelling and limitation of movement in his right ankle, and also purpuric skin lesions in the lower extremity pretibial region. He was asked questions, and he said that he had been having chronic low back pain and morning stiffness for the last few years. His physical examination revealed that he had arthritis in his right ankle, purpuric skin lesions in pretibial regions of both legs, and bilateral FABERE/FADIR positivity. The sacroiliac joint imaging and MRI revealed bilateral sacroiliitis findings, and the lateral heel imaging revealed enthesitis. HLA-B27 was positive. Skin biopsy from lower skin lesions was reported to be consistent with leukocytoclastic vasculitis. Based on clinical, laboratory, radiological, and pathological examinations, the patient was diagnosed with ankylosing spondylitis and leukocytoclastic vasculitis. Administration of corticosteroid, salazopyrin, and nonsteroid anti-inflammatory medications was started. Notable clinical and laboratory regression was observed during his checks 3 months later. PMID:24653851

  9. Eosinophils in vasculitis: characteristics and roles in pathogenesis.

    PubMed

    Khoury, Paneez; Grayson, Peter C; Klion, Amy D

    2014-08-01

    Eosinophils are multifunctional granular leukocytes that are implicated in the pathogenesis of a wide variety of disorders, including asthma, helminth infection, and rare hypereosinophilic syndromes. Although peripheral and tissue eosinophilia can be a feature of many types of small-vessel and medium-vessel vasculitis, the role of eosinophils has been best studied in eosinophilic granulomatosis with polyangiitis (EGPA), where eosinophils are a characteristic finding in all three clinical stages of the disorder. Whereas numerous studies have demonstrated an association between the presence of eosinophils and markers of eosinophil activation in the blood and tissues of patients with EGPA, the precise role of eosinophils in disease pathogenesis has been difficult to ascertain owing to the complexity of the disease process. In this regard, results of clinical trials using novel agents that specifically target eosinophils are providing the first direct evidence of a central role of eosinophils in EGPA. This Review focuses on the aspects of eosinophil biology most relevant to the pathogenesis of vasculitis and provides an update of current knowledge regarding the role of eosinophils in EGPA and other vasculitides.

  10. Pathways to renal biopsy and diagnosis among patients with ANCA small-vessel vasculitis

    PubMed Central

    Poulton, Caroline J.; Nachman, Patrick H.; Hu, Yichun; McGregor, JulieAnne G.; Jennette, J. Charles; Falk, Ronald J.; Hogan, Susan L.

    2013-01-01

    Objectives Antineutrophil cytoplasmic antibody small-vessel vasculitis (ANCA-SVV) is an autoimmune systemic process increasingly recognised since the advent of antibody testing for the disease. Prompt diagnosis and institution of immunosuppressive therapy has been shown to improve patient outcome. The goal of this study was to better understand how patients navigate the health care system from symptom presentation to biopsy diagnosis, and to study the effects of prompt versus delayed diagnosis. Methods Disease symptoms and number of physicians seen prior to renal biopsy were assessed for 127 ANCA-SVV patients. Direct, delayed, and quest pathways to diagnosis and treatment of vasculitis were defined for both patients and providers. Kruskal-Wallis and Fisher exact tests were used to evaluate continual measures and compare categorical variables across pathways. Results Among patients who sought direct care, physician delay in referral to a nephrologist was common (49/127, 71%, p=0.0023). Patients who delayed seeking care also experienced a delayed diagnosis 57% of the time (p=0.0023). Patients presenting with prodromal flu or upper respiratory involvement were more likely to have a delay/quest patient pathway (56% and 55%, respectively) than a direct patient pathway (44%, p=0.033 and 45%, p=0.019, respectively). There was a trend for patients with more severe loss of renal function to have a more direct referral to a nephrologist. Conclusion Delay in diagnosis of ANCA SVV may be due to lack of or non-specific symptoms, especially in patients who present with non-renal manifestations of disease. Better algorithms are needed to identify extra-renal manifestations, expedite diagnosis and improve patient outcomes. PMID:23343774

  11. Acquired immune deficiency syndrome and systemic lupus erythematosis: potential causes of surgical emergencies of the hand.

    PubMed

    Wirth, Johnny J; Sheka, Kedambady P; Gheewala, Anup; Rowe, Norman M

    2008-07-01

    Compartment Syndrome (CS) is a disease that has 2 etiologies, that of acute events and that of chronic. It occurs when the pressure in a fascia-encased compartment exceeds the profusion pressure in tissue. The end result, when left untreated, is muscle and nerve ischemia that can cause significant morbidity. Nerve paralysis, muscle necrosis and fibrosis and, when occurring in an extremity, loss of the limb are some of the potential outcomes of missed diagnosis. This case series involves 2 cases of CS that where caused by vasculitis with etiologies of human immunodeficiency virus and systemic lupus erythematosis. Autoimmune vasculitis has many systemic and local manifestations, but to our knowledge CS has not been described as one of its sequelae. The following is literature review and presentation of these 2 cases.

  12. Rapid improvement of distal vasculitis in PAN related to hepatitis B with alprostadil infusion: a case report.

    PubMed

    Lim, Mie-Jin; Kwon, Seong Ryul; Lee, Seunghee; Park, Won

    2006-08-01

    Polyarteritis nodosa (PAN) related to hepatitis B is an uncommon vasculitis that is sometimes associated with the rapid progression of distal ischemia. A few recent reports have proposed the use of antiviral therapy. However, there is not yet a consensus for the standard treatment of this disease entity and none of these treatments have been focused on fast symptomatic improvement. We describe here a 39-year-old female patient with PAN related to hepatitis B infection who completely recovered from the acutely progressing ischemic manifestations of her distal extremities with the use of alprostadil infusion (prostaglandin E1). The reactivation of her hepatitis B infection after glucocorticoid and cyclophosphamide therapy was successfully managed by the antiviral lamuvudine therapy. Most importantly, the vasodilator together with the conventional therapy may be desirable in the early stages of the disease before irreversible ischemic tissue damage can occur.

  13. Quercetin Inhibits Inflammasome Activation by Interfering with ASC Oligomerization and Prevents Interleukin-1 Mediated Mouse Vasculitis

    PubMed Central

    Domiciano, Talita P.; Wakita, Daiko; Jones, Heather D.; Crother, Timothy R.; Verri, Waldiceu A.; Arditi, Moshe; Shimada, Kenichi

    2017-01-01

    Interleukin-1β (IL-1β) is a highly inflammatory cytokine that significantly contributes to both acute and chronic inflammatory diseases. The secretion of IL-1β requires a unique protease, caspase-1, which is activated by various protein platforms called inflammasomes. Data suggests a key role for mitochondrial reactive oxygen species for inflammasome activation. Flavonoids constitute a group of naturally occurring polyphenolic molecules with many biological activities, including antioxidant effects. In this study, we investigated the effect of three flavonoids, quercetin (QUC), naringenin, and silymarim on inflammasome activation. We found that QUC inhibits IL-1β secretion by both the NLRP3 and AIM2 inflammasome in a dose dependent manner, but not the NLRC4 inflammasome. QUC inhibition of the inflammasome was still observed in Atg16l1 knockout macrophages, indicating that QUC’s effect was autophagy independent. Since QUC inhibited both NLRP3 and AIM2 inflammasomes but not NLRC4, we assessed ASC speck formation. QUC reduced ASC speck formation and ASC oligomerization compared with controls. Additionally, QUC inhibited IL-1β in Cryopyrin-Associated Periodic Syndromes (CAPS) macrophages, where NLRP3 inflammasome is constitutively activated. In conclusion, QUC inhibits both the NLRP3 and AIM2 inflammasome by preventing ASC oligomerization and may be a potential therapeutic candidate for Kawasaki disease vasculitis and other IL-1 mediated inflammatory diseases. PMID:28148962

  14. Quercetin Inhibits Inflammasome Activation by Interfering with ASC Oligomerization and Prevents Interleukin-1 Mediated Mouse Vasculitis.

    PubMed

    Domiciano, Talita P; Wakita, Daiko; Jones, Heather D; Crother, Timothy R; Verri, Waldiceu A; Arditi, Moshe; Shimada, Kenichi

    2017-02-02

    Interleukin-1β (IL-1β) is a highly inflammatory cytokine that significantly contributes to both acute and chronic inflammatory diseases. The secretion of IL-1β requires a unique protease, caspase-1, which is activated by various protein platforms called inflammasomes. Data suggests a key role for mitochondrial reactive oxygen species for inflammasome activation. Flavonoids constitute a group of naturally occurring polyphenolic molecules with many biological activities, including antioxidant effects. In this study, we investigated the effect of three flavonoids, quercetin (QUC), naringenin, and silymarim on inflammasome activation. We found that QUC inhibits IL-1β secretion by both the NLRP3 and AIM2 inflammasome in a dose dependent manner, but not the NLRC4 inflammasome. QUC inhibition of the inflammasome was still observed in Atg16l1 knockout macrophages, indicating that QUC's effect was autophagy independent. Since QUC inhibited both NLRP3 and AIM2 inflammasomes but not NLRC4, we assessed ASC speck formation. QUC reduced ASC speck formation and ASC oligomerization compared with controls. Additionally, QUC inhibited IL-1β in Cryopyrin-Associated Periodic Syndromes (CAPS) macrophages, where NLRP3 inflammasome is constitutively activated. In conclusion, QUC inhibits both the NLRP3 and AIM2 inflammasome by preventing ASC oligomerization and may be a potential therapeutic candidate for Kawasaki disease vasculitis and other IL-1 mediated inflammatory diseases.

  15. Arterial distensibility in children and teenagers: normal evolution and the effect of childhood vasculitis

    PubMed Central

    Cheung, Y; Brogan, P; Pilla, C; Dillon, M; Redington, A

    2002-01-01

    Background: Polyarteritis nodosa is a necrotising vasculitis of the medium sized and small muscular arteries. The inflammatory and subsequent reparative processes may alter the arterial mechanical properties. The effect of vasculitic damage on arterial distensibility has never been explored however. Aim: To determine the normal values and the effect of childhood vasculitis on arterial distensibility in children and teenagers. Methods: Distensibility of the brachioradial arterial segment was studied using pulse wave velocity (PWV ∝1/√distensibility), in 13 children with polyarteritis nodosa at a median age of 11.8 (range 4.9–16) years. As a control group, 155 healthy schoolchildren (6–18 years, 81 boys) were studied. PWV was assessed using a photoplethysmographic technique; blood pressure was measured by an automatic sphygmomanometer (Dinamap). Data from patients were expressed as z scores adjusted for age and compared to a population mean of 0 by a single sample t test. Determinants of PWV in normal children were assessed by univariate and multivariate linear regression analyses. Results: Age, height, weight, and systolic blood pressure correlated individually with the brachioradial PWV. Multivariate analysis identified age as the only independent determinant. Ten of the patients were in clinical remission, while three had evidence of disease activity at the time of study. The PWV in the patient group as a whole was significantly greater than those in healthy children (mean z score +0.99). Raised C reactive protein concentration (>2 mg/dl) in the three patients with active disease was associated with a higher PWV when compared to those in remission (z score +2.78 v +0.45). The diastolic blood pressure of the patients was higher than those of the controls (z score +1.04) while the systolic pressure was similar (z score -0.36). Conclusions: PWV in the brachioradial arterial segment increases gradually during childhood independent of body weight, height, mass

  16. Acute coronary vasospasm in a patient with eosinophilic granulomatosis with polyangiitis following NSAID administration

    PubMed Central

    Benallegue, Naïl; Lozach, Pierre; Belizna, Cristina; Lavigne, Christian; Urbanski, Geoffrey

    2016-01-01

    Abstract Eosinophilic with polyangiitis (EGPA, formerly known as Churg-Strauss syndrome) is a rare systemic disease characterized by a small-vessel necrotizing vasculitis. Cardiac manifestations are broad-ranging and are associated with a poor prognosis. Coronary vasospasm is uncommon. Here, we report a case of an acute coronary vasospasm in a patient with EGPA after corticosteroids withdrawal and nonsteroidal antiinflammatory drug (NSAID) introduction. This patient was initially misdiagnosed as bradykinin-mediated angioedema. A 30-year-old man presented with recurrence of abdominal pain and acute dyspnea. NSAID administration for pain during a flare was followed by coronary vasospasms leading to cardiac arrest. Corticosteroid treatment was recently interrupted by the patient. This case reports a rare cardiac complication of EGPA. NSAID might contribute to coronary vasospasm by eosinophilic degranulation in EGPA. Moreover, corticosteroid compliance must be emphasized among patients who display EGPA with high cardiac risk to prevent fatal issues. PMID:27893661

  17. The role of pain, perseverative cognition and goal adjustment in vasculitis-associated fatigue.

    PubMed

    Mayor, Helen; Reidy, John

    2016-06-14

    Fatigue is a common symptom associated with vasculitis and contributes significantly to impaired quality of life. Motivational control theory suggests a role for perseverative cognition and goal adjustment in fatigue. Therefore, this study investigated these potential predictors of fatigue in individuals with vasculitis. A total of 249 participants completed online questionnaires assessing fatigue, perseverative cognition, goal disengagement and goal reengagement, in addition to demographic and disease-related variables. Hierarchical regression analysis found only pain, sleep disturbance, disease activity and perseverative cognition to significantly predict fatigue. This highlights the importance of psychological factors in determining fatigue in those with vasculitis.

  18. An unusual cause of acute kidney injury due to oxalate nephropathy in systemic scleroderma.

    PubMed

    Mascio, Heather M; Joya, Christie A; Plasse, Richard A; Baker, Thomas P; Flessner, Michael F; Nee, Robert

    2015-08-01

    Oxalate nephropathy is an uncommon cause of acute kidney injury. Far rarer is its association with scleroderma, with only one other published case report in the literature. We report a case of a 75-year-old African-American female with a history of systemic scleroderma manifested by chronic pseudo-obstruction and small intestinal bacterial overgrowth (SIBO) treated with rifaximin, who presented with acute kidney injury with normal blood pressure. A renal biopsy demonstrated extensive acute tubular injury with numerous intratubular birefringent crystals, consistent with oxalate nephropathy. We hypothesize that her recent treatment with rifaximin for SIBO and decreased intestinal transit time in pseudo-obstruction may have significantly increased intestinal oxalate absorption, leading to acute kidney injury. Oxalate nephropathy should be considered in the differential diagnosis of acute kidney injury in scleroderma with normotension, and subsequent evaluation should be focused on bowel function to include alterations in gut flora due to antibiotic administration.

  19. Acute inpatient presentation of scurvy.

    PubMed

    Swanson, Allison M; Hughey, Lauren C

    2010-10-01

    Scurvy is a well-known disease of vitamin C deficiency that still occurs in industrialized countries. The clinical manifestations of follicular hyperkeratosis, perifollicular petechiae, corkscrew hairs, and easy bruising are due to defective collagen synthesis and can be mistaken for small vessel vasculitis. Populations at risk for development of scurvy include elderly patients, alcohol and drug users, individuals who follow restrictive diets or have eating disorders, patients with malabsorption, and individuals with mental illness. We report an acute case of scurvy presenting in the inpatient/hospital setting with clinical findings initially thought to represent vasculitis. A high index of suspicion for scurvy must be kept in the appropriate clinical context, and a thorough medical history and physical examination are vital to make the diagnosis.

  20. The role of ZAP70 kinase in acute lymphoblastic leukemia infiltration into the central nervous system.

    PubMed

    Alsadeq, Ameera; Fedders, Henning; Vokuhl, Christian; Belau, Nele M; Zimmermann, Martin; Wirbelauer, Tim; Spielberg, Steffi; Vossen-Gajcy, Michaela; Cario, Gunnar; Schrappe, Martin; Schewe, Denis M

    2017-02-01

    Central nervous system infiltration and relapse are poorly understood in childhood acute lymphoblastic leukemia. We examined the role of zeta-chain-associated protein kinase 70 in preclinical models of central nervous system leukemia and performed correlative studies in patients. Zeta-chain-associated protein kinase 70 expression in acute lymphoblastic leukemia cells was modulated using short hairpin ribonucleic acid-mediated knockdown or ectopic expression. We show that zeta-chain-associated protein kinase 70 regulates CCR7/CXCR4 via activation of extracellular signal-regulated kinases. High expression of zeta-chain-associated protein kinase 70 in acute lymphoblastic leukemia cells resulted in a higher proportion of central nervous system leukemia in xenografts as compared to zeta-chain-associated protein kinase 70 low expressing counterparts. High zeta-chain-associated protein kinase 70 also enhanced the migration potential towards CCL19/CXCL12 gradients in vitro CCR7 blockade almost abrogated homing of acute lymphoblastic leukemia cells to the central nervous system in xenografts. In 130 B-cell precursor acute lymphoblastic leukemia and 117 T-cell acute lymphoblastic leukemia patients, zeta-chain-associated protein kinase 70 and CCR7/CXCR4 expression levels were significantly correlated. Zeta-chain-associated protein kinase 70 expression correlated with central nervous system disease in B-cell precursor acute lymphoblastic leukemia, and CCR7/CXCR4 correlated with central nervous system involvement in T-cell acute lymphoblastic leukemia patients. In multivariate analysis, zeta-chain-associated protein kinase 70 expression levels in the upper third and fourth quartiles were associated with central nervous system involvement in B-cell precursor acute lymphoblastic leukemia (odds ratio=7.48, 95% confidence interval, 2.06-27.17; odds ratio=6.86, 95% confidence interval, 1.86-25.26, respectively). CCR7 expression in the upper fourth quartile correlated with central

  1. The role of ZAP70 kinase in acute lymphoblastic leukemia infiltration into the central nervous system

    PubMed Central

    Alsadeq, Ameera; Fedders, Henning; Vokuhl, Christian; Belau, Nele M.; Zimmermann, Martin; Wirbelauer, Tim; Spielberg, Steffi; Vossen-Gajcy, Michaela; Cario, Gunnar; Schrappe, Martin; Schewe, Denis M.

    2017-01-01

    Central nervous system infiltration and relapse are poorly understood in childhood acute lymphoblastic leukemia. We examined the role of zeta-chain-associated protein kinase 70 in preclinical models of central nervous system leukemia and performed correlative studies in patients. Zeta-chain-associated protein kinase 70 expression in acute lymphoblastic leukemia cells was modulated using short hairpin ribonucleic acid-mediated knockdown or ectopic expression. We show that zeta-chain-associated protein kinase 70 regulates CCR7/CXCR4 via activation of extracellular signal-regulated kinases. High expression of zeta-chain-associated protein kinase 70 in acute lymphoblastic leukemia cells resulted in a higher proportion of central nervous system leukemia in xenografts as compared to zeta-chain-associated protein kinase 70 low expressing counterparts. High zeta-chain-associated protein kinase 70 also enhanced the migration potential towards CCL19/CXCL12 gradients in vitro. CCR7 blockade almost abrogated homing of acute lymphoblastic leukemia cells to the central nervous system in xenografts. In 130 B-cell precursor acute lymphoblastic leukemia and 117 T-cell acute lymphoblastic leukemia patients, zeta-chain-associated protein kinase 70 and CCR7/CXCR4 expression levels were significantly correlated. Zeta-chain-associated protein kinase 70 expression correlated with central nervous system disease in B-cell precursor acute lymphoblastic leukemia, and CCR7/CXCR4 correlated with central nervous system involvement in T-cell acute lymphoblastic leukemia patients. In multivariate analysis, zeta-chain-associated protein kinase 70 expression levels in the upper third and fourth quartiles were associated with central nervous system involvement in B-cell precursor acute lymphoblastic leukemia (odds ratio=7.48, 95% confidence interval, 2.06–27.17; odds ratio=6.86, 95% confidence interval, 1.86–25.26, respectively). CCR7 expression in the upper fourth quartile correlated with

  2. What does the future hold for clinical studies in vasculitis?

    PubMed

    Hoffman, G S

    2011-05-01

    The era prior to 1990 was a time of careful observation of disease presentation, course, outcomes and meticulous pathology studies. These mainly single-centre studies introduced new life-saving therapies for drugs still used effectively today. In the 1970-1980s, cyclophosphamide (CyP) added to glucocorticosteroids (GCS) was shown to be life-saving. The trade-off was often severe adverse events. Some forms of vasculitis were found not as ominous as thought initially. Some could be treated with safer drugs [e.g. methotrexate (MTX)]. However, whether mild or severe, patients were not cured. From 1990 to the present large collaborative networks have provided studies were not possible heretofore. Randomized controlled trials captured and manipulated vast amounts of data, banked biological specimens and shared these resources and intellectual capital, moving the field forward at an extraordinary pace. We now know that even for severe forms of granulomatosis and polyangiitis [granulomatosis with polyangiitis (GPA), Wegener's granulomatosus (WG)], microscopic polyangiitis (MPA) and Churg-Strauss syndrome (CSS), we do not need to use CyP for extended periods. We have learned recently that rituximab is as effective as CyP for severe WG and MPA. We should never again see the permanent toxicities born from years of chronic CyP use. However, short courses of CyP remain useful and can be life-saving. Step-down therapy from CyP is now a standard of care, perhaps to be replaced by rituximab in the future. If one accepts the premise that there are few cures at present for idiopathic large- and small-vessel vasculitis, we will serve our patients well if we can determine the most effective initial therapy that leads to a maintenance strategy for remission with least risk. Ultimately, we wish to identify causes of vasculitis so they can be used as a wedge to secure cures. Unmet needs and strategies are as follows: (1) to increase the numbers of vasculitis-trained physicians; (2) to

  3. Advances in pathogenesis and treatment of ANCA-associated vasculitis.

    PubMed

    Kallenberg, Cees G M

    2014-10-01

    Anti-neutrophil cytoplasmic autoantibodies (ANCA) directed to proteinase 3 (PR3-ANCA) and myeloperoxidase (MPO-ANCA) are sensitive and specific markers for their associated diseases, granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis) and microscopic polyangiitis (MPA), respectively. Clinical observations suggest but do not prove that ANCA are involved in the pathogenesis of GPA and MPA. In vivo and in vitro experimental data strongly suggest if not prove that MPO-ANCA underlie the pathological lesions seen in MPO-ANCA associated MPA. This is less clear for PR3-ANCA associated GPA in which, besides small-vessel vasculitis, granulomatous inflammation is apparent. Here, cellular immunity appears to play an additional role. Insight into the pathogenetic events involved in these diseases has resulted in new ways of treatment that target the specific pathways that underlie the development of the lesions.

  4. Maintenance of clinical remission in ANCA-associated vasculitis.

    PubMed

    Luqmani, Raashid

    2013-02-01

    A fundamental change in management of antineutrophil cytoplasmic antibody-associated vasculitis in the past 10 years is the early focussed use of aggressive immunosuppression, using regimens comprised of widely available medications. Using a clinical framework to quantify morbidity, we can induce remission in most patients within 3-6 months using glucocorticoids plus methotrexate, cyclophosphamide or rituximab, with additional plasmapheresis when indicated. Difficulty in maintaining remission probably relates to the difference between true pathophysiological remission and the absence of clinical, serological or radiological evidence of disease activity. For surviving patients, the cumulative problems of relapse, burden of disease, or its treatment are coupled with pre-existing diseases or new conditions arising since diagnosis. Initial early control should reduce subsequent damage, but what effect it will have on relapse is not clear. In the absence of a cure, future trials should focus on reducing toxicity and comorbidity as well as controlling disease.

  5. Neutrophilic Dermatoses in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

    PubMed Central

    de Boysson, Hubert; Martin Silva, Nicolas; de Moreuil, Claire; Néel, Antoine; de Menthon, Mathilde; Meyer, Olivier; Launay, David; Pagnoux, Christian; Guillevin, Loïc; Puéchal, Xavier; Bienvenu, Boris; Aouba, Achille

    2016-01-01

    Abstract A few reports suggest combination of ANCA-associated vasculitis (AAV) and neutrophilic dermatoses (ND). We aimed to describe the main characteristics of patients presenting with both AAV and ND in a French cohort and through a systematic literature review, and to discuss the possible common pathogenic process involved. We conducted a retrospective study of patients with both conditions. Patients were selected via the French Internal Medicine Society (SNFMI) and the French Vasculitis Study Group (FVSG). A literature review focusing on a combination of both conditions, concentrated only on publications with well-established diagnoses and individual detailed data. Seventeen patients diagnosed with AAV and ND were identified in this cohort. Twelve patients had granulomatosis with polyangiitis (GPA), 4 had microscopic polyangiitis (MPA) and one had eosinophilic GPA (EGPA). Eight patients, all with GPA, displayed pyoderma gangrenosum (PG). Sweet's syndrome was observed in 6 patients (4 with MPA, one with GPA and one with EGPA) and erythema elevatum diutinum in the other three (2 with GPA and 1 with MPA). The literature review identified 33 additional patients with both conditions, including 26 with GPA. Altogether, of the 50 patients (17 from our study and 33 from the literature review), 33 (66%) patients presented with PG associated with GPA in 29 cases (89%). Corticosteroids were the first-line treatment in conjunction with an immunosuppressive agent in most cases. Outcomes were good and a total of 15 patients experienced a relapse. Patients who relapsed were more likely to have ear, nose and throat manifestation than patients who did not [12/15 (80%) relapsing patients vs. 15/35 (43%) non-relapsing patients; p = 0.03)]. In our stud, the most frequent association concerned GPA and PG. ND should be considered and specifically researched within the spectrum of cutaneous manifestations observed in AAV. PMID:26986103

  6. Risk-Based Classification System of Patients With Newly Diagnosed Acute Lymphoblastic Leukemia

    ClinicalTrials.gov

    2017-02-13

    Adult B Acute Lymphoblastic Leukemia; Adult T Acute Lymphoblastic Leukemia; Childhood B Acute Lymphoblastic Leukemia; Childhood T Acute Lymphoblastic Leukemia; Untreated Adult Acute Lymphoblastic Leukemia; Untreated Childhood Acute Lymphoblastic Leukemia

  7. Vasculitis in patients with inflammatory bowel diseases: A study of 32 patients and systematic review of the literature

    PubMed Central

    Sy, Alice; Khalidi, Nader; Dehghan, Natasha; Barra, Lillian; Carette, Simon; Cuthbertson, David; Hoffman, Gary S.; Koening, Curry L.; Langford, Carol A.; McAlear, Carol; Moreland, Larry; Monach, Paul A.; Seo, Philip; Specks, Ulrich; Sreih, Antoine; Ytterberg, Steven R.; Van Assche, Gert; Merkel, Peter A.; Pagnoux, Christian

    2016-01-01

    Background Published small case series suggest that inflammatory bowel disease [IBD; Crohn’s disease (CD) or ulcerative colitis (UC)] and vasculitis co-occur more frequently than would be expected by chance. Objectives To describe this association by an analysis of a large cohort of carefully studied patients and through a systematic literature review. Methods Patients with both IBD and vasculitis enrolled in the Vasculitis Clinical Research Consortium (VCRC) Longitudinal Studies, followed in Canadian Vasculitis research network (CanVasc) centers and/or in the University of Toronto’s IBD clinic were included in this case series. A systematic literature review of patients with IBD and vasculitis involved a PubMed search through February 2014. The main characteristics of patients with Takayasu arteritis (TAK) and IBD were compared to those in patients with TAK without IBD followed in the VCRC. Results The study identified 32 patients with IBD and vasculitis: 13 with large-vessel vasculitis [LVV; 12 with TAK, 1 with giant cell arteritis (GCA); 8 with CD, 5 with UC]; 8 with ANCA-associated vasculitis [AAV; 6 granulomatosis with polyangiitis (GPA), 2 with eosinophilic granulomatosis with polyangiitis (EGPA)]; 5 with isolated cutaneous vasculitis; and 6 with other vasculitides. Patients with LVV and AAV were mostly female (18/21). The diagnosis of IBD preceded that of vasculitis in 12/13 patients with LVV and 8/8 patients with AAV. The review of the literature identified 306 patients with IBD and vasculitis: 144 with LVV (133 TAK; 87 with IBD preceding LVV), 19 with AAV [14 GPA, 1 EGPA, 4 microscopic polyangiitis (MPA)], 66 with isolated cutaneous vasculitis, and 77 with other vasculitides. Patients with IBD and TAK were younger and had more frequent headaches, constitutional symptoms, or gastrointestinal symptoms compared to those patients in the VCRC who had TAK without IBD. Conclusions These findings highlight the risk of vasculitis, especially TAK, in patients

  8. Successful treatment of acute systemic anaphylaxis in a western lowland gorilla (Gorilla gorilla gorilla).

    PubMed

    Hayman, David T S; King, Tony; Cameron, Kenneth

    2010-09-01

    This brief communication describes the successful treatment of acute systemic anaphylaxis in a wild-born but captive infant western lowland gorilla (Gorilla gorilla gorilla) in the Republic of Congo. The infant demonstrated signs of acute respiratory distress, lingual swelling, and reaction to intradermal tuberculin, given 55 hr earlier. Details of the treatment with steroids, anesthetic induction, and i.v. epinephrine are all reported, and potential antigens that may have initiated the anaphylactic shock are discussed.

  9. [Two cases of severe cutaneous vasculitis with thrombocytopenia associated with hepatic cirrhosis: autoimmune and infective-inflammatory component with endothelial lesion and restrictive pulmonary pattern in one case].

    PubMed

    de Andrade Júnior, D R; Warth, M do P; Morrone, L F; Calich, I; de Andrade, D R

    1992-01-01

    Two clinical cases of female patients with hepatic cirrhosis and autoimmune multisystemic involvement with infectious intercurrent are reported. Case 1 presented infective endocarditis and erysipelas on the left thigh. In the course of the clinical picture a cutaneous vasculitis developed in the same place together with autoimmune thrombocytopenia, leukopenia and pulmonary restrictive picture with inflammatory pattern. There are also elevate immune complexes and complement consumption. Case 2 presented erysipelas on the left thigh cutaneous vasculitis and complement consumption. In Case 1 the infective endocarditis was treated with antibiotic therapy during 4 weeks followed by 1 mg/kg corticoid (Prednisone) with thrombocytopenia and leukopenia reversion. Case 2 presented an improvement with antibiotic only. The relation between chronic liver diseases and systemic autoimmune phenomena is commented, special attention being paid to the cutaneous, hematological and pulmonary affection.

  10. IL-17 production by CSF lymphocytes as a biomarker for cerebral vasculitis

    PubMed Central

    Thom, Vivien; Schmid, Sabrina; Gelderblom, Mathias; Hackbusch, Romy; Kolster, Manuela; Schuster, Simon; Thomalla, Götz; Keminer, Oliver; Pleß, Ole; Bernreuther, Christian; Glatzel, Markus; Wegscheider, Karl; Gerloff, Christian

    2016-01-01

    Objective: To explore the possibility of using interleukin-17 (IL-17) production by CD4+ T cells in the CSF as a potential biomarker for cerebral vasculitis in stroke patients. Methods: In this consecutive case study, we performed prospective analysis of CSF and blood in patients admitted to a university medical center with symptoms of stroke and suspected cerebral vasculitis. Flow cytometry was performed for intracellular detection of inflammatory cytokines in peripheral blood lymphocytes and expanded T cells from CSF. Results: CSF CD4+ lymphocytes from patients with cerebral vasculitis showed significantly higher levels of the proinflammatory cytokine IL-17 compared to patients with stroke not due to vasculitis or with other, noninflammatory neurologic diseases. There was no difference in the production of interferon-γ in the CSF and no overall differences in the relative frequencies of peripheral immune cells. Conclusions: Intracellular IL-17 in CSF cells is potentially useful in discriminating cerebral vasculitis as a rare cause in patients presenting with ischemic stroke. Classification of evidence: This study provides Class II evidence that an increased proportion of IL-17-producing CD4+ cells in CSF of patients presenting with stroke symptoms is indicative of cerebral vasculitis (sensitivity 73%, 95% confidence interval [CI] 39–94%; specificity 100%, 95% CI 74%–100%). PMID:27144213

  11. Promoting patient-centred fundamental care in acute healthcare systems.

    PubMed

    Feo, Rebecca; Kitson, Alison

    2016-05-01

    Meeting patients' fundamental care needs is essential for optimal safety and recovery and positive experiences within any healthcare setting. There is growing international evidence, however, that these fundamentals are often poorly executed in acute care settings, resulting in patient safety threats, poorer and costly care outcomes, and dehumanising experiences for patients and families. Whilst care standards and policy initiatives are attempting to address these issues, their impact has been limited. This discussion paper explores, through a series of propositions, why fundamental care can be overlooked in sophisticated, high technology acute care settings. We argue that the central problem lies in the invisibility and subsequent devaluing of fundamental care. Such care is perceived to involve simple tasks that require little skill to execute and have minimal impact on patient outcomes. The propositions explore the potential origins of this prevailing perception, focusing upon the impact of the biomedical model, the consequences of managerial approaches that drive healthcare cultures, and the devaluing of fundamental care by nurses themselves. These multiple sources of invisibility and devaluing surrounding fundamental care have rendered the concept underdeveloped and misunderstood both conceptually and theoretically. Likewise, there remains minimal role clarification around who should be responsible for and deliver such care, and a dearth of empirical evidence and evidence-based metrics. In explicating these propositions, we argue that key to transforming the delivery of acute healthcare is a substantial shift in the conceptualisation of fundamental care. The propositions present a cogent argument that counters the prevailing perception that fundamental care is basic and does not require systematic investigation. We conclude by calling for the explicit valuing and embedding of fundamental care in healthcare education, research, practice and policy. Without this

  12. Cross-Sector Review of Drivers and Available 3Rs Approaches for Acute Systemic Toxicity Testing

    PubMed Central

    Seidle, Troy; Robinson, Sally; Holmes, Tom; Creton, Stuart; Prieto, Pilar; Scheel, Julia; Chlebus, Magda

    2010-01-01

    Acute systemic toxicity studies are carried out in many sectors in which synthetic chemicals are manufactured or used and are among the most criticized of all toxicology tests on both scientific and ethical grounds. A review of the drivers for acute toxicity testing within the pharmaceutical industry led to a paradigm shift whereby in vivo acute toxicity data are no longer routinely required in advance of human clinical trials. Based on this experience, the following review was undertaken to identify (1) regulatory and scientific drivers for acute toxicity testing in other industrial sectors, (2) activities aimed at replacing, reducing, or refining the use of animals, and (3) recommendations for future work in this area. PMID:20484382

  13. Leukocytoclastic vasculitis in an adolescent with ulcerative colitis: Report of a case and review of the literature

    PubMed Central

    Butts, G Tyler; Bishop, Phyllis R; Wyatt, Julie P

    2014-01-01

    An adolescent female with long-standing, difficult-to-control ulcerative colitis developed leukocytoclastic vasculitis, a rare cutaneous extra-intestinal manifestation of the inflammatory bowel disease. The authors provide a literature review on leukocytoclastic vasculitis complicating ulcerative colitis. Furthermore, the clinical features of leukocytoclastic vasculitis are compared and contrasted with the more common cutaneous extra-intestinal manifestations of inflammatory bowel disease, erythema nodosum, and pyoderma gangrenosum. PMID:27489650

  14. 76 FR 51475 - Medicare Program; Hospital Inpatient Prospective Payment Systems for Acute Care Hospitals and the...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-08-18

    ...We are revising the Medicare hospital inpatient prospective payment systems (IPPS) for operating and capital-related costs of acute care hospitals to implement changes arising from our continuing experience with these systems and to implement certain statutory provisions contained in the Patient Protection and Affordable Care Act and the Health Care and Education Reconciliation Act of 2010......

  15. 75 FR 50041 - Medicare Program; Hospital Inpatient Prospective Payment Systems for Acute Care Hospitals and the...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-08-16

    ...We are revising the Medicare hospital inpatient prospective payment systems (IPPS) for operating and capital-related costs of acute care hospitals to implement changes arising from our continuing experience with these systems and to implement certain provisions of the Affordable Care Act and other legislation. In addition, we describe the changes to the amounts and factors used to determine......

  16. 77 FR 60315 - Medicare Program; Hospital Inpatient Prospective Payment Systems for Acute Care Hospitals and the...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-10-03

    ...-AR12 Medicare Program; Hospital Inpatient Prospective Payment Systems for Acute Care Hospitals and the Long-Term Care Hospital Prospective Payment System and Fiscal Year 2013 Rates; Hospitals' Resident Caps for Graduate Medical Education Payment Purposes; Quality Reporting Requirements for Specific...

  17. 78 FR 15882 - Medicare Program; Hospital Inpatient Prospective Payment Systems for Acute Care Hospitals and the...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-03-13

    ...-AR12 Medicare Program; Hospital Inpatient Prospective Payment Systems for Acute Care Hospitals and the Long Term Care Hospital Prospective Payment System and Fiscal Year 2013 Rates; Hospitals' Resident Caps for Graduate Medical Education Payment Purposes; Quality Reporting Requirements for Specific...

  18. 77 FR 63751 - Medicare Program; Hospital Inpatient Prospective Payment Systems for Acute Care Hospitals and the...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-10-17

    ...; Hospital Inpatient Prospective Payment Systems for Acute Care Hospitals and the Long-Term Care Hospital Prospective Payment System and Fiscal Year 2013 Rates; Hospitals' Resident Caps for Graduate Medical Education Payment Purposes; Quality Reporting Requirements for Specific Providers and for Ambulatory...

  19. East Coast Fever Caused by Theileria parva Is Characterized by Macrophage Activation Associated with Vasculitis and Respiratory Failure

    PubMed Central

    Schneider, David A.; Frevert, Charles W.; Nelson, Danielle D.; Morrison, W. Ivan; Knowles, Donald P.

    2016-01-01

    Respiratory failure and death in East Coast Fever (ECF), a clinical syndrome of African cattle caused by the apicomplexan parasite Theileria parva, has historically been attributed to pulmonary infiltration by infected lymphocytes. However, immunohistochemical staining of tissue from T. parva infected cattle revealed large numbers of CD3- and CD20-negative intralesional mononuclear cells. Due to this finding, we hypothesized that macrophages play an important role in Theileria parva disease pathogenesis. Data presented here demonstrates that terminal ECF in both Holstein and Boran cattle is largely due to multisystemic histiocytic responses and resultant tissue damage. Furthermore, the combination of these histologic changes with the clinical findings, including lymphadenopathy, prolonged pyrexia, multi-lineage leukopenia, and thrombocytopenia is consistent with macrophage activation syndrome. All animals that succumbed to infection exhibited lymphohistiocytic vasculitis of small to medium caliber blood and lymphatic vessels. In pulmonary, lymphoid, splenic and hepatic tissues from Holstein cattle, the majority of intralesional macrophages were positive for CD163, and often expressed large amounts of IL-17. These data define a terminal ECF pathogenesis in which parasite-driven lymphoproliferation leads to secondary systemic macrophage activation syndrome, mononuclear vasculitis, pulmonary edema, respiratory failure and death. The accompanying macrophage phenotype defined by CD163 and IL-17 is presented in the context of this pathogenesis. PMID:27195791

  20. Hereditary leukoencephalopathy with axonal spheroids: a spectrum of phenotypes from CNS vasculitis to parkinsonism in an adult onset leukodystrophy series

    PubMed Central

    Jaunmuktane, Zane; Sheerin, Una-Marie; Phadke, Rahul; Brandner, Sebastian; Milonas, Ionnis; Dean, Andrew; Bajaj, Nin; McNicholas, Nuala; Costello, Daniel; Cronin, Simon; McGuigan, Chris; Rossor, Martin; Fox, Nick; Murphy, Elaine; Chataway, Jeremy; Houlden, Henry

    2016-01-01

    Background Hereditary diffuse leukoencephalopathy with neuroaxonal spheroids (HDLS) is a hereditary, adult onset leukodystrophy which is characterised by the presence of axonal loss, axonal spheroids and variably present pigmented macrophages on pathological examination. It most frequently presents in adulthood with dementia and personality change. HDLS has recently been found to be caused by mutations in the colony stimulating factor-1 receptor (CSF1R) gene. Methods In this study, we sequenced the CSF1R gene in a cohort of 48 patients from the UK, Greece and Ireland with adult onset leukodystrophy of unknown cause. Results Five pathogenic mutations were found, including three novel mutations. The presentations ranged from suspected central nervous system (CNS) vasculitis to extrapyramidal to cognitive phenotypes. The case histories and imaging are presented here, in addition to neuropathological findings from two cases with novel mutations. Conclusion We estimate that CSF1R mutations account for 10% of idiopathic adult onset leukodystrophies and that genetic testing for CSF1R mutations is essential in adult patients presenting with undefined CNS vasculitis or a leukodystrophy with prominent neuropsychiatric signs or dementia. PMID:25935893

  1. Neuro-Behçet disease presenting with acute psychosis in an adolescent.

    PubMed

    Patel, Puja; Steinschneider, Mitchell; Boneparth, Alexis; Lantos, George

    2014-09-01

    Behçet disease is a systemic vasculitis of unknown etiology that can affect the neurologic system. Neuro-Behçet disease is not well defined in children and adolescents, and the diagnosis is difficult to make in this population as they often present with insufficient symptoms to meet diagnostic criteria. Psychiatric symptoms as the initial manifestation of neuro-Behçet disease has rarely been reported. We describe a 17-year-old boy who presented with acute psychosis and was subsequently diagnosed with neuro-Behçet disease. A rare combination of both cerebral venous thrombosis and parenchymal central nervous system involvement was identified by neuroimaging. Although treatment guidelines for neuro-Behçet disease are limited, the patient made demonstrative clinical and radiographic improvement with a combination of corticosteroids, anticoagulation, and immunosuppressants, including a tumor necrosis factor-α (TNFα) blocking agent.

  2. A peptide for targeted, systemic delivery of imaging and therapeutic compounds into acute brain injuries

    PubMed Central

    Mann, Aman P.; Scodeller, Pablo; Hussain, Sazid; Joo, Jinmyoung; Kwon, Ester; Braun, Gary B.; Mölder, Tarmo; She, Zhi-Gang; Kotamraju, Venkata Ramana; Ranscht, Barbara; Krajewski, Stan; Teesalu, Tambet; Bhatia, Sangeeta; Sailor, Michael J.; Ruoslahti, Erkki

    2016-01-01

    Traumatic brain injury (TBI) is a major health and socio-economic problem, but no pharmacological agent is currently approved for the treatment of acute TBI. Thus, there is a great need for advances in this field. Here, we describe a short peptide (sequence CAQK) identified by in vivo phage display screening in mice with acute brain injury. The CAQK peptide selectively binds to injured mouse and human brain, and systemically injected CAQK specifically homes to sites of brain injury in mouse models. The CAQK target is a proteoglycan complex upregulated in brain injuries. Coupling to CAQK increased injury site accumulation of systemically administered molecules ranging from a drug-sized molecule to nanoparticles. CAQK-coated nanoparticles containing silencing oligonucleotides provided the first evidence of gene silencing in injured brain parenchyma by systemically administered siRNA. These findings present an effective targeting strategy for the delivery of therapeutics in clinical management of acute brain injuries. PMID:27351915

  3. A peptide for targeted, systemic delivery of imaging and therapeutic compounds into acute brain injuries

    NASA Astrophysics Data System (ADS)

    Mann, Aman P.; Scodeller, Pablo; Hussain, Sazid; Joo, Jinmyoung; Kwon, Ester; Braun, Gary B.; Mölder, Tarmo; She, Zhi-Gang; Kotamraju, Venkata Ramana; Ranscht, Barbara; Krajewski, Stan; Teesalu, Tambet; Bhatia, Sangeeta; Sailor, Michael J.; Ruoslahti, Erkki

    2016-06-01

    Traumatic brain injury (TBI) is a major health and socio-economic problem, but no pharmacological agent is currently approved for the treatment of acute TBI. Thus, there is a great need for advances in this field. Here, we describe a short peptide (sequence CAQK) identified by in vivo phage display screening in mice with acute brain injury. The CAQK peptide selectively binds to injured mouse and human brain, and systemically injected CAQK specifically homes to sites of brain injury in mouse models. The CAQK target is a proteoglycan complex upregulated in brain injuries. Coupling to CAQK increased injury site accumulation of systemically administered molecules ranging from a drug-sized molecule to nanoparticles. CAQK-coated nanoparticles containing silencing oligonucleotides provided the first evidence of gene silencing in injured brain parenchyma by systemically administered siRNA. These findings present an effective targeting strategy for the delivery of therapeutics in clinical management of acute brain injuries.

  4. Immune Mechanisms in Medium and Large Vessel Vasculitis

    PubMed Central

    Weyand, Cornelia M.; Goronzy, Jörg J.

    2014-01-01

    Summary Vasculitis of the medium and large arteries, most often presenting as giant cell arteritis (GCA), is an infrequent, but potentially fatal type of immune-mediated vascular disease. The site of the aberrant immune reaction, the mural layers of the artery, is strictly defined by vascular dendritic cells, endothelial cells, vascular smooth muscle cells and fibroblasts which engage in an interaction with T cells and macrophages to ultimately cause luminal stenosis or aneurysmal wall damage of the vessel. A multitude of effector cytokines, all known as critical mediators in host-protective immunity, has been identified in the vasculitic lesions. Two dominant cytokine clusters, one centering on the IL-6/IL-17 axis, the other on the IL-12/IFN-γ axis, have been connected with disease activity. These two clusters appear to serve different roles in the vasculitic process. The IL-6/IL-17 cluster is highly responsive to standard corticosteroid therapy, whereas the IL-12/IFN-γ cluster is resistant to steroid-mediated immunosuppression. The information exchange between vascular and immune cells and stabilization of the vasculitic process involves members of the NOTCH receptor and ligand family. Focusing on elements in the tissue context of GCA, instead of broadly suppressing host immunity, may allow for a more tailored therapeutic approach and spare patients the unwanted side-effects of aggressive immunosuppression. PMID:24189842

  5. Pulmonary granulomatous vasculitis induced by insoluble particulates: a case report.

    PubMed

    Hammar, Samuel P; Williams, M Glenn; Dodson, Ronald F

    2003-01-01

    The authors report a case of a 39-year-old woman who sustained an injury to her left knee requiring arthroscopic surgical medial menisectomy and ganglionic block for reflex sympathetic dystrophy syndrome. Approximately 1 year after injury, the patient presented with an elevated white blood cell count and fever and was diagnosed to have a psoas muscle abscess, which was treated with antibiotics. She was also taking 4 different oral medications that contained microcrystalline cellulose as a filter. Approximately 1 month after being diagnosed with the psoas muscle abscess, the patient developed shortness of breath, marked weakness, diaphoresis, and intermittent emesis. She became hypotensive and tachyneic and expired. Postmortem examination showed granulomatous vasculitis with extensive occlusions of pulmonary arteries by birefringent crystalline material identified to be cellulose histochemically and by analytical electron microscopy evaluation. This case report describes the ultrastructural and chemical features of various medicinal tablet fillers and compares them to pure samples. This report also demonstrates the usefulness of analytical electron microscopy in accurately identifying birefringent material in lung tissue.

  6. Alternative approaches for identifying acute systemic toxicity: Moving from research to regulatory testing.

    PubMed

    Hamm, Jon; Sullivan, Kristie; Clippinger, Amy J; Strickland, Judy; Bell, Shannon; Bhhatarai, Barun; Blaauboer, Bas; Casey, Warren; Dorman, David; Forsby, Anna; Garcia-Reyero, Natàlia; Gehen, Sean; Graepel, Rabea; Hotchkiss, Jon; Lowit, Anna; Matheson, Joanna; Reaves, Elissa; Scarano, Louis; Sprankle, Catherine; Tunkel, Jay; Wilson, Dan; Xia, Menghang; Zhu, Hao; Allen, David

    2017-01-06

    Acute systemic toxicity testing provides the basis for hazard labeling and risk management of chemicals. A number of international efforts have been directed at identifying non-animal alternatives for in vivo acute systemic toxicity tests. A September 2015 workshop, Alternative Approaches for Identifying Acute Systemic Toxicity: Moving from Research to Regulatory Testing, reviewed the state-of-the-science of non-animal alternatives for this testing and explored ways to facilitate implementation of alternatives. Workshop attendees included representatives from international regulatory agencies, academia, nongovernmental organizations, and industry. Resources identified as necessary for meaningful progress in implementing alternatives included compiling and making available high-quality reference data, training on use and interpretation of in vitro and in silico approaches, and global harmonization of testing requirements. Attendees particularly noted the need to characterize variability in reference data to evaluate new approaches. They also noted the importance of understanding the mechanisms of acute toxicity, which could be facilitated by the development of adverse outcome pathways. Workshop breakout groups explored different approaches to reducing or replacing animal use for acute toxicity testing, with each group crafting a roadmap and strategy to accomplish near-term progress. The workshop steering committee has organized efforts to implement the recommendations of the workshop participants.

  7. Vascular Access System for Continuous Arterial Infusion of a Protease Inhibitor in Acute Necrotizing Pancreatitis

    SciTech Connect

    Ganaha, Fumikiyo; Yamada, Tetsuhisa; Yorozu, Naoya; Ujita, Masuo; Irie, Takeo; Fukuda, Yasushi; Fukuda, Kunihiko; Tada, Shimpei

    1999-09-15

    We used a vascular access system (VAS) for continuous arterial infusion (CAI) of a protease inhibitor in two patients with acute necrotizing pancreatitis. The infusion catheter was placed into the dorsal pancreatic artery in the first patient and into the gastroduodenal artery in the second, via a femoral artery approach. An implantable port was then connected to the catheter and was secured in a subcutaneous pocket prepared in the right lower abdomen. No complications related to the VAS were encountered. This system provided safe and uncontaminated vascular access for successful CAI for acute pancreatitis.

  8. Alveolar hemorrhage as the initial presentation of systemic lupus erythematosus

    PubMed Central

    de Holanda, Bruna A.; Barreto, Isabela G. Menna; de Araujo, Isadora S. Gomes

    2016-01-01

    Alveolar hemorrhage (AH) is a rare syndrome that can often occur in autoimmune diseases, blood clotting disorders, infection or by acute inhalation injury, presenting rapid evolution and high mortality, especially with late diagnosis and treatment. Among the autoimmune diseases, there are reported cases in patients with primary antiphospholipid syndrome (PAPS), vasculitis and systemic lupus erythematosus (SLE). An early diagnosis is an essential tool in the successful management of this complication, requiring aggressive treatment based on vigorous immunosuppression and broad-spectrum antibiotic. We describe here a case of alveolar hemorrhage associated with glomerulonephritis as the open presentation in a patient with SLE. PMID:27994272

  9. Alveolar hemorrhage as the initial presentation of systemic lupus erythematosus.

    PubMed

    de Holanda, Bruna A; Barreto, Isabela G Menna; de Araujo, Isadora S Gomes; de Araujo, Daniel B

    2016-01-01

    Alveolar hemorrhage (AH) is a rare syndrome that can often occur in autoimmune diseases, blood clotting disorders, infection or by acute inhalation injury, presenting rapid evolution and high mortality, especially with late diagnosis and treatment. Among the autoimmune diseases, there are reported cases in patients with primary antiphospholipid syndrome (PAPS), vasculitis and systemic lupus erythematosus (SLE). An early diagnosis is an essential tool in the successful management of this complication, requiring aggressive treatment based on vigorous immunosuppression and broad-spectrum antibiotic. We describe here a case of alveolar hemorrhage associated with glomerulonephritis as the open presentation in a patient with SLE.

  10. Acute fulminant neuropathy in a patient with Churg-Strauss syndrome.

    PubMed Central

    Ng, K. K.; Yeung, H. M.; Loo, K. T.; Chan, H. M.; Wong, C. K.; Li, P. C.

    1997-01-01

    We report a patient with an acute neuropathy initially mimicking Guillain-Barré syndrome, both clinically and electrophysiologically. Persistent eosinophilia, positive antineutrophil cytoplasmic antibody and eosinophilic vasculitis in sural nerve biopsy later confirmed Churg-Strauss syndrome. Since vasculitic neuropathy can present in an acute and fulminant form, the role of early antibody testing and sural nerve biopsy in atypical cases of acute neuropathy is emphasized. Images Figure PMID:9156128

  11. Evaluation of the importance of astrocytes when screening for acute toxicity in neuronal cell systems.

    PubMed

    Woehrling, E K; Hill, E J; Coleman, M D

    2010-02-01

    Reliable, high throughput, in vitro preliminary screening batteries have the potential to greatly accelerate the rate at which regulatory neurotoxicity data is generated. This study evaluated the importance of astrocytes when predicting acute toxic potential using a neuronal screening battery of pure neuronal (NT2.N) and astrocytic (NT2.A) and integrated neuronal/astrocytic (NT2.N/A) cell systems derived from the human NT2.D1 cell line, using biochemical endpoints (mitochondrial membrane potential (MMP) depolarisation and ATP and GSH depletion). Following exposure for 72 h, the known acute human neurotoxicants trimethyltin-chloride, chloroquine and 6-hydroxydopamine were frequently capable of disrupting biochemical processes in all of the cell systems at non-cytotoxic concentrations. Astrocytes provide key metabolic and protective support to neurons during toxic challenge in vivo and generally the astrocyte containing cell systems showed increased tolerance to toxicant insult compared with the NT2.N mono-culture in vitro. Whilst there was no consistent relationship between MMP, ATP and GSH log IC(50) values for the NT2.N/A and NT2.A cell systems, these data did provide preliminary evidence of modulation of the acute neuronal toxic response by astrocytes. In conclusion, the suitability of NT2 neurons and astrocytes as cell systems for acute toxicity screening deserves further investigation.

  12. Acute Ozone-Induced Pulmonary and Systemic Metabolic ...

    EPA Pesticide Factsheets

    Acute ozone exposure increases circulating stress hormones and induces metabolic alterations in animals and humans. We hypothesized that the increase of adrenal-derived stress hormones is necessary for both ozone-induced metabolic effects and lung injury. Male Wistar-Kyoto rats underwent adrenal demedullation (DEMED), total bilateral adrenalectomy (ADREX), or sham surgery (SHAM). After a 4 day recovery, rats were exposed to air or ozone (1ppm), 4h/day for 1 or 2 days. Circulating adrenaline levels dropped to nearly zero in DEMED and ADREX rats relative to air-exposed SHAM. Corticosterone levels tended to be low in DEMED rats and dropped to nearly zero in ADREX rats. Adrenalectomy in air-exposed rats caused modest changes in metabolites and lung toxicity parameters. Ozone-induced hyperglycemia and glucose intolerance were markedly attenuated in DEMED rats with nearly complete reversal in ADREX rats. Ozone increased circulating epinephrine and corticosterone in SHAM but not in DEMED or ADREX rats. Free fatty acids (p=0.15) and branched-chain amino acids increased after ozone exposure in SHAM but not in DEMED or ADREX rats. Lung minute volume was not affected by surgery or ozone but ozone-induced labored breathing was less pronounced in ADREX rats. Ozone-induced increases in lung protein leakage and neutrophilic inflammation were markedly reduced in DEMED and ADREX rats (ADREX>DMED). Ozone-mediated decreases in circulating white blood cells in SHAM were not obser

  13. [The glutathione system in the blood of rats and morphological changes of the pancreas under experimental acute and chronic pancreatitis].

    PubMed

    Makarchuk, V A; Ushakova, H O; Krylova, O O

    2013-01-01

    In experiment on laboratory rats the models of acute and chronic pancreatitis were developed to study the changes of lipoperoxidation-antioxidant protection system depending on morphological changes of the pancreas. The acute and chronic pancreatitis is accompanied with intensification of lipoperoxidation and gradual inhibition of antioxidant system due to development of subsequent chronization of the pathological process.

  14. Echocardiographic diagnosis of systemic AA amyloidosis presenting with acute liver failure.

    PubMed

    Morelli, Sergio; Bonfiglio, Daniele; Galasso, Laura

    2010-08-01

    We report the case of a 73-year-old man admitted to our hospital for acute hepatic failure. Antemortem diagnosis of systemic AA amyloidosis was made because of typical electrocardiographic and echocardiographic findings, in the absence of the classic clinical picture of kidney involvement.

  15. 76 FR 19365 - Medicare Program; Hospital Inpatient Prospective Payment Systems for Acute Care Hospitals and...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-04-07

    ... Inpatient Prospective Payment Systems for Acute Care Hospitals and Fiscal Year 2011 Final Wage Indices...), HHS. ACTION: Notice. SUMMARY: This notice contains the final fiscal year (FY) 2011 wage indices and... the expiration date for certain geographic reclassifications and special exception wage...

  16. Acute Ozone-Induced Pulmonary and Systemic Metabolic Effects are Diminished in Adrenalectomized Rats

    EPA Science Inventory

    Acute ozone exposure increases circulating stress hormones and induces peripheral metabolic alterations in animals and humans. We hypothesized that the increase of adrenal-derived stress hormones is necessary for ozone-induced systemic metabolic effects and lung injury. Male Wis...

  17. Evaluation of Clinical Alvarado Scoring System and CT Criteria in the Diagnosis of Acute Appendicitis.

    PubMed

    Gunes Tatar, Idil; Yilmaz, Kerim Bora; Sahin, Alpaslan; Aydin, Hasan; Akinci, Melih; Hekimoglu, Baki

    2016-01-01

    Aim. The aim was to evaluate the clinical Alvarado scoring system and computed tomography (CT) criteria for the diagnosis of acute appendicitis. Material and Methods. 117 patients with acute abdominal pain who underwent abdominal CT were enrolled in this retrospective study. Patient demographics, clinical Alvarado scoring, CT images, and pathologic results of the patients were evaluated. Results. 39 of the 53 patients who were operated on had pathologically proven acute appendicitis. CT criteria of appendiceal diameter, presence of periappendiceal inflammation, fluid, appendicolith, and white blood cell (WBC) count were significantly correlated with the inflammation of the appendix. The best cut-off value for appendiceal diameter was 6.5 mm. The correlation between appendiceal diameter and WBC count was 80% (P = 0.01 < 0.05). The correlation between appendiceal diameter and Alvarado score was 78.7% (P = 0.01 < 0.05). Conclusion. Presence of CT criteria of appendiceal diameter above 6.5 mm, periappendiceal inflammation, fluid, and appendicolith should prompt the diagnosis of acute appendicitis. Since patients with acute appendicitis may not always show the typical signs and symptoms, CT is a helpful imaging modality for patients with relatively low Alvarado score and leukocytosis and when physical examination is confusing.

  18. Progress towards a core set of outcome measures in small-vessel vasculitis. Report from OMERACT 9.

    PubMed

    Merkel, Peter A; Herlyn, Karen; Mahr, Alfred D; Neogi, Tuhina; Seo, Philip; Walsh, Michael; Boers, Maarten; Luqmani, Raashid

    2009-10-01

    The past decade has seen a substantial increase in the number and quality of clinical trials of new therapies for vasculitis, including randomized, controlled, multicenter trials that have successfully incorporated measures of disease activity and toxicity. However, because current treatment regimens for severe disease effectively induce initial remission and reduce mortality, future trials will focus on any of several goals including: (a) treatment of mild-moderate disease; (b) prevention of chronic damage; (c) reduction in treatment toxicity; or (d) more subtle differences in remission induction or maintenance. Thus, new trials will require outcome measure instruments that are more precise and are better able to detect effective treatments for different disease states and measure chronic manifestations of disease. The OMERACT Vasculitis Working Group comprises international clinical investigators with expertise in vasculitis who, since 2002, have worked collaboratively to advance the refinement of outcome measures in vasculitis, create new measures to address domains of illness not covered by current research approaches, and harmonize outcome assessment in vasculitis. The focus of the OMERACT group to date has been on outcome measures in small-vessel vasculitis with an overall goal of creating a core set of outcome measures for vasculitis, each of which fulfills the OMERACT filter of truth, discrimination, feasibility, and identifying additional domains requiring further research. This process has been informed by several ongoing projects providing data on outcomes of disease activity, disease-related damage, multidimensional health-related quality of life, and patient-reported ratings of the burden of vasculitis.

  19. Cocaine-induced vasculitis with cutaneous manifestation: A recurrent episode after 2 years

    PubMed Central

    Swe, Thein; Pervil-Ulysse, Mona; Baqui, AAM A.

    2016-01-01

    Cocaine is a popular recreational drug in the United States, and up to 70% of the seized cocaine contains levamisole which is an antihelminthic that can cause cutaneous vasculitis with necrosis and positive antineutrophil cytoplasmic antibodies (ANCAs). Here, we report a unique case of recurrent cocaine-induced vasculitis in a patient who smokes cocaine for more than 20 years. A 38-year-old woman complained of painful erythematous rash in her right arm and right thigh which appeared some hours after smoking cocaine. Physical examination revealed tender, erythematous base, retiform purpura with necrosis and bullae. Serological test showed high atypical perinuclear ANCA titer of 1:320 and antimyeloperoxidase antibody level of 20.4 U/mL. Cocaine-induced vasculitis should be one of the differential diagnoses in cocaine abusers who present with painful rash and areas of necrosis. Early diagnosis is important since it is an emerging public health concern. PMID:28217616

  20. Leukocytoclastic Vasculitis Secondary to Pyridostigmine (Mestinon): Report of a Possible First Case

    PubMed Central

    Singh, Gunveen; Hodgson, Tim; Clarke, David E

    2017-01-01

    Introduction Pyridostigmine is an acetylcholinesterase inhibitor commonly used in the treatment of myasthenia gravis. We describe a patient who developed a rash after recently being started on pyridostigmine and give a general review of leukocytoclastic vasculitis. Case Presentation A 91-year-old man was diagnosed with ocular myasthenia gravis. He was started on pyridostigmine, and 2 weeks later he developed a rash. The rash was biopsied and found to be secondary to leukocytoclastic vasculitis; the pyridostigmine was stopped, loratadine was started, and the rash resolved. Discussion Leukocytoclastic vasculitis is commonly caused by a hypersensitivity reaction to medications, or it can be associated with certain medical conditions. We present a brief review of the most common medications and medical conditions known to cause this reaction, but to our knowledge this is the first description of pyridostigmine causing this reaction. PMID:28080955

  1. Systems biomarkers as acute diagnostics and chronic monitoring tools for traumatic brain injury

    NASA Astrophysics Data System (ADS)

    Wang, Kevin K. W.; Moghieb, Ahmed; Yang, Zhihui; Zhang, Zhiqun

    2013-05-01

    Traumatic brain injury (TBI) is a significant biomedical problem among military personnel and civilians. There exists an urgent need to develop and refine biological measures of acute brain injury and chronic recovery after brain injury. Such measures "biomarkers" can assist clinicians in helping to define and refine the recovery process and developing treatment paradigms for the acutely injured to reduce secondary injury processes. Recent biomarker studies in the acute phase of TBI have highlighted the importance and feasibilities of identifying clinically useful biomarkers. However, much less is known about the subacute and chronic phases of TBI. We propose here that for a complex biological problem such as TBI, multiple biomarker types might be needed to harness the wide range of pathological and systemic perturbations following injuries, including acute neuronal death, neuroinflammation, neurodegeneration and neuroregeneration to systemic responses. In terms of biomarker types, they range from brain-specific proteins, microRNA, genetic polymorphism, inflammatory cytokines and autoimmune markers and neuro-endocrine hormones. Furthermore, systems biology-driven biomarkers integration can help present a holistic approach to understanding scenarios and complexity pathways involved in brain injury.

  2. Central nervous system granulomastous phlebitis with limited extracranial involvement of the heart and lungs: An autopsy case.

    PubMed

    Mlakar, Jernej; Zorman, Jerneja Videčnik; Matičič, Mojca; Vrabec, Matej; Alibegović, Armin; Popović, Mara

    2016-02-01

    Primary angiitis of the central nervous system is a rare condition, usually with an insidious onset. There is a wide variety of histological types (granulomatous, lymphocytic or necrotizing vasculitis) and types of vessel involved (arteries, veins or both). Most cases are idiopathic. We describe a first case of idiopathic granulomatous central nervous system phlebitis with additional limited involvement of the heart and lung, exclusively affecting small and medium sized veins in a 22-year-old woman, presenting as a sub acute headache. The reasons for this peculiar limitation of inflammation to the veins and the involvement of the heart and lungs are unknown.

  3. Non lineal respiratory systems mechanics simulation of acute respiratory distress syndrome during mechanical ventilation.

    PubMed

    Madorno, Matias; Rodriguez, Pablo O

    2010-01-01

    Model and simulation of biological systems help to better understand these systems. In ICUs patients often reach a complex situation where supportive maneuvers require special expertise. Among them, mechanical ventilation in patients suffering from acuter respiratory distress syndrome (ARDS) is specially challenging. This work presents a model which can be simulated and use to help in training of physicians and respiratory therapists to analyze the respiratory mechanics in this kind of patients. We validated the model in 2 ARDS patients.

  4. Alpha-1 Antitrypsin Deficiency Presenting with MPO-ANCA Associated Vasculitis and Aortic Dissection

    PubMed Central

    van Schaik, Jan; Crobach, Stijn L. P.; van Rijswijk, Catharina S. P.; Rotmans, Joris I.

    2017-01-01

    The combination of alpha-1 antitrypsin (AAT) deficiency, ANCA-vasculitis, and aortic aneurysm has been rarely described in literature. We report an eventually fatal case in a 70-year-old patient who initially presented with giant cell arteritis and ANCA associated glomerulonephritis. Several years later, he presented with aortic dissection due to large vessel vasculitis, raising the suspicion of AAT deficiency, as two first-line relatives had chronic obstructive pulmonary disease, while they never smoked. This diagnosis was confirmed by AAT electrophoresis and immunohistochemistry on a temporal artery biopsy. Considering AAT deficiency in these cases might lead to a more timely diagnosis. PMID:28367219

  5. Large Vessel Vasculitis with an Isolated Lesion of a Single-lobe Pulmonary Artery.

    PubMed

    Kitajima, Takamasa; Marumo, Satoshi; Shoji, Tsuyoshi; Huang, Cheng-Long; Yuba, Yoshiaki; Fukui, Motonari

    2016-01-01

    Chronic pulmonary arterial obstructions are caused mostly by chronic pulmonary artery thromboembolism and rarely by vasculitis or intimal sarcoma of the pulmonary artery. We herein report an unusual case of a 42-year-old woman with a solitary obstruction of the pulmonary artery in the right lower lobe of her lung. Because we could not exclude the possibility of intimal sarcoma, middle and lower lobectomy was performed. The resected specimens revealed large vessel vasculitis (LVV) and an isolated lesion in the right lower lobe pulmonary artery. LVV should therefore be considered in the differential diagnosis for single pulmonary arterial stenosis or obstruction.

  6. Interferon-gamma-induced local leukocytoclastic vasculitis at the subcutaneous injection site*

    PubMed Central

    Wang, Fang; Liu, Juan-Hua; Zhao, Yu-Kun; Luo, Di-Qing

    2016-01-01

    Cutaneous reactions associated with interferons (IFNs) treatment are either localized or generalized. The most common presentation of localized reactions at IFNs injection site is usually an erythematous patch or plaque. Local leukocytoclastic vasculitis presenting with cutaneous necrosis is extremely rare. We report a 19-year-old man with hepatitis B who had local leukocytoclastic vasculitis induced by interferon-gama injection at the injection site. After changing the injection sites and using the combined treatment of prednisone and colchicine, the previous lesion healed and no other cutaneous lesion occurred. We also made a mini review of such cases.

  7. Avoiding "Rash" Conclusions: Challenge of IgA Vasculitis in Adults.

    PubMed

    Hu, S; Birg, A; Hovaida, M; Gavin, M W; McCarthy, D

    2017-04-01

    IgA vasculitis is primarily a pediatric disease that is rarely encountered in adults. With adults, gastrointestinal manifestations are quite common, yet are nonspecific and may overlap with other diseases, particularly Crohn's disease, which can make the diagnosis a challenging task. Treatment is controversial given the disease course is usually self-limited with few serious complications. We present a case of IgA vasculitis in an adult patient with limited extraintestinal findings illustrating the complexity of arriving at the correct diagnosis.

  8. Role of the innate immune system in acute viral myocarditis.

    PubMed

    Huang, Chien-Hua; Vallejo, Jesus G; Kollias, George; Mann, Douglas L

    2009-05-01

    Although the adaptive immune system is thought to play an important role in the pathogenesis of viral myocarditis, the role of the innate immune system has not been well defined. To address this deficiency, we employed a unique line of mice that harbor a genomic "knock in" of a mutated TNF gene lacking the AU rich element (TNF(ARE/ARE)) that is critical for TNF mRNA stability and translation, in order to examine the contribution of the innate immune system in encephalomyocarditis-induced myocarditis (EMCV). Heterozygous mice (TNF(ARE/+)) were infected with 500 plaque-forming units of EMCV. TNF(ARE/+)mice had a significantly higher 14-day mortality and myocardial inflammation when compared to littermate control mice. Virologic studies showed that the viral load at 14 days was significantly lower in the hearts of TNF(ARE/+) mice. TNF(ARE/+) mice had an exaggerated proinflammatory cytokine and chemokine response in the heart following EMCV infection. Modulation of the innate immune response in TNF(ARE/+) mice by the late administration of prednisolone resulted in a significant improvement in survival and decreased cardiac inflammation, whereas early administration of prednisolone resulted in a blunted innate response and increased mortality in littermate control mice. Viewed together, these data suggest that the duration and degree of activation of the innate immune system plays a critical role in determining host outcomes in experimental viral myocarditis.

  9. The Innate Immune System in Acute and Chronic Wounds

    PubMed Central

    MacLeod, Amanda S.; Mansbridge, Jonathan N.

    2016-01-01

    Significance: This review article provides an overview of the critical roles of the innate immune system to wound healing. It explores aspects of dysregulation of individual innate immune elements known to compromise wound repair and promote nonhealing wounds. Understanding the key mechanisms whereby wound healing fails will provide seed concepts for the development of new therapeutic approaches. Recent Advances: Our understanding of the complex interactions of the innate immune system in wound healing has significantly improved, particularly in our understanding of the role of antimicrobials and peptides and the nature of the switch from inflammatory to reparative processes. This takes place against an emerging understanding of the relationship between human cells and commensal bacteria in the skin. Critical Issues: It is well established and accepted that early local inflammatory mediators in the wound bed function as an immunological vehicle to facilitate immune cell infiltration and microbial clearance upon injury to the skin barrier. Both impaired and excessive innate immune responses can promote nonhealing wounds. It appears that the switch from the inflammatory to the proliferative phase is tightly regulated and mediated, at least in part, by a change in macrophages. Defining the factors that initiate the switch in such macrophage phenotypes and functions is the subject of multiple investigations. Future Directions: The review highlights processes that may be useful targets for further investigation, particularly the switch from M1 to M2 macrophages that appears to be critical as dysregulation of this switch occurs during defective wound healing. PMID:26862464

  10. Functional biomarkers for the acute effects of alcohol on the central nervous system in healthy volunteers

    PubMed Central

    Zoethout, Remco W M; Delgado, Wilson L; Ippel, Annelies E; Dahan, Albert; van Gerven, Joop M A

    2011-01-01

    The central nervous system (CNS) effects of acute alcohol administration have been frequently assessed. Such studies often use a wide range of methods to study each of these effects. Unfortunately, the sensitivity of these tests has not completely been ascertained. A literature search was performed to recognize the most useful tests (or biomarkers) for identifying the acute CNS effects of alcohol in healthy volunteers. All tests were grouped in clusters and functional domains. Afterwards, the effect of alcohol administration on these tests was scored as improvement, impairment or as no effect. Furthermore, dose–response relationships were established. A total number of 218 studies, describing 342 different tests (or test variants) were evaluated. Alcohol affected a wide range of CNS domains. Divided attention, focused attention, visuo-motor control and scales of feeling high and of subjective drug effects were identified as the most sensitive functional biomarkers for the acute CNS effects of alcohol. The large number of CNS tests that are used to determine the effects of alcohol interferes with the identification of the most sensitive ones and of drug–response relationships. Our results may be helpful in selecting rational biomarkers for studies investigating the acute CNS effects of alcohol or for future alcohol- interaction studies. PMID:21284693

  11. [Autochthonous acute viral and bacterial infections of the central nervous system (meningitis and encephalitis)].

    PubMed

    Pérez-Ruiz, Mercedes; Vicente, Diego; Navarro-Marí, José María

    2008-07-01

    Rapid diagnosis of acute viral and bacterial infections of the central nervous system (meningitis and encephalitis) is highly important for the clinical management of the patient and helps to establish early therapy that may solve life-threatening situations, to avoid unnecessary empirical treatments, to reduce hospital stay, and to facilitate appropriate interventions in the context of public health. Molecular techniques, especially real-time polymerase chain reaction, have become the fastest and most sensitive diagnostic procedures for autochthonous viral meningitis and encephalitis, and their role is becoming increasingly important for the diagnosis and control of most frequent acute bacterial meningitides. Automatic and closed systems may encourage the widespread and systematic use of molecular techniques for the diagnosis of these neurological syndromes in most laboratories.

  12. [Acute adverse effects in transfusion. Proposals for the hemosurveillance system].

    PubMed

    Baptista González, Héctor

    2013-01-01

    The management model based on risk prevention has become a major influence in shaping policies for transfusion safety. There are approximately sixty interactions between the health worker and the patient during the transfusion process,representing the number of times where you have the opportunity to make a mistake.We present an analysis of the weaknesses of the National Blood System, with particular attention to the haemovigilance donor and patient. The proposals include the implementation of the National Blood containing the need to establish from the National Blood Safety, significant changes in the regulatory framework and the internal regulations of the Ministry of Health, the CNTS and COFEPRIS. Is required to promote and coordinate the collection of accurate information from the committees of transfusion medicine, which will be accompanied by an initial diagnosis from the National Survey of Blood. Requires notice to other forms of funding to ensure the viability of the projects operating blood bank. Finally, as a strategic resource, the blood is of public, so access should not be restricted.

  13. Aerococcus viridans infection presenting as cutaneous vasculitis in an immunocompetent patient

    PubMed Central

    Sofi, Fayaz; Ahmad, Mushtaq; Kuchay, Abid

    2016-01-01

    Background Aerococcus viridans organisms are Gram-positive cocci that are widely distributed in hospital environments and room air. These bacteria have infrequently been encountered as human pathogens causing bacteremia, endocarditis and urinary tract infections. The significance of these bacteria may be overlooked due to their fastidious growth, and they are often confused with other strains of streptococci or staphylococci. Case report We present a case of Aerococcus viridans manifesting as cutaneous vasculitis in an immunocompetent patient. A 30-year-old female patient was admitted to hospital after two weeks history of fever, chills and papular rash over the limbs and trunk. The clinical diagnosis of vasculitis was made. Investigations revealed elevated leucocytosis (21.7 × 109/l) with 81% of neutrophils, and an elevated erythrocyte sedimentation rate or 60 mm/h. Serum anti-neutrophil cytoplasmic antibodies (ANCAs) were not found. Blood culture showed growth of Aerococcus viridans. Histopathological assessment of skin biopsy revealed cutaneous vasculitis. Conclusions To date, no clinical case report of this kind has been reported implicating Aerococcus viridans in cutaneous vasculitis. Increased awareness and more studies of this genus should lead to the identification of its potential role in human infections. PMID:28115783

  14. Rituximab in induction therapy for anti-neutrophil cytoplasmic antibody (ANCA) vasculitis.

    PubMed

    Niles, J

    2011-05-01

    Anti-neutrophil cytoplasmic antibodies (ANCA) have been associated with a spectrum of vasculitis that includes granulomatous polyangiitis (formerly known as Wegener's granulomatosis), microscopic polyangiitis, the Churg-Strauss syndrome, primary pauciimmune necrotizing and crescentic glomerulonephritis and related forms of vasculitis. In vitro, in vivo and clinical evidence support the conclusion that ANCA participate in the pathophysiology of this disease spectrum. Rituximab is a potent tool that can interrupt B cell-mediated immunity without major compromise of T cell-mediated immunity. Thus, it has great appeal as a tool to interrupt antibody-mediated autoimmune disease. The results of two prospective randomized trials confirm that rituximab can be effective as part of induction therapy for active ANCA-associated vasculitis. The safety profile for rituximab appears favourable relative to cyclophosphamide and steroids. However, there remain many patients who require individualized adjustments of ancillary therapy, as breakthrough disease, relapses and infectious complications do occur. Based on our current knowledge, rituximab should now be incorporated as part of induction therapy in many patients with ANCA-associated vasculitis. However, more work is needed to determine how rituximab may best be integrated into the overall immunosuppression of these patients.

  15. Bilateral renal artery occlusion in a patient with primary antiphospholipid antibody syndrome: thrombosis, vasculitis or both?

    PubMed

    Ames, P R; Cianciaruso, B; Bellizzi, V; Balletta, M; Lubrano, E; Scarpa, R; Brancaccio, V

    1992-11-01

    A 43-year-old man presented with oliguria and hypertension. Renal arteriography showed bilateral renal artery occlusion. Circulating antiphospholipid antibodies were found together with a change in natural anticoagulant plasma levels. Immunofluorescence of examined vessels showed immune complex vasculitis. We discuss the pathogenetic mechanism leading to the development of this rare occlusive event.

  16. Epitope specificity determines pathogenicity and detectability in ANCA-associated vasculitis

    EPA Science Inventory

    ABSTRACT BACKGROUND Anti-neutrophil cytoplasmic autoantibodies (ANCA) specific for myeloperoxidase (MPO) or proteinase 3 (PR3) are detectable in >90% of patients with ANCA-associated vasculitis (AAV). ANCA titers do not correlate well with disease activity. In vivo and in vi...

  17. Thrombosis associated with cytomegalovirus infection in patients with ANCA-positive vasculitis.

    PubMed

    Wolf, G; Porth, J; Stahl, R A

    2001-11-01

    Three cases of venous thrombosis with pulmonary embolism in two patients associated with underlying antineutrophil cytoplasmic autoantibody (ANCA)-positive vasculitis and reactivated cytomegalovirus (CMV) infection are described. In vitro studies previously have shown that infection of endothelium with CMV increases the release of procoagulant factors and stimulates the expression of adhesion molecules. Because the endothelial cell plays a pivotal role in maintaining the equilibrium between procoagulant and anticoagulant states, injury by ANCA-positive vasculitis and additional infection with CMV may ignite a local thrombosis easily. Although venous thrombosis is uncommon in CMV infection (eg, in the immunosuppressed state after organ transplantation), the combination of vasculitis and reactivated CMV infection may have contributed to injury of the vessel wall with subsequent development of thrombosis. A better awareness of this association could improve morbidity and may lead to prevention of potentially life-threatening pulmonary embolism. Patients with ANCA-positive vasculitis and CMV infection may profit from prophylactic anticoagulant therapy with heparin or low-molecular-weight heparin.

  18. Life-threatening ANCA-positive vasculitis associated with rickettsial infection.

    PubMed

    Nickerson, Ashley; Marik, Paul Ellis

    2012-05-30

    We present a 47-year-old Caucasian fire fighter who developed multisystem organ failure in the setting of a positive antineutrophil cytoplasmic autoantibody (myeloperoxidase) as well as confirmed Rocky Mountain spotted fever by skin biopsy PCR. This case provided a diagnostic challenge, a rare association of a Rickettsia infection and autoimmune vasculitis as well as a unique management approach.

  19. Acute Radiation Syndrome Severity Score System in Mouse Total-Body Irradiation Model.

    PubMed

    Ossetrova, Natalia I; Ney, Patrick H; Condliffe, Donald P; Krasnopolsky, Katya; Hieber, Kevin P

    2016-08-01

    Radiation accidents or terrorist attacks can result in serious consequences for the civilian population and for military personnel responding to such emergencies. The early medical management situation requires quantitative indications for early initiation of cytokine therapy in individuals exposed to life-threatening radiation doses and effective triage tools for first responders in mass-casualty radiological incidents. Previously established animal (Mus musculus, Macaca mulatta) total-body irradiation (γ-exposure) models have evaluated a panel of radiation-responsive proteins that, together with peripheral blood cell counts, create a multiparametic dose-predictive algorithm with a threshold for detection of ~1 Gy from 1 to 7 d after exposure as well as demonstrate the acute radiation syndrome severity score systems created similar to the Medical Treatment Protocols for Radiation Accident Victims developed by Fliedner and colleagues. The authors present a further demonstration of the acute radiation sickness severity score system in a mouse (CD2F1, males) TBI model (1-14 Gy, Co γ-rays at 0.6 Gy min) based on multiple biodosimetric endpoints. This includes the acute radiation sickness severity Observational Grading System, survival rate, weight changes, temperature, peripheral blood cell counts and radiation-responsive protein expression profile: Flt-3 ligand, interleukin 6, granulocyte-colony stimulating factor, thrombopoietin, erythropoietin, and serum amyloid A. Results show that use of the multiple-parameter severity score system facilitates identification of animals requiring enhanced monitoring after irradiation and that proteomics are a complementary approach to conventional biodosimetry for early assessment of radiation exposure, enhancing accuracy and discrimination index for acute radiation sickness response categories and early prediction of outcome.

  20. Double-button Fixation System for Management of Acute Acromioclavicular Joint Dislocation

    PubMed Central

    Torkaman, Ali; Bagherifard, Abolfazl; Mokhatri, Tahmineh; Haghighi, Mohammad Hossein Shabanpour; Monshizadeh, Siamak; Taraz, Hamid; Hasanvand, Amin

    2016-01-01

    Background: Surgical treatments for acromioclavicular (AC) joint dislocation present with some complications. The present study was designed to evaluate the double-button fixation system in the management of acute acromioclavicular joint dislocation. Methods: This cross sectional study, done between February 2011 to June 2014, consisted of 28 patients who underwent surgical management by the double-button fixation system for acute AC joint dislocation. Age, sex, injury mechanism, dominant hand, side with injury, length of follow up, time before surgery, shoulder and hand (DASH), constant and visual analogue scale (VAS) scores, and all complications of the cases during the follow up were recorded. Results: The mean age of patients was 33.23±6.7 years. Twenty four patients (85.71%) were male and four (14.28%) were female. The significant differences were observed between pre-operation VAS, constant shoulder scores and post-operation measurements. There were not any significant differences between right and left coracoclavicular, but two cases of heterotrophic ossifications were recorded. The mean follow-up time was 16.17±4.38 months. Conclusion: According to the results, the double-button fixation system for management of acute acromioclavicular joint dislocation has suitable results and minimal damage to the soft tissues surrounding the coracoclavicular ligaments. PMID:26894217

  1. Bacillus cereus bacteremia in an adult with acute leukemia.

    PubMed

    Funada, H; Uotani, C; Machi, T; Matsuda, T; Nonomura, A

    1988-03-01

    Bacillus cereus, which used to be considered non-pathogenic, was isolated from the blood of a patient with acute leukemia who was receiving intensive chemotherapy. Fatal bacteremia developed with a clinical syndrome of acute gastroenteritis, followed by both meningoencephalitis with subarachnoid hemorrhage and multiple liver abscesses probably caused by infective vasculitis. Surveillance stool cultures revealed colonization with the organism prior to the onset of diarrhea, and repetitive blood cultures were found to be positive. Thus, this case suggested some new important clinicopathologic features of true B. cereus bacteremia complicating acute leukemia.

  2. [Validation of a diagnostic scoring system (Ohmann score) in acute appendicitis].

    PubMed

    Zielke, A; Sitter, H; Rampp, T A; Schäfer, E; Hasse, C; Lorenz, W; Rothmund, M

    1999-07-01

    A diagnostic scoring system, recently published by Ohmann et al. in this journal, was validated by analyzing the clinicopathological data of a consecutive series of 2,359 patients, admitted for suspicion of acute appendicitis. The results of the scoring system were compared to the results of clinical evaluation by junior (provisional) and senior surgeons (final clinical diagnosis). To assess the diagnostic ability of the score, the accuracy and positive predictive value were defined as the major diagnostic performance parameters; the rate of theoretical negative laparotomies and that of diagnostic errors served as the major procedural performance parameters. Of 2,359 patients admitted for suspected acute appendicitis, 662 were proven to have acute appendicitis by histology, for a prevalence of 28%. The overall sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of the provisional clinical diagnosis were 0.50, 0.94, 0.77, 0.83, and 0.82; 0.93, for the score 0.63, 0.93, 0.77, 0.86 and 0.84, and for the final clinical diagnosis 0.90, 0.94, 0.85, 0.96, and 0.93, respectively. Of the main diagnostic performance parameter, the accuracy of the score was significantly better than that of provisional clinical diagnosis (P < 0.05, chi 2 test). The score yielded a rate of negative appendecomies and laparotomies of 14.3 and 12.3%. With respect to the rate of overlooked cases of acute apendicitis, the score demonstrated a superior performance, with only 6 cases missed (0.9%). However, the number of patients with acute appendicitis, including those with perforated disease, who were not identified by the score, was almost four times that of the final clinical diagnosis (245 vs 63). With regard to the main procedural performance parameter, the score resulted in a significantly smaller number of diagnostic errors than the provisional clinical investigator (P < 0.05, chi 2 test). The results of this study indicate that the diagnostic scoring

  3. Systemic Lupus Erythematosus, Radiotherapy, and the Risk of Acute and Chronic Toxicity: The Mayo Clinic Experience

    SciTech Connect

    Pinn, Melva E.; Gold, Douglas G. M.; Petersen, Ivy A.; Osborn, Thomas G.; Brown, Paul D.; Miller, Robert C.

    2008-06-01

    Purpose: To determine the acute and chronic toxic effects of radiotherapy in patients with systemic lupus erythematosus (SLE). Methods and Materials: Medical records of 21 consecutive patients with SLE, who had received 34 courses of external beam radiotherapy and one low-dose-rate prostate implant, were retrospectively reviewed. Patients with discoid lupus erythematosus were excluded. Results: Median survival was 2.3 years and median follow-up 5.6 years. Eight (42%) of 19 patients evaluable for acute toxicity during radiotherapy experienced acute toxicity of Grade 1 or greater, and 4 (21%) had acute toxicity of Grade 3 or greater. The 5- and 10-year incidence of chronic toxicity of Grade 1 or greater was 45% (95% confidence interval [CI], 22-72%) and 56% (95% CI, 28-81%), respectively. The 5- and 10-year incidence of chronic toxicity of Grade 3 or greater was 28% (95% CI, 18-60%) and 40% (95% CI, 16-72%), respectively. Univariate analysis showed that chronic toxicity of Grade 1 or greater correlated with SLE renal involvement (p < 0.006) and possibly with the presence of five or more American Rheumatism Association criteria (p < 0.053). Chronic toxicity of Grade 3 or greater correlated with an absence of photosensitivity (p < 0.02), absence of arthritis (p < 0.03), and presence of a malar rash (p < 0.04). Conclusions: The risk of acute and chronic toxicity in patients with SLE who received radiotherapy was moderate but was not prohibitive of the use of radiotherapy. Patients with more advanced SLE may be at increased risk for chronic toxicity.

  4. [Effects of Tanshinone IIa on cytokines and platelets in immune vasculitis and its mechanism].

    PubMed

    Li, Xiao-Jing; Zhou, Min; Li, Xiao-Hui; Xu, You-Hua; Liu, Hong; Yang, Mo

    2009-02-01

    The objective of this study was to investigate the effect of Tanshinone IIa on IL-1beta, IL-6 and TNF-alpha cytokines in immune vasculitis and platelets, as well as their relationship. The model of immune vasculitis of rabbits were established by intravenous injection of bovine serum albumin twice. Experiment was divided into 4 groups: control group, model group, tanshinone IIa-treated group and aspirin-treated group. The platelet count, platelet aggregation of peripheral blood were determined. The levels of serum IL-1beta, IL-6, TNF-alpha were detected by ELISA. The pathological changes of immune vasculitis were analyzed by hematoxylin & eosin staining, elastic fibers staining and electron microscopy. The results showed that the levels of IL-1beta and TNF-alpha in model group were significantly higher than those in normal group (p < 0.05), while the level of IL-6 was not significantly different between various groups. The serum level of IL-1beta was correlated with platelet number, while serum levels IL-1beta and TNF-alpha were both correlated with the platelet aggregation. The treatment with tanshinone IIa could significantly decrease the serum levels of IL-1beta, TNF-alpha and platelet number, and the efficacy of tanshinone IIa was same as aspirin. The tanshinone IIa and aspirin both could alleviate the vessels damage in patients with immune vasculitis. It is concluded that the tanshinone IIa may diminish the inflammation damage of vessels in patients with immune vasculitis through the inhibition of cytokines and platelets.

  5. Plasma exchange for renal vasculitis and idiopathic rapidly progressive glomerulonephritis: a meta-analysis

    PubMed Central

    Walsh, Michael; Catapano, Fausta; Szpirt, Wladimir; Thorlund, Kristian; Bruchfeld, Annette; Guillevin, Loic; Haubitz, Marion; Merkel, Peter A.; Peh, Chen Au; Pusey, Charles; Jayne, David

    2011-01-01

    Background Plasma exchange may be effective adjunctive treatment for renal vasculitis. We performed a systematic review and meta-analysis of randomized control trials of plasma exchange for renal vasculitis. Study Design Systematic review and meta-analysis of manuscripts identified from electronic databases, bibliographies, and studies identified by experts. Data was abstracted in parallel by two reviewers. Setting & Population Adults with idiopathic renal vasculitis or rapidly progressive glomerulonephritis Selection Criteria for Studies Randomized controlled trials that compared standard care with standard care plus adjuvant plasma exchange in adult patients with either renal vasculitis or idiopathic rapidly progressive glomerulonephritis. Intervention Adjuvant plasma exchange Outcome Composite of end-stage renal disease or death. Results We identified 9 trials including 387 patients. In a fixed-effects model the pooled relative risk of end-stage renal disease or death was 0.80 for patients treated with adjunctive plasma exchange compared to standard care alone (95% confidence interval 0.65 to 0.99; p=0.04). No significant heterogeneity was detected (p=0.5; I2=0%). The effect of plasma exchange did not differ significantly across the range of baseline serum creatinine values (p=0.7) or number of plasma exchange treatments (p=0.8). The relative risk for end-stage renal disease was 0.64 (95% confidence interval 0.47 to 0.88; p=0.006) while the relative risk for death alone was 1.01 (95% confidence interval 0.71 to 1.4; p=0.9). Limitations Although the primary result was statistically significant, there is insufficient statistical information to reliable determine if plasma exchange reduces the composite of end-stage renal disease or death. Conclusions Plasma exchange may reduce the composite endpoint of end-stage renal disease or death in renal vasculitis. Further trials are required given the limited data available. PMID:21194817

  6. Detection of Anti-Pentraxin-3 Autoantibodies in ANCA-Associated Vasculitis

    PubMed Central

    Guilpain, Philippe; Jeannin, Pascale; Pignon, Pascale; Blanchard, Simon; Garo, Erwan; Jaillon, Sébastien; Chevailler, Alain; Renier, Gilles; Puéchal, Xavier; Bottazzi, Barbara; Mantovani, Alberto; Delneste, Yves; Augusto, Jean-François

    2016-01-01

    Objectives Pentraxin 3 (PTX3), in common with myeloperoxidase and proteinase 3, is stored in human neutrophil granules and is expressed on apoptotic neutrophil surface. We therefore investigated the presence of anti-PTX3 autoantibodies (aAbs) in the sera of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) patients. Methods Presence of anti-PTX3 autoantibodies was analysed by a specific enzyme-linked immunosorbent assay in sera from 150 patients with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (EGPA), and in sera of 227 healthy subjects (HS), 40 systemic sclerosis (SSc) patients, and 25 giant cell arteritis patients (GCA). Using indirect immunofluorescence on fixed human neutrophils, we also analyzed the staining pattern associated with the presence of anti-PTX3 aAbs. Results Anti-PTX3 aAbs were detected in 56 of 150 (37.3%) of the AAV patients (versus 12 of 227 (5.3%) of HS, p<0.001) and, interestingly, in 7 of 14 MPO and PR3 ANCA negative AAV patients. Moreover, by indirect immunofluorescence on fixed neutrophils, anti-PTX3 aAbs gave rise to a specific cytoplasmic fluorescence pattern distinct from the classical cytoplasmic (c-ANCA), perinuclear (p-ANCA), and atypical (a-ANCA) pattern. Anti-PTX3 aAbs levels were higher in patients with active AAV as compared to patients with inactive disease. Conclusion Our work suggests that PTX3 is as a novel ANCA antigen. Anti-PTX3 aAbs appear thus as a promising novel biomarker in the diagnosis of AAV, including in patients without detectable MPO and PR3 ANCA. PMID:26797217

  7. [Role of genetic polymorphisms of the hemostatic system in the development of multiple organ dysfunctions in acute disseminated intravascular coagulation].

    PubMed

    Vorob'eva, N A; Kapustin, S I

    2007-01-01

    The paper deals with the hereditary predisposition of the hemostatic system to the severe course of acute disseminated intravascular coagulation (DIC). The results of evaluation of some types of genetic polymorphisms of the factors of coagulation and of the fibrinolytic system are recent and important for clinical application. Analysis of mortality in the fulminant course of acute DIC showed significant differences in the detection rates of the variants of genetic polymorphism in the type I plasminogen activator inhibitor gene, which is indicative of the possible genetic determination of acute DIC.

  8. Acute Hemorrhagic Edema of Infancy.

    PubMed

    Serra E Moura Garcia, C; Sokolova, A; Torre, M L; Amaro, C

    2016-01-01

    Acute Hemorrhagic Edema of Infancy is a small vessel leucocytoclastic vasculitis affecting young infants. It is characterized by large, target-like, macular to purpuric plaques predominantly affecting the face, ear lobes and extremities. Non-pitting edema of the distal extremities and low-grade fever may also be present. Extra-cutaneous involvement is very rare. Although the lesions have a dramatic onset in a twenty-four to forty-eight hour period, usually the child has a non-toxic appearance. In most cases there are no changes in laboratory parameters. The cutaneous biopsy reveals an inflammatory perivascular infiltrate. It is a benign and auto-limited disease, with complete resolution within two to three weeks leaving no sequelae in the majority of cases. No recurrences are described. We report a case of a 42-day old girl admitted at our hospital with Acute Hemorrhagic Edema of Infancy.

  9. An innovative, multidisciplinary, process-driven approach to acute stroke in a community health system network.

    PubMed

    Fessler, Richard D; To, Chiu Yuen; Gordon, Vickie; Stover, Carrie; Dunne, Robert

    2014-01-01

    Stroke is one of the major causes of death and disability in the United States, yet it is undertreated by many major medical centers across the country. Timely recognition and treatment of acute ischemic stroke remains a challenge due to confusing clinical presentations, hospital logistics, communication barriers among providers, and lack of standardized treatment algorithms. By creating a system-wide Code Stroke protocol, St. John Providence Health System improved documentation, increased intravenous tissue plasminogen activator delivery, reduced specialist call-back times, improved door-to-computer tomography scan and door-to-needle time, and identified appropriate patients for endovascular therapy.

  10. Poliomyelitis: immunoglobulin-containing cells in the central nervous system in acute and convalescent phases of the human disease.

    PubMed Central

    Esiri, M M

    1980-01-01

    The immunoperoxidase method has been used to demonstrate the presence of immunoglobulin-containing cells in the central nervous system in acute and convalescent phases of poliomyelitis. These cells were found in considerable numbers in the areas of damage during the acute phase, and persisted at the same sites, though in smaller numbers, during the convalescent phase for at least 8 months. Most of the positively stained cells were plasma cells. IgA was the commonest heavy chain type demonstrated, with lesser amounts also of IgG and, during the acute phase, IgM. In the acute phase more lambda than kappa light chain was demonstrated but in the convalescent phase this ratio was reversed. More light chain than heavy chain was demonstrable during the acute phase. The significance of these results is briefly discussed. Images Fig. 2 PMID:6771081

  11. Human coronavirus NL63 is not detected in the respiratory tracts of children with acute Kawasaki disease.

    PubMed

    Shimizu, Chisato; Shike, Hiroko; Baker, Susan C; Garcia, Francesca; van der Hoek, Lia; Kuijpers, Taco W; Reed, Sharon L; Rowley, Anne H; Shulman, Stanford T; Talbot, Helen K B; Williams, John V; Burns, Jane C

    2005-11-15

    Kawasaki disease (KD) is a self-limited, systemic vasculitis of children for which an infectious trigger is suspected. Recently, an association between KD and human coronavirus (HCoV)-New Haven (NH) was reported, on the basis of polymerase chain reaction (PCR) with primers that also amplified HCoV-NL63. We investigated the possible association between these HCoVs in the respiratory tract and KD by reverse-transcriptase (RT) PCR and viral culture in a geographically and ethnically diverse population. Only 1 (2%) of 48 patients with acute KD was positive by RT-PCR for HCoV-NL63/NH in a nasopharyngeal swab. These data do not support an association between these HCoVs and KD.

  12. Defining nervous system susceptibility during acute and latent herpes simplex virus-1 infection.

    PubMed

    Menendez, Chandra M; Carr, Daniel J J

    2017-03-08

    Herpes simplex viruses are neurotropic human pathogens that infect and establish latency in peripheral sensory neurons of the host. Herpes Simplex Virus-1 (HSV-1) readily infects the facial mucosa that can result in the establishment of a latent infection in the sensory neurons of the trigeminal ganglia (TG). From latency, HSV-1 can reactivate and cause peripheral pathology following anterograde trafficking from sensory neurons. Under rare circumstances, HSV-1 can migrate into the central nervous system (CNS) and cause Herpes Simplex Encephalitis (HSE), a devastating disease of the CNS. It is unclear whether HSE is the result of viral reactivation within the TG, from direct primary infection of the olfactory mucosa, or from other infected CNS neurons. Areas of the brain that are susceptible to HSV-1 during acute infection are ill-defined. Furthermore, whether the CNS is a true reservoir of viral latency following clearance of virus during acute infection is unknown. In this context, this review will identify sites within the brain that are susceptible to acute infection and harbor latent virus. In addition, we will also address findings of HSV-1 lytic gene expression during latency and comment on the pathophysiological consequences HSV-1 infection may have on long-term neurologic performance in animal models and humans.

  13. Activation of PPARα by Wy-14643 ameliorates systemic lipopolysaccharide-induced acute lung injury

    SciTech Connect

    Yoo, Seong Ho; Abdelmegeed, Mohamed A.; Song, Byoung-Joon

    2013-07-05

    Highlights: •Activation of PPARα attenuated LPS-mediated acute lung injury. •Pretreatment with Wy-14643 decreased the levels of IFN-γ and IL-6 in ALI. •Nitrosative stress and lipid peroxidation were downregulated by PPARα activation. •PPARα agonists may be potential therapeutic targets for acute lung injury. -- Abstract: Acute lung injury (ALI) is a major cause of mortality and morbidity worldwide. The activation of peroxisome proliferator-activated receptor-α (PPARα) by its ligands, which include Wy-14643, has been implicated as a potential anti-inflammatory therapy. To address the beneficial efficacy of Wy-14643 for ALI along with systemic inflammation, the in vivo role of PPARα activation was investigated in a mouse model of lipopolysaccharide (LPS)-induced ALI. Using age-matched Ppara-null and wild-type mice, we demonstrate that the activation of PPARα by Wy-14643 attenuated LPS-mediated ALI. This was evidenced histologically by the significant alleviation of inflammatory manifestations and apoptosis observed in the lung tissues of wild-type mice, but not in the corresponding Ppara-null mice. This protective effect probably resulted from the inhibition of LPS-induced increases in pro-inflammatory cytokines and nitroxidative stress levels. These results suggest that the pharmacological activation of PPARα might have a therapeutic effect on LPS-induced ALI.

  14. Relationship of Acute Lung Inflammatory Injury to Fas/FasL System

    PubMed Central

    Neff, Thomas A.; Guo, Ren-Feng; Neff, Simona B.; Sarma, J. Vidya; Speyer, Cecilia L.; Gao, Hongwei; Bernacki, Kurt D.; Huber-Lang, Markus; McGuire, Stephanie; Hoesel, L. Marco; Riedemann, Niels C.; Beck-Schimmer, Beatrice; Zetoune, Firas S.; Ward, Peter A.

    2005-01-01

    There is mounting evidence that apoptosis plays a significant role in tissue damage during acute lung injury. To evaluate the role of the apoptosis mediators Fas and FasL in acute lung injury, Fas (lpr)- or FasL (gld)-deficient and wild-type mice were challenged with intrapulmonary deposition of IgG immune complexes. Lung injury parameters (125I-albumin leak, accumulation of myeloperoxidase, and wet lung weights) were measured and found to be consistently reduced in both lpr and gld mice. In wild-type mice, lung injury was associated with a marked increase in Fas protein in lung. Inflamed lungs of wild-type mice showed striking evidence of activated caspase-3, which was much diminished in inflamed lungs from lpr mice. Intratracheal administration of a monoclonal Fas-activating antibody (Jo2) in wild-type mice induced MIP-2 and KC production in bronchoalveolar lavage fluids, and a murine alveolar macrophage cell line (MH-S) showed significantly increased MIP-2 production after incubation with this antibody. Bronchoalveolar lavage fluid content of MIP-2 and KC was substantially reduced in lpr mice after lung injury when compared to levels in wild-type mice. These data suggest that the Fas/FasL system regulates the acute lung inflammatory response by positively affecting CXC-chemokine production, ultimately leading to enhanced neutrophil influx and tissue damage. PMID:15743781

  15. Titre and affinity of propylthiouracil-induced anti-myeloperoxidase antibodies are closely associated with the development of clinical vasculitis.

    PubMed

    Ye, Hua; Gao, Ying; Guo, Xiao-Hui; Zhao, Ming-Hui

    2005-10-01

    Substantial evidences suggested that propylthiouracil (PTU) could induced anti-myeloperoxidase (MPO) antibodies in sera from patients with hyperthyroidism, however, only a subgroup of the PTU-induced anti-MPO antibody positive patients developed clinical evident vasculitis. The aim of this study is to compare the titres and affinities of PTU induced anti-MPO antibodies in sera from patients with hyperthyroidism with and without clinical vasculitis. Anti-MPO antibody positive sera from patients diagnosed hyperthyroidism with (n = 13) and without (n = 14) clinical evident vasculitis were collected. The titre was determined by MPO-ELISA and expressed as logarithm value (lgT). The affinity constant (aK) of anti-MPO IgG was measured by antigen inhibition assay. The titre and aK values were compared between patients with and without vasculitis. In patients with vasculitis, the mean lgT of anti-MPO antibodies was 3.62 +/- 0.66; the median aK was 4.47 x 10(7)M(-1). In patients without vasculitis, the mean lgT was 2.54 +/- 0.29; the median aK was 0.14 x 10(7)M(-1), and both were significant lower than those in patients with vasculitis (t = 5.464; P = 0.000 & z = -4.373; P = 0.000, respectively). We concluded that the titre and affinity of anti-MPO antibodies might be associated with the development of clinical vasculitis in patients with PTU-induced ANCA.

  16. The development of an outcomes management system for acute medical rehabilitation.

    PubMed

    Cohen, B A; Grigonis, A M; Topper, M E; Morrison, M H

    1997-01-01

    In 1993, Continental Medical Systems, Inc. (CMS), a provider of comprehensive medical rehabilitation, developed the Total Outcomes and Prediction Program (TOPP) to measure and evaluate key medical rehabilitation outcomes, quality indicators, and patient satisfaction at its 37 acute rehabilitation hospitals. The broad purposes of TOPP are to manage patient treatment, improve the cost-effectiveness of care, and provide outcomes reporting for managed care and other interested parties. The challenge was to develop a system which could measure, evaluate, and report medical rehabilitation patient outcomes in a way that could be easily understood by multiple audiences, including payers, accrediting organizations, physicians, patients and families, case managers, and CMS clinical staff. Using data from the Uniform Data System for Medical Rehabilitation database, CMS created descriptive outcomes reports for each hospital and for the corporation overall, including performance statistics, outcomes report cards, and quality report cards. These initial reports, as well as updates, quarterly reports, and special ad hoc requests, provide CMS corporate and hospital staff with statistically valid and reliable information to manage the outcomes of medical rehabilitation treatment. TOPP has assisted CMS with meeting accreditation standards for outcomes management and measurement and has been used in managed care contract negotiations. Future TOPP efforts will integrate resource use data, medical acuity and outcomes from acute, subacute, and outpatient rehabilitation levels into CMS' outcomes reporting system.

  17. Endovascular Management of Acute Pulmonary Embolism Using the Ultrasound-Enhanced EkoSonic System.

    PubMed

    Garcia, Mark J

    2015-12-01

    Acute, symptomatic pulmonary embolism (PE) in the massive and submassive categories continues to be a healthcare concern with significant risk for increased morbidity and mortality. Despite increased awareness and venous thromboembolism prophylaxis, endovascular treatment is still an important option for many of these patients. There are a variety of techniques and devices used for treating PE, but none have been evaluated as extensively as the EkoSonic endovascular system that is also currently the only FDA-approved device for the treatment of pulmonary embolism. This article describes the use of the EkoSonic device for this patient population.

  18. Single-organ vasculitis of the cervix accompanying human papillomavirus infection

    PubMed Central

    Gözüküçük, Murat; Gürsoy, Aslı Yarcı; Kankaya, Duygu; Atabekoglu, Cem

    2016-01-01

    Single-organ vasculitis (SOV) has rarely been reported to involve the female genital tract but mostly the uterine cervix. A 39-year-old woman was diagnosed to have a high-grade cervical intraepithelial lesion and was treated by large loop excision of the transformation zone. Histopathological evaluation of the excised specimen confirmed the diagnosis of cervical intraepithelial neoplasia grade III accompanied by human papillomavirus infection. The excised second specimen showed the evidence of vasculitis of medium-sized vessels of the cervix, which is a quite rare form of SOV. It seems to be important to be aware of the localized form of polyarteritis nodosa limited to the female genital tract to prevent unnecessary immunosuppressive therapies. PMID:28386466

  19. Renal involvement in a syndrome of vasculitis complicating HBsAg negative cirrhosis of the liver.

    PubMed

    Montoliu, J; Darnell, A; Grau, J M; Torras, A; Revert, L

    1985-01-01

    Six HBsAg negative patients with cirrhosis of the liver (CL) presented with recurrent bouts of palpable purpura in the legs due to small vessel leucocytoclastic vasculitis. In addition, all patients had renal failure, proteinuria and microhaematuria. Renal biopsy disclosed either diffuse proliferative (3 cases) or focal necrotising glomerulonephritis with crescents (2 cases). One patient had IgM-IgG mixed cryoglobulinaemia (type II). Four patients died of complications of their CL. Hepatocellular carcinoma was found in 1 case. In the patient without renal biopsy renal function improved following steroids and cyclophosphamide. The pathogenesis of this syndrome of cutaneous vasculitis with severe glomerular involvement in CL is unknown but could be immune-complex mediated.

  20. Postoperative hemorrhagic occlusive retinal vasculitis associated with intracameral vancomycin prophylaxis during cataract surgery.

    PubMed

    Miller, Matthew A; Lenci, Lucas T; Reddy, Chittaranjan V; Russell, Stephen R

    2016-11-01

    We present the case of a 75-year-old man who had uneventful cataract surgery and administration of intracameral vancomycin for endophthalmitis prophylaxis, followed by the same procedure in the fellow eye 1 week later. The patient subsequently developed bilateral hemorrhagic occlusive retinal vasculitis, resulting in profound vision loss in both eyes. A second case of hemorrhagic occlusive retinal vasculitis previously reported from our institution is summarized. That case was characterized by a far milder course, with rapid resolution of vision loss. The 2 cases illustrate the broad range of toxicity potentially associated with intracameral vancomycin, suggest that bilateral administration results in a worse prognosis, and indicate that this disorder may be underrecognized due to the potential for a mild course. We recommend that intracameral vancomycin not be used for endophthalmitis prophylaxis.

  1. The Effects of Plasma Exchange on Severe Vasculitis with Diffuse Alveolar Hemorrhage

    PubMed Central

    Goto, Kimihiko; Nakai, Kentaro; Fujii, Hideki; Nishi, Shinichi

    2017-01-01

    Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis is a life-threatening disease characterized by rapidly progressive glomerulonephritis (RPGN) and diffuse alveolar hemorrhage (DAH). Glucocorticoids and immunosuppressants are commonly used to treat this disease but may induce irreversible side effects, particularly in elderly patients. We herein report the case of a 76-year-old woman with RPGN. After methylprednisolone pulse therapy, DAH occurred, and she required ventilatory support. After plasma exchange, her serum creatinine level improved, and she was discharged with home oxygen therapy. Immunosuppressive agents other than glucocorticoids were not required. In conclusion, plasma exchange with glucocorticoid therapy may be effective in treating severe ANCA-associated vasculitis in elderly patients. PMID:28050000

  2. A severe case of acute exogenous lipoid pneumonia treated with systemic corticosteroid

    PubMed Central

    Yasui, Hideki; Yokomura, Koshi; Suda, Takafumi

    2016-01-01

    Acute exogenous lipoid pneumonia is a rare disorder in adults. A treatment of choice for lipoid pneumonia has not been established, and systemic corticosteroid use remains controversial. We report the case of a 32-year-old man with schizophrenia who presented with kerosene-induced acute exogenous lipoid pneumonia that was treated with a systemic corticosteroid. In this case, supportive therapy did not improve the patient's condition, so systemic corticosteroid therapy was commenced four days after he ingested the kerosene. After corticosteroid commencement, the patient's symptoms and hypoxia improved within a few days. Although some radiological characteristics of this disorder have been reported previously, the process of radiological improvement of exogenous lipoid pneumonia is not well known. In this case, computed tomography findings changed dramatically after corticosteroid therapy was initiated. Extensive bilateral consolidations that were observed on admission improved. Although pneumatoceles developed two weeks after corticosteroid commencement, they were nearly gone after two months of the treatment. While corticosteroid therapy is not suitable for all cases, it should be considered for severe or refractory cases. PMID:27222789

  3. Fatal systemic adenoviral infection superimposed on pulmonary mucormycosis in a child with acute leukemia

    PubMed Central

    Seo, Yu Mi; Hwang-Bo, Seok; Kim, Seong koo; Han, Seung Beom; Chung, Nack-Gyun; Kang, Jin Han

    2016-01-01

    Abstract Background: Although adenovirus (ADV) infection usually causes self-limiting respiratory disorders in immune competent children; severe and systemic ADV infection in children undergoing chemotherapy for leukemia has been continuously reported. Nevertheless, there has been no consensus on risk factors and treatment strategies for severe ADV infection in children undergoing chemotherapy. Case summary: We report a case of a 15-year-old boy with a fatal systemic ADV infection. He had received reinduction chemotherapy for relapsed acute lymphoblastic leukemia under continuing antifungal therapy for previously diagnosed fungal pneumonia. He complained of fever and right shoulder pain 4 days after completing the reinduction chemotherapy. In spite of appropriate antibiotic and antifungal therapy, pneumonia was aggravated and gross hematuria was accompanied. A multiplex polymerase chain reaction test for respiratory viruses was positive for ADV in a blood sample, and a urine culture was positive for ADV. He received oral ribavirin, intravenous immunoglobulin, and intravenous cidofovir therapy; however, he eventually died. Relapsed leukemia, concurrent fungal pneumonia, and delayed cidofovir administration were considered the cause of the grave outcome in this patient. Conclusion: ADV may cause severe infections not only in allogeneic hematopoietic cell transplant recipients, but also in patients undergoing chemotherapy for acute leukemia. The risk factors for severe ADV infection in patients undergoing chemotherapy should be determined in the future studies, and early antiviral therapy should be administered to immune compromised patients with systemic ADV infection. PMID:27749571

  4. Acute effects of shock-type vibration transmitted to the hand-arm system.

    PubMed

    Schäfer, N; Dupuis, H; Hartung, E

    1984-01-01

    The aim of the project was to find out whether shock-type vibration of hand-tools compared to non-impulsive vibration has stronger acute effects on the hand-arm system and therefore needs a stricter evaluation from the occupational health point of view in comparison with the requirements of the Draft International Standard ISO-DIS 5349. Under laboratory conditions, subjects were exposed to simulated vibration of hand-tools (grinder, chain saw, hammer-drill, pneumatic hammer, rivet hammer and nailer). The following evaluation criteria were used: biomechanical transmissibility of the hand-arm system (wrist, elbow joint, shoulder joint); muscle-activity (m. flexor carpi ulnaris, m. biceps, m. triceps); peripheral circulation (skin temperature) and subjective perception (comparison of intensity of standard and test vibrations). The results show no significant difference in acute effects on the hand-arm system between impulsive and non-impulsive type vibrations of the hand-tools tested with respect to the chosen vibration level, short-time exposure (up to 8 min) and evaluation criteria. In summary, therefore, it may be concluded that for the evaluation of shock-type vibration of the hand-tools tested, it is justified to use the existing Draft International Standard ISO-DIS 5349.

  5. Invasive pneumococcal disease complicated by cerebral vasculitis, transient diabetes insipidus and spondylodiscitis.

    PubMed

    Ribeiro, Sofia; Domingues, Vital; Faria, Raquel M; Mendonça, Teresa

    2013-08-19

    Invasive pneumococcal disease (IPD) is a potential life-threatening situation that requires immediate recognition and treatment. Cerebrovascular complications are uncommon and have been reported less frequently in adults than in children. We report a case of 59-year-old man with IPD complicated by cerebral vasculitis, transient central diabetes insipidus and spondylodiscitis. Each of these complications is rare and needs specific approach. Their association is even rarer and to the best of our knowledge this is the first case reported.

  6. Bioinspired engineering study of Plantae vascules for self-healing composite structures.

    PubMed

    Trask, R S; Bond, I P

    2010-06-06

    This paper presents the first conceptual study into creating a Plantae-inspired vascular network within a fibre-reinforced polymer composite laminate, which provides an ongoing self-healing functionality without incurring a mass penalty. Through the application of a 'lost-wax' technique, orthogonal hollow vascules, inspired by the 'ray cell' structures found in ring porous hardwoods, were successfully introduced within a carbon fibre-reinforced epoxy polymer composite laminate. The influence on fibre architecture and mechanical behaviour of single vascules (located on the laminate centreline) when aligned parallel and transverse to the local host ply was characterized experimentally using a compression-after-impact test methodology. Ultrasonic C-scanning and high-resolution micro-CT X-ray was undertaken to identify the influence of and interaction between the internal vasculature and impact damage. The results clearly show that damage morphology is influenced by vascule orientation and that a 10 J low-velocity impact damage event is sufficient to breach the vasculature; a prerequisite for any subsequent self-healing function. The residual compressive strength after a 10 J impact was found to be dependent upon vascule orientation. In general, residual compressive strength decreased to 70 per cent of undamaged strength when vasculature was aligned parallel to the local host ply and a value of 63 per cent when aligned transverse. This bioinspired engineering study has illustrated the potential that a vasculature concept has to offer in terms of providing a self-healing function with minimum mass penalty, without initiating premature failure within a composite structure.

  7. Rituximab versus cyclophosphamide for ANCA-associated vasculitis with renal involvement.

    PubMed

    Geetha, Duvuru; Specks, Ulrich; Stone, John H; Merkel, Peter A; Seo, Philip; Spiera, Robert; Langford, Carol A; Hoffman, Gary S; Kallenberg, Cees G M; St Clair, E William; Fessler, Barri J; Ding, Linna; Tchao, Nadia K; Ikle, David; Jepson, Brett; Brunetta, Paul; Fervenza, Fernando C

    2015-04-01

    Rituximab (RTX) is non-inferior to cyclophosphamide (CYC) followed by azathioprine (AZA) for remission-induction in severe ANCA-associated vasculitis (AAV), but renal outcomes are unknown. This is a post hoc analysis of patients enrolled in the Rituximab for ANCA-Associated Vasculitis (RAVE) Trial who had renal involvement (biopsy proven pauci-immune GN, red blood cell casts in the urine, and/or a rise in serum creatinine concentration attributed to vasculitis). Remission-induction regimens were RTX at 375 mg/m(2) × 4 or CYC at 2 mg/kg/d. CYC was replaced by AZA (2 mg/kg/d) after 3-6 months. Both groups received glucocorticoids. Complete remission (CR) was defined as Birmingham Vasculitis Activity Score/Wegener's Granulomatosis (BVAS/WG)=0 off prednisone. Fifty-two percent (102 of 197) of the patients had renal involvement at entry. Of these patients, 51 were randomized to RTX, and 51 to CYC/AZA. Mean eGFR was lower in the RTX group (41 versus 50 ml/min per 1.73 m(2); P=0.05); 61% and 75% of patients treated with RTX and 63% and 76% of patients treated with CYC/AZA achieved CR by 6 and 18 months, respectively. No differences in remission rates or increases in eGFR at 18 months were evident when analysis was stratified by ANCA type, AAV diagnosis (granulomatosis with polyangiitis versus microscopic polyangiitis), or new diagnosis (versus relapsing disease) at entry. There were no differences between treatment groups in relapses at 6, 12, or 18 months. No differences in adverse events were observed. In conclusion, patients with AAV and renal involvement respond similarly to remission induction with RTX plus glucocorticoids or CYC plus glucocorticoids.

  8. Nationwide epidemiological survey of childhood IgA vasculitis associated hospitalization in the USA.

    PubMed

    Okubo, Yusuke; Nochioka, Kotaro; Sakakibara, Hiroshi; Hataya, Hiroshi; Terakawa, Toshiro; Testa, Marcia; Sundel, Robert P

    2016-11-01

    At the national level, IgA vasculitis-related hospitalizations among children in the USA are scarce. Furthermore, nationwide epidemiology and hospital course of children with IgA vasculitis have not been fully described in the USA, and disparities by race/ethnicity remain unknown. Hospital discharge records of patients aged 19 years or younger were obtained from the 2003, 2006, 2009, and 2012 Kids' Inpatient Database, and they were weighted to estimate the annual hospitalization rates with respect to age, gender, and race/ethnicity in the USA. Annual hospitalization rates were calculated using weighted case estimates and US census data. Negative binomial regression was used to ascertain the factors associated with length of hospital stay. Total annual hospitalization rates showed a significant decreasing trend, ranging from 2.45 per 100,000 children in 2003 to 1.89 per 100,000 children in 2012 (p < 0.001). The peak ages of the hospitalized children with IgA vasculitis were 2 and 7 years, and male-to-female ratios were 1.38-1.44. Factors associated with length of hospital stay were patients' ages (10-14 and 15-19 years), race/ethnicity (Hispanic, Asian, and Pacific Islander), comorbid electrolyte abnormality, GI hemorrhage, intussusception, renal symptoms, and GI symptoms. The annual hospitalization rates for IgA vasculitis are declining in the USA across multiple age groups. GI and renal manifestations are associated with increased length of hospital stay.

  9. Pancreatic ascites hemoglobin contributes to the systemic response in acute pancreatitis.

    PubMed

    Pérez, Salvador; Pereda, Javier; Sabater, Luis; Sastre, Juan

    2015-04-01

    Upon hemolysis extracellular hemoglobin causes oxidative stress and cytotoxicity due to its peroxidase activity. Extracellular hemoglobin may release free hemin, which increases vascular permeability, leukocyte recruitment, and adhesion molecule expression. Pancreatitis-associated ascitic fluid is reddish and may contain extracellular hemoglobin. Our aim has been to determine the role of extracellular hemoglobin in the local and systemic inflammatory response during severe acute pancreatitis in rats. To this end we studied taurocholate-induced necrotizing pancreatitis in rats. First, extracellular hemoglobin in ascites and plasma was quantified and the hemolytic action of ascitic fluid was tested. Second, we assessed whether peritoneal lavage prevented the increase in extracellular hemoglobin in plasma during pancreatitis. Third, hemoglobin was purified from rat erythrocytes and administered intraperitoneally to assess the local and systemic effects of ascitic-associated extracellular hemoglobin during acute pancreatitis. Extracellular hemoglobin and hemin levels markedly increased in ascitic fluid and plasma during necrotizing pancreatitis. Peroxidase activity was very high in ascites. The peritoneal lavage abrogated the increase in extracellular hemoglobin in plasma. The administration of extracellular hemoglobin enhanced ascites; dramatically increased abdominal fat necrosis; upregulated tumor necrosis factor-α, interleukin-1β, and interleukin-6 gene expression; and decreased expression of interleukin-10 in abdominal adipose tissue during pancreatitis. Extracellular hemoglobin enhanced the gene expression and protein levels of vascular endothelial growth factor (VEGF) and other hypoxia-inducible factor-related genes in the lung. Extracellular hemoglobin also increased myeloperoxidase activity in the lung. In conclusion, extracellular hemoglobin contributes to the inflammatory response in severe acute pancreatitis through abdominal fat necrosis and inflammation

  10. Isoniazid Induced Cutaneous Leukocytoclastic Vasculitis in Extra Pulmonary Tuberculosis (Pott’s Spine): A Case Report

    PubMed Central

    V, Dharma Rao; Rampure, Dilip; S, Rama Rao

    2014-01-01

    Anti-tuberculosis drugs frequently result in cutaneous adverse reactions but Isoniazid is known to have least toxic potential for cutaneous reactions. We report a rare case of Isoniazid induced cutaneous leucocytoclastic vasculitis. A 64-year-old male was diagnosed to have Pott’s spine with multiple vertebral body involvement (D8-12 vertebrae). Subsequently, he was treated with first line anti-TB drugs i.e., Isoniazid, Rifampicin, Pyrazinamide and Ethambutol. On the fourth day of treatment with Anti Tuberculosis Treatment (ATT), the patient developed an erythematosus rash over right upper limb not associated with itching or pain, non-blanchable macules and papules over bilateral shins on lower limbs, petechiae on both forearms and hyper pigmented, scaly rash over right axilla and buttocks. The skin biopsy report was consistent with cutaneous leukocytoclastic vasculitis. Although rare, Isoniazid among anti-tuberculosis drugs should be considered as potential cause of drug-induced cutaneous leukocytoclastic vasculitis in the differential diagnosis of erythematosus rash with petechiae. PMID:25302231

  11. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis.

    PubMed

    Yates, M; Watts, R A; Bajema, I M; Cid, M C; Crestani, B; Hauser, T; Hellmich, B; Holle, J U; Laudien, M; Little, M A; Luqmani, R A; Mahr, A; Merkel, P A; Mills, J; Mooney, J; Segelmark, M; Tesar, V; Westman, K; Vaglio, A; Yalçındağ, N; Jayne, D R; Mukhtyar, C

    2016-09-01

    In this article, the 2009 European League Against Rheumatism (EULAR) recommendations for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have been updated. The 2009 recommendations were on the management of primary small and medium vessel vasculitis. The 2015 update has been developed by an international task force representing EULAR, the European Renal Association and the European Vasculitis Society (EUVAS). The recommendations are based upon evidence from systematic literature reviews, as well as expert opinion where appropriate. The evidence presented was discussed and summarised by the experts in the course of a consensus-finding and voting process. Levels of evidence and grades of recommendations were derived and levels of agreement (strengths of recommendations) determined. In addition to the voting by the task force members, the relevance of the recommendations was assessed by an online voting survey among members of EUVAS. Fifteen recommendations were developed, covering general aspects, such as attaining remission and the need for shared decision making between clinicians and patients. More specific items relate to starting immunosuppressive therapy in combination with glucocorticoids to induce remission, followed by a period of remission maintenance; for remission induction in life-threatening or organ-threatening AAV, cyclophosphamide and rituximab are considered to have similar efficacy; plasma exchange which is recommended, where licensed, in the setting of rapidly progressive renal failure or severe diffuse pulmonary haemorrhage. These recommendations are intended for use by healthcare professionals, doctors in specialist training, medical students, pharmaceutical industries and drug regulatory organisations.

  12. Surgical lung biopsy to diagnose Behcet's vasculitis with adult respiratory distress syndrome

    PubMed Central

    Vydyula, Ravikanth; Allred, Charles; Huartado, Mariana; Mina, Bushra

    2014-01-01

    A 34-year-old female presented with fever and abdominal pain. Past medical history includes Crohn's and Behcet's disease. Examination revealed multiple skin ulcerations, oral aphthae, and bilateral coarse rales. She developed respiratory distress with diffuse bilateral alveolar infiltrates on chest radiograph requiring intubation. PaO2/FiO2 ratio was 132. The chest computed tomography revealed extensive nodular and patchy ground-glass opacities. Bronchoalveolar lavage demonstrated a predominance of neutrophils. Methylprednisolone 60 mg every 6 h and broad-spectrum antimicrobials were initiated. No infectious etiologies were identified. Surgical lung biopsy demonstrated diffuse alveolar damage (DAD) mixed with lymphocytic and necrotizing vasculitis with multiple small infarcts and thrombi consistent with Behcet's vasculitis. As she improved, steroids were tapered and discharged home on oral cyclophosphamide. Pulmonary involvement in Behcet's is unusual and commonly manifests as pulmonary artery aneurysms, thrombosis, infarction, and hemorrhage. Lung biopsy findings demonstrating DAD are consistent with the clinical diagnosis of adult respiratory distress syndrome. The additional findings of necrotizing vasculitis and infarcts may have led to DAD. PMID:25378849

  13. Possibly propylthiouracil-induced antineutrophilic cytoplasmic antibody-associated vasculitis manifested as blood coagulation disorders

    PubMed Central

    Yi, Xiao-Yan; Wang, Yao; Li, Qi-Fu; Li, Rong; Yang, Shu-Min; Zhou, Guo-Qing; Wang, Zhi-Hong

    2016-01-01

    Abstract Background: Propylthiouracil is the most common drug used to treat hyperthyroidism. However, this drug could cause a severe disease, antineutrophilic cytoplasmic antibody-associated vasculitis (AAV), which was usually misdiagnosed. Methods: We reported a 60-year-old woman of propylthiouracil-induced AAV manifested as blood coagulation disorders. The patient was admitted because of hyperthyroidism and leukopenia. At the time of hospitalization, she suffered from dry cough, erythema and knee joints ache, and gradually became febrile. And then BP decreased and PLT was reduced with coagulation disorders. ANCA: c-ANCA positive (1:100), p-ANCA positive (1:320), MPO-IgG positive, PR3-IgG positive, GBM-IgG negative. Erythrocyte sedimentation rate and C-reactive protein increased markedly. Chest high-resolution computed tomography (HRCT) showed that scattered spots, patch and ground-glass opacity. Results: Finally, we made a terminal diagnosis of PTU-induced AAV possibly. After drug withdrawal and use of steroid, the patient recovered well and then accepted RAI therapy. As the patient was given imipenem-cilastatin before the reduction of PLT and coagulation disorders, we considered that the hematologic disorders might be caused by antibiotics or a clinical presentation of the vasculitis itself. Conclusion: Drug-induced vasculitis is relatively good prognosis, but early diagnosis and timely withdrawal of associated drugs are the key to the treatment. PMID:27741122

  14. Refractory antineutrophil cytoplasmic antibody-associated vasculitis successfully treated with rituximab: a case report.

    PubMed

    Horai, Yoshiro; Miyamura, Tomoya; Takahama, Soichiro; Hirata, Akie; Nakamura, Masataka; Ando, Hitoshi; Minami, Rumi; Yamamoto, Masahiro; Suematsu, Eiichi

    2010-01-01

    A 63-year-old-man was diagnosed in March 2002 with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis because of mononeuritis multiplex, interstitial pneumonia and a positive finding for myeloperoxidase (MPO)-ANCA. Although treated with prednisolone and oral cyclophosphamide, he suffered repeated remission and deterioration of his conditon, which was complicated by hypertrophic pachymeningitis and sinusitis. In July 2006, he was diagnosed with an exacerbation of ANCA-associated vasculitis because of pyrexia, general malaise, numbness in his face and legs, and elevated serum CRP level. Steroid pulse therapy was thus initiated and the patient's clinical symptoms improved. However, serum CRP levels elevated again (5.18 mg/dl) in September 2006. We began administration of rituximab (500 mg/bodyx4 times) in November 2006 and his symptom and laboratory data significantly improved. The dose of prednisolone was slowly decreased without suffering a relapse. Rituximab has been administered every one year, and good disease control has been achieved. Diagnosis of Wegener's granulomatosis was made from the findings of a nodular lesion in the left lung. Rituximab should be considered for patients with refractory ANCA-associated vasculitis.

  15. Risk of viral acute gastrointestinal illness from nondisinfected drinking water distribution systems.

    PubMed

    Lambertini, Elisabetta; Borchardt, Mark A; Kieke, Burney A; Spencer, Susan K; Loge, Frank J

    2012-09-04

    Acute gastrointestinal illness (AGI) resulting from pathogens directly entering the piping of drinking water distribution systems is insufficiently understood. Here, we estimate AGI incidence from virus intrusions into the distribution systems of 14 nondisinfecting, groundwater-source, community water systems. Water samples for virus quantification were collected monthly at wells and households during four 12-week periods in 2006-2007. Ultraviolet (UV) disinfection was installed on the communities' wellheads during one study year; UV was absent the other year. UV was intended to eliminate virus contributions from the wells and without residual disinfectant present in these systems, any increase in virus concentration downstream at household taps represented virus contributions from the distribution system (Approach 1). During no-UV periods, distribution system viruses were estimated by the difference between well water and household tap virus concentrations (Approach 2). For both approaches, a Monte Carlo risk assessment framework was used to estimate AGI risk from distribution systems using study-specific exposure-response relationships. Depending on the exposure-response relationship selected, AGI risk from the distribution systems was 0.0180-0.0661 and 0.001-0.1047 episodes/person-year estimated by Approaches 1 and 2, respectively. These values represented 0.1-4.9% of AGI risk from all exposure routes, and 1.6-67.8% of risk related to drinking water exposure. Virus intrusions into nondisinfected drinking water distribution systems can contribute to sporadic AGI.

  16. A Case Report of Acute Diverticulitis in “Pseudodiverticulosis” after Hemorpex System® Procedure

    PubMed Central

    2016-01-01

    Introduction. In the last years many mini-invasive approaches were developed in order to reduce postoperative pain and complication after haemorrhoid surgery: one of these alternatives is represented by Hemorpex System, a relatively young technique that combines transanal dearterialization with mucopexy through a dedicated proctoscope. Case Presentation. A 78-year-old male patient was admitted to the Emergency Department for acute urinary retention and elevated temperature. Hemorpex procedure was performed 4 years before. Clinical, endoscopic, and radiological findings demonstrated the presence of multiple diverticula-like structures fulfilled by purulent fluid and a deep alteration of the normal anatomy of the rectum. He was treated following the standard protocol of acute diverticulitis and full recovery from symptoms was achieved. Discussion. Hemorpex System is a young technique, and nowadays-available studies lack long-term follow-up data. Anatomical changes induced by the procedure are consistent and definitive. Our patient luckily demonstrated a prompt response to conservative treatment, but it must be taken into account that, in case of medical treatment failure, surgical approach would be necessary and the actual patient anatomical changes could lead the surgeon to unavoidable threatening maneuvers. PMID:27974987

  17. Both acute and prolonged administration of EPO reduce cerebral and systemic vascular conductance in humans.

    PubMed

    Rasmussen, Peter; Kim, Yu-Sok; Krogh-Madsen, Rikke; Lundby, Carsten; Olsen, Niels V; Secher, Niels H; van Lieshout, Johannes J

    2012-03-01

    Administration of erythropoietin (EPO) has been linked to cerebrovascular events. EPO reduces vascular conductance, possibly because of the increase in hematocrit. Whether EPO in itself affects the vasculature remains unknown; here it was evaluated in healthy males by determining systemic and cerebrovascular variables following acute (30,000 IU/d for 3 d; n=8) and chronic (5000 IU/week for 13 wk; n=8) administration of EPO, while the responsiveness of the vasculature was challenged during cycling exercise, with and without hypoxia. Prolonged administration of EPO increased hematocrit from 42.5 ± 3.7 to 47.6 ± 4.1% (P<0.01), whereas hematocrit was unaffected following acute EPO administration. Yet, the two EPO regimes increased arterial pressure similarly (by 8±4 and 7±3 mmHg, respectively; P=0.01) through reduced vascular conductance (by 7±3 and 5±2%; P<0.05). Also, both EPO regimes widened the arterial-to-jugular O(2) differences at rest as well as during normoxic and hypoxic exercise (P<0.01), which indicated reduced cerebral blood flow despite preserved dynamic cerebral autoregulation, and an increase in middle cerebral artery mean blood flow velocity (P<0.05), therefore, reflected vasoconstriction. Thus, administration of EPO to healthy humans lowers systemic and cerebral conductance independent of its effect on hematocrit.

  18. Acute Lead Exposure Increases Arterial Pressure: Role of the Renin-Angiotensin System

    PubMed Central

    Simões, Maylla Ronacher; Ribeiro Júnior, Rogério F.; Vescovi, Marcos Vinícius A.; de Jesus, Honério C.; Padilha, Alessandra S.; Stefanon, Ivanita; Vassallo, Dalton V.; Salaices, Mercedes; Fioresi, Mirian

    2011-01-01

    Background Chronic lead exposure causes hypertension and cardiovascular disease. Our purpose was to evaluate the effects of acute exposure to lead on arterial pressure and elucidate the early mechanisms involved in the development of lead-induced hypertension. Methodology/Principal Findings Wistar rats were treated with lead acetate (i.v. bolus dose of 320 µg/Kg), and systolic arterial pressure, diastolic arterial pressure and heart rate were measured during 120 min. An increase in arterial pressure was found, and potential roles of the renin-angiotensin system, Na+,K+-ATPase and the autonomic reflexes in this change in the increase of arterial pressure found were evaluated. In anesthetized rats, lead exposure: 1) produced blood lead levels of 37±1.7 µg/dL, which is below the reference blood concentration (60 µg/dL); 2) increased systolic arterial pressure (Ct: 109±3 mmHg vs Pb: 120±4 mmHg); 3) increased ACE activity (27% compared to Ct) and Na+,K+-ATPase activity (125% compared to Ct); and 4) did not change the protein expression of the α1-subunit of Na+,K+-ATPase, AT1 and AT2. Pre-treatment with an AT1 receptor blocker (losartan, 10 mg/Kg) or an ACE inhibitor (enalapril, 5 mg/Kg) blocked the lead-induced increase of arterial pressure. However, a ganglionic blockade (hexamethonium, 20 mg/Kg) did not prevent lead's hypertensive effect. Conclusion Acute exposure to lead below the reference blood concentration increases systolic arterial pressure by increasing angiotensin II levels due to ACE activation. These findings offer further evidence that acute exposure to lead can trigger early mechanisms of hypertension development and might be an environmental risk factor for cardiovascular disease. PMID:21494558

  19. Integrating microRNAs into a system biology approach to acute lung injury.

    PubMed

    Zhou, Tong; Garcia, Joe G N; Zhang, Wei

    2011-04-01

    Acute lung injury (ALI), including the ventilator-induced lung injury (VILI) and the more severe acute respiratory distress syndrome (ARDS), are common and complex inflammatory lung diseases potentially affected by various genetic and nongenetic factors. Using the candidate gene approach, genetic variants associated with immune response and inflammatory pathways have been identified and implicated in ALI. Because gene expression is an intermediate phenotype that resides between the DNA sequence variation and the higher level cellular or whole-body phenotypes, the illustration of gene expression regulatory networks potentially could enhance understanding of disease susceptibility and the development of inflammatory lung syndromes. MicroRNAs (miRNAs) have emerged as a novel class of gene regulators that play critical roles in complex diseases including ALI. Comparisons of global miRNA profiles in animal models of ALI and VILI identified several miRNAs (eg, miR-146a and miR-155) previously implicated in immune response and inflammatory pathways. Therefore, via regulation of target genes in these biological processes and pathways, miRNAs potentially contribute to the development of ALI. Although this line of inquiry exists at a nascent stage, miRNAs have the potential to be critical components of a comprehensive model for inflammatory lung disease built by a systems biology approach that integrates genetic, genomic, proteomic, epigenetic as well as environmental stimuli information. Given their particularly recognized role in regulation of immune and inflammatory responses, miRNAs also serve as novel therapeutic targets and biomarkers for ALI/ARDS or VILI, thus facilitating the realization of personalized medicine for individuals with acute inflammatory lung disease.

  20. Inhalation exposure system used for acute and repeated-dose methyl isocyanate exposures of laboratory animals.

    PubMed

    Adkins, B; O'Connor, R W; Dement, J M

    1987-06-01

    Laboratory animals were exposed by inhalation for 2 hr/day (acute) or 6 hr/day (four consecutive days, repeated dose) to methyl isocyanate (MIC). Exposures were conducted in stainless steel and glass inhalation exposure chambers placed in stainless steel, wire mesh cages. MIC was delivered with nitrogen via stainless steel and Teflon supply lines. Chamber concentrations ranged from 0 to 60 ppm and were monitored continuously with infrared spectrophotometers to 1 ppm and at 2-hr intervals to 20 ppb with a high performance liquid chromatograph equipped with a fluorescence detector. Other operational parameters monitored on a continuous basis included chamber temperature (20-27 degrees C), relative humidity (31-64%), static (transmural) pressure (-0.3 in.), and flow (300-500 L/min). The computer-assistance system interfaced with the inhalation exposure laboratory is described in detail, including the analytical instrumentation calibration system used throughout this investigation.

  1. Sodium nitrite induces acute central nervous system toxicity in guinea pigs exposed to systemic cell-free hemoglobin

    SciTech Connect

    Buehler, Paul W.; Butt, Omer I.; D'Agnillo, Felice

    2011-06-10

    Highlights: {yields} Toxicological implications associated with the use of NaNO{sub 2} therapy to treat systemic cell-free Hb exposure are not well-defined. {yields} Systemic Hb exposure followed by NaNO{sub 2} infusion induces acute CNS toxicities in guinea pigs. {yields} These CNS effects were not reproduced by the infusion of cell-free Hb or NaNO{sub 2} alone. {yields} NaNO{sub 2}-mediated oxidation of cell-free Hb may play a causative role in the observed CNS changes. -- Abstract: Systemic cell-free hemoglobin (Hb) released via hemolysis disrupts vascular homeostasis, in part, through the scavenging of nitric oxide (NO). Sodium nitrite (NaNO{sub 2}) therapy can attenuate the hypertensive effects of Hb. However, the chemical reactivity of NaNO{sub 2} with Hb may enhance heme- or iron-mediated toxicities. Here, we investigate the effect of NaNO{sub 2} on the central nervous system (CNS) in guinea pigs exposed to systemic cell-free Hb. Intravascular infusion of NaNO{sub 2}, at doses sufficient to alleviate Hb-mediated blood pressure changes, reduced the expression of occludin, but not zona occludens-1 (ZO-1) or claudin-5, in cerebral tight junctions 4 h after Hb infusion. This was accompanied by increased perivascular heme oxygenase-1 expression, neuronal iron deposition, increased astrocyte and microglial activation, and reduced expression of neuron-specific nuclear protein (NeuN). These CNS changes were not observed in animals treated with Hb or NaNO{sub 2} alone. Taken together, these findings suggest that the use of nitrite salts to treat systemic Hb exposure may promote acute CNS toxicity.

  2. A Smartphone Client-Server Teleradiology System for Primary Diagnosis of Acute Stroke

    PubMed Central

    2011-01-01

    Background Recent advances in the treatment of acute ischemic stroke have made rapid acquisition, visualization, and interpretation of images a key factor for positive patient outcomes. We have developed a new teleradiology system based on a client-server architecture that enables rapid access to interactive advanced 2-D and 3-D visualization on a current generation smartphone device (Apple iPhone or iPod Touch, or an Android phone) without requiring patient image data to be stored on the device. Instead, a server loads and renders the patient images, then transmits a rendered frame to the remote device. Objective Our objective was to determine if a new smartphone client-server teleradiology system is capable of providing accuracies and interpretation times sufficient for diagnosis of acute stroke. Methods This was a retrospective study. We obtained 120 recent consecutive noncontrast computed tomography (NCCT) brain scans and 70 computed tomography angiogram (CTA) head scans from the Calgary Stroke Program database. Scans were read by two neuroradiologists, one on a medical diagnostic workstation and an iPod or iPhone (hereafter referred to as an iOS device) and the other only on an iOS device. NCCT brain scans were evaluated for early signs of infarction, which includes early parenchymal ischemic changes and dense vessel sign, and to exclude acute intraparenchymal hemorrhage and stroke mimics. CTA brain scans were evaluated for any intracranial vessel occlusion. The interpretations made on an iOS device were compared with those made at a workstation. The total interpretation times were recorded for both platforms. Interrater agreement was assessed. True positives, true negatives, false positives, and false negatives were obtained, and sensitivity, specificity, and accuracy of detecting the abnormalities on the iOS device were computed. Results The sensitivity, specificity, and accuracy of detecting intraparenchymal hemorrhage were 100% using the iOS device with a

  3. [Acute pancreatitis].

    PubMed

    Hecker, M; Mayer, K; Askevold, I; Collet, P; Weigand, M A; Krombach, G A; Padberg, W; Hecker, A

    2014-03-01

    Acute pancreatitis is a potentially fatal disease with individually differing expression of systemic involvement. For this reason early diagnosis with subsequent risk stratification is essential in the clinical management of this frequent gastroenterological disorder. Severe forms of acute pancreatitis occur in approximately 20 % of cases often requiring intensive care monitoring and interdisciplinary therapeutic approaches. In the acute phase adequate fluid replacement and sufficient analgesic therapy is of major therapeutic importance. Concerning the administration of antibiotics and the nutritional support of patients with acute pancreatitis a change in paradigms could be observed in recent years. Furthermore, endoscopic, radiological or surgical interventions can be necessary depending on the severity of the disease and potential complications.

  4. Catheter-directed therapy for acute renal vein thrombosis in systemic lupus erythematosus: A case report.

    PubMed

    Jong, Chien-Boon; Lo, Wei-Yung; Hsieh, Mu-Yang

    2017-02-15

    We report our experience using catheter-directed thrombectomy/thrombolysis (CDT) to treat a patient with acute renal vein thrombosis (RVT) associated with systemic lupus erythematosus (SLE). A 34-year-old woman presented with persistent left flank pain, and a renal ultrasonography examination revealed an enlarged left kidney. Contrast-enhanced computed tomography confirmed the presence of acute left RVT. Because medical treatment failed to relieve her pain and the renal function was deteriorating, we attempted to salvage the occluded left renal vein using an endovascular approach. The pain was completely relieved after a CDT and an overnight urokinase infusion. A follow-up computed tomography examination revealed the complete resolution of the thrombus. The creatinine level returned to normal (1.7-0.4 mg/dL), along with contrast enhancement in the left kidney, and this suggested the preservation of renal function. To our knowledge, this is the first report utilizing CDT to treat SLE-associated RVT. When the renal function is deteriorating, CDT is worth considering for RVT if conventional medical treatment has failed. © 2016 Wiley Periodicals, Inc.

  5. A Biophysical Systems Approach to Identifying the Pathways of Acute and Chronic Doxorubicin Mitochondrial Cardiotoxicity

    PubMed Central

    de Oliveira, Bernardo L.; Niederer, Steven

    2016-01-01

    The clinical use of the anthracycline doxorubicin is limited by its cardiotoxicity which is associated with mitochondrial dysfunction. Redox cycling, mitochondrial DNA damage and electron transport chain inhibition have been identified as potential mechanisms of toxicity. However, the relative roles of each of these proposed mechanisms are still not fully understood. The purpose of this study is to identify which of these pathways independently or in combination are responsible for doxorubicin toxicity. A state of the art mathematical model of the mitochondria including the citric acid cycle, electron transport chain and ROS production and scavenging systems was extended by incorporating a novel representation for mitochondrial DNA damage and repair. In silico experiments were performed to quantify the contributions of each of the toxicity mechanisms to mitochondrial dysfunction during the acute and chronic stages of toxicity. Simulations predict that redox cycling has a minor role in doxorubicin cardiotoxicity. Electron transport chain inhibition is the main pathway for acute toxicity for supratherapeutic doses, being lethal at mitochondrial concentrations higher than 200μM. Direct mitochondrial DNA damage is the principal pathway of chronic cardiotoxicity for therapeutic doses, leading to a progressive and irreversible long term mitochondrial dysfunction. PMID:27870850

  6. Evidence Report: Risk of Acute and Late Central Nervous System Effects from Radiation Exposure

    NASA Technical Reports Server (NTRS)

    Nelson, Gregory A.; Simonsen, Lisa; Huff, Janice L.

    2016-01-01

    Possible acute and late risks to the central nervous system (CNS) from galactic cosmic rays (GCR) and solar particle events (SPE) are concerns for human exploration of space. Acute CNS risks may include: altered cognitive function, reduced motor function, and behavioral changes, all of which may affect performance and human health. Late CNS risks may include neurological disorders such as Alzheimer's disease (AD), dementia and premature aging. Although detrimental CNS changes are observed in humans treated with high-dose radiation (e.g., gamma rays and 9 protons) for cancer and are supported by experimental evidence showing neurocognitive and behavioral effects in animal models, the significance of these results on the morbidity to astronauts has not been elucidated. There is a lack of human epidemiology data on which to base CNS risk estimates; therefore, risk projection based on scaling to human data, as done for cancer risk, is not possible for CNS risks. Research specific to the spaceflight environment using animal and cell models must be compiled to quantify the magnitude of CNS changes in order to estimate this risk and to establish validity of the current permissible exposure limits (PELs). In addition, the impact of radiation exposure in combination with individual sensitivity or other space flight factors, as well as assessment of the need for biological/pharmaceutical countermeasures, will be considered after further definition of CNS risk occurs.

  7. Evaluation of the acute cardiac and central nervous system effects of the fluorocarbon trifluoromethane in baboons

    SciTech Connect

    Branch, C.A.; Goldberg, D.A.; Ewing, J.R.; Butt, S.S.; Gayner, J.; Fagan, S.C.

    1994-12-31

    The gaseous fluorocarbon trifluoromethane has recently been investigated for its potential as an in vivo gaseous indicator for nuclear magnetic resonance studies of brain perfusion. Trifluoromethane may also have significant value as a replacement for chlorofluorocarbon fire retardants. Because of possible species-specific cardiotoxic and anesthetic properties, the toxicological evaluation of trifluoromethane in primates (Papio anubis) is necessary prior to its evaluation in humans. We report the acute cardiac and central nervous system effects of trifluoromethane in eight anesthetized baboons. A dose-response effect was established for respiratory rate, electroencephalogram, and cardiac sinus rate, which exhibited a stepwise decrease from 10% trifluoromethane. No spontaneous arrhythmias were noted, and arterial blood pressure remained unchanged at any inspired level. Intravenous epinephrine infusions (1 {mu}g/kg) induced transient cardiac arrhythmia in 1 animal only at 70% FC-23 (v/v) trifluoromethane. Trifluoromethane appears to induce mild dose-related physiological changes at inspired levels of 30% or more, indicative of an anesthetic effect. These data suggest that trifluoromethane may be safe to use in humans, without significant adverse acute effects, at an inspired level of 30%. 23 refs., 3 figs., 3 tabs.

  8. Acute oxidative stress and systemic Nrf2 activation by the ketogenic diet.

    PubMed

    Milder, Julie B; Liang, Li-Ping; Patel, Manisha

    2010-10-01

    The mechanisms underlying the efficacy of the ketogenic diet (KD) remain unknown. Recently, we showed that the KD increased glutathione (GSH) biosynthesis. Since the NF E2-related factor 2 (Nrf2) transcription factor is a primary responder to cellular stress and can upregulate GSH biosynthesis, we asked whether the KD activates the Nrf2 pathway. Here we report that rats consuming a KD show acute production of H(2)O(2) from hippocampal mitochondria, which decreases below control levels by 3 weeks, suggestive of an adaptive response. 4-Hydroxy-2-nonenal (4-HNE), an electrophilic lipid peroxidation end product known to activate the Nrf2 detoxification pathway, was also acutely increased by the KD. Nrf2 nuclear accumulation was evident in both the hippocampus and liver, and the Nrf2 target, NAD(P)H:quinone oxidoreductase (NQO1), exhibited increased activity in both the hippocampus and liver after 3 weeks. We also found chronic depletion of liver tissue GSH, while liver mitochondrial antioxidant capacity was preserved. These data suggest that the KD initially produces mild oxidative and electrophilic stress, which may systemically activate the Nrf2 pathway via redox signaling, leading to chronic cellular adaptation, induction of protective proteins, and improvement of the mitochondrial redox state.

  9. Pediatric Medical Care System in China Has Significantly Reduced Abandonment of Acute Lymphoblastic Leukemia Treatment

    PubMed Central

    Zhou, Qi; Hong, Dan; Lu, Jun; Zheng, Defei; Ashwani, Neetica

    2015-01-01

    In this study, we have analyzed both administrative and clinical data from our hospital during 2002 to 2012 to evaluate the influence of government medical policies on reducing abandonment treatment in pediatric patients with acute lymphoblastic leukemia. Two policies funding for the catastrophic diseases and the new rural cooperative medical care system (NRCMS) were initiated in 2005 and 2011, respectively. About 1151 children diagnosed with acute lymphoblastic leukemia were enrolled in our study during this period and 316 cases abandoned treatment. Statistical differences in sex, age, number of children in the family, and family financial status were observed. Of most importance, the medical insurance coverage was critical for reducing abandonment treatment. However, 92 cases abandoning treatment after relapse did not show significant difference either in medical insurance coverage or in duration from first complete remission. In conclusion, financial crisis was the main reason for abandoning treatment. Government-funded health care expenditure programs reduced families’ economic burden and thereby reduced the abandonment rate with resultant increased overall survival. PMID:25393454

  10. Acute motor-sensory axonal neuropathy associated with active systemic lupus erythematosus and anticardiolipin antibodies.

    PubMed

    Ubogu, E E; Zaidat, O O; Suarez, J I

    2001-10-01

    Acute motor-sensory axonal neuropathy (AMSAN) is an axonal variant of Guillian-Barré syndrome (GBS) that presents with acute ascending quadriparesis. This has generally been described in association with Campylobacter jejuni infections or with anti-ganglioside antibodies. Known cases have shown a slow recovery and a poor prognosis. We report a case with clinical and electrophysiological evidence of AMSAN in association with active systemic lupus erythematosus (SLE) and anticardiolipin antibodies but not the other associations, with a rapid response to combination immunosuppressant and intravenous immunoglobulin (IVIg) therapy. The association between AMSAN and SLE has not been previously described. This case illustrates that early recognition and the utilization of electrophysiologic techniques may be beneficial in the diagnosis and management of GBS associated with SLE. Fulminant or rapidly progressive cases should be managed in specialized intensive care units. Combination therapy of immunosuppressants and IVIg may be beneficial in non-vasculitic axonal radiculo-neuropathies associated with SLE, resulting in good outcomes.

  11. Systemic thrombolysis with recombinant tissue plasminogen activator in acute ischemic stroke: first Croatian experiences.

    PubMed

    Matijević, Vesna; Alvir, Domagoj; Malojčić, Branko; Unušić, Lea; Supe, Svjetlana; Boban, Marina; Bujan-Kovač, Andrea; Habek, Mario; Poljaković, Zdravka

    2010-12-01

    In September 2003, recombinant tissue plasminogen activator (rt-PA) for acute treatment of ischemic stroke was finally approved by the Croatian Ministry of Health. For the next 5 years, only three stroke units in the country implemented this therapy in their routine practice until summer 2008, when neurological wards in most Croatian hospitals started to treat acute stroke patients with systemic thrombolysis. We present a 2-year experience of thrombolytic therapy (2006-2008) in the stroke unit of the University Hospital in Zagreb, Croatian largest hospital, serving nearly one-fifth of the citizens of Croatia. Obtained data (vitals at admission and before administration of rt-PA; NIHSS and MRS scores at admission, 2 h and 7th day after rt-PA treatment, "time to door" and "door to needle" intervals, duration of hospital treatment as well as outcomes and complications of our 66 thrombolysed patients) are presented and discussed. We also present our results regarding benefits of this therapy as well as possible reasons for complications noticed.

  12. Photoprotective effect and acute oral systemic toxicity evaluation of the novel heterocyclic compound LQFM048.

    PubMed

    Vinhal, Daniela C; de Ávila, Renato Ivan; Vieira, Marcelo S; Luzin, Rangel M; Quintino, Michelle P; Nunes, Liliane M; Ribeiro, Antonio Carlos Chaves; de Camargo, Henrique Santiago; Pinto, Angelo C; Dos Santos Júnior, Helvécio M; Chiari, Bruna G; Isaac, Vera; Valadares, Marize C; Martins, Tatiana Duque; Lião, Luciano M; de S Gil, Eric; Menegatti, Ricardo

    2016-08-01

    The new heterocyclic derivative LQFM048 (3) (2,4,6-tris ((E)-ethyl 2-cyano-3-(4-hydroxy-3-methoxyphenyl)acrylate)-1,3,5-triazine) was originally designed through the molecular hybridization strategy from Uvinul® T 150 (1) and (E)-ethyl 2-cyano-3-(4hydroxy-3-methoxyphenyl)acrylate (2) sunscreens, using green chemistry approach. This compound was obtained in global yields (80%) and showed an interesting redox potential. In addition, it is thermally stable up to temperatures around 250°C. It was observed that LQFM048 (3) showed a low degradation after 150min of sunlight exposure at 39°C, whereas the extreme radiation conditions induced a considerable photodegradation of the LQFM048 (3), especially when irradiated by VIS and VIS+UVA. During the determination of sun protection factor, LQFM048 (3) showed interesting results, specially as in association with other photoprotective compounds and commercial sunscreen. Additionally, the compound (3) did not promote cytotoxicity for 3T3 fibroblasts. Moreover, it was not able to trigger acute oral systemic toxicity in mice, being classified as a compound with low acute toxicity hazard (2.000mg/kg>LD50<5.000mg/kg). Therefore, this compound synthesized using green chemistry approach is promising showing potential to development of a new sunscreen product with advantage of presenting redox potential, indicating antioxidant properties.

  13. A microfluidic device for continuous sensing of systemic acute toxicants in drinking water.

    PubMed

    Zhao, Xinyan; Dong, Tao

    2013-12-03

    A bioluminescent-cell-based microfluidic device for sensing toxicants in drinking water was designed and fabricated. The system employed Vibrio fischeri cells as broad-spectrum sensors to monitor potential systemic cell toxicants in water, such as heavy metal ions and phenol. Specifically, the chip was designed for continuous detection. The chip design included two counter-flow micromixers, a T-junction droplet generator and six spiral microchannels. The cell suspension and water sample were introduced into the micromixers and dispersed into droplets in the air flow. This guaranteed sufficient oxygen supply for the cell sensors. Copper (Cu2+), zinc (Zn2+), potassium dichromate and 3,5-dichlorophenol were selected as typical toxicants to validate the sensing system. Preliminary tests verified that the system was an effective screening tool for acute toxicants although it could not recognize or quantify specific toxicants. A distinct non-linear relationship was observed between the zinc ion concentration and the Relative Luminescence Units (RLU) obtained during testing. Thus, the concentration of simple toxic chemicals in water can be roughly estimated by this system. The proposed device shows great promise for an early warning system for water safety.

  14. Acute Gastrointestinal Illness Risks in North Carolina Community Water Systems: A Methodological Comparison.

    PubMed

    DeFelice, Nicholas B; Johnston, Jill E; Gibson, Jacqueline MacDonald

    2015-08-18

    The magnitude and spatial variability of acute gastrointestinal illness (AGI) cases attributable to microbial contamination of U.S. community drinking water systems are not well characterized. We compared three approaches (drinking water attributable risk, quantitative microbial risk assessment, and population intervention model) to estimate the annual number of emergency department visits for AGI attributable to microorganisms in North Carolina community water systems. All three methods used 2007-2013 water monitoring and emergency department data obtained from state agencies. The drinking water attributable risk method, which was the basis for previous U.S. Environmental Protection Agency national risk assessments, estimated that 7.9% of annual emergency department visits for AGI are attributable to microbial contamination of community water systems. However, the other methods' estimates were more than 2 orders of magnitude lower, each attributing 0.047% of annual emergency department visits for AGI to community water system contamination. The differences in results between the drinking water attributable risk method, which has been the main basis for previous national risk estimates, and the other two approaches highlight the need to improve methods for estimating endemic waterborne disease risks, in order to prioritize investments to improve community drinking water systems.

  15. Molecular Analysis of Central Nervous System Disease Spectrum in Childhood Acute Lymphoblastic Leukemia

    PubMed Central

    Hicks, Chindo; Sitthi-Amorn, Jitsuda; Douglas, Jessica; Ramani, Ritika; Miele, Lucio; Vijayakumar, Vani; Karlson, Cynthia; Chipeta, James; Megason, Gail

    2016-01-01

    Treatment of the central nervous system (CNS) is an essential therapeutic component in childhood acute lymphoblastic leukemia (ALL). The goal of this study was to identify molecular signatures distinguishing patients with CNS disease from those without the disease in pediatric patients with ALL. We analyzed gene expression data from 207 pediatric patients with ALL. Patients without CNS were classified as CNS1, while those with mild and advanced CNS disease were classified as CNS2 and CNS3, respectively. We compared gene expression levels among the three disease classes. We identified gene signatures distinguishing the three disease classes. Pathway analysis revealed molecular networks and biological pathways dysregulated in response to CNS disease involvement. The identified pathways included the ILK, WNT, B-cell receptor, AMPK, ERK5, and JAK signaling pathways. The results demonstrate that transcription profiling could be used to stratify patients to guide therapeutic decision-making in pediatric ALL. PMID:26997880

  16. Molecular evidence for central nervous system involvement in children with newly diagnosed acute lymphoblastic leukemia.

    PubMed

    Januszkiewicz, D A; Nowak, J S

    1995-01-01

    Central nervous system (CNS) involvement in children with newly diagnosed acute lymphoblastic leukemia (ALL) would have profound implication for the prognosis and accurate stratification of CNS prophylactic therapy. Using PCR technique with specific primers for V, D and J segments of TCRD gene, the pattern of TCRD gene rearrangements in bone marrow lymphoblasts and in cells from cerebrospinal fluid (CSF) have been investigated. The study involved 21 children at the time of diagnosis with B-lineage ALL. In nine of 21 patients incomplete TCRDVD gene rearrangement has been found in CSF cells, which was identical to that observed in bone marrow of the same children. It can be concluded that at least in 43 per cent of all analysed cases, there were signs of CNS involvement in newly diagnosed ALL patients.

  17. Stroke navigator--a clinical decision support system for acute stroke.

    PubMed

    van Zon, Kees; Lord, William P; Lagor, Charles; Theiss, Stephan; Brosig, Torge; Siebler, Mario

    2008-11-06

    The Stroke Navigator is a clinical decision support system aimed at improving the diagnosis and treatment of acute stroke. It combines an audit trail, a differential diagnosis window, an interactive stroke protocol map, and a list of recommendations for hospital staff. It provides a patient-specific overview of the workflow status and of the available clinical findings, with the goal of improving the continuity of care. For this purpose, it uses a workflow engine that was specifically designed to meet the demands of clinical practice. The Stroke Navigator furthermore calculates and displays the probabilities of various stroke differential diagnoses. The demonstration will introduce these and other features by means of a hypothetical patient case. It will also summarize the status of alpha-testing the first prototype.

  18. Systemic Atherosclerotic Inflammation Following Acute Myocardial Infarction: Myocardial Infarction Begets Myocardial Infarction

    PubMed Central

    Joshi, Nikhil V; Toor, Iqbal; Shah, Anoop S V; Carruthers, Kathryn; Vesey, Alex T; Alam, Shirjel R; Sills, Andrew; Hoo, Teng Y; Melville, Adam J; Langlands, Sarah P; Jenkins, William S A; Uren, Neal G; Mills, Nicholas L; Fletcher, Alison M; van Beek, Edwin J R; Rudd, James H F; Fox, Keith A A; Dweck, Marc R; Newby, David E

    2015-01-01

    Background Preclinical data suggest that an acute inflammatory response following myocardial infarction (MI) accelerates systemic atherosclerosis. Using combined positron emission and computed tomography, we investigated whether this phenomenon occurs in humans. Methods and Results Overall, 40 patients with MI and 40 with stable angina underwent thoracic 18F-fluorodeoxyglucose combined positron emission and computed tomography scan. Radiotracer uptake was measured in aortic atheroma and nonvascular tissue (paraspinal muscle). In 1003 patients enrolled in the Global Registry of Acute Coronary Events, we assessed whether infarct size predicted early (≤30 days) and late (>30 days) recurrent coronary events. Compared with patients with stable angina, patients with MI had higher aortic 18F-fluorodeoxyglucose uptake (tissue-to-background ratio 2.15±0.30 versus 1.84±0.18, P<0.0001) and plasma C-reactive protein concentrations (6.50 [2.00 to 12.75] versus 2.00 [0.50 to 4.00] mg/dL, P=0.0005) despite having similar aortic (P=0.12) and less coronary (P=0.006) atherosclerotic burden and similar paraspinal muscular 18F-fluorodeoxyglucose uptake (P=0.52). Patients with ST-segment elevation MI had larger infarcts (peak plasma troponin 32 300 [10 200 to >50 000] versus 3800 [1000 to 9200] ng/L, P<0.0001) and greater aortic 18F-fluorodeoxyglucose uptake (2.24±0.32 versus 2.02±0.21, P=0.03) than those with non–ST-segment elevation MI. Peak plasma troponin concentrations correlated with aortic 18F-fluorodeoxyglucose uptake (r=0.43, P=0.01) and, on multivariate analysis, independently predicted early (tertile 3 versus tertile 1: relative risk 4.40 [95% CI 1.90 to 10.19], P=0.001), but not late, recurrent MI. Conclusions The presence and extent of MI is associated with increased aortic atherosclerotic inflammation and early recurrent MI. This finding supports the hypothesis that acute MI exacerbates systemic atherosclerotic inflammation and remote plaque destabilization

  19. Incidence and risk factors for central nervous system relapse in children and adolescents with acute lymphoblastic leukemia

    PubMed Central

    Cancela, Camila Silva Peres; Murao, Mitiko; Viana, Marcos Borato; de Oliveira, Benigna Maria

    2012-01-01

    Background Despite all the advances in the treatment of childhood acute lymphoblastic leukemia, central nervous system relapse remains an important obstacle to curing these patients. This study analyzed the incidence of central nervous system relapse and the risk factors for its occurrence in children and adolescents with acute lymphoblastic leukemia. Methods This study has a retrospective cohort design. The studied population comprised 199 children and adolescents with a diagnosis of acute lymphoblastic leukemia followed up at Hospital das Clinicas, Universidade Federal de Minas Gerais (HC-UFMG) between March 2001 and August 2009 and submitted to the Grupo Brasileiro de Tratamento de Leucemia da Infância - acute lymphoblastic leukemia (GBTLI-LLA-99) treatment protocol. Results The estimated probabilities of overall survival and event free survival at 5 years were 69.5% (± 3.6%) and 58.8% (± 4.0%), respectively. The cumulative incidence of central nervous system (isolated or combined) relapse was 11.0% at 8 years. The estimated rate of isolated central nervous system relapse at 8 years was 6.8%. In patients with a blood leukocyte count at diagnosis ≥ 50 x 109/L, the estimated rate of isolated or combined central nervous system relapse was higher than in the group with a count < 50 x 109/L (p-value = 0.0008). There was no difference in cumulative central nervous system relapse (isolated or combined) for the other analyzed variables: immunophenotype, traumatic lumbar puncture, interval between diagnosis and first lumbar puncture and place where the procedure was performed. Conclusions These results suggest that a leukocyte count > 50 x 109/L at diagnosis seems to be a significant prognostic factor for a higher incidence of central nervous system relapse in childhood acute lymphoblastic leukemia. PMID:23323068

  20. Septic vasculitis and vasculopathy in some infectious emergencies: the perspective of the histopathologist.

    PubMed

    Tomasini, C

    2015-02-01

    Sepsis is a potentially life-threatening complication of an infection where cutaneous lesions often represent one of the early signs. A myriad of microorganisms including bacteria, fungi, yeasts, viruses, protozoas, helminths and algae can be implicated. A broad spectrum of clinical and histopathologic findings can be observed in the skin and the common denominator is a thrombotic vasculopathy. The pathogenesis of cutaneous septic vasculitis (SV)/vasculopathy is complex and includes five main mechanisms: disseminated intravascular coagulation, direct invasion and occlusion of blood vessel walls by microorganisms, hypersensitivity reaction with immune complex deposition into blood vessel walls, embolism from a distant infectious site and vascular effects of toxins. Herein we describe the clinicopathologic findings of some selected cases of SV recently observed in our hospital, including purpura fulminans, necrotizing fasciitis, cutaneous meningococcemia, malignant syphilis and disseminated alternaria infection. Histopathologically, a wide spectrum of histopathologic changes was observed in skin specimens from the various entities, involving the intensity and composition of the inflammatory infiltrate, the degree of vascular changes and the presence of microorganisms, that ranged from a predominant not inflammatory, thrombotic-occlusive vasculopathy in purpura fulminans to leukocytoclastic vasculitis like changes in cutaneous meningococcemia to a dermal angiomatosis-like pattern in disseminated Alternaria infection. The different pathologic presentations may be related to the microorganism involved, the main pathogenetic mechanism that induced the vascular injury and the individual immunologic burden. Early skin biopsy for histopathologic examination and microbiologic culture is a cornerstone in the diagnosis of life-threatening diseases that present with cutaneous septic vasculitis. Ancillary techniques, such as immunohistochemistry and polymerase chain reaction are

  1. Diffuse alveolar haemorrhage secondary to propylthiouracil-induced vasculitis

    PubMed Central

    Ferreira, Catarina; Costa, Teresa; Marques, Ana Vieira

    2015-01-01

    Propylthiouracil is a drug used to treat hyperthyroidism. It can cause several side effects including pulmonary disorders that, although rare, can be severe. The authors describe the case of a woman treated with propylthiouracil who developed diffuse alveolar haemorrhage with severe respiratory failure and anaemia, which improved with discontinuation of the antithyroid drug and on starting systemic corticosteroid therapy. PMID:25661751

  2. Complement C3 is expressed by mast cells in cutaneous vasculitis and is degraded by chymase.

    PubMed

    Lipitsä, Tiina; Naukkarinen, Anita; Laitala, Joel; Harvima, Ilkka T

    2016-10-01

    The complement factor C3 and chymase released from tryptase(+), chymase(+) mast cells may be involved in the pathogenesis of cutaneous leukocytoclastic vasculitis. To study whether mast cells contain C3 in vasculitis and whether chymase interacts with C3, cryosections from vasculitis biopsies were double-stained histochemically for C3c in tryptase(+) mast cells, as well as for chymase and vessel wall C3c, or they were treated with 5 µg/ml rh-chymase for 24 h followed by immunofluorescence (IF) analysis of C3c, IgG, IgM and IgA. The effect of rh-chymase on purified human C3, C3a and IgG was studied using SDS-PAGE electrophoresis and LAD2 mast cell cultures. The results show that 34.2 ± 17.9, 37.4 ± 15.5 and 43.4 ± 18.6 % (mean ± SD) of the mast cells express C3c immunoreactivity in the healthy skin, initial petechial (IP) and palpable purpura (PP) lesions, respectively. About 9.4-12.1 % of the chymase(+) mast cells were in apparent contact with C3c(+) vessels in IP and PP. The treatment of cryosections with rh-chymase decreased the IF staining of C3c, but not that of immunoglobulins. In SDS-PAGE, 1-10 µg/ml rh-chymase degraded the alpha- and beta-chains of C3, but did not degrade IgG. Unexpectedly, the rh-chymase treatment of C3 produced fragments that resulted in the release of tryptase and histamine from LAD2 cells. However, rh-chymase degraded C3a and consequently inhibited C3a activity on LAD2. In conclusion, mast cells can be one source for C3 in the early and late phases of vasculitis pathogenesis. However, rh-chymase degraded native C3, vessel wall C3c, and biologically active C3a. Therefore, chymase may control C3-related pathology.

  3. Severe meningeal fibrinoid vasculitis associated with Theileria taurotragi infection in two short-horned Zebu cattle.

    PubMed

    Biasibetti, Elena; Sferra, Chiara; Lynen, Godelieve; Di Giulio, Giuseppe; De Meneghi, Daniele; Tomassone, Laura; Valenza, Federico; Capucchio, Maria Teresa

    2016-08-01

    The Authors describe a severe vasculitis with fibrinoid necrosis of the meningeal arteries observed in two brains of indigenous short-horn zebu (Bos indicus) cattle, with bovine cerebral theileriosis (BCT) caused by a tick-transmitted hemoprotozoan, Theileria taurotragi, from Northern Tanzania. In the Author's opinion, the role of T. taurotragi infection in the angiocentric and angiodestructive detected features remains to be evaluated. A possible immunopathologic cancerous mechanism, secondary to the lymphoid deregulation, could be involved. This report suggests further studies to better characterize the lymphoid cell involvement in the pathogenesis of the meningeal vascular lesions by T. taurotragi.

  4. Recurrence of ANCA-associated vasculitis in a patient with kidney trasplant.

    PubMed

    García Cosmes, Pedro; Fraile Gómez, Pilar; Lewczuk, Kamil; Rodríguez González, Marta; Ruiz Ferreras, Elena; Tabernero Fernández, Guadalupe

    2016-01-01

    Renal disease secondary to vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCA) can lead to chronic renal disease requiring renal replacement therapy. In these patients, kidney transplantation offers excellent long-term rates of allograft and patient survival; consequently, they can be trasplanted when the clinical disease activity has remitted. However, the risk of disease relapses in the renal allograft remains, although at lower rates due to modern immunosuppressive regimens. We describe the case of a male patient with extracapillary glomerulonephritis type III C-ANCA (+) who developed a recurrence in the renal allograft 8 years after transplantation. Intensive immunosupression with plasmapheresis controlled the disease.

  5. Infected Aortic Aneurysm Mimicking Anti-proteinase 3-Antineutrophil Cytoplasmic Antibody-associated Vasculitis

    PubMed Central

    Hachiya, Kenta; Wakami, Kazuaki; Yoshida, Atsuhiro; Suda, Hisao; Ohte, Nobuyuki

    2016-01-01

    We herein report an unusual case of an infected descending aortic pseudoaneurysm with luminal pathognomonic oscillating vegetation with serological findings and clinical features mimicking anti-proteinase 3-antineutrophil cytoplasmic antibody-associated vasculitis. The positive blood cultures and imaging findings, including a pseudoaneurysm and vegetations in the aorta, suggested the presence of an infected aortic aneurysm. The patient was successfully treated with antibiotics and endovascular aortic repair. A precise diagnosis is crucial in order to avoid inappropriate therapy such as immunosuppressive treatment, which could result in life-threatening consequences in a patient with an infected aortic aneurysm. PMID:27904110

  6. ANCA-associated vasculitis with dual ANCA positivity in coexistence with mixed connective tissue disease.

    PubMed

    Murakami, Masanori; Shimane, Kenichi; Takahashi, Hiroshi; Tomiyama, Junji; Nagashima, Masakazu

    2013-01-01

    We here report a rare case of dual antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in a 38-year-old Japanese woman previously diagnosed with mixed connective tissue disease. The patient was found to be positive for myeloperoxidase- and proteinase 3-ANCA, and was diagnosed with AAV following admission to hospital with fervescence, polyarthralgia, purpura, and asymmetric numbness of the extremities. Examination of her genetic background revealed that she carried HLA-DR9, which confers risk of both diseases in Japanese populations.

  7. Effects of systemic administration of sitafloxacin on subgingival microflora and antimicrobial susceptibility profile in acute periodontal lesions.

    PubMed

    Tomita, Sachiyo; Kasai, Shunsuke; Ihara, Yuichiro; Imamura, Kentaro; Kita, Daichi; Ota, Koki; Kinumatsu, Takashi; Nakagawa, Taneaki; Saito, Atsushi

    2014-01-01

    The aim of this study was to assess the effect(s) of systemic administration of sitafloxacin on subgingival microbial profiles of acute periodontal lesions. Antimicrobial susceptibility of clinical isolates was also investigated. Patients with acute phases of chronic periodontitis were subjected to clinical examination and microbiological assessment of their subgingival plaque samples by culture technique. Sitafloxacin was then administered (100 mg/day for 5 days) systemically. The clinical and microbiological examinations were repeated 6-8 days after administration. Susceptibilities of clinical isolates to various antimicrobials were determined using the broth and agar dilution methods. From the sampled sites in 30 participants, a total of 355 clinical isolates (34 different bacterial species) were isolated and identified. Parvimonas micra, Prevotella intermedia and Streptococcus mitis were the most prevalent cultivable bacteria in acute sites. Systemic administration of sitafloxacin yielded a significant improvement in clinical and microbiological parameters. Among the antimicrobials tested, sitafloxacin was the most potent against the clinical isolates with an MIC90 of 0.12 μg/ml at baseline. After administration, most clinical isolates were still highly susceptible to sitafloxacin although some increase in MICs was observed. The results suggest that systemic administration of sitafloxacin is effective against subgingival bacteria isolated from acute periodontal lesions.

  8. Exploring the Relationships between the Electronic Health Record System Components and Patient Outcomes in an Acute Hospital Setting

    ERIC Educational Resources Information Center

    Wiggley, Shirley L.

    2011-01-01

    Purpose: The purpose of this study was to examine the relationship between the electronic health record system components and patient outcomes in an acute hospital setting, given that the current presidential administration has earmarked nearly $50 billion to the implementation of the electronic health record. The relationship between the…

  9. Peripheral Vasculitis, Intermediate Uveitis and Interferon Use in Multiple Sclerosis

    PubMed Central

    Kinyas, Şeref; Esgin, Haluk

    2016-01-01

    Multiple sclerosis (MS) is a chronic inflammatory demyelinating disease of the central nervous system. A 40-year-old female patient with a 12-year history of MS was admitted to our clinic with blurred vision and floaters in her right eye for about 1 month. Here, we share the findings and the management of intermediate uveitis and retinal periphlebitis in an MS case being treated with interferon beta-1a for 7 years. PMID:27800257

  10. Central Nervous System Involvement in Adult Acute Lymphoblastic Leukemia: Diagnostic Tools, Prophylaxis, and Therapy

    PubMed Central

    Del Principe, Maria Ilaria; Maurillo, Luca; Buccisano, Francesco; Sconocchia, Giuseppe; Cefalo, Mariagiovanna; De Santis, Giovanna; Di Veroli, Ambra; Ditto, Concetta; Nasso, Daniela; Postorino, Massimiliano; Refrigeri, Marco; Attrotto, Cristina; Del Poeta, Giovanni; Lo-Coco, Francesco; Amadori, Sergio; Venditti, Adriano

    2014-01-01

    In adult patients with acute lymphoblastic leukemia (ALL), Central Nervous System (CNS) involvement is associated with a very poor prognosis. The diagnostic assessment of this condition relies on the use of neuroradiology, conventional cytology (CC) and flow cytometry (FCM). Among these approaches, which is the gold standard it is still a matter of debate. Neuroradiology and CC have a limited sensitivity with a higher rate of false negative results. FCM demonstrated a superior sensitivity over CC, particularly when low levels of CNS infiltrating cells are present. Although prospective studies of a large series of patients are still awaited, a positive finding by FCM appears to anticipate an adverse outcome even if CC shows no infiltration. Current strategies for adult ALL CNS-directed prophylaxis or therapy involve systemic and intrathecal chemotherapy and radiation therapy. An early and frequent intrathecal injection of cytostatic combined with systemic chemotherapy is the most effective strategy to reduce the frequency of CNS involvement. In patients with CNS overt ALL, at diagnosis or upon relapse, allogeneic hematopoietic stem cell transplantation might be considered. This review discusses risk factors, diagnostic techniques for identification of CNS infiltration and modalities of prophylaxis and therapy to manage it. PMID:25408861

  11. A novel high channel-count system for acute multisite neuronal recordings.

    PubMed

    Hofmann, Ulrich G; Folkers, Andre; Mösch, Florian; Malina, Thomas; Menne, Kerstin M L; Biella, Gerardo; Fagerstedt, Patriq; De Schutter, Erik; Jensen, Winnie; Yoshida, Ken; Hoehl, Dirk; Thomas, Uwe; Kindlundh, Maria G; Norlin, Peter; de Curtis, Marco

    2006-08-01

    Multisite recording represents a suitable condition to study microphysiology and network interactions in the central nervous system and, therefore, to understand brain functions. Several different materials and array configurations have been proposed for the development of new probes utilized to record brain activity from experimental animal models. We describe new multisite silicon probes that broaden the currently available application base for neuroscientists. The array arrangement of the probes recording sites was extended to increase their spatial resolution. Probes were integrated with a newly developed electronic hardware and novel software for advanced real-time processing and analysis. The new system, based on 32- and 64-electrode silicon probes, proved very valuable to record field potentials and single unit activity from the olfactory-limbic cortex of the in vitro isolated guinea-pig brain preparation and to acutely record unit activity at multiple sites from the cerebellar cortex in vivo. The potential advantages of the new system in comparison to the currently available technology are discussed.

  12. Lung volume recruitment acutely increases respiratory system compliance in individuals with severe respiratory muscle weakness

    PubMed Central

    Molgat-Seon, Yannick; Hannan, Liam M.; Dominelli, Paolo B.; Peters, Carli M.; Fougere, Renee J.; McKim, Douglas A.; Sheel, A. William

    2017-01-01

    The aim of the present study was to determine whether lung volume recruitment (LVR) acutely increases respiratory system compliance (Crs) in individuals with severe respiratory muscle weakness (RMW). Individuals with RMW resulting from neuromuscular disease or quadriplegia (n=12) and healthy controls (n=12) underwent pulmonary function testing and the measurement of Crs at baseline, immediately after, 1 h after and 2 h after a single standardised session of LVR. The LVR session involved 10 consecutive supramaximal lung inflations with a manual resuscitation bag to the highest tolerable mouth pressure or a maximum of 50 cmH2O. Each LVR inflation was followed by brief breath-hold and a maximal expiration to residual volume. At baseline, individuals with RMW had lower Crs than controls (37±5 cmH2O versus 109±10 mL·cmH2O−1, p<0.001). Immediately after LVR, Crs increased by 39.5±9.8% to 50±7 mL·cmH2O−1 in individuals with RMW (p<0.05), while no significant change occurred in controls (p=0.23). At 1 h and 2 h post-treatment, there were no within-group differences in Crs compared to baseline (all p>0.05). LVR had no significant effect on measures of pulmonary function at any time point in either group (all p>0.05). During inflations, mean arterial pressure decreased significantly relative to baseline by 10.4±2.8 mmHg and 17.3±3.0 mmHg in individuals with RMW and controls, respectively (both p<0.05). LVR acutely increases Crs in individuals with RMW. However, the high airway pressures during inflations cause reductions in mean arterial pressure that should be considered when applying this technique. PMID:28326313

  13. Acute Coronary Syndrome Treatment Costs from the Perspective of the Supplementary Health System

    PubMed Central

    Teich, Vanessa; Piha, Tony; Fahham, Lucas; Squiassi, Haline Bianca; Paloni, Everton de Matos; Miranda, Paulo; Araújo, Denizar Vianna

    2015-01-01

    Background Acute coronary syndrome (ACS) is defined as a “group of clinical symptoms compatible with acute myocardial ischemia”, representing the leading cause of death worldwide, with a high clinical and financial impact. In this sense, the development of economic studies assessing the costs related to the treatment of ACS should be considered. Objective To evaluate costs and length of hospital stay between groups of patients treated for ACS undergoing angioplasty with or without stent implantation (stent+ / stent-), coronary artery bypass surgery (CABG) and treated only clinically (Clinical) from the perspective of the Brazilian Supplementary Health System (SHS). Methods A retrospective analysis of medical claims of beneficiaries of health plans was performed considering hospitalization costs and length of hospital stay for management of patients undergoing different types of treatment for ACS, between Jan/2010 and Jun/2012. Results The average costs per patient were R$ 18,261.77, R$ 30,611.07, R$ 37,454.94 and R$ 40,883.37 in the following groups: Clinical, stent-, stent+ and CABG, respectively. The average costs per day of hospitalization were R$ 1,987.03, R$ 4,024.72, R$ 6,033.40 and R$ 2,663.82, respectively. The average results for length of stay were 9.19 days, 7.61 days, 6.19 days and 15.20 days in these same groups. The differences were significant between all groups except Clinical and stent- and between stent + and CABG groups for cost analysis. Conclusion Hospitalization costs of SCA are high in the Brazilian SHS, being significantly higher when interventional procedures are required. PMID:26559980

  14. Gamma interferon expression during acute and latent nervous system infection by herpes simplex virus type 1.

    PubMed Central

    Cantin, E M; Hinton, D R; Chen, J; Openshaw, H

    1995-01-01

    This study was initiated to evaluate a role for gamma interferon (IFN-gamma) in herpes simplex virus type 1 (HSV-1) infection. At the acute stage of infection in mice, HSV-1 replication in trigeminal ganglia and brain stem tissue was modestly but consistently enhanced in mice from which IFN-gamma was by ablated monoclonal antibody treatment and in mice genetically lacking the IFN-gamma receptor (Rgko mice). As determined by reverse transcriptase PCR, IFN-gamma and tumor necrosis factor alpha transcripts were present in trigeminal ganglia during both acute and latent HSV-1 infection. CD4+ and CD8+ T cells were detected initially in trigeminal ganglia at day 5 after HSV-1 inoculation, and these cells persisted for 6 months into latency. The T cells were focused around morphologically normal neurons that showed no signs of active infection, but many of which expressed HSV-1 latency-associated transcripts. Secreted IFN-gamma was present up to 6 months into latency in areas of the T-cell infiltration. By 9 months into latency, both the T-cell infiltrate and IFN-gamma expression had cleared, although there remained a slight increase in macrophage levels in trigeminal ganglia. In HSV-1-infected brain stem tissue, T cells and IFN-gamma expression were present at 1 month but were gone by 6 months after infection. Our hypothesis is that the persistence of T cells and the sustained IFN-gamma expression occur in response to an HSV-1 antigen(s) in the nervous system. This hypothesis is consistent with a new model of HSV-1 latency which suggests that limited HSV-1 antigen expression occurs during latency (M. Kosz-Vnenchak, J. Jacobson, D.M. Coen, and D.M. Knipe, J. Virol. 67:5383-5393, 1993). We speculate that prolonged secretion of IFN-gamma during latency may modulate a reactivated HSV-1 infection. PMID:7609058

  15. Mesotherapy versus Systemic Therapy in the Treatment of Acute Low Back Pain: A Randomized Trial

    PubMed Central

    Costantino, Cosimo; Marangio, Emilio; Coruzzi, Gabriella

    2011-01-01

    Pharmacological therapy of back pain with analgesics and anti-inflammatory drugs is frequently associated with adverse effects, particularly in the elderly. Aim of this study was to compare mesotherapic versus conventional systemic administration of nonsteroidal anti-inflammatory drugs (NSAIDs) and corticosteroids in patients with acute low back pain. Eighty-four patients were randomized to receive anti-inflammatory therapy according to the following protocols: (a) mesotherapy group received the 1st and 4th day 2% lidocaine (1 mL) + ketoprofen 160 mg (1 mL) + methylprednisolone 40 mg (1 mL), then on 7th, 10th, and 13th day, 2% lidocaine (1 mL) + ketoprofen 160 mg (1 mL) + methylprednisolone 20 mg (1 mL) (b) conventional therapy group received ketoprofen 80 mg × 2/die and esomeprazole 20 mg/die orally for 12 days, methylprednisolone 40 mg/die intramuscularly for 4 days, followed by methylprednisolone 20 mg/die for 3 days, and thereafter, methylprednisolone 20 mg/die at alternate days. Pain intensity and functional disability were assessed at baseline (T0), at the end of treatment (T1), and 6 months thereafter (T2) by using visual analogic scale (VAS) and Roland-Morris disability questionnaire (RMDQ). In both groups, VAS and RMDQ values were significantly reduced at the end of drug treatment and after 6 months, in comparison with baseline. No significant differences were found between the two groups. This suggests that mesotherapy may be a valid alternative to conventional therapy in the treatment of acute low back pain with corticosteroids and NSAIDs. PMID:20953425

  16. Adrenocorticotropic hormone in serial cerebrospinal fluid in man - Subject to acute regulation by the hypothalamic-pituitary-adrenocortical system?

    PubMed

    Kellner, Michael; Wortmann, Viola; Salzwedel, Cornelie; Kober, Daniel; Petzoldt, Martin; Urbanowicz, Tatiana; Pulic, Mersija; Boelmans, Kai; Yassouridis, Alexander; Wiedemann, Klaus

    2016-05-30

    Acute regulation of adrenocorticotropic hormone (ACTH) in cerebrospinal fluid (CSF) by the hypothalamic-pituitary-adrenocortical system has not been investigated in man. In a pilot study in healthy male volunteers we measured ACTH every twenty minutes in serial CSF for three hours after an intravenous placebo, hydrocortisone (100mg) or insulin (2mg/kg) injection. No acute inhibitory or stimulatory effects of these interventions were discovered. Our results corroborate previous findings in rhesus monkeys. The regulation of CSF ACTH and its potential relevance for behavioral alterations in health and disease (e.g. major depression or anorexia nervosa) in humans need further study.

  17. Retinal vasculitis and ocular vitreous metastasis following complete response to PD-1 inhibition in a patient with metastatic cutaneous melanoma.

    PubMed

    Manusow, Joshua S; Khoja, Leila; Pesin, Nataly; Joshua, Anthony M; Mandelcorn, Efrem D

    2014-01-01

    We report on a 36-year-old woman treated with the anti PD-1 antibody Pembrolizumab for metastatic cutaneous melanoma in the first line setting. She achieved a complete response and then relapsed with metastases to the vitreous cavity with an associated angiographically determined retinal vasculitis. Vitreous metastasis without choroidal involvement is unusual and may be due to individual cell extravasation, vitreous hemorrhage containing malignant cells, or direct spread through the optic nerve. This finding highlights the need for immune sanctuary sites to be monitored in the presence of PD-1 inhibition and we hypothesize that the use of PD-1 inhibitor potentiated the patient's angiographically determined retinal vasculitis.

  18. Sodium nitrite induces acute central nervous system toxicity in guinea pigs exposed to systemic cell-free hemoglobin.

    PubMed

    Buehler, Paul W; Butt, Omer I; D'Agnillo, Felice

    2011-06-10

    Systemic cell-free hemoglobin (Hb) released via hemolysis disrupts vascular homeostasis, in part, through the scavenging of nitric oxide (NO). Sodium nitrite (NaNO(2)) therapy can attenuate the hypertensive effects of Hb. However, the chemical reactivity of NaNO(2) with Hb may enhance heme- or iron-mediated toxicities. Here, we investigate the effect of NaNO(2) on the central nervous system (CNS) in guinea pigs exposed to systemic cell-free Hb. Intravascular infusion of NaNO(2), at doses sufficient to alleviate Hb-mediated blood pressure changes, reduced the expression of occludin, but not zona occludens-1 (ZO-1) or claudin-5, in cerebral tight junctions 4h after Hb infusion. This was accompanied by increased perivascular heme oxygenase-1 expression, neuronal iron deposition, increased astrocyte and microglial activation, and reduced expression of neuron-specific nuclear protein (NeuN). These CNS changes were not observed in animals treated with Hb or NaNO(2) alone. Taken together, these findings suggest that the use of nitrite salts to treat systemic Hb exposure may promote acute CNS toxicity.

  19. Atypical systemic lupus erythematosus or Castleman's disease.

    PubMed

    Van de Voorde, K; De Raeve, H; De Block, C E; Van Regenmortel, N; Van Offel, J F; De Clerck, L S; Stevens, W J

    2004-01-01

    Collagen vascular diseases and malignancies have common systemic and immune features. We report a case of a 21 year old female patient with constitutional symptoms, polyserositis, spontaneous rupture of the spleen, leukocytoclastic vasculitis and acute renal failure. The tentative diagnosis of SLE was made because she developed a positive antinuclear factor (1/640), with anti-SSA antibodies and a positive lupus anticoagulans. Two months later a cervical lymphadenopathy occurred while recieving treatment with prednisolone. A lymph node biopsy revealed morphologic features of a SLE, similar to those observed in multicentric Castleman's disease (MCD). MCD is a distinct type of a lymphoproliferative disorder of unknown etiology. The difficulties in differential diagnosis of these two diseases are discussed.

  20. A clinical pathologic study of four adult cases of acute mercury inhalation toxicity

    SciTech Connect

    Kanluen, S.; Gottlieb, C.A. )

    1991-01-01

    We report four cases of fatal mercury vapor inhalation, a rare occurrence. The mercury vapor was released at a private home, where one of the occupants was smelting silver from dental amalgam containing an unknown amount of mercury. Within 24 hours of the incident, all occupants began having shortness of breath necessitating hospital admission. The clinical courses are briefly detailed; however, all included rapid deterioration with respiratory failure. Chest roentgenograms in all four cases were consistent with adult respiratory distress syndrome. All patients were treated with dimercaprol, a mercury chelator, but all died, with survival varying from 9 to 23 days postexposure. Autopsies were performed on all four patients. The lungs in all cases were heavy, firm, and airless. Histologic examination revealed severe diffuse alveolar damage, with variable amounts of fibrosis, conforming with acute lung injury in various stages of organization. Additional postmortem findings included acute proximal renal tubular necrosis, vacuolar hepatoxicity, and a spectrum of central nervous system alterations including multifocal ischemic necrosis, gliosis, and vasculitis.

  1. Sex differences in ischemia/reperfusion-induced acute kidney injury are dependent on the renal sympathetic nervous system.

    PubMed

    Tanaka, Ryosuke; Tsutsui, Hidenobu; Ohkita, Mamoru; Takaoka, Masanori; Yukimura, Tokihito; Matsumura, Yasuo

    2013-08-15

    Resistance to ischemic acute kidney injury has been shown to be higher in female rats than in male rats. We found that renal venous norepinephrine overflow after reperfusion played important roles in the development of ischemic acute kidney injury. In the present study, we investigated whether sex differences in the pathogenesis of ischemic acute kidney injury were derived from the renal sympathetic nervous system using male and female Sprague-Dawley rats. Ischemia/reperfusion-induced acute kidney injury was achieved by clamping the left renal artery and vein for 45 min followed by reperfusion, 2 weeks after contralateral nephrectomy. Renal function was impaired after reperfusion in both male and female rats; however, renal dysfunction and histological damage were more severe in male rats than in female rats. Renal venous plasma norepinephrine levels after reperfusion were markedly elevated in male rats, but were not in female rats. These sex differences were eliminated by ovariectomy or treatment with tamoxifen, an estrogen receptor antagonist, in female rats. Furthermore, an intravenous injection of hexamethonium (25mg/kg), a ganglionic blocker, 5 min before ischemia suppressed the elevation in renal venous plasma norepinephrine levels after reperfusion, and attenuated renal dysfunction and histological damage in male rats, and ovariectomized and tamoxifen-treated female rats, but not in intact females. Thus, the present findings confirmed sex differences in the pathogenesis of ischemic acute kidney injury, and showed that the attenuation of ischemia/reperfusion-induced acute kidney injury observed in intact female rats may be dependent on depressing the renal sympathetic nervous system with endogenous estrogen.

  2. Acute and long-term effects of blood flow restricted training on heat shock proteins and endogenous antioxidant systems.

    PubMed

    Cumming, K T; Ellefsen, S; Rønnestad, B R; Ugelstad, I; Raastad, T

    2016-10-10

    Blood flow restricted exercise (BFRE) with low loads has been demonstrated to induce considerable stress to exercising muscles. Muscle cells have developed a series of defensive systems against exercise-induced stress. However, little is known about acute and long-term effects of BFRE training on these systems. Nine previously untrained females trained low-load BFRE and heavy load strength training (HLS) on separate legs and on separate days to investigate acute and long-term effects on heat shock proteins (HSP) and endogenous antioxidant systems in skeletal muscles. BFRE and HLS increased muscle strength similarly by 12 ± 7% and 12 ± 6%, respectively, after 12 weeks of training. Acutely after the first BFRE and HLS exercise session, αB-crystallin and HSP27 content increased in cytoskeletal structures, accompanied by increased expression of several HSP genes. After 12 weeks of training, this acute HSP response was absent. Basal levels of αB-crystallin, HSP27, HSP70, mnSOD, or GPx1 remained unchanged after 12 weeks of training, but HSP27 levels increased in the cytoskeleton. Marked translocation of HSP to cytoskeletal structures at the commencement of training indicates that these structures are highly stressed from BFRE and HLS. However, as the muscle gets used to this type of exercise, this response is abolished.

  3. Effects of Acute Exposure to Moderate Altitude on Vascular Function, Metabolism and Systemic Inflammation

    PubMed Central

    Stöwhas, Anne-Christin; Latshang, Tsogyal D.; Lo Cascio, Christian M.; Lautwein, Sina; Stadelmann, Katrin; Tesler, Noemi; Ayers, Lisa; Berneis, Kaspar; Gerber, Philipp A.; Huber, Reto; Achermann, Peter; Bloch, Konrad E.; Kohler, Malcolm

    2013-01-01

    Background Travel to mountain areas is popular. However, the effects of acute exposure to moderate altitude on the cardiovascular system and metabolism are largely unknown. Objectives To investigate the effects of acute exposure to moderate altitude on vascular function, metabolism and systemic inflammation. Methods In 51 healthy male subjects with a mean (SD) age of 26.9 (9.3) years, oxygen saturation, blood pressure, heart rate, arterial stiffness, lipid profiles, low density lipoprotein (LDL) particle size, insulin resistance (HOMA-index), highly-sensitive C-reactive protein and pro-inflammatory cytokines were measured at 490 m (Zurich) and during two days at 2590 m, (Davos Jakobshorn, Switzerland) in randomized order. The largest differences in outcomes between the two altitudes are reported. Results Mean (SD) oxygen saturation was significantly lower at 2590 m, 91.0 (2.0)%, compared to 490 m, 96.0 (1.0)%, p<0.001. Mean blood pressure (mean difference +4.8 mmHg, p<0.001) and heart rate (mean difference +3.3 bpm, p<0.001) were significantly higher at 2590 m, compared to 490 m, but this was not associated with increased arterial stiffness. At 2590 m, lipid profiles improved (median difference triglycerides −0.14 mmol/l, p = 0.012, HDL +0.08 mmol/l, p<0.001, total cholesterol/HDL-ratio −0.25, p = 0.001), LDL particle size increased (median difference +0.45 nm, p = 0.048) and hsCRP decreased (median difference −0.18 mg/l, p = 0.024) compared to 490 m. No significant change in pro-inflammatory cytokines or insulin resistance was observed upon ascent to 2590 m. Conclusions Short-term stay at moderate altitude is associated with increased blood pressure and heart rate likely due to augmented sympathetic activity. Exposure to moderate altitude improves the lipid profile and systemic inflammation, but seems to have no significant effect on glucose metabolism. Trial Registration ClinicalTrials.gov NCT01130948 PMID:23936377

  4. [Clinical manifestations of antineutrophil cytoplasmic antibodies associated vasculitis].

    PubMed

    Morović-Vergles, Jadranka; Culo, Melanie-Ivana; Sutić, Anamarija

    2014-01-01

    Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides are rare diseases, with the average of 30 new cases per million inhabitants per year. Their main characteristic is systemic involvement with necrosis of the vessel walls (histological changes showing necrosis of the media and inflammation of adventitia and intima). In some forms granulomas may be found surrounding the vessels. ANCA-associated vasculitides include granulomatosis with polyangiitis (GPA, previously called Wegener's), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA, previously called Churg-Straus). Honorific eponyms are now changing to a disease-descriptive or etiology-based nomenclature. ANCA-associated vasculitides are a distinctive group of vasculitides because they dominantly involve small sized vessels, sometimes even medium sized vessels, are associated with antineutrophil cytoplasmic antibodies with high risk of developing glomerulonephritis and respond well to immunosuppresion with cyclophosphamide.

  5. Diagnostic omission errors in acute paediatric practice: impact of a reminder system on decision-making

    PubMed Central

    Ramnarayan, Padmanabhan; Winrow, Andrew; Coren, Michael; Nanduri, Vasanta; Buchdahl, Roger; Jacobs, Benjamin; Fisher, Helen; Taylor, Paul M; Wyatt, Jeremy C; Britto, Joseph

    2006-01-01

    Background Diagnostic error is a significant problem in specialities characterised by diagnostic uncertainty such as primary care, emergency medicine and paediatrics. Despite wide-spread availability, computerised aids have not been shown to significantly improve diagnostic decision-making in a real world environment, mainly due to the need for prolonged system consultation. In this study performed in the clinical environment, we used a Web-based diagnostic reminder system that provided rapid advice with free text data entry to examine its impact on clinicians' decisions in an acute paediatric setting during assessments characterised by diagnostic uncertainty. Methods Junior doctors working over a 5-month period at four paediatric ambulatory units consulted the Web-based diagnostic aid when they felt the need for diagnostic assistance. Subjects recorded their clinical decisions for patients (differential diagnosis, test-ordering and treatment) before and after system consultation. An expert panel of four paediatric consultants independently suggested clinically significant decisions indicating an appropriate and 'safe' assessment. The primary outcome measure was change in the proportion of 'unsafe' workups by subjects during patient assessment. A more sensitive evaluation of impact was performed using specific validated quality scores. Adverse effects of consultation on decision-making, as well as the additional time spent on system use were examined. Results Subjects attempted to access the diagnostic aid on 595 occasions during the study period (8.6% of all medical assessments); subjects examined diagnostic advice only in 177 episodes (30%). Senior House Officers at hospitals with greater number of available computer workstations in the clinical area were most likely to consult the system, especially out of working hours. Diagnostic workups construed as 'unsafe' occurred in 47/104 cases (45.2%); this reduced to 32.7% following system consultation (McNemar test, p

  6. Experience with a Simplified Computer Based Intensive Care Monitoring System in the Management of Acutely Ill Surgical Patients

    PubMed Central

    Hadley, H. Roger; Rutherford, Harold G.; Smith, Louis L.; Briggs, Burton A.; Neilsen, Ivan R.; Rau, Richard

    1979-01-01

    The need exists for a simplified and ecomonical computer based monitoring system for critically ill surgical patients. Such a system would enjoy widespread use in surgical intensive care units in regional, as well as larger community hospitals. We have assembled such a system which provides digital readout of the usual physiologic parameters, and also provide computer storage of accumulated data for review and evaluation of patient care. The computer provides graphic and digital display and digital printout for subsequent inclusion in the patient records. Most frequent indications for this system include the development of acute respiratory insufficiency or acute circulatory failure due to invasive sepsis and/or severe arteriosclerotic cardiovascular disease. Information most beneficial in patient care included measurement of cardiac output;alveolar arterial oxygen gradient. ImagesFigure 1Figure 5Figure 9Figure 11

  7. The effects of prophylactic treatment of the central nervous system on the intellectual functioning of children with acute lymphocytic leukemia

    SciTech Connect

    Moss, H.A.; Nannis, E.D.; Poplack, D.G.

    1981-07-01

    The effect of central nervous system prophylaxis (cranial radiation and intrathecal chemotherapy) on intellectual function was studied in 24 children with acute lymphocytic leukemia. The Wechsler Intelligence tests were administered to these children and to a sample of their healthy siblings, who served as a comparison group. The mean Full Scale lQ was 98.6 for the patients and 112.5 for the sibling controls (p less than 0.001 level). Those patients who received central nervous system preventive treatment at a young age exhibited a greater decrement in intellectual abilities than did patients who were older when they received this treatment. In contrast, leukemia patients who had not received central nervous system prophylaxis had IQs that did not differ statistically from those of their siblings. These data suggest that central nervous system prophylaxis may have an adverse effect on the intellectual capability of children with acute lymphocytic leukemia.

  8. 75 FR 34614 - Medicare Program; Hospital Inpatient Prospective Payment Systems for Acute Care Hospitals and...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-06-17

    ..., 2010 unless otherwise footnoted).'' c. Third column, the title, ``Table 4J.--Out-Migration Adjustment...) Out-Migration Adjustment for Acute Care Hospitals--FY 2010 (April 1, 2010 through September 30, 2010...: Table 4J--(Abbreviated) Out-Migration Adjustment for Acute Care Hospitals--FY 2010 (April 1,...

  9. Reengineering acute episodic and chronic care delivery: the Geisinger Health System experience.

    PubMed

    Slotkin, Jonathan R; Casale, Alfred S; Steele, Glenn D; Toms, Steven A

    2012-07-01

    Comparative effectiveness research (CER) represents an evolution in clinical decision-making research that allows for the study of heterogeneous groups of patients with complex diseases processes. It has foundations in decision science, reliability science, and health care policy research. Health care finance will increasingly rely on CER for guidance in the coming years. There is increasing awareness of the importance of decreasing unwarranted variation in health care delivery. In the past 7 years, Geisinger Health System has performed broad reengineering of its acute episodic and chronic care delivery models utilizing macrosystem-level application of CER principles. These provider-driven process initiatives have resulted in significant improvement across all segments of care delivery, improved patient outcomes, and notable cost containment. These programs have led to the creation of novel pricing models, and when "hardwired" throughout a care delivery system, they can lead to correct medical decision making by 100% of providers in all patient encounters. Neurosurgery as a specialty faces unique challenges and opportunities with respect to broad adoption and application of CER techniques.

  10. Neuropsychological sequelae of central nervous system prophylaxis in survivors of childhood acute lymphoblastic leukemia

    SciTech Connect

    Said, J.A.; Waters, B.G.; Cousens, P.; Stevens, M.M.

    1989-04-01

    We assessed neuropsychologically 106 children with acute lymphoblastic leukemia (ALL) who had all received cranial irradiation for the prevention of central nervous system (CNS) leukemia 1-13 years previously. Children were assessed for adverse late effects of their therapy, using age-appropriate Wechsler measures of overall intellectual ability and supplementary tests. Forty-five siblings near in age to the patients were tested as controls. The patients who had had the most intensive central nervous system (CNS) prophylaxis were found to have a WISC-R Full Scale IQ 17 points lower than the sibling control group. Performance IQ was more affected than verbal IQ. The patients were more easily distracted and less able to concentrate. The severity of the aftereffects was related to younger age at the time of CNS prophylaxis and to a higher dose of cranial irradiation but not to time since CNS prophylaxis. CNS prophylaxis using a combination of cranial irradiation and intrathecal methotrexate has lowered the incidence of CNS relapse in childhood ALL but is associated with considerable long-term morbidity in survivors.

  11. Acute Resistance Exercise Induces Antinociception by Activation of the Endocannabinoid System in Rats

    PubMed Central

    Galdino, Giovane; Romero, Thiago; da Silva, José Felippe Pinho; Aguiar, Daniele; de Paula, Ana Maria; Cruz, Jader; Parrella, Cosimo; Piscitelli, Fabiana; Duarte, Igor; Di Marzo, Vincenzo; Perez, Andrea

    2014-01-01

    Background Resistance exercise (RE) is also known as strength training, and it is performed to increase the strength and mass of muscles, bone strength and metabolism. RE has been increasingly prescribed for pain relief. However, the endogenous mechanisms underlying this antinociceptive effect are still largely unexplored. Thus, we investigated the involvement of the endocannabinoid system in RE-induced antinociception. Methods Male Wistar rats were submitted to acute RE in a weight-lifting model. The nociceptive threshold was measured by a mechanical nociceptive test (paw pressure) before and after exercise. To investigate the involvement of cannabinoid receptors and endocannabinoids in RE-induced antinociception, cannabinoid receptor inverse agonists, endocannabinoid metabolizing enzyme inhibitors and an anandamide reuptake inhibitor were injected before RE. After RE, CB1 cannabinoid receptors were quantified in rat brain tissue by Western blot and immunofluorescence. In addition, endocannabinoid plasma levels were measured by isotope dilution-liquid chromatography mass spectrometry. Results RE-induced antinociception was prevented by preinjection with CB1 and CB2 cannabinoid receptor inverse agonists. By contrast, preadministration of metabolizing enzyme inhibitors and the anandamide reuptake inhibitor prolonged and enhanced this effect. RE also produced an increase in the expression and activation of CB1 cannabinoid receptors in rat brain tissue and in the dorsolateral and ventrolateral periaqueductal regions and an increase of endocannabinoid plasma levels. Conclusion The present study suggests that a single session of RE activates the endocannabinoid system to induce antinociception. PMID:24977916

  12. Efficacy of mizoribine and prednisolone combination therapy in adult patients with IgA vasculitis.

    PubMed

    Mima, Akira

    2017-03-02

    Immunoglobulin (Ig)A vasculitis (IgAV), formerly known as Henoch-Schönlein purpura, is one of the most common vasculitis caused by an IgA-mediated immune complex. It occurs most frequently in childhood and less commonly in adulthood. As for the treatment of IgAV in adults, there are few studies dealing with the administration and efficacy of intravenous pulse steroid therapy or combination therapy using prednisolone (PSL) and immunosuppressive drugs. Mizoribine (MZB) is a newly developed immunosuppressive drug with few adverse effects; however, there are currently few studies using MZB in adult patients with IgAV. In this study, we evaluated the efficacy of MZB combined with a course of PSL in adult patients with IgAV. Five patients with adult onset IgAV were enrolled in the study. All patients received oral PSL (initial dose 30-50 mg/day), and MZB was administered orally at a single morning dose of 150 mg. We investigated the clinical manifestations and prognosis of these patients receiving the combination therapy of MZB and PSL retrospectively. All patients showed complete or partial remission of proteinuria and microscopic hematuria with the combination therapy of MZB and PSL. Furthermore, no significant adverse effects were observed. Although this study had an uncontrolled small group, our results indicate that the combination of MZB with PSL could be a possible new treatment for adult patients with IgAV.

  13. The role of IgM rheumatoid factor in experimental immune vasculitis.

    PubMed Central

    Floyd, M; Tesar, J T

    1979-01-01

    The effect of IgM rhematoid factor (RF) on reversepassive cutaneous Arthus reaction in rats was studied. The RF was obtained from the serum cryoglobulin of a patient with symptoms of purpura, arthralgia and digital gangrene. The cryoglobulins was of IgG-IgM type and when given i.v it induced a prompt hypocomplementaemia in experimental animals. The purified RF also induced low serum complement levels when injected i.v. along with complexes of non-complement-fixing, aggregated IgG. A reverse passive Arthus reaction was induced by intradermal injection of IgG anti-bovine serum albumin (BSA), followed by an i.v. dose of antigen (Ag). The cutaneous inflammatory reaction was aggravated by simultaneous administration of IgM RF intradermally, but not by IgM without antibody (Ab) properties. Intradermal injection of low concentrations of non-complement-fixing IgG anti-BSA, along with normal human IgM, followed by i.v. injection of BSA, resulted in a complete lack of cutaneous inflammation. At higher Ab concentrations there was only a mild inflammation. However, when IgM RF was substituted for normal IgM and injected with non-complement-fixing anti-BSA, an effective reverse passive cutaneous Arthus reaction and vasculitis was induced. The inflammatory response was greatly suppressed by decomplementation of animals by cobra venom factor. This study provides evidence favouring an inflammatory, complement-dependent role for RF in vasculitis. PMID:157238

  14. Comparison of Existing Clinical Scoring Systems in Predicting Severity and Prognoses of Hyperlipidemic Acute Pancreatitis in Chinese Patients

    PubMed Central

    Qiu, Lei; Sun, Rui Qing; Jia, Rong Rong; Ma, Xiu Ying; Cheng, Li; Tang, Mao Chun; Zhao, Yan

    2015-01-01

    Abstract It is important to identify the severity of acute pancreatitis (AP) in the early course of the disease. Clinical scoring systems may be helpful to predict the prognosis of patients with early AP; however, few analysts have forecast the accuracy of scoring systems for the prognosis in hyperlipidemic acute pancreatitis (HLAP). The purpose of this study was to summarize the clinical characteristics of HLAP and compare the accuracy of conventional scoring systems in predicting the prognosis of HLAP. This study retrospectively analyzed all consecutively diagnosed AP patients between September 2008 and March 2014. We compared the clinical characteristics between HLAP and nonhyperlipidemic acute pancreatitis. The bedside index for severity of acute pancreatitis (BISAP), Ranson, computed tomography severity index (CTSI), and systemic inflammatory response syndrome (SIRS) scores were applied within 48 hours following admission. Of 909 AP patients, 129 (14.2%) had HLAP, 20 were classified as severe acute pancreatitis (SAP), 8 had pseudocysts, 9 had pancreatic necrosis, 30 had pleural effusions, 33 had SIRS, 14 had persistent organ failure, and there was 1 death. Among the HLAP patients, the area under curves for BISAP, Ranson, SIRS, and CTSI in predicting SAP were 0.905, 0.938, 0.812, and 0.834, 0.874, 0.726, 0.668, and 0.848 for local complications, and 0.904, 0.917, 0.758, and 0.849 for organ failure, respectively. HLAP patients were characterized by younger age at onset, higher recurrence rate, and being more prone to pancreatic necrosis, organ failure, and SAP. BISAP, Ranson, SIRS, and CTSI all have accuracy in predicting the prognosis of HLAP patients, but each has different strengths and weaknesses. PMID:26061329

  15. Pathophysiology of ANCA-Associated Small Vessel Vasculitis

    PubMed Central

    2010-01-01

    Antineutrophil cytoplasmic autoantibodies (ANCAs) directed to proteinase 3 (PR3-ANCA) or myeloperoxidase (MPO-ANCA) are strongly associated with the ANCA-associated vasculitides—Wegener’s granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome. Clinical observations, including the efficacy of B-cell depletion via rituximab treatment, support—but do not prove—a pathogenic role for ANCA in the ANCA-associated vasculitides. In vitro experimental studies show that the interplay of ANCA, neutrophils, the alternative pathway of the complement system, and endothelial cells could result in lysis of the endothelium. A pathogenic role for MPO-ANCA is strongly supported by in vivo experimental studies in mice and rats, which also elucidate the pathogenic mechanisms involved in lesion development. Unfortunately, an animal model for PR3-ANCA–associated Wegener’s granulomatosis is not yet available. Here, cellular immunity appears to play a major role as well, particularly via interleukin-17–producing T cells, in line with granulomatous inflammation in the lesions. Finally, microbial factors, in particular Staphylococcus aureus and gram-negative bacteria, seem to be involved in disease induction and expression, but further studies are needed to define their precise role in disease development. PMID:20878509

  16. Exanthema and acute anuric renal failure.

    PubMed

    Resch, M; Banas, B; Endemann, D; Mack, M; Riegger, G A J; Gröne, H J; Krämer, B K

    2006-05-01

    A 15-year-old girl with a history of Kawasaki disease was admitted to our nephrological department due to acute renal failure. Despite antibiotic therapy because of fever and the symptoms of a pharyngitis in the last few days, the girl showed persisting fever and developed arthralgias, an exanthema and a rising serum creatinine as well as anuria. A wide variety of differential diagnoses has to be thought of because of the history of the Kawasaki disease (symptoms like fever, pharyngitis, exanthema and arthralgia), i.e. hemolytic-uremic syndrome, vasculitis, ascending infection, postinfection glomerulonephritis. In consideration of etiologically unclear "rapidly progressive renal failure" with anuria and thrombocytopenia an immediate renal biopsy was done and revealed a severe drug induced acute interstitial nephritis. Due to this diagnosis we treated the patient with corticosteroids. Within 4 weeks serum creatinine declined to 1.8 mg/dl but did not normalize.

  17. [Acute myocardial infarction as Eosinophilic granulomatosis with polyangiitis (formerly Churg Strauss syndrome) initial presentation].

    PubMed

    Sulaiman, Wahinuddin; Seung, Ong Ping; Noor, Sabariah Mohd

    2014-01-01

    Eosinophilic granulomatosis with polyangiitis is a rare primary vasculitic disease characterized by hypereosinophilia, late onset asthma and extravascular eosinophil granulomas. We report a case presented initially with acute myocardial infarction which later only proceed with asthma, skin manifestations and peripheral neuropathy. Laboratory parameters showed hypereosinohpilia with negative perinuclear pattern of antineutrophil cytoplasmic autoantibodies (p-ANCA). Skin biopsy showed leucocytoclastic vasculitis with eosinophilic infiltration while coronary angiography was normal. The patient's symptoms improved with IV methylprednisolone, pulse cyclophosphamide and azathioprine.

  18. Autoantigenic nuclear proteins of a clinically atypical renal vasculitis

    PubMed Central

    Avila, Julio; Acosta, Elisa; Machargo, María-del-Valle; Arteaga, María-Francisca; Gallego, Eduardo; Cañete, Haridian; García-Pérez, José-Javier; Martín-Vasallo, Pablo

    2008-01-01

    Background Systemic vasculitides constitute a heterogeneous group of diseases of autoimmunological origin characterized by inflammation of blood vessels and antibodies that react against autoantigens in a process that ultimately affects blood vessel walls. An important number of these patients present kidney disease. An endeavour of this area of research is the identification of autoantigens involved in these diseases. Accordingly, we used serum from a patient suffering from a microscopic polyangiitis, P-ANCA positive, manifesting a clinically atypical renal necrotizing glomerulonephritis and interstitial nephropathy for the identification of autoantigens; we also determined the prevalence of corresponding autoantibodies in other vasculitides, diabetic microangiopathy and in general population. Methods The patient's serum was used as a probe for the immunoscreening method SEREX to screen a human brain cDNA expression library. Results Four positive clones were isolated and sequenced. Clones Jos002 code for protein HDAC5, Jos014 for TFC4, Jos107 for RTF1, and Jos313 for POLDIP3 polymerase. The four proteins are of nuclear localization. None of them had been reported as autoantigen. Recombinant proteins were synthesised and checked as antigens by western blot with different sera from controls and patients affected with other vasculitides and diabetic microangiopathy as well. Only the serum from the patient origin of this study recognized all recombinant proteins. Conclusion We identify four nuclear proteins, HDAC5, TFC4, RTF1 and POLDIP3 polymerase as new autoantigens that could be used as markers in the diagnosis of subfamilies in immune diseases, although we cannot determine the role of these proteins in the aetiopathogenic process. PMID:18625050

  19. Acute cervico-facial infection in Scotland 2010: patterns of presentation, patient demographics and recording of systemic involvement.

    PubMed

    Byers, J; Lowe, T; Goodall, C A

    2012-10-01

    Acute bacterial cervicofacial infection is a common problem that is most often secondary to dental infection. Most cases present as localised abscesses but some may be associated with serious morbidity including scarring, embarrassment of the airway, SIRS (systemic inflammatory response syndrome), and sepsis syndrome. Fourteen oral surgery or maxillofacial surgery units in Scotland took part in a clinical audit of acute infection during two four-week cycles (August and November) in 2010. Information regarding the patients, signs and symptoms, and management was recorded. Training material was distributed between cycles with information on SIRS, sepsis, and the prescription of antibiotics. Overall, 140 patients presented with acute infection. There was an equal sex distribution and ages ranged from 5 to 87 years. There was an association with deprivation and 36% of patients were from the lowest socioeconomic quintile. Most infections were dental (n=120, 86%), and patients presented with pain and swelling (n=120, 86% and n=134, 96%, respectively) Twenty-three patients (16%) met the criteria for SIRS. A further 23 (16%) had at least one positive SIRS marker with incomplete recording of the remaining markers. Twenty-six patients (19%) had no recorded SIRS markers. Cervicofacial infection can be associated with serious morbidity and mortality, which may be better managed if the systemic signs and symptoms of sepsis are recognised and recorded at presentation. This study showed that the recording of signs of sepsis was variable even with training. Further training of junior staff to recognise severe acute bacterial infection may improve management.

  20. Cerebral vascular findings in PAPA syndrome: cerebral arterial vasculopathy or vasculitis and a posterior cerebral artery dissecting aneurysm.

    PubMed

    Khatibi, Kasra; Heit, Jeremy J; Telischak, Nicholas A; Elbers, Jorina M; Do, Huy M

    2015-06-24

    A young patient with PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne) syndrome developed an unusual cerebral arterial vasculopathy/vasculitis (CAV) that resulted in subarachnoid hemorrhage from a ruptured dissecting posterior cerebral artery (PCA) aneurysm. This aneurysm was successfully treated by endovascular coil sacrifice of the affected segment of the PCA. The patient made an excellent recovery with no significant residual neurologic deficit.

  1. Cerebral vascular findings in PAPA syndrome: cerebral arterial vasculopathy or vasculitis and a posterior cerebral artery dissecting aneurysm.

    PubMed

    Khatibi, Kasra; Heit, Jeremy J; Telischak, Nicholas A; Elbers, Jorina M; Do, Huy M

    2016-08-01

    A young patient with PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne) syndrome developed an unusual cerebral arterial vasculopathy/vasculitis (CAV) that resulted in subarachnoid hemorrhage from a ruptured dissecting posterior cerebral artery (PCA) aneurysm. This aneurysm was successfully treated by endovascular coil sacrifice of the affected segment of the PCA. The patient made an excellent recovery with no significant residual neurologic deficit.

  2. An Open Source 3-D Printed Modular Micro-Drive System for Acute Neurophysiology

    PubMed Central

    Eskandar, Emad N.

    2014-01-01

    Current, commercial, electrode micro-drives that allow independent positioning of multiple electrodes are expensive. Custom designed solutions developed by individual laboratories require fabrication by experienced machinists working in well equipped machine shops and are therefore difficult to disseminate into widespread use. Here, we present an easy to assemble modular micro-drive system for acute primate neurophysiology (PriED) that utilizes rapid prototyping (3-d printing) and readily available off the shelf-parts. The use of 3-d printed parts drastically reduces the cost of the device, making it available to labs without the resources of sophisticated machine shops. The direct transfer of designs from electronic files to physical parts also gives researchers opportunities to easily modify and implement custom solutions to specific recording needs. We also demonstrate a novel model of data sharing for the scientific community: a publicly available repository of drive designs. Researchers can download the drive part designs from the repository, print, assemble and then use the drives. Importantly, users can upload their modified designs with annotations making them easily available for others to use. PMID:24736691

  3. Acute lupus pneumonitis followed by intestinal pseudo-obstruction in systemic lupus erythematosus: A case report

    PubMed Central

    JI, CAIHONG; YU, XING; WANG, YONG; SHI, LUFENG

    2016-01-01

    Intestinal pseudo-obstruction (IpsO) and acute lupus pneumonitis (ALP) are uncommon severe complications of systemic lupus erythematosus (SLE). The present study reports the case of a 26-year-old female who presented with abdominal pain, nausea and vomiting as initial symptoms. Computed tomography (CT) scanning revealed the jejunal wall was thickened and streaky, mimicking the presentation of intestinal obstruction. Following emergency surgery, the patient's general condition was aggravated, with evident limb erythematous rashes. A series of laboratory examinations revealed SLE, and combined with patient's medical history IpsO was diagnosed, with a disease Activity Index score of 10. During the therapeutic period, high fever, dyspnea and oxygen saturation (SaO2) reductions were detected, and CT scans indicated lung infiltration, excluding other causes through a comprehensive infectious work-up and a bronchoalveolar lavage examination. ALP was confirmed and treated with high-dose methylprednisolone and gamma globulin supplement. The patient responded well and was discharged in 2 weeks. In the one-year tapering period and after stopping corticosteroids, the patient recovered well with no relapse detected. In conclusion, the manifestation of IpsO in SLE is rare and represents a challenge for the surgeon to establish the correct diagnosis and avoid inappropriate surgical intervention. ALP may be the consequence of emergency surgery, and immediate high-dose glucocorticoid therapy is recommended. PMID:27347044

  4. Central nervous system complications during treatment of acute lymphoblastic leukemia in a single pediatric institution.

    PubMed

    Parasole, Rosanna; Petruzziello, Fara; Menna, Giuseppe; Mangione, Argia; Cianciulli, Emilio; Buffardi, Salvatore; Marchese, Luciano; Nastro, Anna; Misuraca, Aldo; Poggi, Vincenzo

    2010-06-01

    Central nervous system (CNS) complications during treatment of childhood acute lymphoblastic leukemia (ALL) remain a challenging clinical problem. Outcome improvement with more intensive chemotherapy has significantly increased the incidence and severity of adverse events. This study analyzed the incidence of neurological complications during ALL treatment in a single pediatric institution, focusing on clinical, radiological, and electrophysiological findings. Exclusion criteria included CNS leukemic infiltration at diagnosis, therapy-related peripheral neuropathy, late-onset encephalopathy, or long-term neurocognitive defects. During a 9-year period, we retrospectively collected 27 neurological events (11%) in as many patients, from 253 children enrolled in the ALL front-line protocol. CNS complications included posterior reversible leukoencephalopathy syndrome (n = 10), stroke (n = 5), temporal lobe epilepsy (n = 2), high-dose methotrexate toxicity (n = 2), syndrome of inappropriate antidiuretic hormone secretion (n = 1), and other unclassified events (n = 7). In conclusion, CNS complications are frequent events during ALL therapy, and require rapid detection and prompt treatment to limit permanent damage.

  5. Prolonged pretreatment of mice with cholera toxin, but not isoproterenol, alleviates acute lethal systemic inflammatory response.

    PubMed

    Wang, Jingyang; Guo, Xiangrui; Cao, Junxia; Zhang, Xueying; Zhang, Jiyan; Sun, Dejun; Wang, Qingyang

    2014-11-01

    Isoproterenol, a synthetic non-selective β-adrenergic agonist, is often used during the immediate postoperative period after open heart surgery for its chronotropic and vasodilatory effects. It has been demonstrated that isoproterenol pretreatment followed by immediate LPS administration leads to reduced tumor necrosis factor-α (TNF-α) response in vivo. However, sepsis never happens immediately after the surgery, but rather severe immune dysfunction occurs at least 24h later. It remains elusive what effects isoproterenol might exert to innate immunity during the period. In this scenario, we investigated the effects of 24-h isoproterenol pretreatment on septic shock induced by experimental endotoxemia and bacterial peritonitis, with cholera toxin as another cAMP elevator. Unexpectedly, we found that isoproterenol and cholera toxin exhibited distinct effects in acute lethal systemic inflammatory response. Isoproterenol worsened liver injury without enhancing NK/NKT activity. Meanwhile, cholera toxin but not isoproterenol showed dramatically reduced TNF-α response in LPS induced septic shock. Our data provide a caution for the clinical use of isoproterenol and suggest that isoproterenol has cAMP-independent functions.

  6. Cerebral salt wasting syndrome in children with acute central nervous system injury.

    PubMed

    Jiménez, Raquel; Casado-Flores, Juan; Nieto, Monserrat; García-Teresa, María Angeles

    2006-10-01

    The purpose of this investigation was to describe the causes, clinical pattern, and treatment of cerebral salt wasting syndrome in children with acute central nervous system injury. This retrospective study focused on patients120 mEq/L), and volume depletion. Fourteen patients were identified with cerebral salt wasting syndrome, 12 after a neurosurgical procedure (8 brain tumor, 4 hydrocephalus) and 2 after severe brain trauma. In 11 patients the cerebral salt wasting syndrome was diagnosed during the first 48 hours of admission. Prevalence of cerebral salt wasting syndrome in neurosurgical children was 11.3/1000 surgical procedures. The minimum sodium was 122+/-7 mEq/L, the maximum urine osmolarity 644+/-59 mOsm/kgH2O. The maximum sodium supply was 1 mEq/kg/h (range, 0.1-2.4). The mean duration of cerebral salt wasting syndrome was 6+/-5 days (range 1-9). In conclusion, cerebral salt wasting syndrome can complicate the postoperative course of children with brain injury; it is frequently present after surgery for brain tumors and hydrocephalus and in patients with severe head trauma. Close monitoring of salt and fluid balance is essential to prevent severe neurologic and hemodynamic complications.

  7. Acute toxicity and inactivation tests of CO2 on invertebrates in drinking water treatment systems.

    PubMed

    Yin, Wen-Chao; Zhang, Jin-Song; Liu, Li-Jun; Zhao, Jian-Shu; Li, Tuo

    2011-01-01

    In addition to the esthetic problem caused by invertebrates, researchers are recently starting to be more aware of their potential importance in terms of public health. However, the inactivation methods of invertebrates which could proliferate in drinking water treatment systems are not well developed. The objective of this study is to assess the acute toxicity and inactivation effects of CO2 on familiar invertebrates in water treatment processes. The results of this study revealed that CO2 has a definite toxicity to familiar invertebrates. The values of 24-h LC50 (median lethal concentration) were calculated for each test with six groups of invertebrates. The toxicity of CO2 was higher with increasing concentrations in solution but was lower with the increase in size of the invertebrates. Above the concentration of 1,000 mg/L for the CO2 solution, the 100% inactivation time of all the invertebrates was less than 5 s, and in 15 min, the inactivation ratio showed a gradient descent with a decline in concentration. As seen for Mesocyclops thermocyclopoides, by dosing with a sodium bicarbonate solution first and adding a dilute hydrochloric acid solution 5 min later, it is possible to obtain a satisfactory inactivation effect in the GAC (granular activated carbon) filters.

  8. Acute exposure to 2G phase shifts the rat circadian timing system

    NASA Technical Reports Server (NTRS)

    Hoban-Higgins, T. M.; Murakami, D. M.; Tandon, T.; Fuller, C. A.

    1995-01-01

    The circadian timing system (CTS) provides internal and external temporal coordination of an animal's physiology and behavior. In mammals, the generation and coordination of these circadian rhythms is controlled by a neural pacemaker, the suprachiasmatic nucleus (SCN), located within the hypothalamus. The pacemaker is synchronized to the 24 hour day by time cures (zeitgebers) such as the light/dark cycle. When an animal is exposed to an environment without time cues, the circadian rhythms maintain internal temporal coordination, but exhibit a 'free-running' condition in which the period length is determined by the internal pacemaker. Maintenance of internal and external temporal coordination are critical for normal physiological and psychological function in human and non-human primates. Exposure to altered gravitational environments has been shown to affect the amplitude, mean, and timing of circadian rhythms in species ranging from unicellular organisms to man. However, it has not been determined whether altered gravitational fields have a direct effect on the neural pacemaker, or affect peripheral parameters. In previous studies, the ability of a stimulus to phase shift circadian rhythms was used to determine whether a stimulus has a direct effect on the neural pacemaker. The present experiment was performed in order to determine whether acute exposure to a hyperdynamic field could phase shift circadian rhythms.

  9. RNA-Guided CRISPR-Cas9 System-Mediated Engineering of Acute Myeloid Leukemia Mutations.

    PubMed

    Brabetz, Oliver; Alla, Vijay; Angenendt, Linus; Schliemann, Christoph; Berdel, Wolfgang E; Arteaga, Maria-Francisca; Mikesch, Jan-Henrik

    2017-03-17

    Current acute myeloid leukemia (AML) disease models face severe limitations because most of them induce un-physiological gene expressions that do not represent conditions in AML patients and/or depend on external promoters for regulation of gene expression/repression. Furthermore, many AML models are based on reciprocal chromosomal translocations that only reflect the minority of AML patients, whereas more than 50% of patients have a normal karyotype. The majority of AML, however, is driven by somatic mutations. Thus, identification as well as a detailed molecular and functional characterization of the role of these driver mutations via improved AML models is required for better approaches toward novel targeted therapies. Using the IDH2 R140Q mutation as a model, we present a new effective methodology here using the RNA-guided clustered regularly interspaced short palindromic repeats (CRISPR)-Cas9 system to reproduce or remove AML-associated mutations in or from human leukemic cells, respectively, via introduction of a DNA template at the endogenous gene locus via homologous recombination. Our technology represents a precise way for AML modeling to gain insights into AML development and progression and provides a basis for future therapeutic approaches.

  10. Role of cardiac output and the autonomic nervous system in the antinatriuretic response to acute constriction of the thoracic superior vena cava.

    NASA Technical Reports Server (NTRS)

    Schrier, R. W.; Humphreys, M. H.; Ufferman, R. C.

    1971-01-01

    Study of the differential characteristics of hepatic congestion and decreased cardiac output in terms of potential afferent stimuli in the antinatriuretic effect of acute thoracic inferior vena cava (TIVC) constriction. An attempt is made to see if the autonomic nervous system is involved in the antinatriuretic effect of acute TIVC or thoracic superior vena cava constriction.

  11. Spontaneous ureteral rupture in a patient with systemic lupus erythematosus

    SciTech Connect

    Benson, C.H.; Pennebaker, J.B.; Harisdangkul, V.; Songcharoen, S.

    1983-08-01

    A patient with known systemic lupus erythematosus had fever and symptoms of a lower urinary tract infection. Bone scintigraphy showed left ureteral perforation and necrosis with no demonstrable nephrolithiasis. It is speculated that this episode was due to lupus vasculitis.

  12. Necrotizing vasculitis

    MedlinePlus

    ... and ulcers that do not heal Muscles and joints: Joint pain Leg pain Muscle weakness Brain and nervous ... URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is an ...

  13. Vasculitis Foundation

    MedlinePlus

    ... the past more » View All News Connect with us on social media! Join VF Donate Next Page » ... our community through education, awareness and research. Contact Us Click here to send us an email! Mail: ...

  14. Hypersensitivity Vasculitis

    MedlinePlus

    ... most frequently listed as being associated with the development of HV include: penicillin, cephalosporin, sulfonamide, some medicines used to control blood pressure (loop and thiazide-type diuretics), phenytoin and allopurinol. Infections that may be ...

  15. Extracellular phospholipases A2 in relation to systemic inflammatory response syndrome (SIRS) and systemic complications in severe acute pancreatitis.

    PubMed

    Hietaranta, A; Kemppainen, E; Puolakkainen, P; Sainio, V; Haapiainen, R; Peuravuori, H; Kivilaakso, E; Nevalainen, T

    1999-05-01

    The pathophysiology of severe acute pancreatitis (AP) resembles other conditions with systemic inflammatory response syndrome (SIRS) such as sepsis predisposing to remote organ failure. Because extracellular phospholipases A2 (PLA2) have been implicated in AP, their serum concentrations were analyzed with respect to SIRS and systemic complications in patients with severe AP. The serum samples were collected daily for 12 days in 57 patients with severe AP. SIRS, early organ complications, local complications, and outcome of AP were recorded. Time-resolved fluoroimmunoassays were used for group I and group II PLA2 measurements. Thirty-nine (68.4%) patients fulfilled the criteria of SIRS within 12 days from admission. Pancreatic necrosis was detected in 43 (75.4%) patients. Infected necrosis was found preoperatively or at operation in five (8.8%) patients. Twenty-six (45.6%) and eight (14.0%) patients had respiratory or renal failure, respectively. Seven (12.3%) patients died of their disease. All patients with systemic complications fulfilled the criteria of SIRS. The increasing number of positive SIRS criteria was associated with increased frequency of systemic complications. Pancreatic necrosis was not significantly associated with SIRS. The serum concentration of group II PLA2 was significantly higher in patients with SIRS (p < 0.05) compared with patients without from day 7 onward. The concentration of group II PLA2 increased (p < 0.01) in patients with SIRS but decreased in patients without. The serum concentration of group II PLA2 did not differ significantly with respect to systemic complications. The concentration of group I PLA2 decreased (p < 0.05) similarly in patients with and without SIRS or systemic complications during follow-up, respectively. Early systemic complications of severe AP are associated with SIRS with increasing frequency as the number of positive SIRS criteria increases. Group II PLA2 but not group I PLA2 may have pathophysiologic

  16. Effects of acute and chronic social defeat stress are differentially mediated by the dynorphin/kappa-opioid receptor system

    PubMed Central

    Donahue, Rachel J.; Landino, Samantha M.; Golden, Sam A.; Carroll, F. Ivy; Russo, Scott J.; Carlezon, William A.

    2015-01-01

    Accumulating evidence indicates that kappa-opioid receptors (KORs) and their endogenous ligand dynorphin (DYN) can play important roles in regulating the effects of stress. Here, we examined the role of KOR systems in the molecular and behavioral effects of acute (1-day) and chronic (10-day) social defeat stress (SDS) in mice. We found that acute SDS increased DYN mRNA levels within the nucleus accumbens (NAc), a key element of brain dopamine (DA) systems. In contrast, chronic SDS produced long-lasting decreases in DYN mRNA levels. We then examined if disruption of KOR function would affect development of SDS-induced depressive-like behaviors as measured in the intracranial self-stimulation (ICSS) and social interaction tests. Ablation of KORs from DA transporter (DAT)-expressing neurons delayed the development of SDS-induced anhedonia in the ICSS test, suggesting increased stress resilience. However, administration of the long-lasting KOR antagonist JDTic (30 mg/kg, intraperitoneal) before the SDS regimen did not affect anhedonia, suggesting that disruption of KOR function outside DA systems can oppose stress resilience. Social avoidance behavior measured after the 10-day SDS regimen was not altered by ablation of KORs in DAT-expressing neurons or by JDTic administration before testing. Our findings indicate that KORs expressed in DA systems regulate the effects of acute, but not chronic, social stress. PMID:26110224

  17. A new hospice consulting system for terminal cancer patients in transferring to post-acute care options in Taiwan.

    PubMed

    Chang, P M-H; Liu, Y-Y L; Chao, T-C; Lin, H-L; Chen, M-B; Chen, P-M; Chiou, T-J

    2010-03-01

    The terminal cancer patients increase needs for hospice care day by day. A new hospice consulting system has been developed in Taiwan to provide options for terminal cancer patients in choosing a suitable post-acute hospice care while a combined hospice care system is also given by the consulting team in the acute wards. Hereinafter is our report. From March 2005 to January 2006, 313 terminal cancer patients were analysed. These patients had signed consent forms for palliative treatment and had received consultations from the new hospice consulting system. Multivariate analysis showed that the home care patients had better performance status (P = 0.012), less shortness of breath (P = 0.006), less limbs swelling (P = 0.043), less flatulency (P = 0.000) and less constipation (P = 0.018). Among the 162 patients with regular follow-up, the symptoms/signs were significantly improved after intervention of consulting team in pain (P = 0.000), shortness of breath (P = 0.000), difficulty in sleeping (P = 0.002), nausea (P = 0.004), constipation (P = 0.008), changes in skin (P = 0.024) and adoption (P = 0.000). This new system had significant improvement in the terminal cancer patients' symptoms/signs control in acute wards and could contribute to the care quality of home care patients.

  18. Activation of the contact system and inflammation after thrombolytic therapy in patients with acute myocardial infarction.

    PubMed

    Merlini, Piera Angelica; Cugno, Massimo; Rossi, Marco L; Agricola, Pietro; Repetto, Alessandra; Fetiveau, Raffaella; Diotallevi, Paolo; Canosi, Umberto; Mannucci, Pier Mannuccio; Ardissino, Diego

    2004-04-01

    Thrombolytic therapy activates the contact system, and factor XII activation may activate the coagulation cascade and inflammation. It is not known whether an early inflammatory response is induced by thrombolytic therapy in patients with acute myocardial infarction (AMI). We prospectively measured the plasma levels of activated factor XII, cleaved kininogen, prothrombin fragment 1 + 2 (as indexes of the contact phase and coagulation activation), and interleukin-6 and C-reactive protein (CRP) (as indexes of inflammation) in 39 patients hospitalized for AMI within 12 hours of symptom onset: 26 receiving thrombolytic therapy and 13 heparin alone. Blood samples were collected at baseline and after 90 minutes and 24 hours. Patients undergoing thrombolysis had a significant early increase in activated factor XII (from 2.2 ng/ml at baseline to 4.7 ng/ml after 90 minutes; p = 0.0001), cleaved kininogen (from 26% to 37%; p = 0.001), and fragment 1 + 2 (from 1.4 to 2.1 nmol/L; p = 0.0001), whereas the 24-hour levels were similar to baseline levels. The levels of interleukin-6 significantly increased during the first 90 minutes (from 3.9 to 6.3 microg/ml; p = 0.001), and were even higher after 24 hours (11.9 ng/ml, p = 0.0001). CRP levels increased only after 24 hours (p = 0.0001). There were no changes in these parameters in patients receiving heparin alone, except for a 24-hour increase in interleukin-6 and CRP levels. Thus, in patients with AMI receiving thrombolytic therapy, early activation of inflammation parallels the activation of the contact system and the coagulation cascade, which might contribute to microvascular obstruction and reperfusion injury.

  19. Simultaneous Automated Screening and Confirmatory Testing for Vasculitis-Specific ANCA

    PubMed Central

    Sowa, Mandy; Grossmann, Kai; Knütter, Ilka; Hiemann, Rico; Röber, Nadja; Anderer, Ursula; Csernok, Elena; Bogdanos, Dimitrios P.; Borghi, Maria Orietta; Meroni, Pier Luigi; Schierack, Peter; Reinhold, Dirk; Conrad, Karsten; Roggenbuck, Dirk

    2014-01-01

    Anti-neutrophil cytoplasmic antibodies (ANCA) are the serological hallmark of small vessel vasculitis, so called ANCA-associated vasculitis. The international consensus requires testing by indirect immunofluorescence (IIF) on human ethanol-fixed neutrophils (ethN) as screening followed by confirmation with enzyme-linked immunosorbent assays (ELISAs). This study evaluates the combination of cell- and microbead-based digital IIF analysis of ANCA in one reaction environment by the novel multiplexing CytoBead technology for simultaneous screening and confirmatory ANCA testing. Sera of 592 individuals including 118 patients with ANCA-associated vasculitis, 133 with rheumatoid arthritis, 49 with infectious diseases, 77 with inflammatory bowel syndrome, 20 with autoimmune liver diseases, 70 with primary sclerosing cholangitis and 125 blood donors were tested for cytoplasmic ANCA (C-ANCA) and perinuclear ANCA (P-ANCA) by classical IIF and ANCA to proteinase 3 (PR3) and myeloperoxidase (MPO) by ELISA. These findings were compared to respective ANCA results determined by automated multiplex CytoBead technology using ethN and antigen-coated microbeads for microbead immunoassays. There was a good agreement for PR3- and MPO-ANCA and a very good one for P-ANCA and C-ANCA by classical and multiplex analysis (Cohen's kappa [κ] = 0.775, 0.720, 0.876, 0.820, respectively). The differences between classical testing and CytoBead analysis were not significant for PR3-ANCA, P-ANCA, and C-ANCA (p<0.05, respectively). The prevalence of confirmed positive ANCA findings by classical testing (IIF and ELISA) compared with multiplex CytoBead analysis (IIF and microbead immunoassay positive) resulted in a very good agreement (κ = 0.831) with no significant difference of both methods (p = 0.735). Automated endpoint-ANCA titer detection in one dilution demonstrated a very good agreement with classical analysis requiring dilution of samples (κ = 0.985). Multiplexing by Cyto

  20. Acute presentation of thrombocytopaenia in systemic lupus erythematosus is associated with a high mortality in South Africa.

    PubMed

    Patel, N; Mody, G M

    2014-02-01

    The objective of this study was to determine the pattern of presentation, response to treatment, and outcome in patients with systemic lupus erythematosus (SLE) and thrombocytopaenia (TCP). A retrospective review of the records of patients with SLE and TCP and a matched control group of SLE patients without TCP, seen in the rheumatology department in Durban, South Africa, was performed. The demographic data, clinical findings, laboratory findings, treatment and outcome were recorded. There were 54 patients and an equal number of controls. They comprised 30 Indians and 24 African Blacks, median age of 33 years and female to male ratio 5.8:1. A group of eight patients who initially presented with idiopathic thrombocytopaenic purpura (ITP) and subsequently developed SLE were analysed separately. An acute presentation was noted in 31 patients (57%). Patients with an acute presentation had an increased prevalence of renal disease (77% vs 43.5%; p=0.01) and an increased number of deaths (38.7% vs 4.4%; p=0.004). The majority of patients responded to corticosteroids (68.5%) and splenectomy. There was an increased prevalence of renal disease (p=0.03) and deaths (p=0.004) among patients with TCP. The majority of deaths had an acute presentation ((12/13; 92.3%) (p=0.004)), and were due to infection and active lupus. TCP with an acute presentation is associated with a high mortality and predicts survival in SLE.

  1. Technology transfer with system analysis, design, decision making, and impact (Survey-2000) in acute care hospitals in the United States.

    PubMed

    Hatcher, M

    2001-10-01

    This paper provides the results of the Survey-2000 measuring technology transfer for management information systems in health care. The relationships with systems approaches, user involvement, usersatisfaction, and decision-making were measured and are presented. The survey also measured the levels Internet and Intranet presents in acute care hospitals, which will be discussed in future articles. The depth of the survey includes e-commerce for both business to business and customers. These results are compared, where appropriate, with results from survey 1997 and changes are discussed. This information will provide benchmarks for hospitals to plan their network technology position and to set goals. This is the first of three articles based upon the results of the Srvey-2000. Readers are referred to a prior article by the author that discusses the survey design and provides a tutorial on technology transfer in acute care hospitals.

  2. Artificial and bioartificial liver support systems for acute and acute-on-chronic hepatic failure: A meta-analysis and meta-regression.

    PubMed

    Zheng, Zhen; Li, Xu; Li, Zhiliang; Ma, Xiaochun

    2013-10-01

    Artificial and bioartificial liver support systems (LSSs) appear to be safe and effective in the treatment of acute and acute-on-chronic hepatic failure (AHF and AOCHF); however, individually published studies and previous meta-analyses have revealed inconclusive results. The aim of the present meta-analysis was to derive a more precise estimation of the benefits and disadvantages of artificial and bioartificial LSSs for patients with AHF and AOCHF. A literature search was conducted in the PubMed, Embase, Web of Science and Chinese Biomedical (CBM) databases for publications prior to March 1, 2013. Crude relative risks (RRs) or standardized mean differences (SMDs) with 95% confidence intervals (95% CI) were calculated using either the fixed effects or random effects models. Nineteen randomized controlled trials (RCTs) were included, which comprised a total of 566 patients with AHF and 371 patients with AOCHF. The meta-analysis showed that artificial LSS therapy significantly reduced mortality in patients with AOCHF; however, it had no apparent effect on total mortality in patients with AHF. The results also indicated that the use of bioartificial LSSs was correlated with decreased mortality in patients with AHF. A significant reduction in the bridging to liver transplantation was observed in patients with AOCHF following artificial LSS therapy; however, similar results were not observed in patients with AHF. Patients with AHF and those with AOCHF showed significant reductions in total bilirubin levels following artificial LSS therapy. There were no significantly increased risks of hepatic encephalopathy or bleeding in either the patients with AHF or AOCHF following artificial or bioartificial LSS therapies. Univariate and multivariate meta-regression analyses confirmed that none of the factors explained the heterogeneity. The present meta-analysis indicated that artificial LSSs reduce mortality in patients with AOCHF, while the use of bioartificial LSSs was

  3. Artificial and bioartificial liver support systems for acute and acute-on-chronic hepatic failure: A meta-analysis and meta-regression

    PubMed Central

    ZHENG, ZHEN; LI, XU; LI, ZHILIANG; MA, XIAOCHUN

    2013-01-01

    Artificial and bioartificial liver support systems (LSSs) appear to be safe and effective in the treatment of acute and acute-on-chronic hepatic failure (AHF and AOCHF); however, individually published studies and previous meta-analyses have revealed inconclusive results. The aim of the present meta-analysis was to derive a more precise estimation of the benefits and disadvantages of artificial and bioartificial LSSs for patients with AHF and AOCHF. A literature search was conducted in the PubMed, Embase, Web of Science and Chinese Biomedical (CBM) databases for publications prior to March 1, 2013. Crude relative risks (RRs) or standardized mean differences (SMDs) with 95% confidence intervals (95% CI) were calculated using either the fixed effects or random effects models. Nineteen randomized controlled trials (RCTs) were included, which comprised a total of 566 patients with AHF and 371 patients with AOCHF. The meta-analysis showed that artificial LSS therapy significantly reduced mortality in patients with AOCHF; however, it had no apparent effect on total mortality in patients with AHF. The results also indicated that the use of bioartificial LSSs was correlated with decreased mortality in patients with AHF. A significant reduction in the bridging to liver transplantation was observed in patients with AOCHF following artificial LSS therapy; however, similar results were not observed in patients with AHF. Patients with AHF and those with AOCHF showed significant reductions in total bilirubin levels following artificial LSS therapy. There were no significantly increased risks of hepatic encephalopathy or bleeding in either the patients with AHF or AOCHF following artificial or bioartificial LSS therapies. Univariate and multivariate meta-regression analyses confirmed that none of the factors explained the heterogeneity. The present meta-analysis indicated that artificial LSSs reduce mortality in patients with AOCHF, while the use of bioartificial LSSs was

  4. [Acute pancreatitis due to lupus].

    PubMed

    Hani, Mohamed Aziz; Guesmi, Fethi; Ben Achour, Jamel; Zribi, Riadh; Bouasker, Ibtissem; Zoghlami, Ayoub; Najah, Nabil

    2004-02-01

    Among digestive clinical presentations of systemic lupus erythematosus, acute pancreatitis remains a serious affection with very poor prognosis. To date, pathogenesis is still unclear. We report two cases of fatal acute pancreatitis related to systemic lupus erythematosus.

  5. Induction treatment of previously undiagnosed ANCA-associated vasculitis in a renal transplant patient with Rituximab

    PubMed Central

    Graham-Brown, M. P. M.; Aljayyousi, R.; Baines, R. J.; Burton, J. O.; Brunskill, N. J.; Furness, P.; Topham, P.

    2016-01-01

    We report the case of a 40-year-old female transplant patient with undiagnosed ANCA-associated vasculitis (AAV) and renal allograft dysfunction who achieved disease remission with restoration of transplant function following induction therapy with rituximab. There are currently no trial data looking at the use of rituximab for induction of remission of renal transplant patients with AAV. Although recurrence of AAV following renal transplantation is rare, such patients have invariably had multiple previous exposures to induction and maintenance immunosuppressive regimens, often limiting treatment options post-transplantation. In this case, rituximab was well tolerated with no side effects, and was successful in salvaging transplant function. Optimal treatment regimens for relapsed AAV in the transplant population are not known, and clinical trials are needed to evaluate the efficacy and safety of rituximab at inducing and maintaining disease remission in relapsed AAV following transplantation. PMID:27699052

  6. Varicella-Zoster Virus Vasculopathy: A Case Report Demonstrating Vasculitis using Black-Blood MRI

    PubMed Central

    Shah, Jay; Poonawala, Husain; Keay, Susan K; Serulle, Yafell; Steven, Andrew; Gandhi, Dheeraj; Cole, John W

    2016-01-01

    Infections are rare but important causes of stroke. Among these, varicella zoster virus has been known to cause ischemic stroke. During an attack of herpes zoster ophthalmicus, it has been hypothesized that the virus replicates in the trigeminal ganglion and travels via the trigeminal nerve centrally to cause cerebral vasculopathy. Here we present a case of a 69 year-old Caucasian immunocompromised woman who suffered recurrent ischemic infarcts within the same vascular distribution following an episode of zoster ophthalmicus three months prior. An imaging technique termed black-blood magnetic resonance imaging was utilized to aid in the diagnosis of cerebral vasculitis. The case is used to provide a literature review of the pathogenesis, diagnosis, and treatment of cerebral varicella zoster vasculopathy. In situations where an isolated unilateral cerebral vasculopathy is identified, neurologists are urged to consider varicella zoster as a treatable etiologic agent, as untreated vasculopathy can lead to further strokes. PMID:27065314

  7. Treatment of vasculitis and dermatitis in a 59-yr-old Nile hippopotamus (Hippopotamus amphibius).

    PubMed

    Spriggs, Maria; Reeder, Chris

    2012-09-01

    A 59-yr-old female Nile hippopotamus (Hippopotamus amphibius) was diagnosed and treated for severe dermatitis. Lesions included large areas of depigmentation, erosions, and ulcerations on glabrous skin areas, limbs, and perineal region. Histopathologic lesions included a markedly edematous, focally eroded, ulcerative to necrotic epidermis; foci of keratinocyte apoptosis; and a mixed suppurative dermatitis. Most of the dermal vessels had variable hyalinized walls with plump endothelial cells and frequent intramural neutrophils, and some vessels had vascular thrombi consistent with vasculitis. Culture of the lesions yielded beta-hemolytic Streptococcus, Morganella morgannii, and Enterococcus sp. The hippopotamus was successfully treated with sulfamethoxazole and trimethoprim, amoxicillin, and pentoxifylline for more than 2 mo, and the condition did not recur over the subsequent 16 mo.

  8. Is rituximab effective for induction of remission in ANCA-associated vasculitis?

    PubMed

    Rain, Carmen; Yáñez, Tatiana; Rada, Gabriel

    2015-08-13

    Adding rituximab to the treatment with corticosteroids has been proposed as a therapeutic alternative for inducing remission in anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis, especially when fertility is a concern, or when there is contraindication or intolerance to cyclophosphamide. Searching in Epistemonikos database, which is maintained by screening 30 databases, we identified only one systematic review including three pertinent randomized controlled trials. We combined the evidence using meta-analysis and generated a summary of findings following the GRADE approach. We concluded rituximab may slightly increase induction of remission rate, but it may also increase the risk of infection. It is not clear whether it increases the risk of cancer, or whether increases or decreases mortality because the certainty of the evidence is very low.

  9. Decongestion Strategies and Renin-Angiotensin-Aldosterone System Activation in Acute Heart Failure

    PubMed Central

    Mentz, Robert J.; Stevens, Susanna R.; DeVore, Adam D.; Lala, Anuradha; Vader, Justin M.; AbouEzzeddine, Omar F.; Khazanie, Prateeti; Redfield, Margaret M.; Stevenson, Lynne W.; O'Connor, Christopher M.; Goldsmith, Steven R.; Bart, Bradley A.; Anstrom, Kevin J.; Hernandez, Adrian F.; Braunwald, Eugene; Felker, G. Michael

    2014-01-01

    Background High dose diuretics in patients with acute heart failure (AHF) are thought to activate the renin-angiotensin-aldosterone system (RAAS), and alternative decongestion strategies, such as ultrafiltration (UF), have been proposed to mitigate this RAAS activation. Methods We analyzed 427 AHF patients enrolled in the DOSE-AHF and CARRESS-HF trials. We assessed the relationship between two markers of RAAS activation (plasma renin activity [PRA] and aldosterone) from baseline to 72-96h and decongestion strategy; high vs. low-dose and continuous infusion vs. bolus furosemide for DOSE-AHF and UF vs. stepped pharmacologic care for CARRESS-HF. We determined the relationship between RAAS biomarkers and 60-day outcomes. Results Patients with greater RAAS activation at baseline had lower blood pressures, lower serum sodium, and higher BUN. Continuous infusion furosemide and UF were associated with greater PRA increases (median +1.66 vs. +0.66 ng/mL/h with continuous vs. bolus, P=0.021; +4.05 vs. +0.56 ng/mL/h with UF vs. stepped care, P=0.014). There was no significant difference in RAAS biomarker change with high vs. low-dose diuretics (both P>0.5). Neither baseline log PRA nor log aldosterone was associated with increased death/HF hospitalization (HR for a doubling 1.05; 95% CI: 0.98-1.13, P=0.18 and HR 1.13; 95% CI: 0.99-1.28, P=0.069, respectively). The change in RAAS biomarkers from baseline to 72-96 h was not associated with outcomes (both P>0.5). Conclusions High-dose loop diuretics did not result in greater RAAS activation than low-dose diuretics. UF resulted in greater PRA increase than stepped pharmacologic care. Neither PRA nor aldosterone was significantly associated with short-term outcomes in this cohort. PMID:25543972

  10. Prevalence, severity, and clinical features of acute and chronic pancreatitis in patients with systemic lupus erythematosus.

    PubMed

    Wang, Qiang; Shen, Min; Leng, Xiaomei; Zeng, Xiaofeng; Zhang, Fengchun; Qian, Jiaming

    2016-10-01

    Pancreatitis is a rare, life-threatening complication of systemic lupus erythematosus (SLE). This study aimed to describe the clinical features of acute pancreatitis (AP) and chronic pancreatitis (CP) in patients with SLE. Data of patients who fulfilled the revised criteria of the American Rheumatism Association for diagnosis of SLE were retrospectively analyzed. SLE activity was graded according to the SLE Disease Activity Index. Logistic regression analysis was conducted to find out independent associations. Survival rates were estimated by using Kaplan-Meier plots. This study included 5665 SLE patients admitted between January 1983 and January 2014, of whom 52 patients were diagnosed with pancreatitis. Pancreatitis prevalence in SLE patients was 0.92 % (52/5665). AP (0.8 %, 46/5665) was more prevalent than CP (0.1 %, 6/5665), presented mostly during active SLE, and affected more organs. Hypertriglyceridemia occurred in 76.9 % of AP patients and in none of the CP patients. AP patients were divided into severe (n = 10) or mild (n = 20) cases. The average triglyceride level in severe AP cases was higher than that in mild AP cases (P = 0.006), and the mortality rate of lupus-associated AP was 32.6 % (15/46). Concomitant infections and thrombocytopenia were independently associated with poor prognosis (P < 0.001, P = 0.028, respectively). There were significant differences in the clinical manifestations of AP and CP. Patients with severe AP were found to have a higher incidence of concomitant infection and serum triglyceride levels. Concomitant infections and thrombocytopenia were independent risk factors for poor prognosis.

  11. Acute pancreatitis in juvenile systemic lupus erythematosus: a manifestation of macrophage activation syndrome?

    PubMed

    Campos, L M A; Omori, C H; Lotito, A P N; Jesus, A A; Porta, G; Silva, C A A

    2010-12-01

    Acute pancreatitis (AP) is a rare and life-threatening manifestation of juvenile systemic lupus erythematosus (JSLE). The objective of this study was to evaluate the prevalence and clinical features of AP in our JSLE population. AP was defined according to the presence of abdominal pain or vomiting associated to an increase of pancreatic enzymes and/or pancreatic radiological abnormalities. Of note, in the last 26 years, 5367 patients were followed up at our Pediatric Rheumatology Unit and 263 (4.9%) of them had JSLE diagnosis (ACR criteria). AP was observed in 4.2% (11/263) of JSLE patients. The median of age of the JSLE patients at AP diagnosis was 12.4 years (8.8-17.9). All of them had lupus disease activity at AP onset. Three patients were receiving corticosteroids before AP diagnosis. Interestingly, 10/11 JSLE patients fulfilled preliminary guidelines for macrophage activation syndrome, three of them with macrophage hemophagocytosis in bone marrow aspirate and hyperferritinemia. The hallmark of this syndrome is excessive activation and proliferation of T lymphocytes and macrophages with massive hypersecretion of proinflammatory cytokines and clinically it is characterized by the occurrence of unexplained fever, cytopenia and hyperferritinemia. AP treatment was mainly based on intravenous methylprednisolone. Four JSLE patients with AP died and two developed diabetes mellitus. In conclusion, AP was a rare and severe manifestation in active pediatric lupus. The association between AP and macrophage activation syndrome suggests that the pancreas could be a target organ of this syndrome and that pancreatic enzyme evaluation should also be carried out in all patients.

  12. Acute arterial ischemia in a patient with polyarthritis.

    PubMed

    Soro Marín, Sandra; Júdez Navarro, Enrique; Alamillo Sanz, Antonio Salvador; Sánchez Nievas, Ginés

    Cryoglobulins are immunoglobulins that precipitate at cold temperatures. Their presence can be related to a type of vasculitis referred to as cryoglobulinemia. This condition, especially mixed cryoglobulinemia, has been associated with viral infections like hepatitis C virus in 60%-90% of cases, but it has also been reported in relation to connective tissue diseases, usually resulting in a more severe course. We describe the case of a patient with seronegative polyarthritis who developed acute arterial ischemia in association with cryoglobulinemia, with a good response to rituximab therapy.

  13. Literature Review and Global Consensus on Management of Acute Radiation Syndrome Affecting Nonhematopoietic Organ Systems

    PubMed Central

    Dainiak, Nicholas; Gent, Robert Nicolas; Carr, Zhanat; Schneider, Rita; Bader, Judith; Buglova, Elena; Chao, Nelson; Coleman, C. Norman; Ganser, Arnold; Gorin, Claude; Hauer-Jensen, Martin; Huff, L. Andrew; Lillis-Hearne, Patricia; Maekawa, Kazuhiko; Nemhauser, Jeffrey; Powles, Ray; Schünemann, Holger; Shapiro, Alla; Stenke, Leif; Valverde, Nelson; Weinstock, David; White, Douglas; Albanese, Joseph; Meineke, Viktor

    2013-01-01

    Objectives The World Health Organization convened a panel of experts to rank the evidence for medical countermeasures for management of acute radiation syndrome (ARS) in a hypothetical scenario involving the hospitalization of 100 to 200 victims. The goal of this panel was to achieve consensus on optimal management of ARS affecting nonhematopoietic organ systems based upon evidence in the published literature. Methods English-language articles were identified in MEDLINE and PubMed. Reference lists of retrieved articles were distributed to conferees in advance of and updated during the meeting. Published case series and case reports of ARS, publications of randomized controlled trials of relevant interventions used to treat nonirradiated individuals, reports of studies in irradiated animals, and prior recommendations of subject matter experts were selected. Studies were extracted using the Grading of Recommendations Assessment Development and Evaluation system. In cases in which data were limited or incomplete, a narrative review of the observations was made. Results No randomized controlled trials of medical countermeasures have been completed for individuals with ARS. Reports of countermeasures were often incompletely described, making it necessary to rely on data generated in nonirradiated humans and in experimental animals. A strong recommendation is made for the administration of a serotonin-receptor antagonist prophylactically when the suspected exposure is >2 Gy and topical steroids, antibiotics, and antihistamines for radiation burns, ulcers, or blisters; excision and grafting of radiation ulcers or necrosis with intractable pain; provision of supportive care to individuals with neurovascular syndrome; and administration of electrolyte replacement therapy and sedatives to individuals with significant burns, hypovolemia, and/ orshock. A strong recommendation is made against the use of systemic steroids in the absence of a specific indication. A weak

  14. Effects of acute and chronic systemic methamphetamine on respiratory, cardiovascular and metabolic function, and cardiorespiratory reflexes

    PubMed Central

    Hassan, Sarah F.; Wearne, Travis A.; Cornish, Jennifer L.

    2016-01-01

    Key points Methamphetamine (METH) abuse is escalating worldwide, with the most common cause of death resulting from cardiovascular failure and hyperthermia; however, the underlying physiological mechanisms are poorly understood.Systemic administration of METH in anaesthetised rats reduced the effectiveness of some protective cardiorespiratory reflexes, increased central respiratory activity independently of metabolic function, and increased heart rate, metabolism and respiration in a pattern indicating that non‐shivering thermogenesis contributes to the well‐described hyperthermia.In animals that showed METH‐induced behavioural sensitisation following chronic METH treatment, no changes were evident in baseline cardiovascular, respiratory and metabolic measures and the METH‐evoked effects in these parameters were similar to those seen in saline‐treated or drug naïve animals.Physiological effects evoked by METH were retained but were neither facilitated nor depressed following chronic treatment with METH.These data highlight and identify potential mechanisms for targeted intervention in patients vulnerable to METH overdose. Abstract Methamphetamine (METH) is known to promote cardiovascular failure or life‐threatening hyperthermia; however, there is still limited understanding of the mechanisms responsible for evoking the physiological changes. In this study, we systematically determined the effects on both autonomic and respiratory outflows, as well as reflex function, following acute and repeated administration of METH, which enhances behavioural responses. Arterial pressure, heart rate, phrenic nerve discharge amplitude and frequency, lumbar and splanchnic sympathetic nerve discharge, interscapular brown adipose tissue and core temperatures, and expired CO2 were measured in urethane‐anaesthetised male Sprague‐Dawley rats. Novel findings include potent increases in central inspiratory drive and frequency that are not dependent on METH

  15. Acute Recurrent Pancreatitis in Children: A Study From the Pediatric Health Information System.

    PubMed

    Pant, Chaitanya; Sferra, Thomas J; Lee, Brian R; Cocjin, Jose T; Olyaee, Mojtaba

    2016-03-01

    We investigated acute recurrent pancreatitis (ARP) in children using a national health care database. From 2002 to 2014, 26,435 children had a diagnosis of acute pancreatitis (AP); 10,648 discharges were index hospitalizations. A total of 6159 children had a single hospitalization for AP, whereas 4489 (42%) children underwent 15,787 rehospitalizations. Children experienced a median of 2 ARP-related hospitalizations with a median time between admissions of 86 days. Younger patients with a more severe index episode of AP were at a higher risk of ARP. ARP-related hospitalizations had an increased requirement for intensive care unit care compared with an index episode of AP.

  16. Acute systemic accumulation of acrolein in mice by inhalation at a concentration similar to that in cigarette smoke

    PubMed Central

    Tully, Melissa; Zheng, Lingxing; Acosta, Glen; Tian, Ran; Shi, Riyi

    2015-01-01

    Cigarette smoke is an important environmental factor associated with a wide array of public health concerns. Acrolein, a component of tobacco smoke and a known toxin to various cell types, may be a key pathological factor mediating the adverse effects linked with tobacco smoke. Although acrolein is known to accumulate in the respiratory system after acute nasal exposure, it is not clear if it accumulates systemically, and less is known in the nervous system. The aim of this study was to assess the degree of acrolein accumulation in the circulation and in the spinal cord following acute acrolein inhalation in mice. Using a laboratory-fabricated inhalation chamber, we found elevated urinary 3-HPMA, an acrolein metabolite, and increased acrolein adducts in the spinal cord after weeks of nasal exposure to acrolein at a concentration similar to that in tobacco smoke. The data indicated that acrolein is absorbed into the circulatory system and some enters the nervous system. It is expected that these findings may facilitate further studies to probe the pathological role of acrolein in the nervous system resulting from smoke and other external sources. PMID:25446876

  17. Neurofunctional endpoints assessed in human neuroblastoma SH-SY5Y cells for estimation of acute systemic toxicity

    SciTech Connect

    Gustafsson, Helena; Runesson, Johan; Lundqvist, Jessica; Lindegren, Helene; Axelsson, Viktoria; Forsby, Anna

    2010-06-01

    The objective of the EU-funded integrated project ACuteTox is to develop a strategy in which general cytotoxicity, together with organ-specific toxicity and biokinetic features, are used for the estimation of human acute systemic toxicity. Our role in the project is to characterise the effect of reference chemicals with regard to neurotoxicity. We studied cell membrane potential (CMP), noradrenalin (NA) uptake, acetylcholine esterase (AChE) activity, acetylcholine receptor (AChR) signalling and voltage-operated calcium channel (VOCC) function in human neuroblastoma SH-SY5Y cells after exposure to 23 pharmaceuticals, pesticides or industrial chemicals. Neurotoxic alert chemicals were identified by comparing the obtained data with cytotoxicity data from the neutral red uptake assay in 3T3 mouse fibroblasts. Furthermore, neurotoxic concentrations were correlated with estimated human lethal blood concentrations (LC50). The CMP assay was the most sensitive assay, identifying eight chemicals as neurotoxic alerts and improving the LC50 correlation for nicotine, lindane, atropine and methadone. The NA uptake assay identified five neurotoxic alert chemicals and improved the LC50 correlation for atropine, diazepam, verapamil and methadone. The AChE, AChR and VOCC assays showed limited potential for detection of acute toxicity. The CMP assay was further evaluated by testing 36 additional reference chemicals. Five neurotoxic alert chemicals were generated and orphendrine and amitriptyline showed improved LC50 correlation. Due to the high sensitivity and the simplicity of the test protocol, the CMP assay constitutes a good candidate assay to be included in an in vitro test strategy for prediction of acute systemic toxicity.

  18. Cystitis - acute

    MedlinePlus

    Uncomplicated urinary tract infection; UTI - acute cystitis; Acute bladder infection; Acute bacterial cystitis ... cause. Menopause also increases the risk for a urinary tract infection. The following also increase your chances of having ...

  19. Acute Esophageal Necrosis Presenting With Henoch-Schönlein Purpura.

    PubMed

    Iorio, Natalya; Bernstein, Gregory R; Malik, Zubair; Schey, Ron

    2015-10-01

    A 63-year-old woman with abdominal pain and melena developed a palpable, purpuric rash and acute kidney injury. Skin and kidney biopsy confirmed Henoch-Schönlein purpura. Upper endoscopy revealed diffuse, circumferential, black-appearing mucosa of the esophagus consistent with acute esophageal necrosis (AEN), also known as black esophagus. AEN is a very rare cause of gastrointestinal hemorrhage with a high mortality risk. To our knowledge, there have been no prior reports of AEN associated with Henoch-Schonlein purpura or other vasculitis.

  20. Acute Esophageal Necrosis Presenting With Henoch-Schönlein Purpura

    PubMed Central

    Bernstein, Gregory R.; Malik, Zubair; Schey, Ron

    2015-01-01

    A 63-year-old woman with abdominal pain and melena developed a palpable, purpuric rash and acute kidney injury. Skin and kidney biopsy confirmed Henoch-Schönlein purpura. Upper endoscopy revealed diffuse, circumferential, black-appearing mucosa of the esophagus consistent with acute esophageal necrosis (AEN), also known as black esophagus. AEN is a very rare cause of gastrointestinal hemorrhage with a high mortality risk. To our knowledge, there have been no prior reports of AEN associated with Henoch-Schonlein purpura or other vasculitis. PMID:26504868

  1. Mycoplasma pneumoniae: an aetiological agent of acute haemorrhagic oedema of infancy.

    PubMed

    Di Lernia, Vito

    2014-11-01

    Acute haemorrhagic oedema of infancy (AHEI) is considered a separate clinical entity among cutaneous small vessel vasculitis of childhood. It usually occurs in children younger than 2 years of age, with spontaneous recovery occurring within a few weeks. A history of recent upper respiratory or urinary tract infections or immunisation is found in most patients. Although Mycoplasma pneumoniae has been linked to a wide array of skin eruptions or diseases, it is not recognised as a possible cause of acute haemorrhagic oedema of infancy. The authors report a child with AHEI and a concurrent M. pneumoniae infection.

  2. Acute Ozone-Induced Pulmonary and Systemic Metabolic Effects are Diminished in Adrenalectomized Rats#

    EPA Science Inventory

    Acute ozone exposure increases circulating stress hormones and induces metabolic alterations in animals and humans. We hypothesized that the increase of adrenal-derived stress hormones is necessary for both ozone-induced metabolic effects and lung injury. Male Wistar-Kyoto rats ...

  3. Atypical presentation of pheochromocytoma: Central nervous system pseudovasculitis

    PubMed Central

    Rupala, Ketankumar; Mittal, Varun; Gupta, Rajiv; Yadav, Rajiv

    2017-01-01

    Pheochromocytoma has atypical presentation in 9%–10% of patients. Atypical presentations include myocardial infarction, renal failure, and rarely cerebrovascular events. Various etiologies for central nervous system (CNS) involvement in pheochromocytoma have been described in the literature. A rare association of CNS vasculitis-like features has been described with pheochromocytoma. We report a rare case of pheochromocytoma detected on evaluation for CNS vasculitis-like symptoms. PMID:28197038

  4. Tauroursodeoxycholic acid reduces endoplasmic reticulum stress, acinar cell damage, and systemic inflammation in acute pancreatitis.

    PubMed

    Seyhun, Ersin; Malo, Antje; Schäfer, Claus; Moskaluk, Christopher A; Hoffmann, Ralf-Thorsten; Göke, Burkhard; Kubisch, Constanze H

    2011-11-01

    In acute pancreatitis, endoplasmic reticulum (ER) stress prompts an accumulation of malfolded proteins inside the ER, initiating the unfolded protein response (UPR). Because the ER chaperone tauroursodeoxycholic acid (TUDCA) is known to inhibit the UPR in vitro, this study examined the in vivo effects of TUDCA in an acute experimental pancreatitis model. Acute pancreatitis was induced in Wistar rats using caerulein, with or without prior TUDCA treatment. UPR components were analyzed, including chaperone binding protein (BiP), phosphorylated protein kinase-like ER kinase (pPERK), X-box binding protein (XBP)-1, phosphorylated c-Jun NH(2)-terminal kinase (pJNK), CCAAT/enhancer binding protein homologues protein, and caspase 12 and 3 activation. In addition, pancreatitis biomarkers were measured, such as serum amylase, trypsin activation, edema formation, histology, and the inflammatory reaction in pancreatic and lung tissue. TUDCA treatment reduced intracellular trypsin activation, edema formation, and cell damage, while leaving amylase levels unaltered. The activation of myeloperoxidase was clearly reduced in pancreas and lung. Furthermore, TUDCA prevented caerulein-induced BiP upregulation, reduced XBP-1 splicing, and caspase 12 and 3 activation. It accelerated the downregulation of pJNK. In controls without pancreatitis, TUDCA showed cytoprotective effects including pPERK signaling and activation of downstream targets. We concluded that ER stress responses activated in acute pancreatitis are grossly attenuated by TUDCA. The chaperone reduced the UPR and inhibited ER stress-associated proapoptotic pathways. TUDCA has a cytoprotective potential in the exocrine pancreas. These data hint at new perspectives for an employment of chemical chaperones, such as TUDCA, in prevention of acute pancreatitis.

  5. Application of Computed Tomography Processed by Picture Archiving and Communication Systems in the Diagnosis of Acute Achilles Tendon Rupture

    PubMed Central

    Tian, Jing; Xie, Bing; Zhang, Hao

    2016-01-01

    The applications of CT examination in the diagnosis of the acute Achilles tendon rupture (AATR) were investigated. A total of 36 patients with suspected acute Achilles tendon rupture were tested using physical examination, ultrasound, and 3DCT scanning, respectively. Then, surgery was performed for the patients who showed positive result in at least two of the three tests for AATR. 3DVR, MPR, and the other CT scan image processing and diagnosis were conducted in PACS (picture archiving and communication system). PACS was also used to measure the length of distal broken ends of the Achilles tendon (AT) to tendon calcaneal insertion. Our study indicated that CT has the highest accuracy in diagnosis of acute Achilles tendon complete rupture. The length measurement is matched between PACS and those actually measured in operation. CT not only demonstrates more details directly in three dimensions especially with the rupture involved calcaneal insertion flap but also locates the rupture region for percutaneous suture by measuring the length of distal stump in PACS without the effect of the position of ankle. The accuracy of CT diagnosis for Achilles tendon partial rupture is yet to be studied. PMID:28078295

  6. Effects of Local Pancreatic Renin-Angiotensin System on the Microcirculation of Rat with Severe Acute Pancreatitis

    PubMed Central

    Feng, Ling; Long, Haocheng; Wang, Hui; Feng, Jiarui; Chen, Feixiang

    2015-01-01

    Severe acute pancreatitis (SAP) is normally related to multiorgan dysfunction and local complications. Studies have found that local pancreatic renin-angiotensin system (RAS) was significantly upregulated in drug-induced SAP. The present study aimed to investigate the effects of angiotensin II receptors inhibitor valsartan on dual role of RAS in SAP in a rat model and to elucidate the underlying mechanisms. 3.8% sodium taurocholate (1 ml/kg) was injected to the pancreatic capsule in order for pancreatitis induction. Rats in the sham group were injected with normal saline in identical locations. We also investigated the regulation of experimentally induced SAP on local RAS expression in the pancreas through determination of the activities of serum amylase, lipase and myeloperoxidase, histological and biochemical analysis, radioimmunoassay, fluorescence quantitative PCR and Western blot analysis. The results indicated that valsartan could effectively suppress the local RAS to protect against experimental acute pancreatitis through inhibition of microcirculation disturbances and inflammation. The results suggest that pancreatic RAS plays a critical role in the regulation of pancreatic functions and demonstrates application potential as AT1 receptor antagonists. Moreover, other RAS inhibitors could be a new therapeutic target in acute pancreatitis. PMID:26170733

  7. Facilitators and barriers to doing workplace mental health research: Case study of acute psychological trauma in a public transit system.

    PubMed

    Links, Paul S; Bender, Ash; Eynan, Rahel; O'Grady, John; Shah, Ravi

    2016-03-10

    The Acute Psychological Trauma (APT) Study was a collaboration between an acute care hospital, a specialized multidisciplinary program designed to meet the mental health needs of injured workers, and a large urban public transit system. The overall purpose was to evaluate a Best Practices Intervention (BPI) for employees affected by acute psychological trauma compared to a Treatment as Usual (TAU) group. The specific purpose is to discuss facilitators and barriers that were recognized in implementing and carrying out mental health research in a workplace setting. Over the course of the APT study, a joint implementation committee was responsible for day-to-day study operations and made regular observations on the facilitators and barriers that arose throughout the study. The facilitators to this study included the longstanding relationships among the partners, increased recognition for the need of mental health research in the workplace, and the existence of a community advisory committee. The significant barriers to doing this study of mental health research in the workplace included differences in organizational culture, inconsistent union support, co-interventions, and stigma. Researchers and funding agencies need to be flexible and provide additional resources in order to overcome the barriers that can exist doing workplace mental health research.

  8. Application of Computed Tomography Processed by Picture Archiving and Communication Systems in the Diagnosis of Acute Achilles Tendon Rupture.

    PubMed

    Xue, Hai-Peng; Liu, Xin-Wei; Tian, Jing; Xie, Bing; Yang, Chao; Zhang, Hao; Zhou, Da-Peng

    2016-01-01

    The applications of CT examination in the diagnosis of the acute Achilles tendon rupture (AATR) were investigated. A total of 36 patients with suspected acute Achilles tendon rupture were tested using physical examination, ultrasound, and 3DCT scanning, respectively. Then, surgery was performed for the patients who showed positive result in at least two of the three tests for AATR. 3DVR, MPR, and the other CT scan image processing and diagnosis were conducted in PACS (picture archiving and communication system). PACS was also used to measure the length of distal broken ends of the Achilles tendon (AT) to tendon calcaneal insertion. Our study indicated that CT has the highest accuracy in diagnosis of acute Achilles tendon complete rupture. The length measurement is matched between PACS and those actually measured in operation. CT not only demonstrates more details directly in three dimensions especially with the rupture involved calcaneal insertion flap but also locates the rupture region for percutaneous suture by measuring the length of distal stump in PACS without the effect of the position of ankle. The accuracy of CT diagnosis for Achilles tendon partial rupture is yet to be studied.

  9. Public Health Response Systems In-Action: Learning from Local Health Departments’ Experiences with Acute and Emergency Incidents

    PubMed Central

    Hunter, Jennifer C.; Yang, Jane E.; Crawley, Adam W.; Biesiadecki, Laura; Aragón, Tomás J.

    2013-01-01

    As part of their core mission, public health agencies attend to a wide range of disease and health threats, including those that require routine, acute, and emergency responses. While each incident is unique, the number and type of response activities are finite; therefore, through comparative analysis, we can learn about commonalities in the response patterns that could improve predictions and expectations regarding the resources and capabilities required to respond to future acute events. In this study, we interviewed representatives from more than 120 local health departments regarding their recent experiences with real-world acute public health incidents, such as infectious disease outbreaks, severe weather events, chemical spills, and bioterrorism threats. We collected highly structured data on key aspects of the incident and the public health response, particularly focusing on the public health activities initiated and community partners engaged in the response efforts. As a result, we are able to make comparisons across event types, create response profiles, and identify functional and structural response patterns that have import for future public health preparedness and response. Our study contributes to clarifying the complexity of public health response systems and our analysis reveals the ways in which these systems are adaptive to the character of the threat, resulting in differential activation of functions and partners based on the type of incident. Continued and rigorous examination of the experiences of health departments throughout the nation will refine our very understanding of what the public health response system is, will enable the identification of organizational and event inputs to performance, and will allow for the construction of rich, relevant, and practical models of response operations that can be employed to strengthen public health systems. PMID:24236137

  10. A Case of Rituximab Use as an Induction and Maintenance of Remission in ANCA-Associated Vasculitis

    PubMed Central

    Hafiz, Shahd; Albeity, Abdurahman; Almoallim, Hani

    2016-01-01

    Antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV) is a multisystem autoimmune disease affecting mainly microscopic blood vessels due to circulating autoantibodies against neutrophil cytoplasmic antigens. We report a case of a 57-year-old female patient presenting with hemoptysis, sinusitis, and conjunctivitis. Based on lung biopsy, the diagnosis of antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV) was established. She was put on rituximab as induction and maintenance therapy. She responded initially to rituximab as induction therapy but failed to respond in the maintenance course of the drug. Rituximab was stopped and mycophenolate mofetil was administered. She responded as laboratory c-ANCA titers turned negative and symptoms subsided. There are no randomized clinical trials addressing rituximab effect in induction and remission at the same time. This case report doubts the efficacy of the use of rituximab therapy for both induction and maintenance of remission at the same time, waiting for the results of the ongoing trials. PMID:27006851

  11. Cryoglobulinemia-related vasculitis during effective anti-HCV treatment with PEG-interferon alfa-2b.

    PubMed

    De Blasi, T; Aguilar Marucco, D; Cariti, G; Maiello, A; De Rosa, F G; Di Perri, G

    2008-06-01

    HCV infection may be related to many extrahepatic manifestations including mixed cryoglobulinemia (MC). Clinical manifestations commonly associated to MC include arthralgia, purpura, vasculitis, peripheral neuropathy and renal function abnormalities. Treatment with interferon often leads to remission, especially in virological responders, or to disappearance of MC-related clinical manifestations. We report on a patient with chronic hepatitis C, deficit of G6P-DH, type II MC, who developed a cryoglobulinemic vasculitis with purpura, renal impairment and arterial hypertension, during treatment with PEG-interferon a-2b plus amantadine. The occurrence of purpuric lesions and MC-related nephropathy with increased cryocrit despite negative viremia, in a patient previously asymptomatic, during interferon treatment, is unusual.

  12. A Complicated Course of Acute Viral Induced Pharyngitis, Icteric Hepatitis, Acalculous Cholecystitis, and Skin Rash

    PubMed Central

    Erfani, Seddigheh Sadat

    2016-01-01

    This case reveals the complexities and challenges in the diagnosis of acute Epstein-Barr virus (EBV) infection, indicating the potential relationship between EBV infection and severe icteric hepatitis, acalculous cholecystitis, and lymphocytic vasculitis. We suggest including EBV infectious mononucleosis in the list of differential diagnoses when any of these clinical syndromes (or a combination thereof) occurs without apparent cause, especially in the presence of lymphocytosis. To our knowledge, this is the first report to suggest the possible role of EBV in the pathogenesis of cutaneous lymphocytic vasculitis. Also it is possible that EBV infection triggered the flare-up of the underlying rheumatologic disease. Therefore, it could be assumed that a part of the clinical syndrome (e.g., dermatologic manifestations) might be related to the flare-up of the underlying rheumatologic disease. PMID:27847520

  13. Chronic recurrent multifocal osteomyelitis with Crohn's disease exacerbation and vasculitis after granulocyte colony-stimulating factor therapy.

    PubMed

    Manners, P; Robbins, P

    2000-10-01

    Chronic recurrent multifocal osteomyelitis (CRMO), of unknown etiology, is characterized by recurring non-suppurative lesions of bone in multiple sites, and has been considered to be self-limiting. Reported therapies include prolonged antibiotics, corticosteroids and anti-inflammatory medications. This case is presented to illustrate the following: 1) CRMO may be severe, on-going, and unresponsive to treatment; 2) it may be associated with Crohns' disease; 3) the use of granulocyte colony-stimulating factor (G-CSF) may be associated with severe gastrointestinal vasculitis. A male was treated from ages 11-20 years for CRMO (manifesting as multiple bone lesions), with therapies of variable efficacy (anti-inflammatories, antibiotics, corticosteroids, gammaglobulin and methotrexate). With increasing disruption to his life, a 10-day course of granulocyte colony-stimulating factor (G-CSF) was given with benefit seen on magnetic resonance imaging (MRI). With exacerbation of symptoms one month later, G-CSF was re-commenced but ceased after 3 weeks because of abdominal pain, rectal blood loss, and progression of bone lesions with subsequent removal of portions of ileum, colon and appendix, which showed vasculitis. Months later, a colonoscopy revealed perianastomotic ulcers and continuing gastroenterological ulceration not unlike Crohn's disease. With azathioprine, gut and bone symptoms improved. We conclude that 1) CRMO may adversely affect life for years; 2) proven treatments are unavailable; 3) gastroenterological vasculitis/ Crohn's may be associated with CRMO; 4) MRI is useful for monitoring CRMO; 5) In this patient, G-CSF seemed beneficial initially, but later, vasculitis (possibly Crohn's) manifested, leading to bowel resection; 6) Crohn's disease may have been present for years, masked by corticosteroid, and unmasked by reduction of steroids and use of G-CSF.

  14. Accuracy and Predictability of PANC-3 Scoring System over APACHE II in Acute Pancreatitis: A Prospective Study

    PubMed Central

    Vishnu, Vikram Hubbanageri; Muniyappa, Shridhar; Prasath, Arun

    2017-01-01

    Introduction Acute Pancreatitis (AP) is one of the common conditions encountered in the emergency room. The course of the disease ranges from mild form to severe acute form. Most of these episodes are mild and spontaneously subsiding within 3 to 5 days. In contrast, Severe Acute Pancreatitis (SAP) occurring in around 15-20% of all cases, mortality can range between 10 to 85% across various centres and countries. In such a situation we need an indicator which can predict the outcome of an attack, as severe or mild, as early as possible and such an indicator should be sensitive and specific enough to trust upon. PANC-3 scoring is such a scoring system in predicting the outcome of an attack of AP. Aim To assess the accuracy and predictability of PANC-3 scoring system over APACHE II in predicting severity in an attack of AP. Materials and Methods This prospective study was conducted on 82 patients admitted with the diagnosis of pancreatitis. Investigations to evaluate PANC-3 and APACHE II were done on all the patients and the PANC-3 and APACHE II score was calculated. Results PANC-3 score has a sensitivity of 82.6% and specificity of 77.9%, the test had a Positive Predictive Value (PPV) of 0.59 and Negative Predictive Value (NPV) of 0.92. Sensitivity of APACHE II in predicting SAP was 91.3% and specificity was 96.6% with PPV of 0.91, NPV was 0.96. Conclusion Our study shows that PANC-3 can be used to predict the severity of pancreatitis as efficiently as APACHE II. The interpretation of PANC-3 does not need expertise and can be applied at the time of admission which is an advantage when compared to classical scoring systems.

  15. Elevation of Intact and Proteolytic Fragments of Acute Phase Proteins Constitutes the Earliest Systemic Antiviral Response in HIV-1 Infection

    PubMed Central

    Kramer, Holger B.; Lavender, Kerry J.; Qin, Li; Stacey, Andrea R.; Liu, Michael K. P.; di Gleria, Katalin; Simmons, Alison; Gasper-Smith, Nancy; Haynes, Barton F.; McMichael, Andrew J.; Borrow, Persephone; Kessler, Benedikt M.

    2010-01-01

    The earliest immune responses activated in acute human immunodeficiency virus type 1 infection (AHI) exert a critical influence on subsequent virus spread or containment. During this time frame, components of the innate immune system such as macrophages and DCs, NK cells, β-defensins, complement and other anti-microbial factors, which have all been implicated in modulating HIV infection, may play particularly important roles. A proteomics-based screen was performed on a cohort from whom samples were available at time points prior to the earliest positive HIV detection. The ability of selected factors found to be elevated in the plasma during AHI to inhibit HIV-1 replication was analyzed using in vitro PBMC and DC infection models. Analysis of unique plasma donor panels spanning the eclipse and viral expansion phases revealed very early alterations in plasma proteins in AHI. Induction of acute phase protein serum amyloid A (A-SAA) occurred as early as 5–7 days prior to the first detection of plasma viral RNA, considerably prior to any elevation in systemic cytokine levels. Furthermore, a proteolytic fragment of alpha–1-antitrypsin (AAT), termed virus inhibitory peptide (VIRIP), was observed in plasma coincident with viremia. Both A-SAA and VIRIP have anti-viral activity in vitro and quantitation of their plasma levels indicated that circulating concentrations are likely to be within the range of their inhibitory activity. Our results provide evidence for a first wave of host anti-viral defense occurring in the eclipse phase of AHI prior to systemic activation of other immune responses. Insights gained into the mechanism of action of acute-phase reactants and other innate molecules against HIV and how they are induced could be exploited for the future development of more efficient prophylactic vaccine strategies. PMID:20463814

  16. Effect of acute and chronic bilateral visual deafferentation on c-Fos immunoreactivity in the visual system of adult rats.

    PubMed

    Wiedmann, Rhea; Rosahl, Steffen K; Brinker, Thomas; Samii, Madjid; Nakamura, Makoto

    2013-09-01

    In our study we examined acute and chronic changes in c-Fos expression patterns in the visual system of the rat after complete visual deafferentation. In 20 male Lewis rats, the retro-bulbar part of the optic nerve was sectioned bilaterally. Ten animals underwent c-Fos immunohistochemistry after 3 days and 10 animals after 3 weeks examining time-dependent changes. The control group consisted of 10 animals, which did not undergo any surgical manipulation. c-Fos expression in the rat visual system experienced significant changes after acute and chronic bilateral complete visual deafferentation. Acute decrease in c-Fos level was observed in the ventral lateral geniculate nucleus, intergeniculate leaflet, superficial gray layer of the superior colliculus and layers IV and V of the primary visual cortex. After chronic deafferentation, c-Fos expression was also found to be decreased in the optic and deep layers of the superior colliculus and layer VI of the primary visual cortex. No change in c-Fos expression was observed in the dorsal lateral geniculate nucleus and layers I, II and III of the primary visual cortex. This work shows that secondary complete blindness does not lead to uniform decrease in c-Fos levels in all subcortical and cortical brain regions related to vision. These findings provide important information concerning expression of the immediate-early gene product c-Fos in secondary blind rodent models. It may further serve as a relevant baseline finding when electrical stimulation of the visual system is performed, aiding the assessment of visual neuroprosthesis using c-Fos as a functional mapping tool when evaluating different stimulus parameters in blind rodent models.

  17. Intra-Peritoneal Administration of Mitochondrial DNA Provokes Acute Lung Injury and Systemic Inflammation via Toll-Like Receptor 9

    PubMed Central

    Zhang, Lemeng; Deng, Songyun; Zhao, Shuangping; Ai, Yuhang; Zhang, Lina; Pan, Pinhua; Su, Xiaoli; Tan, Hongyi; Wu, Dongdong

    2016-01-01

    The pathogenesis of sepsis is complex. Mitochondrial dysfunction, which is responsible for energy metabolism, intrinsic apoptotic pathway, oxidative stress, and systemic inflammatory responses, is closely related with severe sepsis induced death. Mitochondria DNA (mtDNA) contain un-methylated cytosine phosphate guanine (CpG) motifs, which exhibit immune stimulatory capacities. The aim of this study was to investigate the role and mechanism of mtDNA release on lipopolysaccharide (LPS) induced acute lung injury (ALI) and systemic inflammation. Following LPS injection, plasma mtDNA copies peak at 8 h. Compared with wild-type (WT) mice, mtDNA in toll like receptor 4 knockout (TLR4 KO) mice were significantly decreased. MtDNA intra-peritoneal administration causes apparent ALI as demonstrated by increased lung injury score, bronchoalveolar lavage fluid (BALF) total protein and wet/dry (W/D) ratio; mtDNA injection also directly provokes systemic inflammation, as demonstrated by increased IL-1β, IL-6, high-mobility group protein B1 (HMGB1) level; while nuclear DNA (nDNA) could not induce apparent ALI and systemic inflammation. However, compared with WT mice, TLR4 KO could not protect from mtDNA induced ALI and systemic inflammation. Specific TLR9 inhibitor, ODN 2088 pretreatment can significantly attenuate mtDNA induced ALI and systemic inflammation, as demonstrated by improved lung injury score, decreased lung wet/dry ratio, BALF total protein concentration, and decreased systemic level of IL-1β, IL-6 and HMGB1. MtDNA administration activates the expression of p-P38 mitogen-activated protein kinases (MAPK) in lung tissue and specific TLR9 inhibitor pretreatment can attenuate this activation. Thus, LPS-induced mtDNA release occurs in a TLR4-dependent manner, and mtDNA causes acute lung injury and systemic inflammation in a TLR9-dependent and TLR4-independent manner. PMID:27589725

  18. Spotlight on rituximab in the treatment of antineutrophil cytoplasmic antibody-associated vasculitis: current perspectives

    PubMed Central

    Moog, Philipp; Thuermel, Klaus

    2015-01-01

    A 54-year-old patient presented to his general practitioner because of strong muscle pain in both thighs. Inflammatory parameters (CRP 16.3 mg/dL) and white blood cells (15 g/L) were elevated. The patient reported a weight loss of 10 kg in 4 weeks. There was no fever or any other specific symptoms. Urine dipstick examination and computed tomography of the chest were unremarkable. Because of increasing symptoms, the patient was referred to our department. Magnetic resonance tomography showed diffuse inflammatory changes of the muscles of both thighs. Neurological examination and electrophysiology revealed axonal sensorimotor neuropathy and ground-glass opacities of both lungs had occurred. Serum creatinine increased to 229 μmol/L within a few days, with proteinuria of 3.3 g/g creatinine. Kidney biopsy showed diffuse pauci-immune proliferative glomerulonephritis. Proteinase 3-specific antineutrophil cytoplasmic antibodies were markedly increased. Birmingham Vasculitis Activity Score was 35. Within 2 days, serum creatinine further increased to 495 μmol/L. Plasma exchange, high-dose glucocorticosteroids, and hemodialysis were started. The patient received cyclophosphamide 1 g twice and rituximab 375 mg/m2 four times according to the RITUXVAS protocol. Despite ongoing therapy, hemodialysis could not be withdrawn and had to be continued over 3 weeks until diuresis normalized. Glucocorticosteroids were tapered to 20 mg after 2 months, and serum creatinine was 133 μmol/L. However, nephritic urinary sediment reappeared. Another dose of 1 g cyclophosphamide was given, and glucocorticosteroids were raised for another 4 weeks. After 6 months, the daily prednisolone dose was able to be tapered to 5 mg. Serum creatinine was 124 μmol/L, proteinuria further decreased to 382 mg/g creatinine, and the Birmingham Vasculitis Activity Score was 0. Maintenance therapy with rituximab 375 mg/m2 every 6 months was started. At the last visit after 8 months, the patient was still in

  19. Effect of acute stress on sexual behavior in female rats: participation of the central angiotensinergic system.

    PubMed

    Cecconello, Ana Lúcia; Raineki, Charlis; Sebben, Vanise; Lucion, Aldo Bolten; Sanvitto, Gilberto Luiz

    2010-03-05

    Stress might influence the reproductive behavior in females, and central angiotensin II (Ang II) is a peptide that plays a role in stress response and in the modulation of sexual behavior. The medial amygdala (MeA), an important structure that regulates this behavior, is strongly involved in stress response. The aim of the present study was to evaluate the effect of acute restraint stress on the night of proestrus on sexual receptivity in female rats and the participation of Ang II and MeA in this effect. Adult female Wistar rats with regular estrous cycles were utilized. The acute stress protocol utilized was the restraint stress for 15 min on the night of proestrus. The participation of Ang II was evaluated by injecting Ang II and Ang II receptor antagonists (losartan and PD12319) into the MeA. The lordosis quotient was recorded. The stress or the microinjection of Ang II into the MeA significantly reduced sexual behavior. The blockade of AT(1) or AT(2) receptors in the MeA prevented the effect of stress and the effect of Ang II microinjection into this nucleus on sexual receptivity. We concluded that acute restraint stress on the night of proestrus reduces sexual behavior in rats, and this effect is mediated by both AT(1) and AT(2) receptors in the MeA.

  20. Secondhand smoke as an acute threat for the cardiovascular system: a change in paradigm.

    PubMed

    Raupach, Tobias; Schäfer, Katrin; Konstantinides, Stavros; Andreas, Stefan

    2006-02-01

    The evidence that active smoking is a risk factor for cardiovascular disease (CVD) and the leading cause of preventable death is overwhelming. However, numerous epidemiological findings indicate that even passive exposure to cigarette smoke may exert detrimental effects on vascular homoeostasis. Recent experimental data provide a deeper insight into the pathophysiological mechanisms linking secondhand smoke (SHS) to CVD. Importantly, most of these effects appear to be characterized by a rapid onset. For example, the relatively low doses of toxins inhaled by passive smoking are sufficient to elicit acute endothelial dysfunction, and these effects may be related, at least in part, to the inactivation of nitric oxide. Moreover, passive smoking may directly impair the viability of endothelial cells and reduce the number and functional activity of circulating endothelial progenitor cells. In addition, platelets of non-smokers appear to be susceptible to pro-aggregatory changes with every passive smoke exposure. Overall, SHS induces oxidative stress and promotes vascular inflammation. Apart from vasoconstriction and thrombus formation, however, the myocardial oxygen balance is further impaired by SHS-induced adrenergic stimulation and autonomic dysfunction. These data strongly suggest that passive smoking is capable of precipitating acute manifestations of CVD (atherothrombosis) and may also have a negative impact on the outcome of patients who suffer acute coronary syndromes.

  1. [Changes in some of the kallikrein-kinin system indices in patients with acute pancreatitis].

    PubMed

    Uchikov, P; Terzieva, D; Shtereva, S; Sirakova, I

    1999-01-01

    Proceeding from the major role played by kinins in the pathophysiology of endogenic intoxication among acute pancreatitis patients (AP), and the conflicting and scarce literature data on the issue, the changes in the level of prekallikrein, high-molecular kininogen, alpha 2-macroglobulin, alpha 1-antitrypsin, plasminogen and carboxypeptidase N in the blood are studied in dynamics at 1, 3, 5, 7, 9 and 14 days after admission of 48 patients with mild, and 121 with severe form of acute pancreatitis. Forty-eight individuals are used for control purpose. PK, KG and plasminogen are assayed using the colorimetric method of the Boehringer Company--Mannheim, KG--by chronometric test of the Sigma Diagnostics Company, CPO N--after Folk's method, as modified by Erdös, alpha 2-MG--by radial immunodiffusion according to Mancini, and alpha 1-AJ--by immunoturbidometric method. As shown by the results, in acute pancreatitis KKS activation occurs, demonstrated by the reduced PK, KG and alpha 2-MG values, and by the statistically significant enhancement of alpha 1-DJ, COP N and plasminogen activity. In patients presenting mild forms the aforementioned changes are rather weakly manifested and transient, while in the serious forms they are markedly expressed and persisting. In either form the deviations are rather pronounced in the first three days of disease. Coinciding with a clinical course characterized by cardiovascular changes similarly strongly manifested.

  2. An evaluation process for an electronic bar code medication administration information system in an acute care unit.

    PubMed

    Bargren, Michelle; Lu, Der-Fa

    2009-01-01

    The purpose of this case study is to present an evaluation process and recommendations for addressing the gaps found with the implementation of a new bar code medication administration (BCMA) technology in a busy acute care hospital unit. The case study analyzes workflow procedures associated with administration of medications in an inpatient labor and delivery care unit before and one year after implementation of BCMA technology. The comparison reveals a twofold increase in workflow procedures for nursing staff because of the new technology. System gaps are identified from a nursing user's perspective, and recommendations are offered to close those gaps.

  3. Progressive disseminated histoplasmosis in systemic lupus erythematosus-an unusual presentation of acute tenosynovitis and a literature review.

    PubMed

    Lim, Sian Yik; Kijsirichareanchai, Kunut; Winn, Richard

    2013-01-01

    Progressive disseminated histoplasmosis is a disease where Histoplasma capsulatum affects multiple organs due to the inability of host cellular immunity to control the infection. Progressive disseminated histoplasmosis mainly involves the bone marrow, liver, and lungs. We report an unusual initial presentation of progressive disseminated histoplasmosis presenting as acute tenosynovitis in a systemic lupus erythematosus (SLE) patient. This report highlights the point that H. capsulatum may present as focal lesions and a high level of suspicion is needed to make the diagnosis, especially in SLE patients. We specifically reviewed reported cases of progressive disseminated histoplasmosis in SLE patients, and a review of the literature is presented.

  4. Effects of acute versus post-acute systemic delivery of neural progenitor cells on neurological recovery and brain remodeling after focal cerebral ischemia in mice.

    PubMed

    Doeppner, T R; Kaltwasser, B; Teli, M K; Bretschneider, E; Bähr, M; Hermann, D M

    2014-08-21

    Intravenous transplantation of neural progenitor cells (NPCs) induces functional recovery after stroke, albeit grafted cells are not integrated into residing neural networks. However, a systematic analysis of intravenous NPC delivery at acute and post-acute time points and their long-term consequences does not exist. Male C57BL6 mice were exposed to cerebral ischemia, and NPCs were intravenously grafted on day 0, on day 1 or on day 28. Animals were allowed to survive for up to 84 days. Mice and tissues were used for immunohistochemical analysis, flow cytometry, ELISA and behavioral tests. Density of grafted NPCs within the ischemic hemisphere was increased when cells were transplanted on day 28 as compared with transplantation on days 0 or 1. Likewise, transplantation on day 28 yielded enhanced neuronal differentiation rates of grafted cells. Post-ischemic brain injury, however, was only reduced when NPCs were grafted at acute time points. On the contrary, reduced post-ischemic functional deficits due to NPC delivery were independent of transplantation paradigms. NPC-induced neuroprotection after acute cell delivery was due to stabilization of the blood-brain barrier (BBB), reduction in microglial activation and modulation of both peripheral and central immune responses. On the other hand, post-acute NPC transplantation stimulated post-ischemic regeneration via enhanced angioneurogenesis and increased axonal plasticity. Acute NPC delivery yields long-term neuroprotection via enhanced BBB integrity and modulation of post-ischemic immune responses, whereas post-acute NPC delivery increases post-ischemic angioneurogenesis and axonal plasticity. Post-ischemic functional recovery, however, is independent of NPC delivery timing, which offers a broad therapeutic time window for stroke treatment.

  5. An animal model to study toxicity of central nervous system therapy for childhood acute lymphoblastic leukemia: Effects on behavior

    SciTech Connect

    Mullenix, P.J.; Kernan, W.J.; Tassinari, M.S.; Schunior, A.; Waber, D.P.; Howes, A.; Tarbell, N.J. )

    1990-10-15

    Central nervous system prophylactic therapy used in the treatment of acute lymphoblastic leukemia can reduce intelligence quotient scores and impair memory and attention in children. Cranial irradiation, intrathecal methotrexate, and steroids are commonly utilized in acute lymphoblastic leukemia therapy. How they induce neurotoxicity is unknown. This study employs an animal model to explore the induction of neurotoxicity. Male and female Sprague-Dawley rats at 17 and 18 days of age were administered 18 mg/kg prednisolone, 2 mg/kg methotrexate, and 1000 cGy cranial irradiation. Another 18-day-old group was administered 1000 cGy cranial irradiation but no drugs. Matching controls received saline and/or a sham exposure to radiation. All animals at 6 weeks and 4 months of age were tested for alterations in spontaneous behavior. A computer pattern recognition system automatically recorded and classified individual behavioral acts displayed during exploration of a novel environment. Measures of behavioral initiations, total time, and time structure were used to compare treated and control animals. A permanent sex-specific change in the time structure of behavior was induced by the prednisolone, methotrexate, and radiation treatment but not by radiation alone. Unlike hyperactivity, the effect consisted of abnormal clustering and dispersion of acts in a pattern indicative of disrupted development of sexually dimorphic behavior. This study demonstrates the feasibility of an animal model delineating the agent/agents responsible for the neurotoxicity of central nervous system prophylactic therapy.

  6. Multi-walled carbon nanotube-physicochemical properties predict the systemic acute phase response following pulmonary exposure in mice.

    PubMed

    Poulsen, Sarah S; Knudsen, Kristina B; Jackson, Petra; Weydahl, Ingrid E K; Saber, Anne T; Wallin, Håkan; Vogel, Ulla

    2017-01-01

    Pulmonary exposure to multi-walled carbon nanotubes (MWCNTs) has been linked to an increased risk of developing cardiovascular disease in addition to the well-documented physicochemical-dependent adverse lung effects. A proposed mechanism is through a strong and sustained pulmonary secretion of acute phase proteins to the blood. We identified physicochemical determinants of MWCNT-induced systemic acute phase response by analyzing effects of pulmonary exposure to 14 commercial, well-characterized MWCNTs in female C57BL/6J mice pulmonary exposed to 0, 6, 18 or 54 μg MWCNT/mouse. Plasma levels of acute phase response proteins serum amyloid A1/2 (SAA1/2) and SAA3 were determined on day 1, 28 or 92. Expression levels of hepatic Saa1 and pulmonary Saa3 mRNA levels were assessed to determine the origin of the acute phase response proteins. Pulmonary Saa3 mRNA expression levels were greater and lasted longer than hepatic Saa1 mRNA expression. Plasma SAA1/2 and SAA3 protein levels were related to time and physicochemical properties using adjusted, multiple regression analyses. SAA3 and SAA1/2 plasma protein levels were increased after exposure to almost all of the MWCNTs on day 1, whereas limited changes were observed on day 28 and 92. SAA1/2 and SAA3 protein levels did not correlate and only SAA3 protein levels correlated with neutrophil influx. The multiple regression analyses revealed a protective effect of MWCNT length on SAA1/2 protein level on day 1, such that a longer length resulted in lowered SAA1/2 plasma levels. Increased SAA3 protein levels were positively related to dose and content of Mn, Mg and Co on day 1, whereas oxidation and diameter of the MWCNTs were protective on day 28 and 92, respectively. The results of this study reveal very differently controlled pulmonary and hepatic acute phase responses after MWCNT exposure. As the responses were influenced by the physicochemical properties of the MWCNTs, this study provides the first step towards designing

  7. Acute Hendra virus infection: Analysis of the pathogenesis and passive antibody protection in the hamster model

    SciTech Connect

    Guillaume, Vanessa; Wong, K. Thong; Looi, R.Y.; Georges-Courbot, Marie-Claude; Barrot, Laura; Buckland, Robin; Wild, T. Fabian; Horvat, Branka

    2009-05-10

    Hendra virus (HeV) and Nipah virus (NiV) are recently-emerged, closely related and highly pathogenic paramyxoviruses. We have analysed here the pathogenesis of the acute HeV infection using the new animal model, golden hamster (Mesocricetus auratus), which is highly susceptible to HeV infection. HeV-specific RNA and viral antigens were found in multiple organs and virus was isolated from different tissues. Dual pathogenic mechanism was observed: parenchymal infection in various organs, including the brain, with vasculitis and multinucleated syncytia in many blood vessels. Furthermore, monoclonal antibodies specific for the NiV fusion protein neutralized HeV in vitro and efficiently protected hamsters from HeV if given before infection. These results reveal the similarities between HeV and NiV pathogenesis, particularly in affecting both respiratory and neuronal system. They demonstrate that hamster presents a convenient novel animal model to study HeV infection, opening new perspectives to evaluate vaccine and therapeutic approaches against this emergent infectious disease.

  8. Fever and increased serum IL-1 activity as a systemic manifestation of acute phototoxicity in New Zealand White rabbits.

    PubMed

    Ansel, J C; Luger, T A; Green, I

    1987-07-01

    Ultraviolet (UV) radiation is a significant environmental hazard for humans and animals. Although the clinical effect of an acute UV exposure such as cutaneous inflammation, malaise, somnolence, chills, and fever have been appreciated for many years, the underlying mechanisms mediating these effects are poorly understood. Since chills and fever are the most dramatic systemic sequelae after a prolonged exposure to UV, we specifically examined the effect of whole-body UV irradiation on core body temperature and serum endogenous pyrogen activity of New Zealand White rabbits, correlating this with serum interleukin 1 (IL-1) activity and alterations of serum divalent cation levels. We found that an acute dose of UV irradiation (Westinghouse FS-40 lamps, 0.2 mJ/cm2/s X 8 h) resulted in a significant increase in the core body temperature 2 h post UV (0.8 degree C), peaking 5 h post UV (1.8 degree C), and returning to normal 24 h post UV. Likewise, the sera from the UV-irradiated rabbits had significant endogenous pyrogen activity when transferred into naive recipient animals, causing an increase in core body temperature within 45 min (0.65 +/- 0.12 degree C), decreasing over the next 2 h, and returning to normal 6 h post injection. No endotoxin contamination was detected in any serum samples. This post-UV febrile response was accompanied by a prolonged increase in serum IL-1 activity (5-10 X) and a significant alteration in serum divalent cation levels, with the rabbits becoming euthermic even as the serum IL-1 levels remained elevated. These findings provide new information concerning the pathogenesis and kinetics of these systemic effects after an acute dose of UV irradiation.

  9. Successful management of acute respiratory failure in an Idiopathic Pulmonary Fibrosis patient using an extracorporeal carbon dioxide removal system.

    PubMed

    Vianello, Andrea; Arcaro, Giovanna; Paladini, Luciana; Iovino, Silvia

    2016-08-01

    Patients with Idiopathic Pulmonary Fibrosis (IPF) requiring Invasive Mechanical Ventilation (IMV) following unsuccessful treatment with Non-Invasive Ventilation (NIV) have a high mortality rate. IMV is, moreover, an independent predictor of poor outcome during the post-transplantation period in patients on waiting lists for Lung Transplantation (LT). Here we describe the successful management of an IPF patient with acute respiratory failure (ARF) using a pump-assisted veno-venous system for extracorporeal CO2 removal (ECCO2R) (ProLUNG® system) as an alternative to endotracheal intubation (ETI) following NIV failure. Given this positive experience, further studies are warranted focusing on the ECCO2R system's tolerability, safety, and efficacy in patients with IPF and severe ARF in whom NIV alone is ineffective.

  10. Anti-oxidized low-density lipoprotein antibodies in myeloperoxidase–positive vasculitis patients preferentially recognize hypochlorite-modified low density lipoproteins

    PubMed Central

    Slot, M C; Theunissen, R; van Paassen, P; Damoiseaux, J G M C; Cohen Tervaert, J W

    2007-01-01

    Many patients surviving vasculitis are prone to accelerated atherosclerosis and often have enhanced levels of antibodies to oxidized low-density lipoprotein (oxLDL). To measure anti-oxLDL antibodies, oxidation of LDL is achieved with copper (Cu) or malondialdehyde (MDA). Because, in vivo, LDL may be oxidized with myeloperoxidase (MPO) or its product hypochlorite, we measured anti-hypochlorite LDL antibodies in patients with vasculitis, haemodialysis patients and healthy controls. A newly developed enzyme-linked immunosorbent assay (ELISA) was used to detect antibodies to oxLDL as modified by hypochlorite. Results are compared with data obtained by standard LDL oxidation using MDA–LDL or Cu–LDL as substrate. Results were compared between anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) patients (n = 93), haemodialysis (HD) patients (n = 59) and healthy controls (HC; n = 43). Furthermore, patients with MPO–ANCA-associated vasculitis (n = 47) were compared with patients with proteinase 3 (PR3)–ANCA associated vasculitis (n = 46). Optimal cut-off points were determined by receiver operator characteristic (ROC) curve analysis. Anti-oxLDL antibodies are enhanced in AAV patients (MDA–LDL and hypochlorite–LDL) and in HD patients (hypochlorite–LDL), when compared to HC. Furthermore, patients with MPO–ANCA-associated vasculitis had higher levels of antibodies to hypochlorite–LDL than patients with PR3–ANCA-associated vasculitis. Our newly developed assay, in which hypochlorite–LDL is used as substrate, seems a more sensitive assay than traditional assays to measure oxLDL antibodies. Furthermore, our results suggest that enhanced MPO-mediated LDL oxidation occurs in patients with MPO–ANCA. PMID:17521320

  11. Kaposi's sarcoma with HHV8 infection and ANCA-associated vasculitis in a hemodialysis patient.

    PubMed

    Fatma, Lilia Ben; Rais, Lamia; Mebazza, Amel; Azzouz, Haifa; Beji, Somaya; Krid, Madiha; Smaoui, Wided; Maiz, Hedi Ben; Zouaghi, Karim; Zitouna, Moncef; Osmane, Amel Ben; Moussa, Fatma Ben

    2013-11-01

    The association between Kaposi's sarcoma (KS) and human herpes virus eight (HHV-8) infection is rarely reported in hemodialysis (HD) patients. We report here the rare association of KS, HHV-8 and hepatitis C virus (HCV) infection as well as syphilis in a HD patient. We report the case of a 72-year-old woman who presented with microscopic polyangiitis with alveolar hemorrhage and pauci-immune necrosing and crescentic glomerulonephritis as well as renal failure requiring HD. Biological tests showed positive HCV and syphilis tests. The patient was treated by HD and intravenous pulse, followed by oral corticosteroids and six cyclophosphamide monthly pulses with remission of the alveolar hemorrhage, but without renal functional recovery as the patient remained HD dependent. Five months after the first treatment administration, she developed extensive purpuric lesions on her lower limbs, abdomen face and neck. A skin biopsy showed KS. The HHV-8 test was positive, with positive polymerase chain reaction-HHV8 in the serum and skin. After immunosuppression withdrawal, the KS skin lesions regressed rapidly without relapse after 12 months of follow-up, but alveolar hemorrhage relapsed after 16 months of follow-up. Our case showed that the immunosuppressed state related to multiple factors such as aging, vasculitis, HHV-8, HCV, syphilis, immunosuppressive therapy and HD may all have contributed to the development of KS in our patient.

  12. Relapsing polychondritis with p-ANCA associated vasculitis: Which triggers the other?

    PubMed Central

    File, Ibolya; Trinn, Csilla; Mátyus, Zsolt; Ujhelyi, László; Balla, József; Mátyus, János

    2014-01-01

    Relapsing polychondritis (RP) is a rare autoimmune disease with chronic inflammatory/destructive lesions of the cartilaginous tissues. In one third of the cases it is associated with other autoimmune disorders, mostly with anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). We report three cases of RP with p-ANCA positive AAV. In the first patient RP developed 1.5 years after the onset of AAV. In the others the signs of RP were present before the onset of severe crescent glomerulonephritis. Patients responded well on steroid and cyclophosphamide. In dialysis dependent cases plasmapheresis was also used successfully. During the 2 and 1.5 years of follow up, they were symptom-free, and had stable glomerular filtration rate. The first patient died after four years of follow-up due to the complications of sudden unset pancytopenia, which raises the possibility of associated hemophagocytic syndrome. In the setting of RP or AAV physicians should always be aware of the possibility of sudden or insidious appearance of the other disease. PMID:25516870

  13. Circulating ADAM17 Level Reflects Disease Activity in Proteinase-3 ANCA-Associated Vasculitis.

    PubMed

    Bertram, Anna; Lovric, Svjetlana; Engel, Alissa; Beese, Michaela; Wyss, Kristin; Hertel, Barbara; Park, Joon-Keun; Becker, Jan U; Kegel, Johanna; Haller, Hermann; Haubitz, Marion; Kirsch, Torsten

    2015-11-01

    ANCA-associated vasculitides are characterized by inflammatory destruction of small vessels accompanied by enhanced cleavage of membrane-bound proteins. One of the main proteases responsible for ectodomain shedding is disintegrin and metalloproteinase domain-containing protein 17 (ADAM17). Given its potential role in aggravating vascular dysfunction, we examined the role of ADAM17 in active proteinase-3 (PR3)-positive ANCA-associated vasculitis (AAV). ADAM17 concentration was significantly increased in plasma samples from patients with active PR3-AAV compared with samples from patients in remission or from other controls with renal nonvascular diseases. Comparably, plasma levels of the ADAM17 substrate syndecan-1 were significantly enhanced in active AAV. We also observed that plasma-derived ADAM17 retained its specific proteolytic activity and was partly located on extracellular microparticles. Transcript levels of ADAM17 were increased in blood samples of patients with active AAV, but those of ADAM10 or tissue inhibitor of metalloproteinases 3, which inhibits ADAMs, were not. We also performed a microRNA (miR) screen and identified miR-634 as significantly upregulated in blood samples from patients with active AAV. In vitro, miR-634 mimics induced a proinflammatory phenotype in monocyte-derived macrophages, with enhanced expression and release of ADAM17 and IL-6. These data suggest that ADAM17 has a prominent role in AAV and might account for the vascular complications associated with this disease.

  14. Current and novel biomarkers in anti-neutrophil cytoplasm-associated vasculitis

    PubMed Central

    Draibe, Juliana Bordignon; Fulladosa, Xavier; Cruzado, Josep Maria; Torras, Joan; Salama, Alan David

    2016-01-01

    Anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) is characterized by a variable disease course, with up to 50% of patients having one relapse within 5 years and many progressing to end-stage organ damage despite modern treatment strategies. Moreover, complications arising from treatment dominate the causes of mortality and morbidity both early and late during disease, especially in the elderly and those with severe renal involvement, and there is additional uncertainty as to how long treatment should be continued. There is, therefore, an urgent clinical need to identify robust biomarkers to better predict treatment responses, risk of disease relapse and eventual complete clinical and immunological quiescence. To date, no such biomarkers exist, but better understanding of disease pathogenesis and the underlying immune dysfunction has provided some potential candidates linked to the discovery of new antibodies, different leukocyte activation states, the role of the alternative complement pathway and markers of vascular activation. With all promising new biomarkers, there is the need to rapidly replicate and validate early findings using large biobanks of samples that could be brought together by leaders in the field. PMID:27478594

  15. Possible intrinsic association of anti-neutrophil cytoplasmic antibody-associated vasculitis coexisting with multiple myeloma

    PubMed Central

    Liu, Huifang; Xiong, Jiachuan; Zhang, Jun; Zhang, Ying; Nie, Ling; Wang, Yiqin; Zhao, Jinghong

    2016-01-01

    Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a life-threatening condition that causes renal failure. Multiple myeloma (MM) is a malignant proliferation of monoclonal plasma cells in the blood that can also cause renal failure. The two diseases have high morbidity and mortality rates in the elderly, with a poor prognosis. A 64-year-old female presented to Xinqiao Hospital (Chonqing, China) with fatigue and a poor appetite that had been apparent for 6 weeks. Laboratory tests revealed a serum creatinine level of 10.31 mg/dl, a cytoplasmic ANCA titer of 1:10, a positive result for myeloperoxidase and a serum globulin level of 3.96 g/dl. A renal biopsy revealed crescent glomerulonephritis, combined with the rapid progression of renal function. Based on these observations (ANCA titer, crescent glomerulonephritis and rapid progression of renal function) a diagnosis of AAV was established. MM was confirmed by serum immunofixation electrophoresis combined with bone marrow aspiration. The present study discusses what is, to the best of our knowledge, the first case of AAV coexisting with MM in order to highlight it as a clinical concern. PMID:27602144

  16. Circulating ADAM17 Level Reflects Disease Activity in Proteinase-3 ANCA-Associated Vasculitis

    PubMed Central

    Bertram, Anna; Lovric, Svjetlana; Engel, Alissa; Beese, Michaela; Wyss, Kristin; Hertel, Barbara; Park, Joon-Keun; Becker, Jan U.; Kegel, Johanna; Haller, Hermann; Haubitz, Marion

    2015-01-01

    ANCA-associated vasculitides are characterized by inflammatory destruction of small vessels accompanied by enhanced cleavage of membrane-bound proteins. One of the main proteases responsible for ectodomain shedding is disintegrin and metalloproteinase domain-containing protein 17 (ADAM17). Given its potential role in aggravating vascular dysfunction, we examined the role of ADAM17 in active proteinase-3 (PR3)-positive ANCA-associated vasculitis (AAV). ADAM17 concentration was significantly increased in plasma samples from patients with active PR3-AAV compared with samples from patients in remission or from other controls with renal nonvascular diseases. Comparably, plasma levels of the ADAM17 substrate syndecan-1 were significantly enhanced in active AAV. We also observed that plasma-derived ADAM17 retained its specific proteolytic activity and was partly located on extracellular microparticles. Transcript levels of ADAM17 were increased in blood samples of patients with active AAV, but those of ADAM10 or tissue inhibitor of metalloproteinases 3, which inhibits ADAMs, were not. We also performed a microRNA (miR) screen and identified miR-634 as significantly upregulated in blood samples from patients with active AAV. In vitro, miR-634 mimics induced a proinflammatory phenotype in monocyte-derived macrophages, with enhanced expression and release of ADAM17 and IL-6. These data suggest that ADAM17 has a prominent role in AAV and might account for the vascular complications associated with this disease. PMID:25788529

  17. Dermatomyositis Leading to Necrotizing Vasculitis: A Perfect Response to Applied Therapy

    PubMed Central

    Akbaryan, Mahmood; Darabi, Farideh; Soltani, Zahra

    2016-01-01

    Dermatomyositis is an idiopathic inflammatory myopathy that cause skin and muscle complications. The ethiology is not understood well yet. Released cytokines including interferon and interleukins are suggested to make inflammatory responses in the skin or muscle. Muscle weakness and skin lesions including heliotrope rash, shawl sign and Gottron’s papules are the most common symptoms. A biopsy (muscle or skin) is always the most reliable method for diagnosis. Corticosteroids in association with immunosuppressive agents are used as standard treatment. The patient was a 30 years old woman who got involved with dermatomyositis for 10 years. She has been under therapy with Methotrexate, Prednisolon and Azathioprine until she came to us suffering from progressive skin lesions. Experiments and examinations were normal except the lesions and detected lipoatrophy. Because of immune cells infiltration and observations necrotizing vasculitis was diagnosed. After three month of high dose prednisolon and intravenous cyclophosphamide therapy the lesions vanished remarkable. True and immediate diagnosis gives physicians the chance not only to assess the best treatment but have adequate time to apply the procedure. However shortening the therapy and diminishing morbidity of the disease need more investigations and efforts. PMID:28190982

  18. Association of retinal vasculitis (Eales' disease) and Meniere-like vestibulocochlear symptoms.

    PubMed

    Wagner, Wolfgang; Fehrmann, Astrid

    2006-02-01

    The objective of this study was to present a previously unknown association of visual loss and Meniere-like vestibuloauditory symptoms in Eales' disease (idiopathic retinal vasculitis) and to give a survey of other conditions affecting both the retina and inner ear. A 32-year-old man diagnosed with Eales' disease presented with recurrent deteriorations of visual acuity and simultaneous recurrent vestibuloauditory symptoms strongly reminiscent of Meniere's disease. Both the recurrent low frequency hearing losses and the losses of visual acuity could be restituted repeatedly by high dose i.v. corticosteroids. A literature review reveals that in a number of other retinopathic diseases associated with Meniere-like symptoms, microvasculopathy is also the pathomechanism. A common pathogenetic cause, e.g., autoimmune-mediated microvasculitis of retinal periphlebitis and vestibulocochlear symptoms, in this case is suggested. The presented case adds new evidence to microvasculopathy being a pathogenetic factor in Meniere's disease. In cases of coincidence of vestibulocochlear symptoms and retinal perivasculitis, a therapeutic attempt with high-dose corticosteroids is advisable.

  19. Dermatomyositis Leading to Necrotizing Vasculitis: A Perfect Response to Applied Therapy.

    PubMed

    Akbaryan, Mahmood; Darabi, Farideh; Soltani, Zahra

    2016-12-01

    Dermatomyositis is an idiopathic inflammatory myopathy that cause skin and muscle complications. The ethiology is not understood well yet. Released cytokines including interferon and interleukins are suggested to make inflammatory responses in the skin or muscle. Muscle weakness and skin lesions including heliotrope rash, shawl sign and Gottron's papules are the most common symptoms. A biopsy (muscle or skin) is always the most reliable method for diagnosis. Corticosteroids in association with immunosuppressive agents are used as standard treatment. The patient was a 30 years old woman who got involved with dermatomyositis for 10 years. She has been under therapy with Methotrexate, Prednisolon and Azathioprine until she came to us suffering from progressive skin lesions. Experiments and examinations were normal except the lesions and detected lipoatrophy. Because of immune cells infiltration and observations necrotizing vasculitis was diagnosed. After three month of high dose prednisolon and intravenous cyclophosphamide therapy the lesions vanished remarkable. True and immediate diagnosis gives physicians the chance not only to assess the best treatment but have adequate time to apply the procedure. However shortening the therapy and diminishing morbidity of the disease need more investigations and efforts.

  20. TRAIL administration down-modulated the acute systemic inflammatory response induced in a mouse model by muramyldipeptide or lipopolysaccharide.

    PubMed

    Marcuzzi, Annalisa; Secchiero, Paola; Crovella, Sergio; Zauli, Giorgio

    2012-10-01

    The potent inducer of apoptosis TRAIL/Apo2 ligand is now under considerations in clinical trials for the treatment of different types of cancer. Since the natural history of cancer is often characterized by microbial infections, we have investigated the effect of recombinant human TRAIL in a mouse model of systemic acute inflammation of microbial origin represented by BALB/c mice treated with either bacterial muramyldipeptide (MDP) or lipopolysaccharide (LPS). When administered intraperitoneally (i.p.), these inflammatory bacterial compounds triggered a severe systemic inflammatory response within 2h, represented by body temperature elevation, increase of circulating serum amyloid-A (SAA) and of the number of leukocytes in the peritoneal cavity. Moreover, both MDP and LPS induced a significant elevation of the circulating levels of several inflammatory cytokines and chemokines. Noteworthy, pre-treatment with recombinant human TRAIL 48 and 72 h before administration of either MDP or LPS, significantly counteracted all acute inflammatory responses, including the elevation of key pro-inflammatory cytokines/chemokines such as IL-1α, IL-6, G-CSF, MCP-1. These data demonstrate for the first time that TRAIL has a potent anti-inflammatory activity, which might be beneficial for the anti-tumoral activity of TRAIL.

  1. [Dipodascus capitatus (Geotrichum capitatum): fatal systemic infection on patient with acute myeloid leukemia].

    PubMed

    Lafayette, Thereza Christina Sampaio; Oliveira, Loiva Therezinha Otonelli; Landell, Melissa; Valente, Patrícia; Alves, Sydney Hartz; Pereira, Waldir Veiga

    2011-10-01

    The infections caused by Dipodascus capitatus are rare, and the treatment is difficult. We reported a case of a patient with acute myeloid leukemia. The fungus was first isolated from hemocultures, and the phenotypic identification was based on mycological methods. The genotyping was carried out by sequencing the region D1/D2 from 26 rDNA. The susceptibility tests were assayed by Etest® and by the microdilution technique. None of the antifungal treatments employed were effective. The patient died on day 17 after the mycological diagnosis. The authors discussed the emergence of such infections as well as the difficulty regarding the diagnosis and treatment.

  2. Time course of systemic oxidative stress and inflammatory response induced by an acute exposure to Residual Oil Fly Ash

    SciTech Connect

    Marchini, T.; Magnani, N.D.; Paz, M.L.; Vanasco, V.; Tasat, D.; González Maglio, D.H.; and others

    2014-01-15

    It is suggested that systemic oxidative stress and inflammation play a central role in the onset and progression of cardiovascular diseases associated with the exposure to particulate matter (PM). The aim of this work was to evaluate the time changes of systemic markers of oxidative stress and inflammation, after an acute exposure to Residual Oil Fly Ash (ROFA). Female Swiss mice were intranasally instilled with a ROFA suspension (1.0 mg/kg body weight) or saline solution, and plasma levels of oxidative damage markers [thiobarbituric acid reactive substances (TBARSs) and protein carbonyls], antioxidant status [reduced (GSH) and oxidized (GSSG) glutathione, ascorbic acid levels, and superoxide dismutase (SOD) activity], cytokines levels, and intravascular leukocyte activation were evaluated after 1, 3 or 5 h of exposure. Oxidative damage to lipids and decreased GSH/GSSG ratio were observed in ROFA-exposed mice as early as 1 h. Afterwards, increased protein oxidation, decreased ascorbic acid content and SOD activity were found in this group at 3 h. The onset of an adaptive response was observed at 5 h after the ROFA exposure, as indicated by decreased TBARS plasma content and increased SOD activity. The observed increase in oxidative damage to plasma macromolecules, together with systemic antioxidants depletion, may be a consequence of a systemic inflammatory response triggered by the ROFA exposure, since increased TNF-α and IL-6 plasma levels and polymorphonuclear leukocytes activation was found at every evaluated time point. These findings contribute to the understanding of the increase in cardiovascular morbidity and mortality, in association with environmental PM inhalation. - Highlights: • An acute exposure to ROFA triggers the occurrence of systemic oxidative stress. • Changes in plasmatic oxidative stress markers appear as early as 1 h after exposure. • ROFA induces proinflammatory cytokines release and intravascular leukocyte activation. • PMN

  3. Lipid peroxidation and antioxidant system in rats acutely treated with acetone.

    PubMed

    Mathias, M G; Almeida, B B de; Bueno, J E; Portari, G V; Jordao, A A

    2010-06-01

    Cascades of metabolic changes leading to acetone production are induced in states of energy catabolism such as starvation or the use of a ketogenic diet. The reduced capacity for cell detoxification or the increased generation of free radicals is responsible for the toxic effect of acetone. The objective of the present study was to determine the effects of acute treatment (AT) with acetone on the oxidative and metabolic status of rats. The AT group (n=16) was treated by gavage with a single administration of 7.0 g acetone/kg body weight at a concentration of 25% (m/v). Eight rats were euthanized 6 h later (AT6) and eight 24 h later (AT24). Acetone levels were determined in blood and urine and oxidative parameters were analyzed by determining thiobarbituric acid reactive species (TBARS, indicators of lipid peroxidation) and reduced glutathione (GSH) and vitamin E as antioxidant parameters. Serum glucose, blood cholesterol and triglycerieds and hepatic fat were also determined. The results indicated a significant difference in the hepatic oxidative parameters, serum glucose and in plasma triglycerides between the groups. Thus, we conclude that the administration of acute acetone doses can promote changes in some biochemical parameters and in the hepatic oxidative profile.

  4. Parasite Manipulation of Its Host's Physiological Reaction to Acute Stress: Experimental Results from a Natural Beetle-Nematode System.

    PubMed

    Davis, Andrew K; Vasquez, David; LeFeuvre, Jake; Sims, Stuart; Craft, Meghan; Vizurraga, Anna

    All animals, whether vertebrate or invertebrate, must be capable of reacting to acute stressors, such as escaping from predators, and most do so with a suite of transient physiological changes that temporarily enhance survival. Some of these changes include mobilization of immune cells and increased cardiac output. A small but growing number of studies have begun to show that certain parasites appear capable of modifying such responses. We addressed this topic using a natural host and parasite system, that is, a nematode (Chondronema passali) that parasitizes horned passalus beetles, Odontotaenius disjunctus (family Passalidae), of the eastern United States. With a series of experiments, we sought to determine whether this parasite affects (1) the immune reaction to stress, (2) the output of stress-induced alarm calls, or (3) the increase in heart rate that occurs in response to acute stressors, with the stressors being mechanical or thermal. Results showed that hemocyte density increased after both stressors in nonparasitized beetles but did not increase in parasitized beetles. While mobilization of immune cells would enhance host immunity during stress, this would also be damaging to the nematode, so this scenario appears to benefit the parasite. We found no evidence that the nematode suppresses the overall reaction to stress (or prevents stress from occurring), since parasitized beetles did not differ from nonparasitized ones in alarm call rates or in heart beat frequency after exposure to mechanical stressors. Suppression of the host's normal immune reaction to stressful stimuli could translate to delayed or even reduced wound healing or pathogen resistance during these events. This project is a rare demonstration of parasite manipulation of host immune response to acute stress and should stimulate further investigations into the interactive nature of stress and parasites.

  5. Medication Nonadherence is Associated with Increased Subsequent Acute Care Utilization among Medicaid Beneficiaries with Systemic Lupus Erythematosus

    PubMed Central

    Feldman, Candace H.; Yazdany, Jinoos; Guan, Hongshu; Solomon, Daniel H.; Costenbader, Karen H.

    2015-01-01

    Objective We examined whether nonadherence to hydroxychloroquine (HCQ) or immunosuppressive medications (IS) was associated with higher subsequent acute care utilization among Medicaid beneficiaries with systemic lupus erythematosus (SLE). Methods We utilized U.S. Medicaid data from 2000–2006 to identify adults 18–64 years with SLE who were new users of HCQ or IS. We defined the index date as receipt of HCQ or IS without use in the prior six months. We measured adherence using the medication possession ratio (MPR), the proportion of days covered by total days supply dispensed, for one-year post-index date. Our outcomes were all-cause and SLE-related emergency department (ED) visits and hospitalizations in the subsequent year. We used multivariable Poisson regression models to examine the association between nonadherence (MPR<80%) and acute care utilization adjusting for sociodemographics and comorbidities. Results We identified 9,600 HCQ new users and 3,829 IS new users with SLE. The mean MPR for HCQ was 47.8% (SD 30.3) and for IS, 42.7% (SD 30.7). 79% of HCQ users and 83% of IS users were nonadherent (MPR<80%). In multivariable models, among HCQ users, the incidence rate ratio (IRR) of ED visits was 1.55 (95% CI 1.43–1.69) and the IRR of hospitalizations was 1.37 (95% CI 1.25–1.50), comparing nonadherers to adherers. For IS users, the IRR of ED visits was 1.64 (95% CI 1.42–1.89) and of hospitalizations was 1.67 (95% CI 1.41–1.96) for nonadherers versus adherers. Conclusion In this cohort, nonadherence to HCQ and IS was common and was associated with significantly higher subsequent acute care utilization. PMID:26097166

  6. The habitus of 'rescue' and its significance for implementation of rapid response systems in acute health care.

    PubMed

    Mackintosh, Nicola; Humphrey, Charlotte; Sandall, Jane

    2014-11-01

    The need to focus on patient safety and improve the quality and consistency of medical care in acute hospital settings has been highlighted in a number of UK and international reports. When patients on a hospital ward become acutely unwell there is often a window of opportunity for staff, patients and relatives to contribute to the 'rescue' process by intervening in the trajectory of clinical deterioration. This paper explores the social and institutional processes associated with the practice of rescue, and implications for the implementation and effectiveness of rapid response systems (RRSs) within acute health care. An ethnographic case study was conducted in 2009 in two UK hospitals (focussing on the medical directorates in each organisation). Data collection involved 180 h of observation, 35 staff interviews (doctors, nurses, health care assistants and managers) and documentary review. Analysis was informed by Bourdieu's logic of practice and his relational concept of the 'field' of the general medical ward. Three themes illustrated the nature of rescue work within the field and collective rules which guided associated occupational distinction practices: (1) the 'dirty work' of vital sign recording and its distinction from diagnostic (higher order) interpretive work; (2) the moral order of legitimacy claims for additional help; and (3) professional deference and the selective managerial control of rescue work. The discourse of rescue provided a means of exercising greater control over clinical uncertainty. The acquisition of 'rescue capital' enabled the social positioning of health care assistants, nurses and doctors, and shaped use of the RRS on the wards. Boundary work, professional legitimation and jurisdictional claims defined the social practice of rescue, as clinical staff had to balance safety, professional and organisational concerns within the field. This paper offers a nuanced understanding of patient safety on the front-line, challenging notions of

  7. Acute disseminated encephalomyelitis.

    PubMed

    Alper, Gulay

    2012-11-01

    Acute disseminated encephalomyelitis is an immune-mediated inflammatory and demyelinating disorder of the central nervous system, commonly preceded by an infection. It principally involves the white matter tracts of the cerebral hemispheres, brainstem, optic nerves, and spinal cord. Acute disseminated encephalomyelitis mainly affects children. Clinically, patients present with multifocal neurologic abnormalities reflecting the widespread involvement in central nervous system. Cerebrospinal fluid may be normal or may show a mild pleocytosis with or without elevated protein levels. Magnetic resonance image (MRI) shows multiple demyelinating lesions. The diagnosis of acute disseminated encephalomyelitis requires both multifocal involvement and encephalopathy by consensus criteria. Acute disseminated encephalomyelitis typically has a monophasic course with a favorable prognosis. Multiphasic forms have been reported, resulting in diagnostic difficulties in distinguishing these cases from multiple sclerosis. In addition, many inflammatory disorders may have a similar presentation with frequent occurrence of encephalopathy and should be considered in the differential diagnosis of acute disseminated encephalomyelitis.

  8. Establishing a dose-response relationship between acute resistance-exercise and the immune system: Protocol for a systematic review.

    PubMed

    Szlezak, Adam Michael; Szlezak, Siri Lauluten; Keane, James; Tajouri, Lotti; Minahan, Clare

    2016-12-01

    Exercise immunology research has traditionally focussed on aerobic-exercise, however it has become apparent in more recent years that resistance-exercise can also considerably affect host immunobiology. To date however, no systematic process has been used to establish a dose-response relationship between resistance-exercise and the immune system. The present systematic review was thus conducted to determine the dose-response effects of a bout of resistance-exercise on acute leukocyte counts. In accordance with the PRISMA guidelines, a systematic literature search was conducted in the electronic databases, PubMed, Web of Science, and Google Scholar, over the date range of 1989-2016. Following the PICO elements, eligibility criteria included: i) participants: healthy humans aged 18-40; ii) intervention: a single bout of resistance-exercise; iii) comparator: at least one comparator group; iv) outcome: acute measures of circulating leukocyte counts. Specific exclusion criteria were also applied. Risk of bias and quality of evidence was assessed using the PEDro scale. Due to the individual designs of the admitted studies, a qualitative analysis (systematic narrative synthesis) was employed in the present review. The results of the present review demonstrate that a single bout of resistance-exercise induces an acute monocytosis, neutrophilia, and lymphocytosis. It became apparent that the reviewed literature either does not consistently specify, or does not describe with sufficient detail, the time-course between the onset of exercise and the collection of blood. We recommend that researchers consider addressing this in future studies, and also collect blood measures during exercise to aid with comparison of temporal effects. Regarding the determination of a dose-response relationship, an acute neutrophilia, monocytosis and lymphocytosis appears to occur more rapidly and to a greater magnitude following a single bout of high-dose vs low-dose resistance

  9. [Circulating immune complexes in acute concussion of the brain].

    PubMed

    Midlenko, A I; Biktimirov, T Z; Garmashov, Iu A; Smirnova, M A; Smol'ianinova, V P

    2000-01-01

    The aim of the study was to study the count of circulating immune complexes (CIC) in the blood of children with acute concussion of the brain. The fact that CIC at high concentrations that can penetrate into the brain through the blood-brain barrier and cause complications as vasculitis, microangiopathy, proliferative processes in the meninges, enlarged ventricles of the brain, and atrophy of its tissue was borne in mind. The studies revealed a significant progressive CIC increase within 3 weeks. For correction of blood CIC levels, laser exposure was applied to the carotid and vertebral arteries and acupuncture points. For comparison, thymaline in age-specific doses was used. Laser radiation showed a significant fall of CIC at days 19-21, particular when applied to the acupuncture points. Thymaline did not affect blood CIC levels. Laser application to the acupuncture points in children with acute brain concussion should reduce the incidence of complications of brain injury disease.

  10. Neuroinflammation and disruption in working memory in aged mice after acute stimulation of the peripheral innate immune system

    PubMed Central

    Chen, Jing; Buchanan, Jessica B.; Sparkman, Nathan L.; Godbout, Jonathan P.; Freund, Gregory G.; Johnson, Rodney W.

    2008-01-01

    Acute cognitive disorders are common in elderly patients with peripheral infections but it is not clear why. Here we injected old and young mice with Escherichia coli lipopolysaccharide (LPS) to mimic an acute peripheral infection and separated the hippocampal neuronal cell layers from the surrounding hippocampal tissue by laser capture microdissection and measured mRNA for several inflammatory cytokines (IL-1β, IL-6, and TNFα) that are known to disrupt cognition. The results showed that old mice had an increased inflammatory response in the hippocampus after LPS compared to younger cohorts. Immunohistochemistry further showed more microglial cells in the hippocampus of old mice compared to young adults, and that more IL-1β-positive cells were present in the dentate gyrus and in the CA1, CA2 and CA3 regions of LPS-treated old mice compared to young adults. In a test of cognition that required animals to effectively integrate new information with a preexisting schema to complete a spatial task, we found that hippocampal processing is more easily disrupted in old animals than in younger ones when the peripheral innate immune system is stimulated. Collectively, the results suggest that aging can facilitate neurobehavioral complications associated with peripheral infections probably by allowing the over expression of inflammatory cytokines in brain areas that mediate cognitive processing. PMID:17951027

  11. Reduced acute inpatient care was largest savings component of Geisinger Health System's patient-centered medical home.

    PubMed

    Maeng, Daniel D; Khan, Nazmul; Tomcavage, Janet; Graf, Thomas R; Davis, Duane E; Steele, Glenn D

    2015-04-01

    Early evidence suggests that the patient-centered medical home has the potential to improve patient outcomes while reducing the cost of care. However, it is unclear how this care model achieves such desirable results, particularly its impact on cost. We estimated cost savings associated with Geisinger Health System's patient-centered medical home clinics by examining longitudinal clinic-level claims data from elderly Medicare patients attending the clinics over a ninety-month period (2006 through the first half of 2013). We also used these data to deconstruct savings into its main components (inpatient, outpatient, professional, and prescription drugs). During this period, total costs associated with patient-centered medical home exposure declined by approximately 7.9 percent; the largest source of this savings was acute inpatient care ($34, or 19 percent savings per member per month), which accounts for about 64 percent of the total estimated savings. This finding is further supported by the fact that longer exposure was also associated with lower acute inpatient admission rates. The results of this study suggest that patient-centered medical homes can lead to sustainable, long-term improvements in patient health outcomes and the cost of care.

  12. Elevated systemic elimination of cimetidine in rats with acute biliary obstruction: the role of renal organic cation transporter OCT2.

    PubMed

    Kurata, Tomohiko; Muraki, Yuichi; Mizutani, Hideki; Iwamoto, Takuya; Okuda, Masahiro

    2010-01-01

    Renal tubular secretion of cationic drugs is dominated by two classes of organic cation transporters, OCT2/SLC22A2 and MATE1/SLC47A1, localized to the basolateral and brush-border membranes of the renal tubular epithelial cells, respectively. However, little is known about the expression and function of these transporters in acute cholestasis. Systemic clearance of cimetidine was significantly higher in rats with bile duct ligation (BDL) for 24 hours than in sham-operated rats, with no significant changes in the volume of distribution between the groups. In addition, net tubular secretory clearance of cimetidine was significantly higher in the BDL rats compared with the sham rats, with no significant changes in the glomerular filtration rate. Moreover, the renal tissue-to-plasma concentration ratio of cimetidine was elevated in BDL rats, although the renal tissue-to-urine clearance ratio of cimetidine was not different between the two groups. The expression level of basolateral organic cation transporter rOCT2 protein in the kidney cortex was markedly higher in BDL rats than that in the sham rats, but that of H+/organic cation antiporter rMATE1 protein in the brush-border membranes was not significantly different between the two groups. These results demonstrate that the renal tubular secretion of cimetidine was increased by acute cholestasis, and this increase was attributable to elevated expression levels of rOCT2 but not of rMATE1 in the rat.

  13. A serial histologic study of the development and progression of acute pancreatitis in the rat.

    PubMed Central

    Rao, S. S.; Watt, I. A.; Donaldson, L. A.; Crocket, A.; Joffe, S. N.

    1981-01-01

    This study was undertaken for the purpose of a serial investigation of the development and progression of the light-microscopic changes of acute pancreatitis and histologic criteria for evaluating pancreatitis. Acute pancreatitis, similar to that found in man, was induced in rats with the use of a closed duodenal loop technique (n = 36). Control rats underwent a laparotomy with mobilization of the duodenum (n = 12). Animals were killed every 2 hours for 24 hours, and a detailed and independent histologic evaluation was made of each. Focal acinar necrosis proceeding to a vasculitis appeared within 2--4 hours before the infiltration of inflammatory cells. Thereafter, the extent of acinar necrosis closely reflected the vasculitis with the later development of the acute inflammation. By the sixteenth hour, these changes were graded as moderate pancreatitis, and by 24 hours the process represented severe hemorrhagic pancreatitis. Vascular changes and acinar necrosis preceded the inflammatory cell infiltrate. The pancreatitis has been quantitated into minimal, moderate, or severe by assessing the severity of edema, acute inflammatory infiltrate, and changes in the vessels, ducts, and acini. Images Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Figure 6 Figure 7 Figure 8 Figure 9 PMID:7223862

  14. Diversity, cellular origin and autoreactivity of antibody-secreting cell expansions in acute Systemic Lupus Erythematosus

    PubMed Central

    Tipton, Christopher M; Fucile, Christopher F; Darce, Jaime; Chida, Asiya; Ichikawa, Travis; Gregoretti, Ivan; Schieferl, Sandra; Hom, Jennifer; Jenks, Scott; Feldman, Ron J; Mehr, Ramit; Wei, Chungwen; Lee, F. Eun-Hyung; Cheung, Wan Cheung; Rosenberg, Alexander F; Sanz, Iñaki

    2015-01-01

    Acute SLE courses with antibody-secreting cells (ASC) surges whose origin, diversity, and contribution to serum autoantibodies remain unknown. Deep sequencing, autoantibody proteome and single-cell analysis demonstrated highly diversified ASC punctuated by VH4-34 clones that produce dominant serum autoantibodies. A fraction of ASC clones contained unmutated autoantibodies, a finding consistent with differentiation outside the germinal centers. A substantial ASC segment derived from a distinct subset of newly activated naïve cells of significant clonality that persist in the circulation for several months. Thus, selection of SLE autoreactivities occurred during polyclonal activation with prolonged recruitment of recently activated naïve B cells. These findings shed light into SLE pathogenesis, help explain the benefit of anti-B cell agents and facilitate the design of future therapies. PMID:26006014

  15. The DAC system and associations with acute leukemias and myelodysplastic syndromes.

    PubMed

    Bug, Gesine; Ottmann, Oliver G

    2010-12-01

    Imbalances of histone acetyltransferase (HAT) and deacetylase activity (DAC) that result in deregulated gene expression are commonly observed in leukemias. These alterations provide the basis for novel therapeutic approaches that target the epigenetic mechanisms implicated in leukemogenesis. As the acetylation status of histones has been linked to transcriptional regulation of genes involved particularly in differentiation and apoptosis, DAC inhibitors (DACi) have attracted considerable attention for treatment of hematologic malignancies. DACi encompass a structurally diverse family of compounds that are being explored as single agents as well as in combination with chemotherapeutic drugs, small molecule inhibitors of signaling pathways and hypomethylating agents. While DACi have shown clear evidence of activity in acute myeloid leukemia, myelodysplastic syndromes and lymphoid malignancies, their precise role in treatment of these different entities remain to be elucidated. Successful development of these compounds as elements of novel targeted treatment strategies for leukemia will require that clinical studies be performed in conjunction with translational research including efforts to identify predictive biomarkers.

  16. Acute Bronchitis

    MedlinePlus

    ... can also cause acute bronchitis. To diagnose acute bronchitis, your health care provider will ask about your symptoms and listen to your breathing. You may also have other tests. Treatments include rest, fluids, and aspirin (for adults) or ...

  17. Serum from patients with systemic vasculitis induces alternatively activated macrophage M2c polarization.

    PubMed

    Ohlsson, Susanne M; Linge, Carl Petrus; Gullstrand, Birgitta; Lood, Christian; Johansson, Asa; Ohlsson, Sophie; Lundqvist, Andrea; Bengtsson, Anders A; Carlsson, Fredric; Hellmark, Thomas

    2014-01-01

    Anti-neutrophil cytoplasmic antibody associated vasculitides (AAV) are conditions defined by an autoimmune small vessel inflammation. Dying neutrophils are found around the inflamed vessels and the balance between infiltrating neutrophils and macrophages is important to prevent autoimmunity. Here we investigate how sera from AAV patients may regulate macrophage polarization and function. Macrophages from healthy individuals were differentiated into M0, M1, M2a, M2b or M2c macrophages using a standardized protocol, and phenotyped according to their expression surface markers and cytokine production. These phenotypes were compared with those of macrophages stimulated with serum from AAV patients or healthy controls. While the healthy control sera induced a M0 macrophage, AAV serum promoted polarization towards the M2c subtype. No sera induced M1, M2a or M2b macrophages. The M2c subtype showed increased phagocytosis capacity compared with the other subtypes. The M2c polarization found in AAV is consistent with previous reports of increased levels of M2c-associated cytokines.

  18. Hypogalactosylation of serum IgG in patients with ANCA-associated systemic vasculitis

    PubMed Central

    HOLLAND, M; TAKADA, K; OKUMOTO, T; TAKAHASHI, N; KATO, K; ADU, D; BEN-SMITH, A; HARPER, L; SAVAGE, C O S; JEFFERIS, R

    2002-01-01

    The triad of small vessel vasculitides (SVV) comprise Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and Churg–Strauss syndrome (CS). All three are associated with presence of circulating IgG antineutrophil cytoplasm antibodies (ANCA) which target autoantigens contained, primarily, within neutrophil azurophilic granules. The widely accepted model of pathogenesis suggests that ANCA activate cytokine-primed neutrophils within the microvasculature, leading to by-stander damage to endothelial cells, and rapid escalation of inflammation with recruitment of mononuclear cells. Activation may be initiated, in vitro, by the coligation of the PR3 or MPO antigen, translocated to the cell surface, and FcγRIIa/FcγRIIIb receptors. This suggests that the IgG subclass profile of ANCA and, possibly, its glycosylation status could influence the inflammatory mechanisms activated. The glycosylation status of total IgG isolated from the sera of patients with WG (13), MPA (6) and CSS (1) was determined by analysis of the released oligosaccharides. A deficit in IgG galactosylation is demonstrated for all patient samples, compared to controls. The mean percentage values for the agalactosylated (G0) oligosaccharides were 57% (SD ± 9·71), 47% (SD ± 4·25) and 28% (SD ± 4·09) for WG, MPO and control samples, respectively. The G0 levels for polyclonal IgG isolated from the sera of both WG and MPA patients were significantly increased compared to controls (P < 0·0001). The major glycoform present therefore is agalactosylated (G0) IgG. In previous studies the G0 glycoform of IgG has been shown to bind and activate mannan binding lectin, and hence to activate the complement cascade, and to facilitate mannose receptor binding and the uptake of IgG complexes by macrophages and dendritic cells. Both of these activities could impact on the processing and presentation of self-antigens in autoimmune disease. PMID:12100039

  19. Intravenous Cyclophosphamide and Plasmapheresis in Dialysis-Dependent ANCA-Associated Vasculitis

    PubMed Central

    Pepper, Ruth J.; Chanouzas, Dimitrios; Tarzi, Ruth; Little, Mark A.; Casian, Alina; Walsh, Michael; Pusey, Charles D.; Harper, Lorraine

    2013-01-01

    Summary Background and objectives Induction therapy with oral cyclophosphamide (CYP) has been a mainstay of treatment in patients with severe renal failure secondary to ANCA-associated vasculitis (AAV). Recent evidence proposes using pulsed intravenous CYP in less severe disease to minimize adverse events. It is unclear if this can be translated to those with dialysis-dependent renal insufficiency. Design, setting, participants, & methods All AAV patients presenting between 2005 and 2010 requiring dialysis at presentation were retrospectively analyzed. Patients were treated with plasma exchange, corticosteroids, and intravenous CYP. Rate of dialysis independence at 3 and 12 months and adverse effects were assessed and compared with the outcome of the plasmapheresis, prednisolone, and oral CYP arm of the randomized MEPEX (methylprednisolone versus plasma exchange) trial. Results Forty-one patients were included. At 3 months, 3 (7.3%) patients had died on dialysis, 12 (29.3%) remained dialysis dependent, and 26 (63.4%) were dialysis independent (creatinine, 2.5 mg/dl; GFR, 26 ml/min per 1.73 m2). Four patients subsequently reached ESRD at a median time of 83 days. Thirty-seven (90%) patients reached 1 year follow-up, 13 (35%) remained dialysis dependent, and 24 (65%) had independent renal function. Eleven patients (27%) had episodes of leukopenia (white cell count <4×109/L) during CYP therapy and 17 (41%) experienced infectious complications. This compares favorably with the dialysis-dependent cohort treated with plasmapheresis in the MEPEX study in which 51% were alive with independent renal function at 1 year. Conclusions Intravenous CYP used with corticosteroids and plasmapheresis may be an effective alternative to oral CYP in patients with dialysis-dependent AAV. PMID:23160261

  20. The epidemiology of antineutrophil cytoplasmic antibody-associated vasculitis in northwestern Turkey.

    PubMed

    Pamuk, Ömer Nuri; Dönmez, Salim; Calayır, Gökçe Büşra; Pamuk, Gülsüm Emel

    2016-08-01

    Epidemiological data about antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is very limited. Until now, there has been no study about the epidemiology of AAV in Turkey. In this study, we evaluated the frequency of AAV in the northeastern part of Turkey. The general clinical features of patients diagnosed with AAV at our center within the last 10 years (2004-2014) were retrospectively recorded down. The incidence rates and the prevalence per 1,000,000 population aged ≥16 years were calculated. In addition, we evaluated the clinical features and survival rates of AAV patients. There were 30 patients with granulomatous polyangiitis (GPA), 15 with microscopic polyangiitis (MPA), and 5 with eosinophilic polyangiitis (EGPA). The overall prevalence of AAV in our region was 69.3/1,000,000 in individuals ≥16 years. Males had a similar prevalence (73.2/1,000,000) with females (65.4/1,000,000). The mean annual incidence rate was 8.1/million for all AAV. The annual incidence of AAV in females was 6.9/million; in males, it was 9.2/million. The annual incidence for GPA was calculated as 4.8/1,000,000, the incidence for MPA was 2.4/1,000,000, and the incidence for CSS was 0.8/1,000,000. Ten-year survival of patients with AAV was 65.3 %. The only independent poor prognostic factor in Cox's multivariate analysis was advanced age at the time of diagnosis (OR 7.5, 95 % CI 10.6-526, p = 0.043). The frequency of all AAV in northwestern Turkey was similar to that in southern Europe; however, it was lower than the frequency in Northern Europe.

  1. Eosinophilic Fasciitis and Acute Encephalopathy Toxicity from Pembrolizumab Treatment of a Patient with Metastatic Melanoma.

    PubMed

    Khoja, Leila; Maurice, Catherine; Chappell, MaryAnne; MacMillan, Leslie; Al-Habeeb, Ayman S; Al-Faraidy, Nada; Butler, Marcus O; Rogalla, Patrik; Mason, Warren; Joshua, Anthony M; Hogg, David

    2016-03-01

    Anti-PD-1 inhibitors have significant activity in metastatic melanoma. Responses often occur early and may be sustained. The optimal duration of treatment with these agents is unknown. Here, we report the case of a 51-year-old woman treated with pembrolizumab, as part of the Keynote-001 trial, as first-line treatment for metastatic disease. She experienced a complete response after 13.8 months of treatment with no adverse events. One month after the last drug infusion and 18 months from starting treatment, the patient presented with eosinophilic fasciitis. She then developed acute confusion and weakness, thought to be due to intracranial vasculitis. High-dose steroids were initiated with resolution of the fasciitis. Aspirin was commenced for presumed vasculitis with resolution of the neurologic symptoms. To our knowledge, there are no previous reports of eosinophilic fasciitis or cerebral vasculitis due to anti-PD-1 agents. This case demonstrates that toxicity may occur in association with pembrolizumab treatment after a prolonged period of treatment without toxicity. Future trials should explore the optimal duration of treatment with pembrolizumab.

  2. Antiphospholipid Syndrome - A Case Report of Pulmonary Thromboembolism, Followed with Acute Myocardial Infarction in Patient with Systemic Sclerosis

    PubMed Central

    Vavlukis, Marija; Kotlar, Irina; Chaparoska, Emilija; Pocesta, Bekim; Pejkov, Hristo; Boshev, Marjan; Kedev, Sasko

    2015-01-01

    AIM: We are presenting an uncommon case of pulmonary embolism, followed with an acute myocardial infarction, in a patient with progressive systemic sclerosis. CASE PRESENTATION: A female 40 years of age was admitted with signs of pulmonary embolism, confirmed with CT scan, which also reviled a thrombus in the right ventricle. The patient had medical history of systemic sclerosis since the age of 16 years. She suffered an ischemic stroke 6 years ago, but she was not taking any anticoagulant or antithrombotic medications ever since. She received a treatment with thrombolytic therapy, and subsequent UFH, but, on the second day after receiving fibrinolysis, she felt chest pain accompanied with ECG changes consistent for ST-segment elevation myocardial infarction (STEMI). Urgent coronary angiography was undertaken, which reviled cloths causing total occlusion in 4 blood vessels, followed with thromboaspiration, but without successful reperfusion. Several hours later the patient developed rapid deterioration with letal ending. During the very short hospital course, blood sampling reviled presence of antiphospholipid antibodies. CONCLUSION: The acquired antiphospholipid syndrome is common condition in patients with systemic autoimmune diseases, but relatively rare in patients with systemic sclerosis. Never the less, we have to be aware of it when treating the patients with systemic sclerosis. PMID:27275312

  3. 78 FR 38679 - Medicare Program; Hospital Inpatient Prospective Payment Systems for Acute Care Hospitals and the...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-06-27

    ... Deep Brain Stimulator (DBS) and Vagus Nerve Stimulator (VNS) Systems (the table is titled ``KEY... System and DBS and VNS Systems Deep brain Vagus nerve RNS system stimulator (DBS) stimulator (VNS) Type... Hours--Continuous. Stimulation target Cortical; varies according to Deep brain nuclei... Ascending...

  4. Unusual Synchronous Presentation of Maxillary Sinus Fibrosarcoma and Gemistocytic Astrocytoma with a Complication Called Leukocytoclastic Vasculitis: A Case Report

    PubMed Central

    Cadir, Bilge; Karahan, Nermin; Nasir, Serdar; Aydin, M. Asim; Turkaslan, S. Suha

    2009-01-01

    Fibrosarcoma of the paranasal sinuses is extremely rare pathology and there is limited report in the literature. We report synchronous presentation of maxillary sinus fibrosarcoma and gemistocytic astrocytoma which is, to our knowledge, unique in the literature. Both tumors metastases to other organ rarely and the metastatic spread of gemistocytic astrocytoma to fibrosarcoma or vice versa have also not been reported in the literature yet. This report discusses the clinical course of the disease, outcome of the treatment approach and survival as well as an unusual occurrence of leukocytoclastic vasculitis during the course of radiotherapy in such unusual presentation. PMID:19756200

  5. [Scanning electron microscopy of peripheral blood erythrocytes in the diagnosis and treatment of hemorrhagic vasculitis in children].

    PubMed

    Nagaeva, T A; Balasheva, I I; Saratikov, A S; Bocharova, M N; Mikhalenko, A N

    2001-01-01

    Twenty-four children aged 3-15 years were examined, 16 of these with cutaneous and articulo-cutaneous hemorrhagic vasculitis (HV) and 8 normal controls. The patients were divided into 2 groups: 10 patients treated by basic therapy and 6 children whose treatment protocols were supplemented by membranoprotector locheine. The children were repeatedly examined 1 month after discharge from hospital. Scanning electron microscopy of peripheral blood erythrocytes provides valuable diagnostic data on erythrocyte membrane morphology and function in children with HV and can serve as a method for monitoring the efficiency of new approaches to therapy of this disease.

  6. Evaluation of a stand-alone computer-aided detection system for acute intra-cranial hemorrhage in emergency environments

    NASA Astrophysics Data System (ADS)

    Fernandez, James; Deshpande, Ruchi; Wang, Ximing; Liu, Brent; Brazaitis, Michael; Munter, Fletcher; Liu, Margaret

    2011-03-01

    Acute intra-cranial hemorrhage (AIH) may result from traumatic brain injury (TBI). Successful management of AIH depends heavily on the speed and accuracy of diagnosis. Timely diagnosis in emergency environments in both civilian and military settings is difficult primarily due to severe time restraints and lack of resources. Often, diagnosis is performed by emergency physicians rather than trained radiologists. As a result, added support in the form of computer-aided detection (CAD) would greatly enhance the decision-making process and help in providing faster and more accurate diagnosis of AIH. This paper discusses the implementation of a CAD system in an emergency environment, and its efficacy in aiding in the detection of AIH.

  7. Safety and tolerability of intrathecal liposomal cytarabine as central nervous system prophylaxis in patients with acute lymphoblastic leukemia.

    PubMed

    Valentin, Angelika; Troppan, Katharina; Pfeilstöcker, Michael; Nösslinger, Thomas; Linkesch, Werner; Neumeister, Peter

    2014-08-01

    Central nervous system recurrence in acute lymphoblastic leukemia (ALL) occurs in up to 15% of patients and is frequently associated with poor outcome. The purpose of our study was to evaluate the efficacy and safety of a slow-release liposomal formulation of cytarabine for intrathecal (IT) meningeal prophylaxis in patients suffering from ALL. Forty patients aged 20-77 years (median 36) were preventively treated with a total of 96 (range 1-6) single doses containing 50 mg of liposomal cytarabine on a compassionate use basis. After a median observation period of 23 months (range 2-118) only two patients experienced a combined medullary-leptomeningeal disease recurrence after primary diagnosis. Except for headache grade 2 in two patients, no specific toxicity attributable to IT liposomal cytarabine application was noted. Long-term neurological side effects were not observed. IT liposomal cytarabine therapy with concomitant dexamethasone appears to be feasible and well tolerated.

  8. Obesity as the initial manifestation of central nervous system relapse of acute lymphoblastic leukemia: case report and literature review.

    PubMed

    Zhang, Li-Dan; Li, Yan-Hong; Ke, Zhi-Yong; Huang, Li-Bin; Luo, Xue-Qun

    2012-01-01

    A 6-year-old boy with acute lymphoblastic leukemia in remission experienced hyperphagia, obesity, and emotional disorders. Cytomorphologic examination of cerebral spinal fluid (CSF) and cranial MRI did not help in differentiating between central nervous system leukemia (CNSL) and other CNS diseases including tuberculosis in this boy. Flow cytometric CSF analysis on repeated lumber puncture detected lymphoblasts, while microscopic CSF examination did not definitively show relapse disease. The diagnosis of CNSL was thus made and confirmed by the response to leukemia treatment. Obesity can be the first manifestation of CNSL and the diagnosis can be challenging. A combination of CSF cytomorphology, CSF flow cytometry, and cranial MRI can be useful in the diagnosis of the disease. Two mechanisms of CNSL-related obesity are discussed based on the literature review.

  9. Psychobiology of PTSD in the Acute Aftermath of Trauma: Integrating Research on Coping, HPA Function and Sympathetic Nervous System Activity

    PubMed Central

    Morris, Matthew C.; Rao, Uma

    2012-01-01

    Research on the psychobiological sequelae of trauma has typically focused on long-term alterations in individuals with chronic posttraumatic stress disorder (PTSD). Far less is known about the nature and course of psychobiological risk factors for PTSD during the acute aftermath of trauma. In this review, we summarize data from prospective studies focusing on the relationships among sympathetic nervous system activity, hypothalamic-pituitary-adrenal function, coping strategies and PTSD symptoms during the early recovery (or non-recovery) phase. Findings from pertinent studies are integrated to inform psychobiological profiles of PTSD-risk in children and adults in the context of existing models of PTSD-onset and maintenance. Data regarding bidirectional relations between coping strategies and stress hormones is reviewed. Limitations of existing literature and recommendations for future research are discussed. PMID:23380312

  10. Effect of lornoxicam therapy on expression of TLR2 and TLR4 mRNA during systemic complications of acute pancreatitis.

    PubMed

    Gorskii, V A; Agapov, M A; Khoreva, M V; Petrov, V A; Kravchenko, A Yu; Battaev, A I

    2014-11-01

    Primary pancreatic injury that occurs in acute pancreatitis leads to necrosis of pancreatic cells and is accompanied by the development systemic inflammatory response of varying severity. Systemic inflammatory response, in turn, can lead to the development of multiple organ dysfunction syndrome and death of patients. The release of damage-associated molecular patterns into the extracellular space is the trigger pathological mechanism underlying these processes. The released patterns exert their effects via Toll-like receptors (TLR). These findings suggest that TLR can be considered a new target for therapeutic intervention in acute pancreatitis. We studied mRNA expression of TLR2 and TLR4 in the peripheral blood mononuclear cells from the patients with acute pancreatitis and showed a decrease in the examined parameters associated with lornoxicam treatment. Anti-mediator therapy decreased mortality in these patients.

  11. Extracorporeal Membrane Oxygenation as Bridge-to-Decision in Acute Heart Failure due to Systemic Light-Chain Amyloidosis

    PubMed Central

    Silva, Jennifer Mancio; Fontes-Carvalho, Ricardo; Valente, Dília; Almeida, Cristiana; Cruz, António José; Tente, David; Coelho, Henrique; Oliveira, Marco; Albuquerque, Aníbal; Ribeiro, Vasco Gama

    2015-01-01

    Patient: Female, 58 Final Diagnosis: Acute hear failure Symptoms: Dispnoea • edema • fatigue Medication: — Clinical Procedure: Bone marrow biopsy • endomyocardial biopsy • abdominal subcutaneous fat biopsy under ECMO support Specialty: Cardiology Objective: Rare disease Background: Cardiac amyloidosis results from the amyloid deposition in heart tissue, either in the context of a systemic disease or as a localized form. Several pro-amyloid proteins can produce amyloid deposits in the heart. Each of these amyloidoses has characteristic clinical (cardiac and extracardiac) features, and a specific diagnosis and treatment. Case Report: A 58-year-old woman who presented with acute heart failure and echocardiographic findings strongly suggestive of infiltrative cardiomyopathy needed percutaneous veno-arterial extracorporeal membrane oxygenation (ECMO) as bridge-to-decision. Amyloid deposition was found on endomyocardial and bone marrow biopsies. Bone marrow plasma cell infiltrate with acute renal lesion and hypercalcemia confirmed the diagnosis of multiple myeloma-associated systemic light-chain amyloidosis (AL). Refractory shock with multi-organic failure syndrome persisted and no improvements in left ventricular function and structure were seen. After extensive discussion by a multidisciplinary team, and with the patients’ family, she was not considered eligible for high-dose chemotherapy and/or autologous stem cell transplantation, heart transplantation, or sequential heart with autologous stem cell transplantation. The patient died a few hours after ECMO withdrawal. During the 14 days of ECMO support no major bleeding or thrombotic complications occurred. Conclusions: The clinician must consider a diagnosis of cardiac amyloidosis in patients with heart failure, a restrictive type of cardiomyopathy with ventricular hypertrophy in the absence of valve abnormalities, or uncontrolled arterial hypertension. Although developments in chemotherapy have greatly

  12. Self-care Barriers Reported by Emergency Department Patients With Acute Heart Failure: A Sociotechnical Systems-based Approach

    PubMed Central

    Holden, Richard J.; Schubert, Christiane C.; Eiland, Eugene C.; Storrow, Alan B.; Miller, Karen F.; Collins, Sean P.

    2015-01-01

    Objective To pilot a sociotechnical systems-based instrument that assesses the prevalence and nature of self-care barriers among patients presenting to the emergency department (ED) with acute heart failure. Methods A semi-structured instrument for measuring self-reported self-care barriers was developed and administered by ED clinicians and non-clinician researchers to 31 ED patients diagnosed with acute heart failure. Responses were analyzed using descriptive statistics and qualitative content analysis. Feasibility was assessed by examining participant cooperation rates, instrument completion times, item nonresponse, and data yield. Results Of 47 distinct self-care barriers assessed, a median of 15 per patient were indicated as “sometimes” or “often” present. Thirty-four specific barriers were reported by over 25% of patients and nine were reported by over 50%. The sources of barriers included the person, self-care tasks, tools and technologies, and organizational, social, and physical contexts. Seven of the top ten most prevalent barriers were related to patient characteristics and the next three to the organizational context (e.g., life disruptions). A preliminary feasibility assessment found few item nonresponses or comprehension difficulties, good cooperation, high data yield from both closed- and open-ended items, but opportunities to reduce median administration time and variability. Conclusions An instrument assessing self-care barriers from multiple system sources can be feasibly implemented in the ED. Further research is required to modify the instrument for widespread use and evaluate its implementation across institutions and cultural contexts. Self-care barriers measurement can be one component of broader inquiry into the distributed health-related “work” activity of patients, caregivers, and clinicians. PMID:25616317

  13. Impact of integrated health system changes, accelerated due to an earthquake, on emergency department attendances and acute admissions: a Bayesian change-point analysis

    PubMed Central

    Schluter, Philip J; Hamilton, Greg J; Deely, Joanne M; Ardagh, Michael W

    2016-01-01

    Objective To chart emergency department (ED) attendance and acute admission following a devastating earthquake in 2011 which lead to Canterbury's rapidly accelerated integrated health system transformations. Design Interrupted time series analysis, modelling using Bayesian change-point methods, of ED attendance and acute admission rates over the 2008–2014 period. Setting ED department within the Canterbury District Health Board; with comparison to two other district health boards unaffected by the earthquake within New Zealand. Participants Canterbury's health system services ∼500 000 people, with around 85 000 ED attendances and 37 000 acute admissions per annum. Main outcome measures De-seasoned standardised population ED attendance and acute admission rates overall, and stratified by age and sex, compared before and after the earthquake. Results Analyses revealed five global patterns: (1) postearthquake, there was a sudden and persisting decrease in the proportion of the population attending the ED; (2) the growth rate of ED attendances per head of population did not change between the pre-earthquake and postearthquake periods; (3) postearthquake, there was a sudden and persisting decrease in the proportion of the population admitted to hospital; (4) the growth rate of hospital admissions per head of the population declined between pre-earthquake and postearthquake periods and (5) the most dramatic reduction in hospital admissions growth after the earthquake occurred among those aged 65+ years. Extrapolating from the projected and fitted deseasoned rates for December 2014, ∼676 (16.8%) of 4035 projected hospital admissions were avoided. Conclusions While both necessarily and opportunistically accelerated, Canterbury's integrated health systems transformations have resulted in a dramatic and sustained reduction in ED attendances and acute hospital admissions. This natural intervention experiment, triggered by an earthquake, demonstrated that

  14. Renal Biopsy Findings in Acute Renal Failure in the Cohort of Patients in the Spanish Registry of Glomerulonephritis

    PubMed Central

    López-Gómez, Juan M.; Rivera, Francisco

    2008-01-01

    Background and objectives: Renal biopsy in acute renal failure of unknown origin provides irreplaceable information for diagnosis, treatment, and prognosis. This study analyzed the frequency and clinicopathologic correlations of renal native biopsied acute renal failure in Spain during the period 1994 through 2006. Design, setting, participants, & measurements: Acute renal failure was defined as a rapid deterioration of glomerular filtration rate, with or without oligoanuria or rapidly progressive renal insufficiency, including acute-on-chronic renal failure. Patients who were younger than 15 yr were considered children, those between 15 and 65 yr adults, and those >65 elderly. Results: Between 1994 and 2006, data on 14,190 native renal biopsies were collected from 112 renal units in Spain. Of these, 16.1% (2281 biopsies) were diagnosed with acute renal failure. The prevalence of the main clinical syndromes was different in the three age groups: Biopsy-confirmed acute renal failure in children was 5.7%, in adults was 12.5%, and in elderly increased significantly to 32.9%. The prevalence of biopsy-confirmed acute renal failure according to cause was as follows: Vasculitis, 23.3%; acute tubulointerstitial nephritis, 11.3%; and crescentic glomerulonephritis types 1 and 2, 10.1%. The prevalence of the different causes differed significantly according to age group. Conclusions: The Spanish Registry of Glomerulonephritis provides useful information about renal histopathology in biopsy-confirmed acute renal failure. The prevalence of vasculitis and crescentic glomerulonephritis is high, especially in elderly patients. These data obtained from a national large registry highlight the value of renal biopsy in undetermined acute renal failure. PMID:18354075

  15. HMGB1 and Histones Play a Significant Role in Inducing Systemic Inflammation and Multiple Organ Dysfunctions in Severe Acute Pancreatitis

    PubMed Central

    Tenhunen, Jyrki; Tonnessen, Tor Inge

    2017-01-01

    Severe acute pancreatitis (SAP) starts as a local inflammation of pancreatic tissue that induces the development of multiple extrapancreatic organs dysfunction; however, the underlying mechanisms are still not clear. Ischemia-reperfusion, circulating inflammatory cytokines, and possible bile cytokines significantly contribute to gut mucosal injury and intestinal bacterial translocation (BT) during SAP. Circulating HMGB1 level is significantly increased in SAP patients and HMGB1 is an important factor that mediates (at least partly) gut BT during SAP. Gut BT plays a critical role in triggering/inducing systemic inflammation/sepsis in critical illness, and profound systemic inflammatory response syndrome (SIRS) can lead to multiple organ dysfunction syndrome (MODS) during SAP, and systemic inflammation with multiorgan dysfunction is the cause of death in experimental SAP. Therefore, HMGB1 is an important factor that links gut BT and systemic inflammation. Furthermore, HMGB1 significantly contributes to multiple organ injuries. The SAP patients also have significantly increased circulating histones and cell-free DNAs levels, which can reflect the disease severity and contribute to multiple organ injuries in SAP. Hepatic Kupffer cells (KCs) are the predominant source of circulating inflammatory cytokines in SAP, and new evidence indicates that hepatocyte is another important source of circulating HMGB1 in SAP; therefore, treating the liver injury is important in SAP. PMID:28316860

  16. Pre-stimulation of the kallikrein system in cisplatin-induced acute renal injury: An approach to renoprotection

    SciTech Connect

    Aburto, Andrés; Barría, Agustín; Cárdenas, Areli; Carpio, Daniel; Figueroa, Carlos D.; Burgos, Maria E.; Ardiles, Leopoldo

    2014-10-15

    Antineoplastic treatment with cisplatin is frequently complicated by nephrotoxicity. Although oxidative stress may be involved, the pathogenic mechanisms responsible for renal damage have not been completely clarified. In order to investigate the role of the renal kinin system in this condition, a group of rats was submitted to high potassium diet to stimulate the synthesis and excretion of tissue kallikrein 1 (rKLK1) previous to an intraperitoneal injection of 7 mg/kg cisplatin. A significant reduction in lipoperoxidation, evidenced by urinary excretion of malondialdehyde and renal immunostaining of hidroxy-nonenal, was accompanied by a decline in apoptosis. Coincident with these findings we observed a reduction in the expression of renal KIM-1 suggesting that renoprotection may be occurring. Stimulation or indemnity of the renal kinin system deserves to be evaluated as a complementary pharmacological measure to diminish cisplatin nephrotoxicity. - Highlights: • Mechanisms of cisplatin-induced-renal damage have not been completely clarified. • Cisplatin induces oxidative stress and apoptosis. • The renal kallikrein-kinin system is protective in experimental acute renal damage. • Kallikrein stimulation reduces oxidative stress and apoptosis induced by cisplatin. • Protection of the kallikrein-kinin system may reduce cisplatin toxicity.

  17. Investigation for role of tissue factor and blood coagulation system in severe acute pancreatitis and associated liver injury.

    PubMed

    Ou, Zhi-Bing; Miao, Chun-Mu; Ye, Ming-Xin; Xing, Ding-Pei; He, Kun; Li, Pei-Zhi; Zhu, Rong-Tao; Gong, Jian-Ping

    2017-01-01

    This study aims to investigate the molecular mechanisms underlying the pathogenesis of severe acute pancreatitis (SAP) and SAP-associated liver injury, we performed an association analysis of the functions of tissue factor (TF) and blood coagulation system in both SAP patients and mouse SAP model. Our results showed that serum TF and tissue factor-microparticle (TF-MP) levels were highly up-regulated in both SAP patients and SAP mouse model, which was accompanied by the dysfunction of blood coagulation system. Besides, TF expression was also highly up-regulated in the Kupffer cells (KCs) of SAP mouse model. After inhibiting KCs in SAP mouse model, the amelioration of blood coagulation system functions was associated with the decrease in serum TF and TF-MPs levels, and the reduction of SAP-associated liver injury was associated with the decrease of TF expression in KCs. In conclusion, the dis-regulated TF expression and associated dysfunction of blood coagulation system are critical factors for the pathogenesis of SAP and SAP-associated liver injury. TF may serve as a potential and effective target for treating SAP and SAP-associated liver injury.

  18. Acute effects of whole-body proton irradiation on the immune system of the mouse

    NASA Technical Reports Server (NTRS)

    Kajioka, E. H.; Andres, M. L.; Li, J.; Mao, X. W.; Moyers, M. F.; Nelson, G. A.; Slater, J. M.; Gridley, D. S.

    2000-01-01

    The acute effects of proton whole-body irradiation on the distribution and function of leukocyte populations in the spleen and blood were examined and compared to the effects of photons derived from a (60)Co gamma-ray source. Adult female C57BL/6 mice were exposed to a single dose (3 Gy at 0.4 Gy/min) of protons at spread-out Bragg peak (SOBP), protons at the distal entry (E) region, or gamma rays and killed humanely at six different times thereafter. Specific differences were noted in the results, thereby suggesting that the kinetics of the response may be variable. However, the lack of significant differences in most assays at most times suggests that the RBE for both entry and peak regions of the Bragg curve was essentially 1.0 under the conditions of this study. The greatest immunodepression was observed at 4 days postexposure. Flow cytometry and mitogenic stimulation analyses of the spleen and peripheral blood demonstrated that lymphocyte populations differ in radiosensitivity, with B (CD19(+)) cells being most sensitive, T (CD3(+)) cells being moderately sensitive, and natural killer (NK1.1(+)) cells being most resistant. B lymphocytes showed the most rapid recovery. Comparison of the T-lymphocyte subsets showed that CD4(+) T helper/inducer cells were more radiosensitive than the CD8(+) T cytotoxic/suppressor cells. These findings should have an impact on future studies designed to maximize protection of normal tissue during and after proton-radiation exposure.

  19. Implementing an interdisciplinary electronic documentation system at two pilot units within an acute care setting.

    PubMed

    van der Wal, Rena; Dalzel, Shelley; Kitzul, Marianne

    2009-01-01

    An electronic interdisciplinary clinical documentation system that includes assessments and some clinical interventions was designed and implemented on two pilot units. This paper describes the procedures for designing the screens, implementing the system, and integrating the electronic documentation system with the workflow of nursing staff. The results of this pilot project are outlined and implications for future efforts are examined.

  20. Acute phase reaction and acute phase proteins*

    PubMed Central

    Gruys, E.; Toussaint, M.J.M.; Niewold, T.A.; Koopmans, S.J.

    2005-01-01

    A review of the systemic acute phase reaction with major cytokines involved, and the hepatic metabolic changes, negative and positive acute phase proteins (APPs) with function and associated pathology is given. It appears that APPs represent appropriate analytes for assessment of animal health. Whereas they represent non-specific markers as biological effect reactants, they can be used for assessing nutritional deficits and reactive processes, especially when positive and negative acute phase variables are combined in an index. When such acute phase index is applied to separate healthy animals from animals with some disease, much better results are obtained than with single analytes and statistically acceptable results for culling individual animals may be reached. Unfortunately at present no cheap, comprehensive and easy to use system is available for assessing various acute phase proteins in serum or blood samples at the same time. Protein microarray or fluid phase microchip technology may satisfy this need; and permit simultaneous analysis of numerous analytes in the same small volume sample and enable integration of information derived from systemic reactivity and nutrition with disease specific variables. Applying such technology may help to solve health problems in various countries not only in animal husbandry but also in human populations. PMID:16252337