Science.gov

Sample records for vasculitis

  1. Hypersensitivity Vasculitis

    MedlinePLUS

    ... Youtube LinkedIn Twitter Facebook Rare Disease Day + Vasculitis Behcet’s Disease Central Nervous System (CNS) Vasculitis Cryoglobulinemia Eosinophilic ... be severe. Kidney inflammation and even more rarely liver, lung, heart and brain injury have occurred in ...

  2. Vasculitis Foundation

    MedlinePLUS

    ... Youtube LinkedIn Twitter Facebook Rare Disease Day + Vasculitis Behcet’s Disease Central Nervous System (CNS) Vasculitis Cryoglobulinemia Eosinophilic ... is a condition that involves inflammation in the blood vessels. The condition occurs if your immune system attacks ...

  3. Pulmonary vasculitis.

    PubMed

    Lally, Lindsay; Spiera, Robert F

    2015-05-01

    Pulmonary vasculitis encompasses inflammation in the pulmonary vasculature with involved vessels varying in caliber from large elastic arteries to capillaries. Small pulmonary capillaries are the vessels most commonly involved in vasculitis affecting the lung. The antineutrophil cytoplasmic antibody-associated vasculitides, which include granulomatosis with polyangiitis (formerly Wegener granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome), are the small vessel vasculitides in which pulmonary vasculitis is most frequently observed and are the major focus of this review. Vasculitic involvement of the large pulmonary vessels as may occur in Behçet syndrome and Takayasu arteritis is also discussed. PMID:25836645

  4. Childhood vasculitis.

    PubMed

    Dillon, M J

    1998-01-01

    Vasculitis can and does occur in childhood. Apart from the relatively common vasculitides (Henoch-Schönlein purpura, Kawasaki disease and in world wide terms Takayasu disease) there are a number of important but comparatively rare disorders affecting children. These include macroscopic and microscopic polyarteritis, cutaneous polyarteritis, Wegener's granulomatosis, Churg-Strauss syndrome, primary angiitis of the central nervous system, hypersensitivity angiitis, hypocomplimentaemic urticarial vasculitis, vasculitis associated with various connective tissue disorders and vasculitis associated with conditions such as Behçets syndrome, familial Mediterranean fever and Cogan's syndrome. Distinguishing these conditions from other disorders is often difficult and requires clinical acumen and appropriate investigative procedures. With modern therapeutic agents, it is possible to implement appropriate therapy but in spite of this, there remains a not inconsequential morbidity and mortality. PMID:9643315

  5. Vasculitis Pregnancy Registry

    ClinicalTrials.gov

    2015-11-06

    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

  6. Ocular vasculitis.

    PubMed

    Espinoza, Gabriela M; Desai, Ankit; Akduman, Levent

    2013-09-01

    Vasculitis is the inflammation of blood vessels that leads to loss of perfusion and ischemia with necrosis. When this occurs in the orbit, the consequences are typically very conspicuous and can be devastating with decreased quality of life and loss of vision. Systemic vasculitides are often related to ophthalmic disorders, which can serve as the first diagnostic manifestation of potentially life-threatening disease. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (e.g., granulomatosis with polyangiitis), Behcet's disease, rheumatoid arthritis, sarcoidosis, and systemic lupus erythematosus are a few of the diseases commonly associated with ocular vasculitis. Collaboration between ophthalmologists and rheumatologists is important in the successful diagnosis and treatment of patients with vasculitis. PMID:23888364

  7. Golfer's vasculitis.

    PubMed

    Kelly, Robert I; Opie, Jacinta; Nixon, Rosemary

    2005-02-01

    A number of patients presented with an erythematous, purpuric rash occurring on the legs in association with playing golf and also after prolonged walks or hikes. Many patients believed that it was an allergic reaction to grasses or insecticides and had sometimes undergone extensive allergy testing. We collected reports of 17 such cases from dermatologists in the state of Victoria, Australia. Patients were interviewed by phone and asked to submit photographs of the rash if possible. Of these, the eruption developed in 15 after playing 18 holes of golf and in three following prolonged hikes. The rash would usually develop over the summer months under hot conditions. Most patients were over 50 years of age when the tendency to develop the eruption began. Biopsies of the rash in the active phase showed leukocytoclastic vasculitis. Patch testing and investigations for potential underlying causes for vasculitis were negative or unremarkable. It would seem that this is a common but poorly documented condition. The clinical presentation and histology would support the conclusion that it represents a leukocytoclastic vasculitis induced by prolonged exercise under hot conditions. The findings would suggest that it occurs in healthy people and extensive investigation with blood tests or allergy testing is inappropriate. We believe the condition should be termed 'golfer's vasculitis', as golf appears to be the most common precipitating event and such a term would enable the condition to become more widely recognized. PMID:15670170

  8. Pediatric Vasculitis Initiative

    ClinicalTrials.gov

    2015-07-16

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

  9. Impact of Vasculitis on Employment and Income

    ClinicalTrials.gov

    2015-12-01

    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

  10. [Vasculitis associated with connectivitis].

    PubMed

    Merazzi, D; Parravicini, D; Lietti, D

    1989-01-01

    A great number of vasculitis in childhood is associated with Rheumatic disease. Vascular lesions involve small vessels of various organs. These are described in systemic lupus erythematosus, juvenile rheumatoid arthritis, dermatomyositis, scleroderma, mixed connective tissue disease, Behçet syndrome, Sjögren syndrome. PMID:2682546

  11. Telmisartan induced urticarial vasculitis.

    PubMed

    Mahajan, Vikram K; Singh, Ravinder; Gupta, Mrinal; Raina, Rashmi

    2015-01-01

    A 53-year-old man developed urticarial vasculitis following ingestion of telmisartan and hydrochlorothiazide combination for hypertension. Treatment with prednisolone and cetirizine was curative, but his lesions recurred when he continued telmisartan and hydrochlorothiazide against medical advice. Re-challenge with the same doses of telmisartan precipitated similar lesions with telmisartan and not with hydrochlorothiazide. This uncommon cutaneous adverse reaction of angiotensin II receptor blockers has implication for the clinicians as more such cases may become apparent with their wider use than in premarketing studies. PMID:26600649

  12. Telmisartan induced urticarial vasculitis

    PubMed Central

    Mahajan, Vikram K.; Singh, Ravinder; Gupta, Mrinal; Raina, Rashmi

    2015-01-01

    A 53-year-old man developed urticarial vasculitis following ingestion of telmisartan and hydrochlorothiazide combination for hypertension. Treatment with prednisolone and cetirizine was curative, but his lesions recurred when he continued telmisartan and hydrochlorothiazide against medical advice. Re-challenge with the same doses of telmisartan precipitated similar lesions with telmisartan and not with hydrochlorothiazide. This uncommon cutaneous adverse reaction of angiotensin II receptor blockers has implication for the clinicians as more such cases may become apparent with their wider use than in premarketing studies.

  13. Tubercular retinal vasculitis.

    PubMed

    Patricio, Maria Sara; Portelinha, Joana; Passarinho, Maria Picoto; Guedes, Marta Esteves

    2013-01-01

    Intraocular tuberculosis (TB) infection can have different clinical manifestations including retinal vasculitis. It more frequently involves the veins and is associated with retina haemorrhages and neovascularisation. The diagnosis may be difficult and presumptive being based on clinical findings and evidence of systemic TB infection. The authors present a case of a 61-year-old woman with blurred vision and floaters in her left eye for 6 years, associated with recurrent vitreous haemorrahages. A temporal branch retinal vein occlusion was presumed. Four years later her right eye was also involved. Her best-corrected visual acuity (BCVA) was 20/50 in both eyes. Fundoscopic examination showed bilateral venous occlusion with vascular staining on fluorescein angiography suggestive of vasculitis secondary to Eales Disease (ED). The interferon gamma release assay (IGRA-QuantiFERON-TB Gold) was positive and antituberculosis treatment (ATT) was started. Her final BCVA was 20/20 bilaterally, without recurrences over a follow-up of 15 months. The use of ATT is likely to reduce recurrent vitreous haemorrhages and eliminate future recurrences. PMID:23737572

  14. Imaging spectrum of CNS vasculitis.

    PubMed

    Abdel Razek, Ahmed Abdel Khalek; Alvarez, Hortensia; Bagg, Stephen; Refaat, Sherif; Castillo, Mauricio

    2014-01-01

    Cerebral vasculitis is characterized by inflammation of the walls of blood vessels and may affect vessels of any size. The pathogenesis of vasculitis remains poorly understood. Vasculitis may affect large vessels (Takayasu arteritis, giant cell arteritis), medium-sized vessels (Kawasaki disease, polyarteritis nodosa), small vessels (immunoglobulin A vasculitis, microscopic polyangiitis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis), or variable-sized vessels (Behçet disease, Cogan syndrome). Primary angiitis of the central nervous system (CNS) is an idiopathic disorder with no evidence of generalized inflammation that may simulate reversible cerebral vasoconstriction syndromes. Vasculitis may be secondary to systemic disease, infection, malignancy, drug use, or radiation therapy. Imaging findings vary from small ischemic changes to frank infarction, hemorrhage, and white matter edema and may show contrast material enhancement. The cerebral arteries may demonstrate a beaded appearance with variable degrees of stenosis, occlusion, and contrast enhancement of the vessel wall. Correlation of imaging findings with clinical presentation and laboratory test results helps establish the diagnosis of CNS vasculitis. PMID:25019429

  15. Vasculitis and the gastrointestinal tract.

    PubMed

    Geboes, K; Dalle, I

    2002-01-01

    Vasculitis, defined as a non-infectious inflammatory disorder of blood vessels, can affect vessels of any type in any organ. The gastrointestinal (GI) tract may thus also be involved. In systemic disorders as mixed connective tissue disease (MCTD) and systemic lupus erythematodes (SLE), patients may present with symptoms of gastrointestinal disfunction such as motility disorders, caused by alterations in the connective tissue. True vasculitis however also occurs in the GI tract. Severe, occlusive damage often leads to ischemia that may result in ulceration and perforation. Non-occlusive vascular disease may lead to vascular leakage resulting in oedema and haemorrhage. Those patients often present with diarrhoea or symptoms of bleeding. GI involvement is frequent in Henoch-Schönlein purpura and also often noted in polyarteritis nodosa (PAN), microscopic polyangiitis, Wegener's syndrome and Churg-Strauss syndrome. Furthermore, GI vasculitis has also been described in giant cell arteritis, Takayasu's disease, Buerger's disease and leucocytoclastic vasculitides as essential mixed cryoglubulinemia, lupus vasculitis, rheumatoid disease, MCTD, drug-induced vasculitis and Behçet's disease. The diagnosis and classification of vasculitis relies upon a combination of clinical, serological, haematological, radiological and histological findings. Establishing a precise diagnosis can be difficult but is important because treatment and prognosis can be highly variable. PMID:12619427

  16. Diagnostic approach to patients with suspected vasculitis

    PubMed Central

    Suresh, E

    2006-01-01

    Vasculitis presents several diagnostic challenges. Firstly, patients could present with protean clinical manifestations with a wide spectrum ranging from isolated cutaneous vasculitis to multisystem involvement. Secondly, there are several medical conditions that could mimic the presentation of vasculitis. The range of differential diagnosis is therefore broad. Thirdly, vasculitis could occur as a primary disorder or be secondary to various medical conditions. It becomes important to differentiate them, as treatment of some forms of vasculitis such as those that are secondary to infection or drugs, is different from that of primary vasculitis. Fourthly, there are several different forms of vasculitis. Some are benign and self limiting, while others have the potential to threaten vital organ function and life. It follows that a rational approach is required during evaluation of patients with suspected vasculitis. PMID:16891436

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

    ClinicalTrials.gov

    2015-10-19

    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

  18. Gastroenterology Cases of Cutaneous Leukocytoclastic Vasculitis

    PubMed Central

    Arabaci, Elif; Yildiz, Kemal; Cakirca, Mustafa; Cikrikcioglu, Mehmet Ali; Ergun, Fatih; Danalioglu, Ahmet; Kocaman, Orhan; Senturk, Hakan

    2013-01-01

    Rarely, leukocytoclastic vasculitis can result from ischemic colitis, inflammatory bowel disease, and cryoglobulinemia. There is no established standard for the treatment of leukocytoclastic vasculitis associated with gastroenterologic diseases. This paper presents three cases of leukoytoclastic vasculitis, each of which is associated with a different gastroenterologic condition: ischemic colitis, Crohn's disease, and chronic hepatitis C. Each condition went into remission by treatment of leukocytoclastic vasculitis, regardless of the underlying disease. PMID:24250337

  19. 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.

  20. Single-Organ Gallbladder Vasculitis

    PubMed Central

    Hernández-Rodríguez, José; Tan, Carmela D.; Rodríguez, E. René; Hoffman, Gary S.

    2014-01-01

    Abstract Systemic vasculitis (SV) involving abdominal structures usually has a poor prognosis. Gallbladder vasculitis (GV) has been reported as part of SV (GB-SV) and focal single-organ vasculitis (GB-SOV). We analyzed clinical and histologic characteristics of patients with GV to identify features that differentiate GB-SOV from the systemic forms of GV. To identify affected patients with GV we used pathology databases from our institution and an English-language PubMed search. Clinical manifestations, laboratory and histologic features, treatment administered, and outcomes were recorded. Patients were divided in 2 groups, GB-SOV and GB-SV. As in previous studies of single-organ vasculitis, GB-SOV was only considered to be a sustainable diagnosis if disease beyond the gallbladder was not apparent after a follow-up period of at least 6 months. Sixty-one well-characterized patients with GV were included (6 from our institution). There was no significant sex bias (32 female patients, 29 male). Median age was 52 years (range, 18–94 yr). GB-SOV was found in 20 (33%) and GB-SV in 41 (67%) patients. No differences were observed in age, sex frequency, or duration of gallbladder symptoms between groups. Past episodes of recurrent right-upper quadrant or abdominal pain and lithiasic cholecystitis were more frequent in GB-SOV patients, whereas acalculous cholecystitis occurred more often in GB-SV. In GB-SV, gallbladder-related symptoms occurred more often concomitantly with or after the systemic features, but they sometimes appeared before SV was fully developed (13.5%). Constitutional and musculoskeletal symptoms were reported only in GB-SV patients. Compared to GB-SOV, GB-SV patients presented more often with fever (62.5% vs 20%; p?=?0.003) and exhibited higher erythrocyte sedimentation rate levels (80?±?28 vs 37?±?25?mm/h, respectively; p?=?0.006). All GB-SV patients required glucocorticoids and 50% of them also received cytotoxic agents. Mortality in GB-SV was higher than in GB-SOV (35.5% vs 10%; p?=?0.05). Nongranulomatous inflammation with fibrinoid necrosis of medium-sized vessels occurred equally in both groups (>90%). Forms of SV affecting the gallbladder included polyarteritis nodosa (n?=?10), hepatitis B virus-associated vasculitis (n?=?8), cryoglobulinemic (essential or hepatitis C virus-associated) vasculitis (n?=?6), vasculitis associated with autoimmune diseases (n?=?6), microscopic polyangiitis (n?=?4), eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (n?=?4), IgA vasculitis (Henoch-Schönlein) (n?=?2), and giant cell arteritis (n?=?1). GV is uncommon. Its histology most often consists of a nongranulomatous necrotizing vasculitis affecting medium-sized vessels. GB-SOV is usually discovered after routine cholecystectomy performed because of the presence of local symptoms, gallstone-associated cholecystitis, and contrary to GB-SV, GB-SOV is usually not associated with systemic symptoms. Acute phase reactants and surrogate markers of autoimmunity are usually normal or negative in GB-SOV. GB-SOV does not require systemic antiinflammatory or immunosuppressive therapy; surgery is adequate to achieve cure. GB-SV always warrants immunosuppressant therapy and is associated with high mortality. The finding of GV may precede the generalized manifestations of SV. Therefore, once GV is discovered, studies to determine disease extent and a vigilant follow-up are mandatory. PMID:25500710

  1. 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

  2. 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. PMID:25399945

  3. Disease assessment in systemic vasculitis.

    PubMed

    Luqmani, Raashid Ahmed

    2015-04-01

    The anti-neutrophil cytoplasm antibody-associated vasculitides are complex multi-system disorders with many overlapping clinical features. Their outcome has been transformed by effective immunosuppression, preventing death in over 70% of cases. The quality of survival is affected by the disease course, which is characterized by a significant likelihood of relapse in 38%, chronic effects from the disease and its treatment, as well as emerging or worsening comorbidity, all of which contribute to the patient's clinical condition and outcome. Whilst imaging and laboratory testing including histology are important aspects of diagnosis, they are of limited value in assessing response to therapy or subsequent disease course. We have developed standardized validated clinical methods to quantify disease activity and damage; we are developing effective measures of patient experience to complement these procedures. This approach provides a rational basis for clinical management as well as being essential in the conduct of clinical trials and studies in vasculitis, by providing reproducible definitions of relapse, remission and response to therapy for patients with systemic vasculitis. Clinical assessment remains the current gold standard for evaluating disease progress, but requires regular training to ensure standardization. The development of biomarkers in future may produce a more accurate description of disease and identify potential targets for therapy as well as predictors of response to drugs. PMID:25662796

  4. New insight into the rheumatoid vasculitis.

    PubMed

    Cojocaru, M; Cohocaru, Inimioara Mihaela; Chico, B

    2015-01-01

    Vasculitis in rheumatoid arthritis (rheumatoid vasculitis, RV) has a heterogeneous clinical presentation that includes skin disorders, neuropathy, eye symptoms and systemic inflammation. Rheumatoid vasculitis is an unusual complication of longstanding, severe rheumatoid arthritis (RA). While RA affects the body's joints, vasculitis is a condition in which blood vessels become inflamed. Rheumatoid vasculitis occurs in approximately 2 to 5% of patients who have RA. The blood vessels most often involved are arteries that bring blood to the skin, nerves, and internal organs. Veins can also be involved. Rheumatoid vasculitis is skin condition that is a typical feature of RA, presenting as peripheral vascular lesions that are localized (purpura, cutaneous ulceration, and gangrene of the distal parts of the extremities). The cause of RV is unknown, but given the prominence of immune components and the pathologic changes in involved blood vessels, an autoimmune process is suggested. Compared to other forms of vasculitis, there has been relativejy little research in recent years on the specific entity of RV. There is some evidence that the incidence of RV has decreased over the past several decades, perhaps because of a better treatment of the underlying RA. In the present review, we discuss the clinical features, laboratory tests, the pathogenesis of RV. PMID:26402981

  5. [Diagnostic imaging of central nervous system vasculitis].

    PubMed

    Yokota, Hajime; Yamada, Kei

    2015-03-01

    Vasculitis involving the central nervous system presents with infarction and hemorrhage, which are often nonspecific findings. Laboratory examinations are essential for diagnosis of vasculitis in addition to comprehensive and systematic review of the clinical course. Although most findings tend to be nonspecific, enhancement and thickening of the vascular wall indicate vasculitis. Visualization of the vascular wall requires selection of the appropriate imaging modality and mode of image acquisition. Contrast-enhanced CT, MRI, and FDG-PET are useful for visualizing large vessel vasculitis, while CT, MRI, and angiography are effective for medium vessel vasculitis. The use of ultrasound is limited to evaluating vessels on the body surface. Although relatively thick vessels can be demonstrated by angiography, complete survey of small vessels is difficult. Here, we summarize the characteristics of each imaging modality and imaging findings of typical vasculitides-Takayasu arteritis, giant cell arteritis, ANCA-associated vasculitis, Behçet's disease, primary angiitis of the CNS, and vasculitis associated with systemic disease. Differential diagnoses are also shown, including infective endocarditis, tuberculous meningitis, Ehlers-Danlos syndrome, and reversible cerebral vasoconstriction syndrome. PMID:25846439

  6. Vasculitis

    MedlinePLUS

    ... by the American College of Rheumatology Communications and Marketing Committee. This information is provided for general education ... Lists Supporters About Us Leadership Careers at ACR Social Media Newsroom Annual Reports & Financial Statements Policies & Guidelines ...

  7. Urticarial Vasculitis in Northern Spain

    PubMed Central

    Loricera, Javier; Calvo-Río, Vanesa; Mata, Cristina; Ortiz-Sanjuán, Francisco; González-López, Marcos A.; Alvarez, Lino; González-Vela, M. Carmen; Armesto, Susana; Fernández-Llaca, Héctor; Rueda-Gotor, Javier; González-Gay, Miguel A.; Blanco, Ricardo

    2014-01-01

    Abstract Urticarial vasculitis (UV) is a subset of cutaneous vasculitis (CV), characterized clinically by urticarial skin lesions of more than 24 hours’ duration and histologically by leukocytoclastic vasculitis. We assessed the frequency, clinical features, treatment, and outcome of a series of patients with UV. We conducted a retrospective study of patients with UV included in a large series of unselected patients with CV from a university hospital. Of 766 patients with CV, UV was diagnosed in 21 (2.7%; 9 male and 12 female patients; median age, 35 yr; range, 1–78 yr; interquartile range, 5–54 yr). Eight of the 21 cases were aged younger than 20 years old. Potential precipitating factors were upper respiratory tract infections and drugs (penicillin) (n = 4; in all cases in patients aged <20 yr), human immunodeficiency virus (HIV) infection (n = 1), and malignancy (n = 1). Besides urticarial lesions, other features such as palpable purpura (n = 7), arthralgia and/or arthritis (n = 13), abdominal pain (n = 2), nephropathy (n = 2), and peripheral neuropathy (n = 1) were observed. Hypocomplementemia (low C4) with low C1q was disclosed in 2 patients. Other abnormal laboratory findings were leukocytosis (n = 7), increased erythrocyte sedimentation rate (n = 6), anemia (n = 4), and antinuclear antibody positivity (n = 2). Treatment included corticosteroids (n = 12), antihistaminic drugs (n = 6), chloroquine (n = 4), nonsteroidal antiinflammatory drugs (n = 3), colchicine (n = 2), and azathioprine (n = 1). After a median follow-up of 10 months (interquartile range, 2–38 mo) recurrences were observed in 4 patients. Apart from 1 patient who died because of an underlying malignancy, the outcome was good with full recovery in the remaining patients. In conclusion, our results indicate that UV is rare but not exceptional. In children UV is often preceded by an upper respiratory tract infection. Urticarial lesions and joint manifestations are the most frequent clinical manifestation. Low complement serum levels are observed in a minority of cases. The prognosis is generally good, but depends on the underlying disease. PMID:24378743

  8. Orlistat-induced bullous leukocytoclastic vasculitis.

    PubMed

    Lazic, Tamara; Fonder, Margaret; Robinson-Bostom, Leslie; Wilkel, Caroline S; Della Torre, Laura

    2013-03-01

    Cutaneous leukocytoclastic vasculitis (LCV), also known as small-vessel vasculitis, is a process thought to be related to the presence of circulating immune complexes. Leukocytoclastic vasculitis is thought to be idiopathic in up to 50% of cases, but other common causes and associated disorders include certain medications, most frequently antibiotics; infections; collagen-vascular disease; paraproteinemias; and rarely neoplasia. We report a patient with cutaneous LCV induced by orlistat, a pancreatic lipase inhibitor that works as a weight-loss agent by decreasing the absorption of dietary fat. PMID:23617088

  9. [Primary central nervous system vasculitis].

    PubMed

    Schuster, S; Magnus, T

    2015-12-01

    Primary angiitis of the central nervous system (PACNS) is a rare disorder. However, it is often considered in the differential diagnosis of vascular or inflammatory CNS diseases. Diagnosis is challenging, as specific biomarkers are lacking and the clinical presentation can be variable. A definitive diagnosis can only be established by biopsy of the inflammatory changes in the vascular wall. Alternatively, the diagnosis of PACNS can also be based on the synopsis of clinical, radiological, and laboratory findings. Different subtypes of PACNS have been described in recent years, depending on the size of the affected vessels or histopathological patterns. Based on selective literature research in the database PubMed on the subject of CNS vasculitis, this article reviews the diagnostic characteristics and differential diagnosis of the condition. We suggest a diagnostic algorithm customized to the size of the affected vessels. Lastly, therapeutic options and the outcome of PACNS are briefly outlined. PMID:26589203

  10. Thrombosis in vasculitis: from pathogenesis to treatment.

    PubMed

    Emmi, Giacomo; Silvestri, Elena; Squatrito, Danilo; Amedei, Amedeo; Niccolai, Elena; D'Elios, Mario Milco; Della Bella, Chiara; Grassi, Alessia; Becatti, Matteo; Fiorillo, Claudia; Emmi, Lorenzo; Vaglio, Augusto; Prisco, Domenico

    2015-01-01

    In recent years, the relationship between inflammation and thrombosis has been deeply investigated and it is now clear that immune and coagulation systems are functionally interconnected. Inflammation-induced thrombosis is by now considered a feature not only of autoimmune rheumatic diseases, but also of systemic vasculitides such as Behçet's syndrome, ANCA-associated vasculitis or giant cells arteritis, especially during active disease. These findings have important consequences in terms of management and treatment. Indeed, Behçet'syndrome requires immunosuppressive agents for vascular involvement rather than anticoagulation or antiplatelet therapy, and it is conceivable that also in ANCA-associated vasculitis or large vessel-vasculitis an aggressive anti-inflammatory treatment during active disease could reduce the risk of thrombotic events in early stages. In this review we discuss thrombosis in vasculitides, especially in Behçet's syndrome, ANCA-associated vasculitis and large-vessel vasculitis, and provide pathogenetic and clinical clues for the different specialists involved in the care of these patients. PMID:25883536

  11. 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

  12. HIV diagnosis in a patient presenting with vasculitis.

    PubMed

    Sharif, M; Hameed, S; Akin, I; Natarajan, U

    2016-02-01

    A patient with digital ischaemia and gangrene was treated with iloprost and antiplatelets for two weeks. His vasculitic screen was negative except for a positive HIV test. His vasculitis improved three weeks after treatment with antiretroviral medications. Though vasculitis is well known to be associated with HIV infection, very few cases of HIV present as vasculitis. PMID:25769887

  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 encountered by clinicians. The most common underlying malignancy is generally hematologic. In these cases the presence of cytopenias and immature cells may be red flags for the diagnosis of cancer. In patients with paraneoplastic cutaneous vasculitis, the prognosis depends on the underlying neoplasia. PMID:24145696

  14. Immunologic Cerebral Vasculitis and Extrapulmonary Tuberculosis: An Uncommon Association

    PubMed Central

    Wang, Yiyi; Li, Qian; Zhen, Xiaohan; Liu, Yuan

    2015-01-01

    Infection can cause cerebral vasculitis not only by direct invasion of the vessel wall, but by immune complex deposition, or through secondary cryoglobulineamia. There are also two types of cerebral vasculitis associated with tuberculosis (TB). In TB treatment, cerebral vasculitis caused by immunologic injury received little attention than vasculitis due to direct invasion of TB infection. We report a case in a young woman who presented with fever, generalized lymphadenopathy, stroke-like events, movement disorder and coma, which was found to be active, lymph node TB with immunologic cerebral vacuities without tuberculosis meningitis. PMID:26500938

  15. Immunologic Cerebral Vasculitis and Extrapulmonary Tuberculosis: An Uncommon Association.

    PubMed

    Wang, Yiyi; Li, Qian; Zhen, Xiaohan; Liu, Yuan; Wu, Qi

    2015-09-01

    Infection can cause cerebral vasculitis not only by direct invasion of the vessel wall, but by immune complex deposition, or through secondary cryoglobulineamia. There are also two types of cerebral vasculitis associated with tuberculosis (TB). In TB treatment, cerebral vasculitis caused by immunologic injury received little attention than vasculitis due to direct invasion of TB infection. We report a case in a young woman who presented with fever, generalized lymphadenopathy, stroke-like events, movement disorder and coma, which was found to be active, lymph node TB with immunologic cerebral vacuities without tuberculosis meningitis. PMID:26500938

  16. Vasculitis Syndromes of the Central and Peripheral Nervous Systems

    MedlinePLUS

    ... system, including the central (CNS) and peripheral (PNS) nervous systems. Vasculitis disorders, or syndromes, of the CNS and PNS are ... for Vasculitis Syndromes of the Central and Peripheral Nervous Systems Clinical Trials At NIH ... Organization for Rare Disorders (NORD) 55 Kenosia Avenue Danbury, CT 06810 orphan@ ...

  17. Idiopathic granulomatous vasculitis: response to immunosuppressive therapy.

    PubMed Central

    Alguacil-Garcia, G F; Moreno-Requena, J; Martinez-Albadalejo, M; Hallal-Hachem, H; Gonzalez-Pina, B; de Paco-Moya, M

    1995-01-01

    A case of idiopathic granulomatous vasculitis (disseminated visceral giant cell arteritis) is described in an old woman, the seventh case of this rare disorder reported to date. The main organ affected was the liver and, to our knowledge, this is the first patient to be diagnosed while still alive and the only case to have received medical treatment. It is also the first time that muscular involvement has been documented in this condition. Cyclophosphamide treatment resulted in disappearance of symptoms and increase in weight. The patient died of an unrelated condition. Images PMID:7665707

  18. [Vasculitis: New nomenclature of the Chapel Hill consensus conference 2012].

    PubMed

    Holl-Ulrich, K

    2014-11-01

    Within the last years, many advances have been made in the understanding of the etiopathology of vasculitis as well as of different disease courses. The revised 2012 Chapel Hill consensus conference (CHCC) nomenclature reflects current knowledge on the etiopathology in addition to the descriptive principles of vessel size and types of inflammation. The anti-neutrophil cytoplasmic antibody (ANCA)-associated forms of vasculitis have been separated as a group, as opposed to immune complex small vessel vasculitis. When consensus was achieved eponyms have been replaced by systematic names, such as granulomatosis with polyangiitis (Wegener's granulomatosis) or eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Moreover, clinically important but less well-known types of vasculitis have now been included in the CHCC nomenclature. This article presents the changes and summarizes the results of important new articles on the clinical picture and morphology of vasculitis. PMID:25269877

  19. Childhood vasculitis hospitalizations in Spain, 1997-2011.

    PubMed

    Villaverde-Hueso, A; Alonso, V; Morales-Piga, A; Hens-Pérez, M; Abaitua, I; Posada-de-la-Paz, M

    2014-05-01

    The aim of this study is to describe the childhood vasculitis hospital burden in Spain (1997-2011), considering type of disease, hospitalization rates and time trends. Data were obtained from the National Discharges Basic Minimum Data Set (National Patient Data Base). Inpatient events of children younger than 15 years of age were analyzed. Principal diagnosis of vasculitis were selected according Ninth Revision of the International Classification of Diseases: Takayasu arteritis, Polyarteritis nodosa, Kawasaki disease, Wegener`s granulomatosis, Churg-Strauss syndrome, and Henoch-Schönlein purpura. A total of 14518 children hospitalizations related to vasculitis were identified in Spain from 1997 to 2011. The average hospitalization rate for children was 13.33±1.71 per 100,000. Henoch-Schönlein purpura and Kawasaki disease were the most common type of vasculitis, hospitalization rates were 11.00 and 3.97 per 100,000 children, respectively. Other vasculitis hospitalizations are much rare in childhood. Average length of stay was 6.04 days and estimated cost per inpatient hospital care was 2,847€. Hospital case fatality rate was 0.05% for overall vasculitis. In conclusion, epidemiological data of childhood vasculitis are useful both to health decision-making and to identify research priorities. PMID:24940860

  20. 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. PMID:25986911

  1. Genetically Distinct Subsets within ANCA-Associated Vasculitis

    PubMed Central

    Lyons, Paul A.; Rayner, Tim F.; Trivedi, Sapna; Holle, Julia U.; Watts, Richard A.; Jayne, David R.W.; Baslund, Bo; Brenchley, Paul; Bruchfeld, Annette; Chaudhry, Afzal N.; Tervaert, Jan Willem Cohen; Deloukas, Panos; Feighery, Conleth; Gross, Wolfgang L.; Guillevin, Loic; Gunnarsson, Iva; P, Lorraine Harper M.R.C; Hrušková, Zdenka; Little, Mark A.; Martorana, Davide; Neumann, Thomas; Ohlsson, Sophie; Padmanabhan, Sandosh; Pusey, Charles D.; Salama, Alan D.; Sanders, Jan-Stephan F.; Savage, Caroline O.; Segelmark, Mårten; Stegeman, Coen A.; Tesa?, Vladimir; Vaglio, Augusto; Wieczorek, Stefan; Wilde, Benjamin; Zwerina, Jochen; Rees, Andrew J.; Clayton, David G.; Smith, Kenneth G.C.

    2013-01-01

    BACKGROUND Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis is a severe condition encompassing two major syndromes: granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis) and microscopic polyangiitis. Its cause is unknown, and there is debate about whether it is a single disease entity and what role ANCA plays in its pathogenesis. We investigated its genetic basis. METHODS A genomewide association study was performed in a discovery cohort of 1233 U.K. patients with ANCA-associated vasculitis and 5884 controls and was replicated in 1454 Northern European case patients and 1666 controls. Quality control, population stratification, and statistical analyses were performed according to standard criteria. RESULTS We found both major-histocompatibility-complex (MHC) and non-MHC associations with ANCA-associated vasculitis and also that granulomatosis with polyangiitis and microscopic polyangiitis were genetically distinct. The strongest genetic associations were with the antigenic specificity of ANCA, not with the clinical syndrome. Anti–proteinase 3 ANCA was associated with HLA-DP and the genes encoding ?1-antitrypsin (SERPINA1) and proteinase 3 (PRTN3) (P = 6.2×10?89, P = 5.6×10?12, and P = 2.6×10?7, respectively). Anti–myeloperoxidase ANCA was associated with HLA-DQ (P = 2.1×10?8). CONCLUSIONS This study confirms that the pathogenesis of ANCA-associated vasculitis has a genetic component, shows genetic distinctions between granulomatosis with polyangiitis and microscopic polyangiitis that are associated with ANCA specificity, and suggests that the response against the autoantigen proteinase 3 is a central pathogenic feature of proteinase 3 ANCA–associated vasculitis. These data provide preliminary support for the concept that proteinase 3 ANCA–associated vasculitis and myeloperoxidase ANCA–associated vasculitis are distinct autoimmune syndromes. (Funded by the British Heart Foundation and others.) PMID:22808956

  2. Atherosclerotic factors in PR3 pulmonary vasculitis.

    PubMed

    Zycinska, K; Wardyn, K; Zielonka, T M; Nitsch-Osuch, A; Smolarczyk, R; Zarzycki, S; Demkow, U; Lukas, W; Pirogowicz, I

    2013-01-01

    Autoimmune disease such as systemic lupus erythematosus or rheumatoid arthritis are connected with higher risk of atherosclerosis and cardiovascular complications and mortality. This results from inflammatory damage to the vessel wall by vasculitis. The aim of the present study was to evaluate whether patients with Wegener's granulomatosis (WG) and pulmonary involvement have an increased prevalence of atherosclerotic disease as characterized traditional risk factors. Twenty one patients with WG in remission and 15 control subject were entered to the study. Traditional risk factor for cardiovascular disease such as hyperglycemia, hypertension, smoking, obesity, and dyslipidemia were assessed. Both systolic and diastolic blood pressure were higher in WG patients (p<0.025). Total cholesterol, LDL and TG levels were markedly elevated in 18 of the 21 in pulmonary WG patients. Compared with controls, plasma levels of hsCRP were raised in WG patients; 3.68 (0.79-9.75) mg/l vs. 0.14 (0.12-0.59) mg/l (p<0.01). We conclude that non-pharmacological and pharmacological treatments of traditional risk factors are crucial to prevent cardiovascular disease in WG patients and thus should be part of therapy to control WG activity and damage caused by it. PMID:22826078

  3. Drug-induced vasculitis: a clinical and pathological review.

    PubMed

    Radi?, M; Martinovi? Kaliterna, D; Radi?, J

    2012-01-01

    Drug-induced vasculitis is an inflammation of blood vessels caused by the use of various pharmaceutical agents. Vasculitis causes changes in the walls of blood vessels, including thickening, weakening, narrowing and scarring. Inflammation can be short-term (acute) or long-term (chronic) and can be so severe that the tissues and organs supplied by the affected vessels do not get enough blood. The shortage of blood can result in organ and tissue damage, even death. Drug-induced vasculitis is the most common form of vasculitis. The differential diagnosis between drug-induced and idiopathic vasculitic conditions may be difficult in the individual patient. Withdrawal may be helpful to distinguish between these syndromes. Withdrawal of the offending agent alone is often sufficient to induce prompt resolution of clinical manifestations, obviating the need for immunosuppressive and anti-inflammatory drugs. Increasing understanding of the pathophysiological characteristics of all inflammatory vasculitides should lead to better diagnostic and therapeutic approaches to drug-induced vasculitis. PMID:22271809

  4. [Systemic vasculitis: study of 27 cases in Senegal].

    PubMed

    Ndongo, S; Diallo, S; Tiendrebeogo, J; Diop, I B; Tall, A; Pouye, A; Ka, M M; Diop, T M

    2010-06-01

    Studies on vasculitis in black Africa are rare. The purpose of this report is to describe a retrospective study of systemic vasculitis managed in the internal medicine, ORL and cardiolology departments of the Aristide le Dantec University Hospital in Dakar, Senegal from 1995 to 2007. A series of 27 cases involving 7 men and 20 women with a mean age of 49 years was compiled. Primary vasculitis included Horton disease in 3 cases, Wegener disease in 2, Takayasu disease in 1, and Buerger disease in 1. Secondary vasculitis included mixed cryoglobulinemia with Gougerot Sjögren syndrome in 7 cases, primary Goujeröt syndrome in 4, rheumatoid arthritis in 3, nodosa periarteritis with hepatitis B in 2, SHARP syndrome in 1, and polymyositis in 1. The remaining two cases involved abdominal periaortitis including one associated with retrosperitoneal fibrosis and tuberculosis and the other with spondylarthropathy. Corticotherapy in combination with anticoagulants, immunosuppressive therapy, and surgery, when necessary, allowed effective management in 24 cases. The findings of this study show that systemic vasculitis can have numerous etiologies and indicate that secondary forms are the most common. Appropriate care modalities are needed to prevent severe outcome in Senegalese hospitals. PMID:20734595

  5. Systemic vasculitis: an annual critical digest of the most recent literature.

    PubMed

    Della Rossa, Alessandra; Cioffi, Elisa; Elefante, Elena; Ferro, Francesco; Parma, Alice; Vagelli, Roberta; Talarico, Rosaria

    2014-01-01

    Herewith we provide our annual digest of the recent literature on systemic vasculitis in which we reviewed all the articles published during the last 12 months on large-, medium- and small-vessel vasculitis, and selected the most relevant studies regarding the epidemiology, pathogenesis and management of systemic vasculitis. In particular, we focused the attention on giant cell arteritis, ANCA-associated vasculitis and cryoglobulinaemia. PMID:24854379

  6. 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

  7. Endoscopic and radiographic features of gastrointestinal involvement in vasculitis.

    PubMed

    Hokama, Akira; Kishimoto, Kazuto; Ihama, Yasushi; Kobashigawa, Chiharu; Nakamoto, Manabu; Hirata, Tetsuo; Kinjo, Nagisa; Higa, Futoshi; Tateyama, Masao; Kinjo, Fukunori; Iseki, Kunitoshi; Kato, Seiya; Fujita, Jiro

    2012-03-16

    Vasculitis is an inflammation of vessel walls, followed by alteration of the blood flow and damage to the dependent organ. Vasculitis can cause local or diffuse pathologic changes in the gastrointestinal (GI) tract. The variety of GI lesions includes ulcer, submucosal edema, hemorrhage, paralytic ileus, mesenteric ischemia, bowel obstruction, and life-threatening perforation.The endoscopic and radiographic features of GI involvement in vasculitisare reviewed with the emphasis on small-vessel vasculitis by presenting our typical cases, including Churg-Strauss syndrome, Henoch-Schönlein purpura, systemic lupus erythematosus, and Behçet's disease. Important endoscopic features are ischemic enterocolitis and ulcer. Characteristic computed tomographic findings include bowel wall thickening with the target sign and engorgement of mesenteric vessels with comb sign. Knowledge of endoscopic and radiographic GI manifestations can help make an early diagnosis and establish treatment strategy. PMID:22442741

  8. 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

  9. Ischemic Retinal Vasculitis Associated with Cataract Surgery and Intracameral Vancomycin

    PubMed Central

    Lenci, Lucas T.; Chin, Eric K.; Carter, Christi; Russell, Stephen R.; Almeida, David R. P.

    2015-01-01

    Recently, there have been reports suggesting that intracameral vancomycin has been associated with retinal vasculitis; some have described this phenomenon as postoperative hemorrhagic occlusive retinal vasculitis. We present a case of a 65-year-old woman who underwent uncomplicated phacoemulsification and posterior chamber intraocular lens implantation followed by intracameral antibiotic prophylaxis. Unlike prior reports, this report demonstrates a case of mild visual reduction and minimal inflammation with subtle but complete unilateral peripheral retinal ischemia associated with cataract surgery and intracameral vancomycin, suggesting a spectrum of toxicity that may be underrecognized. PMID:26618015

  10. Drug-Induced Vasculitis: New Insights and a Changing Lineup of Suspects.

    PubMed

    Grau, Rafael G

    2015-12-01

    An increasing number of therapeutic agents have been associated with a vasculitic syndrome. This usually involves small vessels, primarily capillaries, venules, and arterioles in leukocytoclastic vasculitis, small-vessel disease similar to an antineutrophil cytoplasmic antibody-related vasculitis, or mid-sized muscular arteries in a polyarteritis-like picture. Antineutrophil cytoplasmic antibodies are present in many cases of vasculitis regardless of the size of the vessel involved. Monoclonal antibodies used to treat many autoimmune disorders have become the most common agents associated with drug-induced vasculitis. Important advances in epigenetics, genetics, and neutrophil apoptosis are providing new insights into the pathogenesis of both drug-induced vasculitis and idiopathic vasculitis. Although management has not changed significantly in the past few years where withdrawal of the offending agent is the primary intervention, increasing awareness of drug-induced vasculitis can lead to earlier diagnosis and prevention of severe organ damage and fatalities. PMID:26503355

  11. 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

  12. A Case of Cerebral Vasculitis Associated with Ulcerative Colitis

    PubMed Central

    Raj, Naveen; Arkebauer, Matthew; Waters, Barry; Dickinson, Brucha

    2015-01-01

    Ulcerative colitis (UC) is a chronic, debilitating condition characterized by inflammation of the colonic mucosa. It is regarded as a systemic inflammatory disorder that can affect a number of organ systems. Central nervous system disease associated with UC is a rare sequela of inflammatory bowel disease, occurring in less than 5% of cases. These manifestations include arterial and venous thrombosis, leukoencephalitis, seizures, and vasculitis. We present a case of a 61-year-old female with a two-year history of well-controlled ulcerative colitis, who developed altered mental status and weakness. On brain imaging, she was found to have cerebral lesions which were biopsied. Histopathology subsequently revealed coagulative necrosis and inflammation characteristic of vasculitis. Rheumatology serologies were negative, and the patient was started on steroids that dramatically improved her neurological function, with no residual deficits, and led to resolution of the brain lesions. PMID:26557402

  13. 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. PMID:24752347

  14. Pharmacological approaches to CNS vasculitis: where are we at now?

    PubMed

    Pagnoux, Christian; Hajj-Ali, Rula A

    2016-01-01

    The diagnosis and treatment of central nervous system (CNS) vasculitis is extremely challenging. Several conditions can mimic CNS vasculitis and require totally different treatment. CNS vasculitis, once confirmed, may result from infections or systemic diseases that will warrant specific treatments, or, more rarely, be primary and isolated (PCNSV). Prospective trials to help determine the optimal treatment for PCNSV are lacking, but data from several cohorts have provided seminal data on its management. The consensus is to use glucocorticoids as first-line agents, combined with additional immunosuppressants for the most severe cases, mainly cyclophosphamide for induction, followed by less-toxic maintenance therapy with azathioprine, methotrexate, or mycophenolate mofetil. The recent identification of PCNSV subgroups and predictors of outcomes might help in deciding the adequate treatment for each patient, keeping in mind that these data are based on a small number of patients. Other agents and biologics can be considered for patients with relapsing and/or refractory disease, but evidence is limited. In practice, the diagnosis must be re-questioned in patients with PCNSV refractory to standard treatment, especially with diagnoses not based on pathology. PMID:26559201

  15. Levamisole/Cocaine Induced Systemic Vasculitis and Immune Complex Glomerulonephritis

    PubMed Central

    Garg, Lohit; Gupta, Sagar; Swami, Abhishek; Zhang, Ping

    2015-01-01

    Levamisole is an antihelminthic and immunomodulator medication that was banned by the USFDA in 1998. It has been increasingly used to adulterate cocaine due to its psychotropic effects and morphological properties. Adverse reactions including cutaneous vasculitis, thrombocytopenia, and agranulocytosis have been well described. Despite systemic vasculitis in this setting, renal involvement is uncommon. We report here a case of ANCA positive systemic vasculitis with biopsy proven immune complex mediated glomerulonephritis likely secondary to levamisole/cocaine. A 40-year-old Caucasian male with no past medical history presented with 3-week history of fatigue, skin rash, joint pains, painful oral lesions, oliguria, hematuria, worsening dyspnea on exertion, and progressive lower extremity edema. He had a history of regular tobacco and cocaine use. Lab testing revealed severe anemia, marked azotemia, deranged electrolytes, and 4.7?gm proteinuria. Rheumatologic testing revealed hypocomplementemia, borderline ANA, myeloperoxidase antibody, and positive atypical p-ANCA. Infectious and other autoimmune workup was negative. Kidney biopsy was consistent with immune mediated glomerulonephritis and showed mesangial proliferation and immune complex deposition consisting of IgG, IgM, and complement. High dose corticosteroids and discontinuing cocaine use resulted in marked improvement in rash, mucocutaneous lesions, and arthritis. There was no renal recovery and he remained hemodialysis dependent. PMID:26290761

  16. Cytomegalovirus-related necrotising vasculitis mimicking Henoch-Schönlein syndrome.

    PubMed

    D'Alessandro, Matteo; Buoncompagni, Antonella; Minoia, Francesca; Coccia, Maria C; Martini, Alberto; Picco, Paolo

    2014-01-01

    Viral vasculitides have been previously reported in the literature, the role of infections in their pathogenesis ranging from direct cause to trigger event. Here we report the case of a 3-year-old immunocompetent girl who developed a systemic vasculitis leading to ileal perforation, mimicking a full blown picture of Henoch-Schönlein purpura. High dosage steroid treatment was started, with good response. The anatomopathological examination of the resected gastrointestinal tract showed features of necrotising vasculitis and cytomegalovirus (CMV)-related inclusion bodies in the endothelial cells, with direct correlation to vascular damage. The causative role of viral infection was revealed by the presence of CMV DNA in patient's blood and positive IgG titer against the virus. Steroid therapy was then tapered: the patient achieved clinical remission, which still persists after a six-months follow-up. Our report suggests that CMV vasculitis is probably more frequent than previously thought, even in immunocompetent patients, with a protean clinical presentation, mimicking other types of vasculitides. PMID:24854375

  17. Correlation between Fluorescein Angiographic Findings and Visual Acuity in Behçet Retinal Vasculitis

    PubMed Central

    Kim, Min; Kwon, Hee Jung; Choi, Eun Young; Kim, Sung Soo; Koh, Hyoung Jun

    2015-01-01

    Purpose To identify significant fluorescein angiographic (FA) characteristics associated with visual acuity (VA) in Behçet retinal vasculitis. Materials and Methods Retrospective review of 86 eyes of 48 patients (age: 35.6±10.2 years) with Behçet retinal vasculitis were performed. VA and FA findings as well as correlation between them were assessed. Results The mean initial VA of eyes with posterior pole-involved vasculitis (63 eyes; 73.3%) was significantly worse than that of those with peripheral vasculitis (23 eye; 26.7%) (logarithm of the minimum angle of resolution VA: 0.554±0.572 vs. 0.078±0.148; p<0.0001). Subgroup analysis revealed a more severe and diffuse pattern of vascular leakage in posterior pole-involved vasculitis compared to peripheral vasculitis (p<0.0001). Retinal vascular leakage (?=0.345; p<0.0001), optic disc hyperfluorescence (?=0.147; p=0.032), and macular leakage (?=0.107; p=0.047) were significantly associated with worse initial VA. During the follow up (mean: 33.3±17.9 months), the change of leakage showed no significant correlation with change of VA in posterior pole-involved vasculitis (?=0.199, p=0.092). Conclusion Posterior pole involvement, the degree of retinal vascular leakage, optic disc hyperfluorescence, and macular leakage are significantly associated with VA in Behçet retinal vasculitis. PMID:26069134

  18. Corticosteroids in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

    PubMed

    Keller, Sarah F; Miloslavsky, Eli M

    2016-02-01

    Glucocorticoids (GCs) have been the cornerstone of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) therapy since their advent in the 1950s. There is considerable variation in their use, both with respect to dose and duration. Given considerable treatment-related morbidity and mortality, refining the role of GCs is becoming increasingly important. This article discusses the current role of GCs in various phases of AAV treatment, including remission induction, maintenance therapy, treatment of relapses, and the use of local GCs. It discusses current controversies relating to GC use as well as research efforts that seek to reduce GC toxicity in AAV. PMID:26611553

  19. Severe bilateral ischemic retinal vasculitis following cataract surgery.

    PubMed

    Nicholson, Laura B; Kim, Brian T; Jardón, Javier; Townsend-Pico, William; Santos, Carmen; Moshfeghi, Andrew A; Albini, Thomas A; Eliott, Dean; Sobrin, Lucia

    2014-01-01

    This report describes two cases of severe, bilateral ischemic retinal vasculitis following cataract surgeries at different surgical centers. In both cases, the patient underwent bilateral cataract surgeries, performed 1 week apart for each eye. In the perioperative period following the second of the two surgeries, both patients developed severe, bilateral intraocular inflammation and profound vision loss. The underlying cause of this adverse response remains unknown. The authors suggest that the severe inflammatory reaction could be related to an intraoperative intracameral vancomycin injection. PMID:25127533

  20. Nodular Vasculitis That Developed during Etanercept (Enbrel) Treatment in a Patient with Psoriasis

    PubMed Central

    Park, Seung-Bae; Chang, In-Kyu; Im, Myung; Lee, Young; Kim, Chang-Deok; Seo, Young-Joon

    2015-01-01

    Nodular vasculitis was introduced by Montgomery for cases of erythema induratum-like lesions that were not associated with tuberculosis. Nodular vasculitis has been associated with both nontuberculous infections and noninfectious conditions. However, there has been no report on the development of nodular vasculitis during tumor necrosis factor-? inhibitor treatment. A 28-year-old man visited our clinic for the treatment of severe psoriasis with a 20-year history. Subcutaneous injection of etanercept (25 mg, twice weekly) was started. One year later, erythematous nodules developed on his lower leg. A skin biopsy showed lobular panniculitis with extensive necrosis and vasculitis. To exclude latent tuberculosis, an assay specific for Mycobacterium tuberculosis antigens was performed, with a negative result. After stopping etanercept under the diagnosis of nodular vasculitis associated with etanercept, the lesions gradually disappeared, leaving depressed scars in 3 months. There has been no recurrence after 6 months of follow-up. PMID:26512176

  1. Pauci-Immune Crescentic Glomerulonephritis: An ANCA-Associated Vasculitis

    PubMed Central

    Syed, Rafeel; Rehman, Amina; Valecha, Gautam; El-Sayegh, Suzanne

    2015-01-01

    Rapidly progressive glomerulonephritis (RPGN) is a syndrome signified by a precipitous loss of renal function, with features of glomerulonephritis including dysmorphic erythrocyturia and glomerular proteinuria. RPGN is associated with extensive crescent formation, and, thus, the clinical term RPGN is often used interchangeably with the pathologic term crescentic glomerulonephritis (CGN). From an immunopathologic standpoint, primary RPGN is divided into pauci-immune GN (PICG), anti-GBM GN, and immune complex GN. PICG, the most common etiology of primary RPGN, refers to a necrotizing glomerulonephritis with few or no immune deposits by immunofluorescence (IF) or electron microscopy (EM). In most patients, pauci-immune CGN is a component of a systemic small vessel vasculitis such as granulomatosis with polyangiitis (GPA). Approximately 90% of patients with PICG have circulating ANCA antibodies, leading to the nomenclature ANCA-associated vasculitis (AAV). Recent research has identified several other antibodies associated with PICG, which is now understood to be a complex spectrum of disease with considerable overlap in terms of clinical phenotype and outcomes. In addition, several genetic and environmental factors have recently been implicated in the pathogenesis of this disorder. With new prognostic classifications, enhanced understanding of immunopathologic mechanisms, and novel treatment paradigms, clinical and experimental interest in PICG remains high. PMID:26688808

  2. Multiple evanescent white dot syndrome associated with retinal vasculitis

    PubMed Central

    Takahashi, Akihiro; Saito, Wataru; Hashimoto, Yuki; Ishida, Susumu

    2015-01-01

    Purpose A recent study revealed thickening of the inner retinal layers in acute stage of multiple evanescent white dot syndrome (MEWDS); however, the pathogenesis is still unknown. We report two cases with MEWDS whose funduscopy showed obvious retinal vasculitis. Methods Case reports. Results Healthy myopic 16- and 27-year-old women were the cases under study. In both cases, funduscopic examination revealed multiple, faint, small, subretinal white dots at the posterior pole to the midperiphery and macular granularity oculus dexter. Retinal vascular sheathing was also observed at midperiphery. Late-phase fluorescein angiography revealed leakages corresponding to the vascular sheathing. Enhanced depth imaging optical coherence tomography revealed the discontinuity of the ellipsoid zone corresponding to the white dots and increased macular choroidal thickness. One month later, these white dots and retinal sheathing spontaneously resolved in both cases. Three months later, impairments of the outer retinal morphology and the visual acuity were restored. Conclusion These results suggest that retinal vasculitis possibly plays a role in the pathogenesis of thickened inner retinal layers in acute stage of MEWDS. PMID:26451125

  3. Update on the treatment of ANCA associated vasculitis.

    PubMed

    Smith, Rona M

    2015-06-01

    The introduction of glucocorticoids and cyclophosphamide has transformed ANCA associated vasculitis (AAV) from a fatal to a largely treatable condition. Over the past 40 years, considerable progress has been made in refining immunosuppressive regimens with a focus on minimising toxicity. As knowledge of the pathogenesis of AAV grows, it is mirrored by the availability of biological agents. Lymphocyte and cytokine targeted agents have been evaluated for the treatment of AAV and are entering the routine therapeutic arena with the potential to improve patient outcomes. Rituximab has transformed management of AAV in the past decade. However, there remains unmet need in the treatment of AAV; the majority of patients will relapse within five years of diagnosis despite maintenance immunosuppression; a small number of patients remain refractory to current therapies and treatment toxicity continues to contribute to mortality and chronic disability. As in rare diseases, treatment advances in vasculitis depend on international collaborative research networks to both establish an evidence base for newer agents and develop recommendations for optimal patient management. PMID:26021670

  4. 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

  5. Pulmonary arterial hypertension associated with rare cause of ANCA-associated vasculitis misdiagnosed as idiopathic one

    PubMed Central

    Li, Yi; Yi, Qun

    2015-01-01

    Pulmonary arterial hypertension (PAH) and ANCA-associated vasculitis (AAV) are both rare and complex diseases with poor prognosis especially when misdiagnosis. We report a rare case of a young woman presented with idiopathic pulmonary arterial hypertension (IPAH), but who was later found to have PAH associated with AAV. This case reminds us the very importance of differential diagnosis to PAH patients and the identification of rare cause likes ANCA-associated vasculitis. To our knowledge, this patient represents the first published case of misdiagnosis of PAH secondary to ANCA-associated vasculitis. PMID:26629233

  6. Gemcitabine-induced large vessel vasculitis demonstrated by PET CT: a rare, important side effect.

    PubMed

    Eyre, Toby A; Gooding, Sarah; Patel, Ishita; Moore, Niall; Hatton, Chris; Collins, Graham P

    2014-06-01

    Gemcitabine is a nucleoside analogue used widely across haemato-oncology. Side effects are generally predictable, and typically consist of cytopenia, nausea, and infection. As the present case clearly demonstrates, gemcitabine is in rare cases associated with life-threatening large vessel vasculitis, which can involve the aorta. It is important to consider gemcitabine-induced vasculitis in non-specifically unwell patients with raised inflammatory markers and fever of unknown origin, with or without signs of vascular compromise. Early recognition, cessation of gemcitabine therapy, and high-dose steroids are critical for a good outcome. PET CT is valuable to diagnose large vessel vasculitis and monitor treatment response. PMID:24584910

  7. Theodore E. Woodward Award. Do ANCA cause small vessel vasculitis?

    PubMed Central

    Falk, R. J.

    2001-01-01

    ANCA may be a pathogenetic force, but to date, support for this contention remains indirect. Active immunization with antigen or passive transfer of ANCA has not reproduced small vessel vasculitis (SVV). It is more than likely that if ANCA are pathogenetic, they are involved as one of many simultaneously occurring mechanisms acting in concert with other synergistic inflammatory mediators of disease. These include not only environmental factors such as infection or environmental toxins such as silica, but also genetic factors that are only now being described. The paradigm for this autoimmune process must include several events that occur simultaneously or sequentially, including ANCA, leukocyte activation and injured endothelium. Images Fig. 1 Fig. 2 PMID:11413776

  8. Rituximab versus Cyclophosphamide for ANCA-Associated Vasculitis

    PubMed Central

    Stone, John H.; Merkel, Peter A.; Spiera, Robert; Seo, Philip; Langford, Carol A.; Hoffman, Gary S.; Kallenberg, Cees G.M.; St. Clair, E. William; Turkiewicz, Anthony; Tchao, Nadia K.; Webber, Lisa; Ding, Linna; Sejismundo, Lourdes P.; Mieras, Kathleen; Weitzenkamp, David; Ikle, David; Seyfert-Margolis, Vicki; Mueller, Mark; Brunetta, Paul; Allen, Nancy B.; Fervenza, Fernando C.; Geetha, Duvuru; Keogh, Karina A.; Kissin, Eugene Y.; Monach, Paul A.; Peikert, Tobias; Stegeman, Coen; Ytterberg, Steven R.; Specks, Ulrich

    2011-01-01

    BACKGROUND Cyclophosphamide and glucocorticoids have been the cornerstone of remission-induction therapy for severe antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis for 40 years. Uncontrolled studies suggest that rituximab is effective and may be safer than a cyclophosphamide-based regimen. METHODS We conducted a multicenter, randomized, double-blind, double-dummy, noninferiority trial of rituximab (375 mg per square meter of body-surface area per week for 4 weeks) as compared with cyclophosphamide (2 mg per kilogram of body weight per day) for remission induction. Glucocorticoids were tapered off; the primary end point was remission of disease without the use of prednisone at 6 months. RESULTS Nine centers enrolled 197 ANCA-positive patients with either Wegener’s granulomatosis or microscopic polyangiitis. Baseline disease activity, organ involvement, and the proportion of patients with relapsing disease were similar in the two treatment groups. Sixty-three patients in the rituximab group (64%) reached the primary end point, as compared with 52 patients in the control group (53%), a result that met the criterion for noninferiority (P<0.001). The rituximab-based regimen was more efficacious than the cyclophosphamide-based regimen for inducing remission of relapsing disease; 34 of 51 patients in the rituximab group (67%) as compared with 21 of 50 patients in the control group (42%) reached the primary end point (P = 0.01). Rituximab was also as effective as cyclophosphamide in the treatment of patients with major renal disease or alveolar hemorrhage. There were no significant differences between the treatment groups with respect to rates of adverse events. CONCLUSIONS Rituximab therapy was not inferior to daily cyclophosphamide treatment for induction of remission in severe ANCA-associated vasculitis and may be superior in relapsing disease. (Funded by the National Institutes of Allergy and Infectious Diseases, Genentech, and Biogen; ClinicalTrials.gov number, NCT00104299.) PMID:20647199

  9. 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.

  10. Coronary artery vasculitis: assessment with cardiac multi-detector computed tomography.

    PubMed

    Jeon, Chang Ho; Kim, Yeo Koon; Chun, Eun Ju; Kim, Jeong A; Yong, Hwan Seok; Doo, Kyung Won; Choi, Sang Il

    2015-06-01

    Coronary artery vasculitis is rare and comprises an array of inflammatory diseases. It often results in severe and life-threatening complications, including coronary artery aneurysm, coronary artery stenosis, intraluminal thrombosis, and microcirculation abnormalities. These may occur at a young age and are often silent in the early phases. Invasive coronary angiography is the gold standard for diagnosing coronary artery disease (CAD); however, multi-detector computed tomography (MDCT) is now widely regarded as a powerful non-invasive tool for the detection of CAD. It is important for clinicians to recognize the various CT findings associated with coronary artery vasculitis in order to promote accurate diagnosis and proper patient management. The purpose of this article is to present an overview of the conditions associated with coronary artery vasculitis, with an emphasis on etiology and cardiac MDCT diagnosis of CAD. Cardiac MDCT is clinically useful and can provide information for the accurate diagnosis and treatment of coronary vasculitis. PMID:25841665

  11. Isolated non-necrotising granulomatous vasculitis of the gall bladder- a rare entity.

    PubMed

    Prasaad, Priavadhana Rajan; Shekhar, Shubhranshu; Priyadharshini, S Anu

    2014-10-01

    Gall bladder diseases are a significant cause of morbidity and mortality worldwide. Gall bladder diseases comprise a wide spectrum of disease entities including non specific inflammatory diseases, acute and chronic cholecystitis, follicular cholecystitis, granulomatous cholecystitis, metaplasic and dysplastic diseases of the gall bladder mucosa, gall bladder polyps and carcinomas. Here, we describe an unusual and a rare case of granulomatous vasculitis of the gall bladder incidentally diagnosed in a 38-year-old female, in a routine cholecystectomy specimen. Granulomatous vasculitis has been reported as a part of localised vasculitis of the gastrointestinal tract in the literature. The case is presented here for the rarity of the diagnosis of an isolated non-necrotising granulomatous vasculitis of the gall bladder. PMID:25478351

  12. Rare adverse effect of a common drug: nitrofurantoin-induced ANCA-associated vasculitis.

    PubMed

    Agarwal, Abhishek; Agrawal, Abhinav; Nathan, Karim; Roy, Satyajeet

    2015-01-01

    Nitrofurantoin is commonly prescribed to treat urinary tract infections (UTI). Reported adverse effects include gastrointestinal, pulmonary, hepatic and neurological disorders. We report a case of a 67-year-old woman treated for UTI with nitrofurantoin who presented with antineutrophilic cytoplasmic antibody (ANCA)-associated renal and skin vasculitis 3?days after starting treatment. The symptoms resolved following withdrawal of the drug and treatment with steroids. This is the first reported case of nitrofurantoin causing ANCA-associated vasculitis. PMID:25935915

  13. Mycophenolate mofetil in the treatment of SLE and systemic vasculitis: experience at a single university center.

    PubMed

    Lourdudoss, C; Vollenhoven, R van

    2014-03-01

    Mycophenolate mofetil (MMF) is used off-label for systemic lupus erythematosus (SLE) and systemic vasculitis. The study aim was to investigate clinical use and treatment results with MMF in patients with SLE and systemic vasculitis. This study included patients with SLE or systemic vasculitis with ongoing or previous MMF treatment. Data on treatment outcome were obtained through medical record reviews. A total of 135 of 648 (21%) patients with SLE and 43 of 455 (9%) patients with systemic vasculitis had ongoing or previous MMF treatment. Among SLE patients, the most common organ manifestation at baseline (treatment start) was renal involvement (50%). Most of the systemic vasculitis patients had Wegener's granulomatosis (GPA) (65%). Median dose of MMF was 2000?mg/day. Glucocorticoid (GC) doses were significantly reduced during MMF treatment from 21.7?mg/day at baseline to 8.3?mg/day at 12 months (p?vasculitis patients. MMF appeared to be effective with a reasonable survival-on-drug and a GC-sparing effect. PMID:24399811

  14. Incidence of Leukocytoclastic Vasculitis, 1996–2010: A Population-Based Study in Olmsted County, Minnesota

    PubMed Central

    Arora, Amrita; Wetter, David A.; Gonzalez-Santiago, Tania M.; Davis, Mark D. P.; Lohse, Christine M.

    2014-01-01

    Objective To determine the population-based incidence of leukocytoclastic vasculitis (LCV). Patients and Methods Retrospective population-based study of all Olmsted County, Minnesota, residents with a skin biopsy–proven diagnosis of LCV from January 1, 1996, through December 31, 2010. Results A total of 84 patients (mean age at diagnosis, 48.3 years) with newly diagnosed, skin biopsy–proven LCV (43 women, 41 men) were identified. The incidence rate (age and sex adjusted to the 2000 US white population) was 4.5 per 100,000 person-years (95% confidence interval, 3.5–5.4). The incidence of LCV increased significantly with age at diagnosis (P<.001) and did not differ between female and male patients. Subtypes of LCV were cutaneous small-vessel vasculitis (CSVV), 38 patients (45%); IgA vasculitis, 25 (30%); urticarial vasculitis, 10 (12%); cryoglobulinemic vasculitis, 3 (4%); and antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis, 8 (10%). LCV was idiopathic in 29 of 38 CSVV patients (76%) and 24 of 25 IgA vasculitis patients (96%). Thirty-nine of 84 patients (46%) had systemic involvement, with the renal system most commonly involved (17/39; 44%). Twenty-four of 80 patients (30%) with follow-up data available had recurrent disease. Compared with the Minnesota white population, observed survival in the incident LCV cohort was significantly poorer than expected (P<.001), including the subset of patients with idiopathic CSVV (P=.03). Conclusion The incidence of LCV was higher than that in previously published studies. Idiopathic LCV was more common in our population-based cohort than previously described. Overall survival was significantly poorer and should be explored further in future studies. PMID:24981218

  15. Endothelial nitric oxide synthase gene polymorphisms in Behçet's disease and rheumatic diseases with vasculitis

    PubMed Central

    Kim, J; Chang, H; Lee, S; Kim, J; Kim, K; Lee, S; Chung, W

    2003-01-01

    Objective: To assess potential associations between Korean Behçet's disease (BD) or other rheumatic diseases with vasculitis and two polymorphisms of the endothelial nitric oxide synthase (eNOS) gene, which include the Glu298Asp polymorphism in exon 7 and a variable number of tandem repeats (VNTR) polymorphism in intron 4. Methods: 65 patients with BD, 27 with rheumatic diseases with vasculitis, and 80 controls were studied. Analyses of the Glu298Asp polymorphism in exon 7 and VNTR polymorphism in intron 4 of the eNOS gene were made by the polymerase chain reaction (PCR)-restriction fragment length polymorphism technique and PCR genotyping, respectively. Additionally, HLA-B51 typing was performed in the BD group and controls by a two step PCR sequence-specific primers method. Results: Significant differences in Glu298Asp genotype frequencies were found between the BD or vasculitis groups and the controls (BD group v controls: pcorr=0.006; vasculitis group v controls: p<0.001). The Asp298 frequency was much higher in the BD and vasculitis groups than in the controls. Even after stratification of the BD group based on the results of HLA-B51 testing, a significant association of the Glu298Asp polymorphism was still found (p=0.002, Mantel-Haenszel weighted odds ratio 4.3, 95% confidence interval 1.7 to 10.9). Distribution of the genotype frequencies in two eNOS gene polymorphisms was similar in connective tissue diseases-associated vasculitis and primary vasculitic syndromes. In contrast, distribution of alleles and genotypes of VNTR polymorphism did not differ between BD or vasculitis groups and the controls. Conclusion: The Glu298Asp polymorphism in exon 7 of the eNOS gene seems to be a susceptibility gene for Korean BD and other rheumatic diseases. PMID:14583572

  16. Interstitial Keratitis, Vertigo, and Vasculitis: Typical Cogan's Syndrome.

    PubMed

    Azami, Ahad; Maleki, Nasrollah; Kalantar Hormozi, Mohammadreza; Tavosi, Zahra

    2014-01-01

    Cogan's syndrome (CS) is a chronic inflammatory disorder of unknown etiology that most commonly affects young adults. Clinical hallmarks are bilateral interstitial keratitis and vestibuloauditory dysfunction. Association between CS and systemic vasculitis as well as aortitis also exists. The diagnosis of CS is based upon presence of characteristic inflammatory eye disease and vestibuloauditory dysfunction. We describe classic Cogan's syndrome in a 47-year-old female from Ardabil. The patient was admitted with headache, vertigo, nausea, vomiting, right leg claudication, musculoskeletal pains, bilateral hearing loss, and blindness for the past two months. Ophthalmologic examination revealed that visual acuity was 0.1 bilaterally. Conjunctival hyperemia, bilateral cataract, and interstitial keratitis were detected with a slit lamp examination. Pure tone audiogram (PTA) and auditory brain stem response (ABR) showed bilateral sensorineural hearing loss. The other differential diagnosis of CS was studied and ruled out. Pulse i.v. methylprednisolone and cyclophosphamide were given and were followed by oral prednisolone and cyclophosphamide. Clinical follow-up showed partial improvement. PMID:24715922

  17. Healing rate and bacterial necrotizing vasculitis in venous leg ulcers.

    PubMed

    Piérard-Franchimont, C; Paquet, P; Arrese, J E; Piérard, G E

    1997-01-01

    Morbidity associated with venous leg ulcers is important in the elderly. The biological processes involved during attempts at healing are much more complex than in most models of experimental wounds. In addition, there is still controversy on deleterious effects elicited by both microorganisms and antiseptics on cells involved in the healing process. Using histology, immunohistochemistry and iterative computerized planimetry, we evaluated the bacterial load, the inflammatory aspects and the healing rate of leg ulcers present in 15 eligible women aged from 57 to 73 years. Each patient had at least 2 chronic ulcers treated with hydrocolloid dressing alone or in combination with daily applications of povidone-iodine solution (PVP-I). The weekly reduction in wound area was superior for hydrocolloid+PVP-I treatment than in hydrocolloid-treated ulcers. After a 4-week treatment, hydrocolloid-treated ulcers contained clumps of microorganisms and showed massive infiltration by phagocytes including Mac 387+ and factor XIIIa+ cells. Leukocytoclastic vasculitis was present as well. These features were less pronounced in ulcers treated with hydrocolloid+PVP-I. In sum, a broad-spectrum antimicrobial such as PVP-I may be beneficial in reducing deleterious bacteria-related inflammation. As a result, the healing rate leg ulcers is enhanced. PMID:9252771

  18. 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

  19. Sjogren's syndrome complicated with IgA nephropathy and leukocytoclastic vasculitis.

    PubMed

    Tsai, Tsung-Chang; Chen, Chen-Yin; Lin, Wei-Tung; Lee, Wei-Jen; Chen, Hung-Chun

    2008-01-01

    We report a case of primary Sjogren's syndrome (SS) with cutaneous leukocytoclastic vasculitis and IgA nephropathy. The accurate diagnosis of SS was established based on objective signs and symptoms of ocular and oral dryness, a characteristic appearance of a biopsy sample from a minor salivary gland, and the presence of anti-SS-A autoantibody. A second autoimmune disorder was not present, so the diagnosis of primary SS was established. A histologic finding of skin biopsy of purpuric lesion was typical for leukocytoclastic vasculitis. Renal biopsy was performed for nephrotic range proteinuria. The pathologic finding of renal biopsy was IgA glomerulonephritis with crescent formation. The patient was treated with small doses of glucocorticoids and maintenance hemodialysis. Leukocytoclastic vasculitis is one of the most characteristic extraglandular manifestations of SS. However, IgA nephropathy associated with SS and leukocytoclastic vasculitis is a rare finding. SS patients with glomerulonephritis present a more diverse outcome, even requiring hemodialysis. Therefore, renal biopsy is warranted in SS with glomerulonephritis and systemic vasculitis. PMID:18704825

  20. Clinical and serological features of severe vasculitis in rheumatoid arthritis: prognostic implications.

    PubMed Central

    Geirsson, A J; Sturfelt, G; Truedsson, L

    1987-01-01

    Sixteen patients with classic rheumatoid arthritis (RA) complicated by severe vasculitis were studied and compared with a matched control group of 16 RA patients without vasculitis. Seven of the patients with vasculitis died within 4 to 120 months (median 32 months) after developing vasculitic symptoms. Gangrene of digits and extremities, bowel ulcers or bowel perforation, or both, and cardiac involvement were more common among the patients who died than among those with a more favourable course. The present data suggest that large vessel vasculitis in RA is associated with high frequency of arteriosclerotic vascular disease. The serum concentrations of complement components C3 and C4 were lower, and concentrations of IgM rheumatoid factor, complement activating rheumatoid factor, and C1q binding immune complexes (C1q solid and C1q fluid phase assay) were significantly higher among vasculitic patients than in the control group. Laboratory data provided little prognostic information with regard to rheumatoid vasculitis, with the exception that IgM and IgG rheumatoid factors were significantly higher among patients with fatal course of disease than in those who achieved remission. PMID:3689000

  1. Genome-Wide Association Study of Hepatitis C Virus- and Cryoglobulin-Related Vasculitis

    PubMed Central

    Zignego, Anna Linda; Wojcik, Genevieve L.; Cacoub, Patrice; Visentini, Marcella; Casato, Milvia; Mangia, Alessandra; Latanich, Rachel; Charles, Edgar; Gragnani, Laura; Terrier, Benjamin; Piazzola, Valeria; Dustin, Lynn B.; Khakoo, Salim I.; Busch, Michael P.; Lauer, George M.; Kim, Arthur Y.; Alric, Laurent; Thomas, David L.; Duggal, Priya

    2014-01-01

    Objectives The host genetic basis of mixed cryoglobulin vasculitis is not well understood and has not been studied in large cohorts. A genome-wide association study was conducted among 356 HCV RNA positive individuals with cryoglobulin-related vasculitis and 447 ethnically-matched, HCV RNA positive controls. Methods All cases had both serum cryoglobulins as well as a vasculitis syndrome. A total of 899,641 markers from the Illumina HumanOmni1-Quad chip were analyzed using logistic regression adjusted for sex, as well as genetically-determined ancestry. Replication of select single nucleotide polymorphisms (SNPs) was conducted using 91 cases and 180 controls, adjusting for sex and country of origin. Results The most significant associations were identified on chromosome 6 near the NOTCH4 and MHC class II genes. A genome-wide significant association was detected on chromosome 6 at SNP rs9461776 (OR= 2.16, p=1.16E-07) between HLA-DRB1 and DQA1: this association was further replicated in additional independent samples (meta-analysis p=7.1×10?9). Conclusions A genome-wide significant association with cryoglobulin related vasculitis was identified with SNPs near NOTCH4 and MHC Class II genes. The two regions are correlated and it is difficult to disentangle which gene is responsible for the association with MC vasculitis in this extended MHC region. PMID:25030430

  2. Idiopathic hypereosinophilic syndrome: a new cause of vasculitis of the central nervous system.

    PubMed

    Rice, C M; Kurian, K M; Renowden, S; Whiteway, A; Price, C; Scolding, N J

    2015-05-01

    Idiopathic hypereosinophilic syndrome (IHES) is a primary haematological condition characterised by persistent, otherwise unexplained hypereosinophilia sufficient to cause organ damage. Various neurological complications are reported, but very few have mentioned CNS pathology and none has included CNS vasculitis. Our objective here is to report IHES as a new cause of histopathologically confirmed CNS vasculitis. A 39-year-old man presented with a relapsing sub-acute encephalopathy, with severe headaches, confusion and drowsiness, myoclonus, ataxia and papilloedema. He had a history of nephrotic syndrome 18 years earlier, stable for the past 5 years on low-dose corticosteroids and low-dose tacrolimus (2 mg bd); lichen planus, and (15 years previously) aloplecia totalis. On admission, he had a marked peripheral eosinophilia (up to 9.1 × 10(9)/dL), which—it subsequently became clear—had been intermittently present for 16 years. After extensive investigation, biopsies of brain and bone marrow confirmed diagnoses of cerebral vasculitis, with lymphocytic and macrophage (but not eosinophilic) cellular infiltration of blood vessel walls, and IHES. CNS vasculitis can therefore now be added to the list of neurological complications of IHES. A dramatic and sustained neurological improvement, and likewise of the eosinophilia, following treatment with corticosteroids and cyclophosphamide, emphasises the tractability of this newly described form of CNS vasculitis. PMID:25843450

  3. Urinary Biomarkers in Relapsing Antineutrophil Cytoplasmic Antibody-associated Vasculitis

    PubMed Central

    Lieberthal, Jason G.; Cuthbertson, David; Carette, Simon; Hoffman, Gary S.; Khalidi, Nader A.; Koening, Curry L.; Langford, Carol A.; Maksimowicz-McKinnon, Kathleen; Seo, Philip; Specks, Ulrich; Ytterberg, Steven R.; Merkel, Peter A.; Monach, Paul A.

    2015-01-01

    Objective Glomerulonephritis (GN) is common in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), but tools for early detection of renal involvement are imperfect. We investigated 4 urinary proteins as markers of active renal AAV: alpha-1 acid glycoprotein (AGP), kidney injury molecule-1 (KIM-1), monocyte chemoattractant protein-1 (MCP-1), and neutrophil gelatinase-associated lipocalin (NGAL). Methods Patients with active renal AAV (n = 20), active nonrenal AAV (n = 16), and AAV in longterm remission (n = 14) were identified within a longitudinal cohort. Urinary biomarker concentrations (by ELISA) were normalized for urine creatinine. Marker levels during active AAV were compared to baseline remission levels (from 1–4 visits) for each patient. Areas under receiver-operating characteristic curves (AUC), sensitivities, specificities, and likelihood ratios (LR) comparing disease states were calculated. Results Baseline biomarker levels varied among patients. All 4 markers increased during renal flares (p < 0.05). MCP-1 discriminated best between active renal disease and remission: a 1.3-fold increase in MCP-1 had 94% sensitivity and 89% specificity for active renal disease (AUC = 0.93, positive LR 8.5, negative LR 0.07). Increased MCP-1 also characterized 50% of apparently nonrenal flares. Change in AGP, KIM-1, or NGAL showed more modest ability to distinguish active renal disease from remission (AUC 0.71–0.75). Hematuria was noted in 83% of active renal episodes, but also 43% of nonrenal flares and 25% of remission samples. Conclusion Either urinary MCP-1 is not specific for GN in AAV, or it identifies early GN not detected by standard assessment and thus has potential to improve care. A followup study with kidney biopsy as the gold standard is needed. PMID:23547217

  4. An approach to the diagnosis and management of systemic vasculitis revised version with tracked changes removed

    PubMed Central

    Miller, A; Chan, M; Wiik, A; Misbah, S A; Luqmani, R A

    2010-01-01

    The systemic vasculitides are a complex and often serious group of disorders which, while uncommon, require careful management in order to ensure optimal outcome. In most cases there is no known cause. Multi-system disease is likely to be fatal without judicious use of immunosuppression. A prompt diagnosis is necessary to preserve organ function. Comprehensive and repeated disease assessment is a necessary basis for planning therapy and modification of treatment protocols according to response. Therapies typically include glucocorticoids and, especially for small and medium vessel vasculitis, an effective immunosuppressive agent. Cyclophosphamide is currently the standard therapy for small vessel multi-system vasculitis, but other agents are now being evaluated in large randomized trials. Comorbidity is common in patients with vasculitis, including the cumulative effects of potentially toxic therapy. Long-term evaluation of patients is important in order to detect and manage relapses. PMID:20070316

  5. Capecitabine-induced leukocytoclastic vasculitis under neoadjuvant chemotherapy for locally advanced colorectal cancer

    PubMed Central

    Kee, Bryan K.; Tetzlaff, Michael T.; Wolff, Robert A.

    2015-01-01

    We describe a case of capecitabine-induced leukocytoclastic vasculitis in a patient with locally advanced rectal cancer under curative neoadjuvant concurrent chemoradiation using capecitabine. After 5 days of the initiation of capecitabine the patient developed a pruritic maculopapular rash in her extremities consistent with vasculitis which was confirmed on skin biopsy without any signs of systemic involvement. Capecitabine was held and the rash was treated with topical steroids with complete resolution of both rash and pruritus. Due to a lack of other alternative chemotherapeutic options and the cutaneous-only involvement of vasculitis, the capecitabine was re-introduced. Two days later, the patient developed an identical maculopapular rash with a similar distribution. Prednisone was initiated while the capecitabine was continued with complete resolution of the rash. The patient successfully completed her curative neoadjuvant chemoradiation therapy treatment without the need to permanently discontinue the capecitabine. PMID:26029464

  6. Systemic Levamisole-Induced Vasculitis in a Cocaine User without Cutaneous Findings: A Consideration in Diagnosis

    PubMed Central

    Baptiste, Gillian G.; Alexopoulos, Anastasia-Stefania; Masud, Tahsin; Bonsall, Joanna M.

    2015-01-01

    Levamisole is a known immunomodulating agent frequently used as a cutting agent in cocaine consumed in the United States today. Numerous cases of anti-neutrophil cytoplasmic antibody (ANCA) vasculitis connected with the use of levamisole-adulterated cocaine have previously been reported in the literature, classically characterized by a retiform purpuric rash. We report a case of a crack-cocaine user without cutaneous abnormalities who developed ANCA-associated glomerulonephritis that progressed to renal failure. This case demonstrates the difficulties in solidifying the diagnosis of levamisole-induced vasculitis in the absence of cutaneous findings and the need to pursue more testing to establish causality in ANCA-associated vasculitis that has potential for severe end-organ damage in patients who continue to use cocaine. PMID:26635879

  7. 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

  8. Intravascular crystal deposition: an early clue to the diagnosis of type 1 cryoglobulinemic vasculitis.

    PubMed

    Gammon, Bryan; Longmire, Michelle; DeClerck, Brittney

    2014-09-01

    Cutaneous small vessel vasculitis (CSVV) is a nonspecific finding with an extensive differential diagnosis. It is critically important to distinguish skin-limited presentations of CSVV from severe life-threatening systemic vasculitides presenting with CSVV as an initial manifestation. It can be challenging to determine which patients presenting with CSVV are at risk for systemic disease. Standard histopathologic evaluation, direct immunofluorescence, and serologic evaluation is typically required to exclude a systemic vasculitis. Type 1 cryoglobulinemia may rarely present with CSVV. Herein, we report a case of type 1 cryoglobulinemia in the setting of occult multiple myeloma. CSVV with prominent intravascular crystal formation was noted. The presence of intravascular crystals in the setting of CSVV may represent an important early clue to the diagnosis of type 1 cryoglobulinemic vasculitis. PMID:25147987

  9. IgA vasculitis associated with anti-TNF-? inhibitors in a patient with Crohn's disease.

    PubMed

    Nishikawa, Jun; Hosokawa, Ayumu; Akashi, Momoko; Mihara, Hiroshi; Nanjo, Sohachi; Yoshita, Hiroki; Ando, Takayuki; Kajiura, Shinya; Fujinami, Haruka; Sugiyama, Toshiro

    2015-01-01

    Anti-TNF-? inhibitors have been widely used in the treatment of inflammatory bowel disease. Although they have good clinical efficacy and tolerance, they remain a matter of concern because they cause drug-induced autoimmune disorders as side effects. Here, we report a case of a patient with Crohn's disease who developed IgA vasculitis after infliximab and adalimumab treatment. A 17-year-old male with Crohn's disease who had received scheduled infliximab treatment for the preceding 19 months complained of purpura on his lower limbs. He was diagnosed with infliximab-induced IgA vasculitis. Switching infliximab to adalimumab resulted in rapid improvement of the condition. However, 21 months after switching to adalimumab, his purpura recurred. Drug-induced IgA vasculitis is a rare complication caused by infliximab and adalimumab; however, diagnosis in the early phase and appropriate management of patients receiving anti-TNF-? inhibitors is critical to a successful patient outcome. PMID:26440688

  10. Value of anti-infective chemoprophylaxis in primary systemic vasculitis: what is the evidence?

    PubMed Central

    2009-01-01

    Although infections are a major concern in patients with primary systemic vasculitis, actual knowledge about risk factors and evidence concerning the use of anti-infective prophylaxis from clinical trials are scarce. The use of high dose glucocorticoids and cyclophosphamide pose a definite risk for infections. Bacterial infections are among the most frequent causes of death, with Staphylococcus aureus being the most common isolate. Concerning viral infections, cytomegalovirus and varicella-zoster virus reactivation represent the most frequent complications. The only prophylactic measure that is widely accepted is trimethoprim/sulfamethoxazole to avoid Pneumocystis jiroveci pneumonia in small vessel vasculitis patients with generalised disease receiving therapy for induction of remission. PMID:19886977

  11. Management of Large-Vessel Vasculitis With FDG-PET

    PubMed Central

    Soussan, Michael; Nicolas, Patrick; Schramm, Catherine; Katsahian, Sandrine; Pop, Gabriel; Fain, Olivier; Mekinian, Arsene

    2015-01-01

    Abstract We aimed to clarify the role of 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) in the management of large-vessel vasculitis (LVV), focusing on 3 issues which are as follows: describe and determine the different FDG-PET criteria for the diagnosis of vascular inflammation, the performance of FDG-PET for the diagnosis of large-vessel inflammation in giant cell arteritis (GCA) patients, and the performance of FDG-PET to evaluate the disease inflammatory activity in Takayasu arteritis (TA) patients. MEDLINE, Cochrane Library, and EMBASE database were searched for articles that evaluated the value of FDG-PET in LVV, from January 2000 to December 2013. Inclusion criteria were American College of Rheumatology criteria for GCA or TA, definition PET positivity threshold, and >4 cases included. Sensitivity (Se) and specificity (Sp) of FDG-PET for the diagnosis of large-vessel inflammation were calculated from each included individual study, and then pooled for meta-analysis with a random-effects model. Twenty-one studies (413 patients, 299 controls) were included in the systematic review. FDG-PET showed FDG vascular uptake in 70% (288/413) of patients and 7% (22/299) of controls. Only vascular uptake equal to or higher than the liver uptake was significantly different between GCA/TA patients and controls (P?

  12. Cocaine-Induced Delayed Recurrent Vasculitis: A 4-Year Follow-Up

    PubMed Central

    Yogarajah, Meera; Pervil-Ulysse, Mona; Sivasambu, Bhradeev

    2015-01-01

    Patient: Female, 51 Final Diagnosis: Cocaine induced vasculitis Symptoms: — Medication: — Clinical Procedure: None Specialty: Rheumatology Objective: Rare disease Background: Cocaine is a highly abused substance in United States with almost 70 % of cocaine adulterated with levamisole. It is known to cause vasculitis involving multiple organs due to its direct toxic effect and by the contribution of levamisole or a combined effect of both. Case Report: A 51-year-old woman complained of painful erythematous rash in her hands and lower extremities that started few hours after smoking cocaine and progressed to blistering dark lesions in her lower extremities. She denied any other systemic complaints. Although she has been smoking cocaine for more than 35 years, these skin eruptions started only 4 years ago. Examination revealed tender retiform purpura in the hand and tender retiform purpura with hemorrhagic bulla in the legs. Initially, she had only a significantly positive atypical p-ANCA and later developed combined positivity of both Myeloperoxidase (MPO) and Anti-proteinase-3(PR3) antibodies with a p-ANCA pattern on immunofluorescence. We report a unique case of cocaine (likely contaminated with levamisole)-induced delayed recurrent vasculitis with varying vasculitic antibodies over the years. Conclusions: This case highlights the fact that patients can develop cocaine-related vasculitis after many years of uneventful abuse. Cocaine, with its adulterant levamisole, has the propensity to trigger diverse immunological reactions, which is evident by the varying antibody profile seen in the same patient over time. PMID:26003170

  13. [Acute abdomen due to mesenteric vasculitis as a presentation form of systemic lupus erythematosus].

    PubMed

    Palma, S; Arellano, R; Melkonian, E; Guzmán, L

    1996-01-01

    We report a previously healthy 24 years old woman, that presented with an acute abdomen due to a mesenteric vasculitis. The diagnostic study demonstrated the presence of a systemic lupus erythematosus with a type IV nephropathy. Posterior to intestinal resection and immunosuppressive therapy, the patient had a satisfactory evolution. PMID:8762624

  14. Toxoplasmosis associated with digital vasculitis and immunodeficiency--a dilemma in diagnosis.

    PubMed Central

    Ellis, M. E.; Webb, A. K.; Dunbar, E. M.

    1984-01-01

    We present a case of acquired toxoplasmosis associated with severe digital vasculitis--a previously unreported finding. The rise in Toxoplasma gondii antibody titres may have been attenuated by impaired cellular immunity, and this poses a problem for diagnosis. PMID:6483704

  15. [A case of ANCA-associated vasculitis with esophageal stenosis and pseudodiverticulosis].

    PubMed

    Suzuki, Soh; Ohishi, Manabu; Katayama, Norimasa; Koizumi, Hiroshi; Namiki, Masao

    2015-01-01

    A previously healthy 67-year-old woman visited our institution because of cough that had persisted for 3 months, fever at night, left tinnitus, and hearing loss from 1 month prior. She lost 2 kg in weight over the last 6 months, and her C-reactive protein level and leukocyte count were increased. Computed tomography revealed multiple small nodules in both lung fields. She was admitted to our hospital for further examination to determine the optimal medical treatment. Because the myeloperoxidase antineutrophil cytoplasmic antibody (ANCA) titer was 132.5 U/mL and microscopic hematuria was observed, vasculitis was considered. A percutaneous kidney biopsy was performed and revealed microscopic polyangiitis. For this reason, the case was diagnosed as ANCA-associated vasculitis. Esophageal stenosis and pseudodiverticulosis were observed on upper gastrointestinal endoscopy. Pathological changes due to inflammation were observed in the biopsy and esophagography. Pulse therapy with methylprednisolone at 1,000 mg/day was administered to the patient as the first-line treatment, followed by prednisolone at 40 mg/day as remission induction treatment. Bloody urine and lung shadows disappeared, and the steroid dose was tapered at hospital discharge. The patient's hearing ability and the esophageal pathological change that was considered to be ANCA-associated vasculitis were improved. Although a few studies have reported that esophageal stenosis and pseudodiverticulosis accompany ANCA-associated vasculitis, we think that examination for the possible presence of gastrointestinal tract lesions is important. PMID:26625471

  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. Systemic antineutrophil cytoplasmic antibody vasculitis in a patient with chronic lymphocytic leukemia: quite a rare diagnosis.

    PubMed

    Pamuk, Gülsüm Emel; Uyanik, Mehmet Sevki; Demir, Muzaffer; Tekgündüz, Emre; Turgut, Burhan; Soy, Mehmet

    2007-08-01

    There might be rheumatic manifestations of malignant diseases, especially those of the hematological type. Until now, antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis in chronic lymphocytic leukemia (CLL) has been reported on only very few occasions. Here, we present our patient with Rai stage II CLL who came to us with constitutional symptoms. She turned out to have hematuria with dysmorphic erythrocytes and developed hemoptysis. She had pulmonary-renal syndrome and was diagnosed with p-ANCA positive microscopic polyangiitis. She is currently using prednisolone and cyclophosphamide and undergoing regular hemodialysis. Constitutional symptoms in patients with hematological malignancies should make the physicians consider systemic vasculitis after exclusion of disease-related complications. PMID:17010431

  18. Indomethacin-Related Leukocytoclastic Vasculitis: A Case Report and Review of Literature

    PubMed Central

    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. PMID:23525826

  19. [Large vessel involvement in ANCA-associated vasculitis: Report of one case].

    PubMed

    Vega, Jorge; Guarda, Francisco J

    2015-09-01

    Pauci-immune glomerulonephritis in systemic vasculitides usually have anti-neuthrophil cytoplasmic antibodies (ANCA). However, vasculitides of large vessels such as Takayasu's and giant cell (temporal) arteritis do not. Exceptionally ANCA(+) small vessel vasculitides are associated with large vessel vasculitis. It may be a coincidence or both vasculitides have a common pathogenesis. We report a 30 years old woman on hemodialysis due to a chronic glomerulonephritis ANCA(+) diagnosed nine years ago. Eight years later, she presented with an aortitis with severe stenosis of distal aorta and vasculitis of left subclavian artery. She was treated with adrenal steroids and cyclophosphamide. During the ensuing five years she has been stable and without signs of reactivation of the disease. PMID:26530205

  20. 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. PMID:25381429

  1. Letrozole-induced necrotising leukocytoclastic small vessel vasculitis: First report of a case in the UK

    PubMed Central

    Pathmarajah, Pirunthan; Shah, Karishma; Taghipour, Kathy; Ramachandra, Su; Thorat, Mangesh A; Chaudhry, Ziaullah; Patkar, Vivek; Peters, Francesca; Connor, Thomas; Spurrell, Emma; Tobias, Jeffrey S; Vaidya, Jayant S

    2015-01-01

    Introduction Letrozole, an aromatase inhibitor, is a commonly used neo-adjuvant drug to treat hormone-sensitive breast cancer. There have been a few cases of aromatase inhibitor induced vasculitis but the first case of letrozole-induced vasculitis was reported from Switzerland in 2014 (Digklia et al.) [1]. Presentation of case We report the case of a 72-year-old woman with a small breast cancer. She was started on pre-operative letrozole (2.5 mg/d) whilst awaiting surgery. Ten days later she presented with burning pain and purpuric skin lesions which progressed to extensive ischaemic superficial necrosis of the lower limb skin, resolving over 3–4 months after local and systemic steroids. Histologically, it showed leucocytoclasis with evidence of eosinophilia consistent with a diagnosis of cutaneous leukocytoclastic small vessel vasculitis. Discussion The initial clinical presentation was severe burning pain around the ankles and a spreading violaceous rash. Letrozole was stopped. Wide local excision (lumpectomy) and sentinel node biopsy were postponed because of the accompanying pneumonitis and gastrointestinal upset, and were carried out 3.5 months later. Fortunately, the tumour size did not increase, but appeared to reduce, and axillary lymph nodes remained negative, i.e., this patient’s cancer outcome does not seem to have been jeopardized. Conclusion Leukocytoclastic vasculitis is a hypersensitivity reaction that is usually self-resolving, though our case needed systemic steroid treatment. Letrozole is a commonly used drug in clinical practice and prescribers should be aware of this rare side effect, which in our case delayed treatment without any apparent harm and possibly reduced tumour size. PMID:26432999

  2. Animal models of anti-neutrophil cytoplasmic antibody-associated vasculitis

    PubMed Central

    Coughlan, A M; Freeley, S J; Robson, M G

    2012-01-01

    OTHER ARTICLES PUBLISHED ON ANCA IN THIS ISSUE How anti-neutrophil cytoplasmic autoantibodies activate neutrophils. Clinical and Experimental Immunology 2012, 169: 220–8. Antibodies against neutrophil proteins myeloperoxidase (MPO) and proteinase 3 are thought to cause disease in anti-neutrophil cytoplasmic antibody (ANCA) vasculitis. There have been a number of recent developments in the animal models of ANCA vasculitis in both mice and rats. These include models based on an immune response to MPO generated in MPO-deficient mice, with other models using MPO-sufficient mice and rats. In addition, there is a report of the use of humanized mice where immunodeficient mice have been engrafted with human haematopoietic stem cells and injected with patient ANCA. Antibodies to another protein lysosomal-associated protein-2 have been found in patients with ANCA vasculitis, and evidence from a rat model suggests that they are also pathogenic. These models all have advantages and disadvantages, which are discussed. We also consider what these models have taught us about the pathogenesis of ANCA vasculitis. Experiments using genetically modified mice and pharmacological inhibition have given insights into disease mechanisms and have identified potential therapeutic targets. Toll-like receptor stimulation modifies disease by acting both at the level of tissue injury and in the generation of the autoimmune response. Complement is also potentially important with data to support the role of the alternative pathway and C5a in particular. Intracellular pathways have been examined, with a role showing p38 mitogen-activated protein kinase and phosphatidylinositol 3-kinase ?. Serine proteases are now known to contribute to disease by release of interleukin-1? in ANCA-activated neutrophils and monocytes. Other potential therapies studied in these models include the use of bortezemib and strategies to modify antibody glycosylation. PMID:22861362

  3. Comparability of patients with ANCA-associated vasculitis enrolled in clinical trials or in observational cohorts

    PubMed Central

    Pagnoux, Christian; Carette, Simon; Khalidi, Nader A.; Walsh, Michael; Hiemstra, Thomas F.; Cuthbertson, David; Langford, Carol; Hoffman, Gary S.; Koening, Curry L.; Monach, Paul A.; Moreland, Larry; Mouthon, Luc; Seo, Phil; Specks, Ulrich; Ytterberg, Steven; Westman, Kerstin; Hoglund, Peter; Harper, Lorraine; Flossmann, Oliver; Luqmani, Raashid; Savage, Caroline; Rasmussen, Niels; de Groot, Kirstin; Tesar, Vladimir; Jayne, David; Merkel, Pater A.; Guillevin, Loic

    2015-01-01

    Objective To analyse the differences between patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) entered into randomised clinical trials (RCTs) and those followed in large observational cohorts. Methods The main characteristics and outcomes of patients with generalised and/or severe GPA or MPA with a five-factor score ?1 enrolled in the French Vasculitis Study Group (FVSG) or the US-Canadian-based Vasculitis Clinical Research Consortium cohorts were compared to those enrolled in one of 2 FVSG clinical RCTs (WEG91, WEGENT) or 3 European Vasculitis Society clinical trials (CYCLOPS, CYCAZAREM, IMPROVE). Results 657 patients (65.3% with GPA) in RCTs were compared to 437 in cohorts (90.6% with GPA). RCT patients were older at diagnosis than the cohort patients (56.6±13.9 vs. 46.8±17.3 years), had higher Birmingham vasculitis activity score (19.5±9.1 vs. 16.9±7.4), and more frequent kidney disease (84.0% vs. 54.9%) but fewer ear, nose, and throat symptoms (56.8% vs. 72.2%). At 56 months post-diagnosis, mortality and relapse rates, adjusted for age and renal function, were higher for patients with GPA in RCTs vs. cohorts (10.7% vs. 2.5% [p=0.001] and 22.5% vs. 15.6% [p=0.03], respectively) but similar for patients with MPA (6.2% vs. 6.6% [p=0.92] and 16.6% vs. 10.1% [p=0.39], respectively). Conclusion Patients with GPA or MPA in RCTs and those in observational cohorts show important differences that should be remembered when interpreting results based on these study populations. PMID:26016754

  4. Risks of treatments and long-term outcomes of systemic ANCA-associated vasculitis.

    PubMed

    Flossmann, Oliver

    2015-06-01

    Patients with ANCA-associated vasculitis (AAV) have an increased risk of premature death and organ failure. Treatment strategies with immune-suppressive drugs need to balance disease control and prevention of relapse against the risk of side effects in particular infection and malignancy. The longer-term outcome of patients with AAV who participated in several randomised controlled trials has been published in recent years. The results of these and other newer studies will be the focus of this review. PMID:26009242

  5. Ureteric vasculitis, an unusual presentation of polyarteritis nodosa: a case report.

    PubMed

    Jois, Ramesh; Gupta, Anant; Krishnamurthy, Santosh

    2015-06-01

    A 22 year old female presented with vasculitic skin rash. She was incidentally found to be hypertensive and had proteinuria. Skin biopsy showed leuco-cytoclastic vasculitis. On Imaging, there was left hydronephrosis, hydroureter with bilateral multiple foci of ureteric stenosis and micro-aneurysms in relation to interlobar arteries of kidney. A diagnosis of classical Polyarteritis Nodosa (cPAN) with multi-level ureteric stenosis was made. She was treated with Glucocorticoids, Cyclophosphamide, following which Azathioprine was given. PMID:25925589

  6. Duodenal hemorrhage and dermal vasculitis associated with mixed connective tissue disease.

    PubMed

    Hirose, W; Nakane, H; Misumi, J; Sugi, T; Hara, M; Kawagoe, M; Nakamura, H

    1993-01-01

    We describe a 23-year-old woman with mixed connective tissue disease (MCTD) who developed duodenal bleeding responsive to treatment with corticosteroid and recurrent skin eruptions due to leukocytoclastic vasculitis. The endoscopic findings revealed patchy, reddened, edematous, friable mucosa with scattered small ulcers and oozing of blood in the second portion of the duodenum. Endoscopic pictures from the onset to healing are presented. This appears to be the first recorded occurrence of such findings in MCTD. PMID:8441150

  7. Kidney lesions associated with systemic vasculitis: report of 25 cases in morocco.

    PubMed

    Abdou, N; El Mustapha, F; Ghislaine, M; Khalid, Z; Benyounes, R; Mohamed, B G; Khadija, H; Squalli, S; Driss, Z

    2001-01-01

    In this retrospective study, we report 25 patients with renal injury caused by systemic vasculitis. These patients were hospitalized at the department of nephrology-hemodialysis in Ibn Rochd hospital from 1985 to 1998. The mean age of the patients was 36 years (range 3 to 57 years) with male predominance (68%). The clinical presentations included cutaneous purpura in 80% of the patients, arthritis in 60% and pulmonary hemorrhage in 20%. The renal injury manifested as abnormal urinary sediment in 20 of the study patients (80%), nephritic syndrome in 14 (56%), renal failure in nine (36%). Three patients (12%) had renal failure as the initial presentation followed by the other manifestations of vasculitis. The anti-neutrophil cytoplasmic antibodies (ANCA) have been checked in only six patients of whom four were positive. Henoch-Schonlein purpura was the most common clinical sign in 11 patients (44%) followed by Behcet's disease in five (20%). The treatment was variable in type and duration but generally included corticoids alone or in combination with immunosuppressants. The prognosis of the renal injury was variable. The renal function remained stable in those with Henoch-Schonlein purpura, but was less favorable with the other etiologies with more predominance of renal failure and end-stage renal disease. This retrospective analysis of our experience is given to throw light on the pattern of vasculitis in our region. PMID:18209403

  8. Increased serum levels of soluble vascular endothelial-cadherin in patients with systemic vasculitis.

    PubMed

    Chen, Tao; Guo, Zai-Pei; Cao, Na; Qin, Sha; Li, Meng-Meng; Jia, Rui-Zhen

    2014-08-01

    Henoch-Schönlein purpura (HSP) is a commonest systemic vasculitis (SV) in childhood characterized by an inflammatory reaction directed at vessels. Endothelial damage and perivascular leukocyte infiltrates are vital in the development of HSP. Vascular endothelial (VE)-cadherin is an endothelial cell-specific adhesion molecule, which plays critical roles in angiogenesis and endothelial integrity. Herein, we investigated the serum levels of soluble VE-cadherin (sVE-cadherin) in patients with HSP and other forms of SV. The serum levels of sVE-cadherin in 30 patients with HSP, together with patients with urticarial vasculitis, allergic vasculitis, Behcet disease, psoriasis vulgaris (PV) and atopic dermatitis (AD) and 26 health controls were measured by enzyme-linked immunosorbent assay. Serum levels of sVE-cadherin were significantly increased in patients with HSP in acute stage and patients with other forms of SV but not in patients with PV or AD. Moreover, Serum sVE-cadherin levels in HSP patients were correlated with the severity of this disease and serum concentrations of IgA anticardiolipin antibodies and vascular endothelial growth factor. Taken together, we show firstly that serum sVE-cadherin is abnormally increased in HSP patients. Increased serum levels of sVE-cadherin might be a novel biomarker for evaluating the severity of HSP and useful for identifying the presence of SV in inflammatory skin conditions. PMID:24469639

  9. Immunomodulation with eicosapentaenoic acid supports the treatment of autoimmune small-vessel vasculitis

    PubMed Central

    Hirahashi, Junichi; Kawahata, Kimito; Arita, Makoto; Iwamoto, Ryo; Hishikawa, Keiichi; Honda, Mie; Hamasaki, Yoshifumi; Tanaka, Mototsugu; Okubo, Koshu; Kurosawa, Miho; Takase, Osamu; Nakakuki, Masanori; Saiga, Kan; Suzuki, Kazuo; Kawachi, Shoji; Tojo, Akihiro; Seki, George; Marumo, Takeshi; Hayashi, Matsuhiko; Fujita, Toshiro

    2014-01-01

    Small-vessel vasculitis is a life-threatening autoimmune disease that is frequently associated with anti-neutrophil cytoplasmic antibodies (ANCAs). Conventional immunotherapy including steroids and cyclophosphamide can cause serious adverse events, limiting the efficacy and safety of treatment. Eicosapentaenoic acid (EPA), a key component of fish oil, is an omega-3 polyunsaturated fatty acid widely known to be cardioprotective and beneficial for vascular function. We report two elderly patients with systemic ANCA-associated vasculitis (AAV) in whom the administration of EPA in concert with steroids safely induced and maintained remission, without the use of additioal immunosuppressants. To explore the mechanisms by which EPA enhances the treatment of AAV, we employed SCG/Kj mice as a spontaneous murine model of AAV. Dietary enrichment with EPA significantly delayed the onset of crescentic glomerulonephritis and prolonged the overall survival. EPA-derived anti-inflammatory lipid mediators and their precursors were present in the kidney, plasma, spleen, and lungs in the EPA-treated mice. Furthermore, a decrease in ANCA production and CD4/CD8-double negative T cells, and an increase in Foxp3+ regulatory T cells in the lymph nodes of the kidney were observed in the EPA-treated mice. These clinical and experimental observations suggest that EPA can safely support and augment conventional therapy for treating autoimmune small-vessel vasculitis. PMID:25230773

  10. 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. PMID:26195577

  11. Circulating Markers of Vascular Injury and Angiogenesis in ANCA-Associated Vasculitis

    PubMed Central

    Monach, Paul A; Tomasson, Gunnar; Specks, Ulrich; Stone, John H; Cuthbertson, David; Krischer, Jeffrey; Ding, Linna; Fervenza, Fernando C; Fessler, Barri J; Hoffman, Gary S; Ikle, David; Kallenberg, Cees GM; Langford, Carol A; Mueller, Mark; Seo, Philip; St.Clair, E William; Spiera, Robert; Tchao, Nadia; Ytterberg, Steven R; Gu, Yi-Zhong; Snyder, Ronald D; Merkel, Peter A

    2011-01-01

    Objective To identify biomarkers that distinguish between active ANCA-associated vasculitis (AAV) and remission in a manner superior or complementary to established markers of systemic inflammation. Methods Markers of vascular injury and angiogenesis were measured before and after treatment in a large clinical trial in AAV. 163 subjects enrolled in the Rituximab in ANCA-Associated Vasculitis (RAVE) trial were studied. Serum levels of E-selectin, ICAM-3, MMP1, MMP3, MMP9, P-selectin, thrombomodulin, and VEGF were measured at study screening (time of active disease) and at month 6. ESR and CRP levels had been measured at the time of the clinical visit. The primary outcome was the difference in marker level between screening and month 6 among patients in remission (BVAS/WG score of 0) at month 6. Results All subjects had severe active vasculitis (mean BVAS/WG score 8.6 +/? 3.2 SD) at screening. Among the 123 subjects clinically in remission at month 6, levels of all markers except E-selectin showed significant declines. MMP3 levels were also higher among the 23 subjects with active disease at month 6 than among the 123 subjects in remission. MMP3 levels correlated weakly with ESR and CRP. Conclusion Many markers of vascular injury and angiogenesis are elevated in severe active AAV and decline with treatment, but MMP3 appears to distinguish active AAV from remission better than the other markers studied. Further study of MMP3 is warranted to determine its clinical utility in combination with conventional markers of inflammation and ANCA titers. PMID:21953143

  12. Dysregulation of autoantigen genes in ANCA-associated vasculitis involves alternative transcripts and new protein synthesis.

    PubMed

    McInnis, Elizabeth A; Badhwar, Anshul K; Muthigi, Akhil; Lardinois, Olivier M; Allred, S Colby; Yang, Jiajin; Free, Meghan E; Jennette, J Charles; Preston, Gloria A; Falk, Ronald J; Ciavatta, Dominic J

    2015-02-01

    Proteinase 3 (PR3) and myeloperoxidase (MPO) are two major autoantigens in patients with vasculitis with ANCA. The genes encoding these autoantigens are abnormally expressed in peripheral granulocytes of patients with active ANCA-associated vasculitis. This study provides evidence that this transcriptional dysregulation results in a variety of mRNA processing events from the PRTN3 gene locus. In addition to elevated levels of PR3 message, leukocyte RNA from patients contained PR3 transcripts with an alternative 3' untranslated region. Furthermore, we detected usage of an alternative transcription start site within intron 1 of the PRTN3 gene locus that coincided with active disease (odds ratio, 3.3; 95% confidence interval, 1.3 to 8.4; P=0.01). This promoter may be developmentally regulated, because it was active in normal human bone marrow, multiple leukemia cell lines, MCF-7 cells, and subjects after GM-CSF treatment but not subjects with a neutrophil left shift. This transcript, which lacks exon 1 of PRTN3, encodes a 24-kD protein (p24(PR3/MBN)) with a sequence similar to that previously described for myeloblastin. Notably, PR3, p24(PR3/MBN), and MPO were synthesized in cultured neutrophils from patients with active ANCA-associated vasculitis, indicating that increased transcription results in newly synthesized autoantigens in peripheral neutrophils of patients. The synthesis of p24(PR3/MBN) seems to expand the autoantigen repertoire, because immunoblots showed that sera from patients recognized p24(PR3/MBN). These findings emphasize the importance of transcriptional dysregulation of the autoantigen in autoimmune disease. PMID:25060059

  13. Mycobacterium tuberculosis Infection Is Associated with the Development of Erythema Nodosum and Nodular Vasculitis

    PubMed Central

    Chen, Lianjun; Zhang, Qiao’an; Luo, Xiaoqun; Zhang, Wenhong

    2013-01-01

    Background Mycobacterium tuberculosis (MTB) infection has been suggested to contribute to the pathogenesis of erythema nodosum (EN) and nodular vasculitis (NV), the classic forms of panniculitis. However, there is little evidence to demonstrate the presence of MTB in the skin lesions. This study is aimed at evaluating the association between MTB infection and the development of EN and NV in a Chinese population. Methods A total of 107 patients (36 EN, 27 NV, and 44 others) with vasculitis and 40 control cases with other skin diseases were recruited and their skin lesion samples were subjected to real time polymerase chain reaction (PCR) analysis of the IS6110 and mpt64 gene fragments of MTB. Their blood mononuclear cells were tested for MTB antigen-specific IFN-? responses by QuantiFERON®-TB Gold In-Tube (IT) assays. Results PCR analysis revealed that 7/23 (30.4%) and 7/18 (38.9%) of the EN and NV samples were positive for the IS6110 DNA, respectively, which were significantly higher than 3/34 (8.8%) of other vasculitis (OV) and 3/40 (7.5%) of the control samples (p<0.05). The nested Real-Time PCR assay indicated that 6/7 (86%) of the IS6110-positive EN samples, all of the IS6110-positive NV and control samples, but only 1/3 of the IS6110-positive OV samples, were positive for the mpt64 gene. Similarly, 19/32 (59.4%) of the EN patients, 20/26 (76.9%) of the NV patients, and 17/36 (47.2%) of the OV patients were positive for MTB antigen-specific IFN-? responses, which were significantly higher than 6/40 (15%) of the controls (p<0.05). Conclusion Our data strongly suggest that MTB infection and active TB are associated with the development of NV and EN in Chinese. PMID:23650522

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

    PubMed Central

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

    2013-01-01

    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. PMID:23960149

  15. Bortezomib in type I cryoglobulinemic vasculitis: are we acting too late?

    PubMed

    Ramirez, Giuseppe A; Campochiaro, Corrado; Salmaggi, Chiara; Pagliula, Gaia; D'Aliberti, Teresa; Marcatti, Magda; Tresoldi, Moreno; Praderio, Luisa

    2015-01-01

    Type II and type III cryoglobulinemic vasculitis (CV) are characterized by a deranged immune function due to concomitant chronic infections or rheumatic disorders. Conversely, type I CV is caused by plasma cell dyscrasia. Bortezomib is a proteasome inhibitor that is largely employed as a first-line treatment for multiple myeloma. The use of bortezomib in cases of monoclonal gammopathy of undetermined significance (MGUS)-related refractory type I CV has been reported in only four patients. In the current report, we discuss the efficacy of bortezomib treatment in a patient with type I CV, with a focus on the suitability and early application of this drug. PMID:25948361

  16. Adenocarcinoma of the seminal vesicles complicated by antineutrophil cytoplasmic antibody vasculitis: a case report and review of the literature

    PubMed Central

    2013-01-01

    Introduction Adenocarcinoma of the seminal vesicles is a very rare malignancy, with less than 100 cases reported worldwide. It is documented to have a poor prognosis, with the majority of patients developing metastatic disease, most commonly in the prostate, bladder and rectum. Currently there is no standard treatment for metastatic disease and the limited reports of treatment with radiotherapy, chemotherapy and hormonal (anti-androgenic) therapy show that they are generally of modest benefit. The association between malignancy and an increased risk of autoimmune vasculitis has been demonstrated in a number of malignancies, but to date there have been no documented cases of adenocarcinoma of the seminal vesicles associated with anti-neutrophil cytoplasmic antibody vasculitis. Case presentation In this report we describe the case of a 55-year-old Caucasian man with metastatic adenocarcinoma of the seminal vesicles. He previously had received chemotherapy treatment for advanced testicular cancer and later presented with hemospermia. He subsequently developed c-antineutrophil cytoplasmic antibody vasculitis requiring intensive immunosuppression and renal dialysis. Conclusion Adenocarcinoma of the seminal vesicles is a rare diagnosis and our case is more unusual in that our patient previously had chemotherapy treatment for advanced testicular cancer and went on to develop severe antineutrophil cytoplasmic antibody vasculitis when diagnosed with metastatic seminal vesicle cancer. This case illustrates that autoimmune vasculitis can occur in any patient with malignancy and an early referral to the renal team combined with renal biopsy can assist in the earlier diagnosis and more successful management of these rare events. This case should be of interest to oncologists, renal physicians, urologists and general physicians who encounter patients presenting with hemospermia or vasculitis. PMID:23452402

  17. Coronary vasculitis with acute myocardial infarction in a young woman with systemic lupus erythematosus.

    PubMed

    Caracciolo, Eugene A; Marcu, Constantin B; Ghantous, Andre; Donohue, Thomas J; Hutchinson, Gordon

    2004-04-01

    Myocardial infarction in patients with systemic lupus erythematosus (SLE) is most commonly a consequence of atherosclerosis. Coronary vasculitis with aneurysms is a rare cause of myocardial ischemia in SLE. We present a case of a 22-year-old woman with a 4-year history of SLE who was admitted with acute onset of chest pain. Although initially treated for lupus pericarditis, she was subsequently found to have an acute myocardial infarction. Cardiac catheterization revealed multiple areas of aneurysmal coronary dilatation and only moderate stenoses of the secondary branches.In view of the angiographic findings, coronary revascularization was not indicated. Anticoagulant therapy was initiated as a result of the presence of large aneurysmal coronary dilatations, which are predisposed to in situ thrombosis and distal embolization. The coronary vasculitis was treated with immunosuppressive therapy. Measures aimed at secondary prevention of coronary artery disease, including optimization of lipid profile, blood pressure control, and prevention of left ventricular postinfarct remodeling, were initiated and continued indefinitely. PMID:17043468

  18. Dominantly inherited cutaneous small-vessel lymphocytic vasculitis maps to chromosome 6q26-q27.

    PubMed

    Sellick, Gabrielle S; Coleman, Richard J; Webb, Emily L; Chow, Jade; Bevan, Steven; Rosbotham, Jane L; Houlston, Richard S

    2005-10-01

    Outside the context of hereditary deficiencies of complement and IgA, Mendelian inherited predisposition to small vessel lymphocytic vasculitis (SVLV) has rarely been documented. Here we report a large, multigenerational family segregating symmetrical cutaneous SVLV affecting the cheeks, thighs and hands. In all affected family members the disease presented in early infancy and there was no evidence for an association with systemic disease. Skin biopsy of lesions showed a lymphocytic vasculitis with red blood cell extravasation. Complementary studies, with extensive investigation focused on dysfunction of the immunological system were negative. The pattern of inheritance of SVLV in the family was compatible with an autosomal dominantly acting disease gene with incomplete penetrance. To localize the disease causing gene in the family a genome-wide linkage search was conducted using a high-density SNP array. Haplotype construction and analysis of recombination events permitted the minimal interval defining the disease locus to be refined to a 4.7 Mb region on chromosome 6q26-q27. The genes CCR6 and GPR31, which map to the linked region represent plausible candidates for the disease on the basis of their biological function. Extensive screening of both genes by mutational analysis failed to identify a deleterious mutation in the family. PMID:16133183

  19. Moderator's view: Should all patients with ANCA-associated vasculitis be primarily treated with rituximab?

    PubMed

    Tesar, Vladimir

    2015-07-01

    Experience with rituximab in patients with new ANCA-associated vasculitis (AAV) is still very limited, especially in patients with severe (organ- or life-threatening) AAV. Rituximab may be more effective in anti-PR3 AAV, but potentially less effective in some granulomatous manifestations of AAV. We do not know what the response is to rituximab on the tissue level. Rituximab induction needs to be followed by maintenance treatment, and potentially very long rituximab maintenance may result in higher risk of rituximab-related complications (e.g. decrease in IgG levels). Long-term experience with rituximab in AAV is insufficient. Treatment with rituximab is more expensive than the standard treatment with cyclophosphamide and corticosteroids and seems to be cost-effective only in patients primarily treated with cyclophosphamide. Rituximab can be used in some newly diagnosed patients with AAV (e.g. women with child-bearing potential, or patients with active vasculitis and severe infection), but with the available information, it may be too early to use it as a first-line treatment in all new AAV patients. PMID:25999373

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

    PubMed

    Huang, Q; Liu, Y L

    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, ?2-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. PMID:26679670

  1. PF-1355, a mechanism-based myeloperoxidase inhibitor, prevents immune complex vasculitis and anti-glomerular basement membrane glomerulonephritis.

    PubMed

    Zheng, Wei; Warner, Roscoe; Ruggeri, Roger; Su, Chunyan; Cortes, Christian; Skoura, Athanasia; Ward, Jessica; Ahn, Kay; Kalgutkar, Amit; Sun, Dexue; Maurer, Tristan S; Bonin, Paul D; Okerberg, Carlin; Bobrowski, Walter; Kawabe, Thomas; Zhang, Yanwei; Coskran, Timothy; Bell, Sammy; Kapoor, Bhupesh; Johnson, Kent; Buckbinder, Leonard

    2015-05-01

    Small vessel vasculitis is a life-threatening condition and patients typically present with renal and pulmonary injury. Disease pathogenesis is associated with neutrophil accumulation, activation, and oxidative damage, the latter being driven in large part by myeloperoxidase (MPO), which generates hypochlorous acid among other oxidants. MPO has been associated with vasculitis, disseminated vascular inflammation typically involving pulmonary and renal microvasculature and often resulting in critical consequences. MPO contributes to vascular injury by 1) catabolizing nitric oxide, impairing vasomotor function; 2) causing oxidative damage to lipoproteins and endothelial cells, leading to atherosclerosis; and 3) stimulating formation of neutrophil extracellular traps, resulting in vessel occlusion and thrombosis. Here we report a selective 2-thiouracil mechanism-based MPO inhibitor (PF-1355 [2-(6-(2,5-dimethoxyphenyl)-4-oxo-2-thioxo-3,4-dihydropyrimidin-1(2H)-yl)acetamide) and demonstrate that MPO is a critical mediator of vasculitis in mouse disease models. A pharmacokinetic/pharmacodynamic response model of PF-1355 exposure in relation with MPO activity was derived from mouse peritonitis. The contribution of MPO activity to vasculitis was then examined in an immune complex model of pulmonary disease. Oral administration of PF-1355 reduced plasma MPO activity, vascular edema, neutrophil recruitment, and elevated circulating cytokines. In a model of anti-glomerular basement membrane disease, formerly known as Goodpasture disease, albuminuria and chronic renal dysfunction were completely suppressed by PF-1355 treatment. This study shows that MPO activity is critical in driving immune complex vasculitis and provides confidence in testing the hypothesis that MPO inhibition will provide benefit in treating human vasculitic diseases. PMID:25698787

  2. Rheumatoid Vasculitis

    MedlinePLUS

    ... feet) , arteries of the fingers and toes causing digital ischemia, and eyes with scleritis. Scleritis (inflammation of ... complication requires urgent treatment with immunosuppressive medications. Figure: Digital ischemia – this image shows a blood flow deficiency ...

  3. ANCA-Associated Vasculitis in a Patient with Systematic Sclerosis and Sjögren's Syndrome: A Case Report

    PubMed Central

    Kubota, Keiichi; Ueno, Toshiharu; Mise, Koki; Hazue, Ryo; Suwabe, Tatsuya; Kikuchi, Koichi; Hoshino, Junichi; Sumida, Keiichi; Hayami, Noriko; Takaichi, Kenmei; Fujii, Takeshi; Ohashi, Kenichi; Nonomura, Yoshinori; Ubara, Yosifumi

    2015-01-01

    A 65-year-old woman with a limited form of systematic sclerosis (SSc) and Sjögren's syndrome (SS) was admitted to our hospital for the evaluation of renal dysfunction. Her serum creatinine was 1.6 mg/dl, proteinuria was 1.6 g/day, and the urine sediment contained 20–29 erythrocytes/high-power field. Myeloperoxidase anti-neutrophil cytoplasmic antibodies, anti-SS-A/SS-B antibodies and anti-centromere antibodies were positive. A renal biopsy showed focal necrotizing glomerulonephritis with focal interstitial lymphoplasmacytic infiltration. A diagnosis of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) was made. A steroid therapy was initiated and AAV subsided. This is a rare case of AAV in a patient with anti-centromere-positive limited SSc and SS. PMID:26120576

  4. 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. PMID:23082536

  5. Is Levamisole-Induced Vasculitis a Relegated Diagnostic Possibility? A Case Report and Review of Literature

    PubMed Central

    Patnaik, Soumya; Balderia, Percy; Vanchhawng, Lisa; Markazi, Peyman; Wykretowicz, Jedrzej; Perloff, Sarah

    2015-01-01

    Patient: Female, 38 Final Diagnosis: Levamisole induced vasculitis Symptoms: Skin and joint Medication: — Clinical Procedure: None Specialty: Toxicology Objective: Unusual clinical course Background: Levamisole, a veterinary anti-helminthic, is a common adulterant in cocaine. Levamisole-induced vasculopathy (LIV) is a relatively new entity, and is being increasingly recognized since it was first reported in 2010. Although cutaneous findings, agranulocytosis, and positive antineutrophil cytoplasmic antibodies (ANCA) are characteristic, the full clinical picture and appropriate management remain unclear. Case Report: A 38-year-old woman presented with malaise and a pruritic, painful rash on all extremities, right ankle pain, and effusion and necrosis of the right 2nd and 3rd finger tips. After extensive work-up, we determined that she had LIV. Conclusions: Arthritis-dermatitis syndrome in cocaine users should raise suspicion for LIV. Although some features are characteristic, the full clinical spectrum is yet to be described. Management is supportive. PMID:26406869

  6. Combination anti-VEGF and corticosteroid therapy for idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome.

    PubMed

    Sawhney, Gagan K; Payne, John F; Ray, Robin; Mehta, Sonia; Bergstrom, Chris S; Yeh, Steven

    2013-11-01

    Vision loss associated with the idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome most commonly occurs from macular edema or complications related to neovascularization. The authors present a case of advanced IRVAN associated with a massive exudative response characterized by peripheral retinal telangiectasias, exudative retinal detachment, and macular edema with lipid maculopathy. The patient was managed successfully with visual acuity from hand motion to 20/150 using a combination of local corticosteroids, intravitreal bevacizumab, panretinal photocoagulation, and eventually pars plana vitrectomy for progressive vitreomacular traction. VEGF- and non-VEGF-mediated mechanisms appear to be involved in the pathogenesis of IRVAN given the efficacy of combination therapy. [ophthalmic surg lasers imaging retina. 2013;44:599-602.]. PMID:24221466

  7. ANCA-Associated Vasculitis in a Patient with Systematic Sclerosis and Sjögren's Syndrome: A Case Report.

    PubMed

    Kubota, Keiichi; Ueno, Toshiharu; Mise, Koki; Hazue, Ryo; Suwabe, Tatsuya; Kikuchi, Koichi; Hoshino, Junichi; Sumida, Keiichi; Hayami, Noriko; Takaichi, Kenmei; Fujii, Takeshi; Ohashi, Kenichi; Nonomura, Yoshinori; Ubara, Yosifumi

    2015-01-01

    A 65-year-old woman with a limited form of systematic sclerosis (SSc) and Sjögren's syndrome (SS) was admitted to our hospital for the evaluation of renal dysfunction. Her serum creatinine was 1.6 mg/dl, proteinuria was 1.6 g/day, and the urine sediment contained 20-29 erythrocytes/high-power field. Myeloperoxidase anti-neutrophil cytoplasmic antibodies, anti-SS-A/SS-B antibodies and anti-centromere antibodies were positive. A renal biopsy showed focal necrotizing glomerulonephritis with focal interstitial lymphoplasmacytic infiltration. A diagnosis of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) was made. A steroid therapy was initiated and AAV subsided. This is a rare case of AAV in a patient with anti-centromere-positive limited SSc and SS. PMID:26120576

  8. Immune- and ribosome-related genes were associated with systemic vasculitis.

    PubMed

    Gan, S J; Ye, B; Qian, S X; Zhang, C; Mao, J Q; Li, K; Tang, J D

    2015-02-01

    This study aimed to investigate the molecular mechanism of systemic vasculitis via bioinformatics analysis. Gene express profile of E-GEOD-16945 (13 Takayasu arteritis samples and 13 control samples) was downloaded from European Bioinformatics Institute (EBI) database. Differentially expressed genes (DEGs) were screened between Takayasu arteritis and normal controls (|log FC| > 1). Basic local alignment search tool (BLASTX) was used for the Clusters of Orthologous Groups (COG) classification of DEGs. Gene ontology analysis was performed for the DEGs (P < 0.05). A gene expression network was built with DEGs. Mcode in Cytoscape software was used to extract modules from the network (degree ? 2, K-core ? 2 and adjusted P-value < 0.05) followed by pathway analysis using GenMAPP (false discovery rate < 0.05). A total of 747 DEGs were identified. There were 16 significant GO function terms enriched with DEGs, of which immune and defence response was the most significant GO term. Totally, three modules were extracted from gene expression network, including one module constituted with upregulated genes and two modules constituted with downregulated genes. Furthermore, human leucocyte antigen (HLA)-DRB1, HLA-DPA1, HLA-DPB1, HLA-DOA and HLA-DRA in the downregulated modules were significantly linked to immune-related pathways (intestinal immune network for IgA production and systemic lupus erythematosus pathways), while ribosomal protein L 31 (RPL31), RPS3A and RPL9 in the upregulated module were enriched in ribosome pathway. The immune-related pathways, ribosome pathway, immune-related genes including (HLA-DRB1, HLA-DPA1, HLA-DPB1, HLA-DOA and HLA-DRA) and ribosome-related genes (RPL31, RPS3A and RPL9) might be involved in systemic vasculitis. PMID:25410188

  9. Farm Exposure as a Differential Risk Factor in ANCA-Associated Vasculitis

    PubMed Central

    Willeke, P.; Schlüter, B.; Sauerland, C.; Becker, H.; Reuter, S.; Jacobi, A.; Schotte, H.

    2015-01-01

    Objective To investigate the association of farm exposure and the development of ANCA-associated vasculitis (AAV). Methods One hundred eighty-nine well defined patients with AAV (n = 119 with granulomatosis with polyangiitis [GPA], n = 48 with microscopic polyangiitis [MPA], n = 22 patients with eosinophilic granulomatosis with polyangiitis [EGPA]) and 190 controls (n = 119 patients with rheumatoid arthritis, n = 71 with large vessel vasculitis) were interrogated using a structured questionnaire. Factors investigated were occupation, farm exposure, contact to different livestock, participation in harvesting, residence next to a farm, MRSA status, and contact to domestic pets at disease onset or ever before. The odds ratio (OR) and 95% confidence interval [95%CI] were calculated for each item. Results Univariate analysis revealed a strong association of AAV with regular farm exposure; OR 3.44 [95%CI 1.43–8.27]. AAV was also associated with regular contact to cattle 4.30 (1.43–8.27), pigs 2.75 (1.12–6.75) and MRSA carriage 3.38 (1.11–10.3). This association was stronger in the subgroup of GPA patients. OR in this group for farm exposure was 4.97; [2.02–12.2], for cattle 6.71 [95% CI 2.19–20.7], for pigs 4.34 [1.75–10.9], and MRSA carriage 5.06 [1.62–15.8]). There was no significant association of MPA or EGPA with these parameters. Conclusion A significant association between farm exposure or farm animal exposure and AAV especially in the subgroup of patients with GPA has been identified. This suggests that these entities are distinct and have different triggers for the immune process. PMID:26339905

  10. 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

    2015-12-23

    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. PMID:26511022

  11. 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.

  12. Primary central nervous system vasculitis preceded by granulomatous hypophysitis: Case report with a review of the literature

    PubMed Central

    Huang, Meng; Steele, William J.; Baskin, David S.

    2015-01-01

    Background: Primary central nervous system (CNS) vasculitis is an idiopathic inflammatory process that selectively affects CNS vasculature without a systemic inflammatory response, and causes luminal obstruction with resultant ischemia of recipient tissue. Its varying clinical symptoms and signs depend on the caliber of vessels involved and distribution and location of the affected structures. Granulomatous hypophysitis (GH) is an autoimmune inflammatory process typically affecting women, and usually presents with hypopituitarism, and at times, diabetes insipidus, and/or visual loss. Both entities are rare CNS diseases, which, to our knowledge, have never been previously reported in the same patient. Case Description: We present a unique case of chronic progressive primary CNS vasculitis causing limbic encephalopathy in a 30-year-old male with only a history of medication-controlled hypertension. He initially presented 4 months prior with nonspecific neurological complaints and was found to have a homogenously enhancing and enlarged pituitary, which was biopsy proven to be GH. Conclusion: This rather unique presentation highlights the need to maintain a high index of suspicion for underlying PCNS vasculitis in a patient who does not fit the typical demographic for isolated GH. PMID:26539311

  13. Hepatotropic Viral Infection Associated Systemic Vasculitides—Hepatitis B Virus Associated Polyarteritis Nodosa and Hepatitis C Virus Associated Cryoglobulinemic Vasculitis

    PubMed Central

    Sharma, Aman; Sharma, Kusum

    2013-01-01

    Two hepatotropic viruses have been shown to have causal relationship with systemic vasculitis-hepatitis B with classical polyarteritis nodosa and hepatitis C with cryoglobulinemic vasculitis. The present paper provides an updated overview on the clinical presentations and management of these vasculitides. HBV associated PAN patients have higher weight loss, peripheral neuropathy, mononeuritis multiplex, abdominal pain, gastrointestinal manifestations requiring surgery, cardiomyopathy, orchitis, hypertension, and/or elevated transaminase levels. Microaneurysms are also more common in mesenteric artery. Skin manifestations, however are less common. These patients also have a severe disease as suggested by higher five factor score and higher BVAS. Though relapses are less common, mortality is higher in patients with HBV PAN as compared to non HBV PAN. Plasmapheresis has a role in treatment in clearing off immune complexes. The common clinical manifestations of HCV associated cryoglobulinemic vasculitis are skin lesions, peripheral neuropathy, glomerulonephritis, arthritis, and sicca symptoms. Though combination therapy comprising of pegylated interferon ? and ribavirin is the first line of management, immunotherapy is needed for severe or life threatening manifestations. Recent randomized trials have shown the efficacy of rituximab in such situations. PMID:25755502

  14. Peripheral CD5+ B Cells in Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

    PubMed Central

    Unizony, Sebastian; Lim, Noha; Phippard, Deborah J.; Carey, Vincent J.; Miloslavsky, Eli M.; Tchao, Nadia K.; Iklé, David; Asare, Adam L.; Merkel, Peter A.; Monach, Paul A.; Seo, Philip; St Clair, E. William; Langford, Carol A.; Spiera, Robert; Hoffman, Gary S.; Kallenberg, Cees G. M.; Specks, Ulrich; Stone, John H.

    2015-01-01

    Objective CD5+ B cells have been conceptualized as a possible surrogate for Breg cells. The aim of the present study was to determine the utility of CD5+ B cells as biomarkers in antineutrophil cytoplasmic antibody–associated vasculitis (AAV). Methods The absolute and relative numbers (percentages) of CD5+ B cells (explanatory variables) were measured longitudinally during 18 months in 197 patients randomized to receive either rituximab (RTX) or cyclophosphamide (CYC) followed by azathioprine (AZA) for the treatment of AAV (Rituximab in ANCA-Associated Vasculitis [RAVE] trial). Outcome variables included disease activity (status of active disease versus complete remission), responsiveness to induction therapy, disease relapse, disease severity, and, in RTX-treated patients, relapse-free survival according to the percentage of CD5+ B cells detected upon B cell repopulation. Results CD5+ B cell numbers were comparable between the treatment groups at baseline. After an initial decline, absolute CD5+ B cell numbers progressively increased in patients in the RTX treatment arm, but remained low in CYC/AZA-treated patients. In both groups, the percentage of CD5+ B cells increased during remission induction and slowly declined thereafter. During relapse, the percentage of CD5+ B cells correlated inversely with disease activity in RTX-treated patients, but not in patients who received CYC/AZA. No significant association was observed between the numbers of CD5+ B cells and induction treatment failure or disease severity. The dynamics of the CD5+ B cell compartment did not anticipate disease relapse. Following B cell repopulation, the percentage of CD5+ B cells was not predictive of time to flare in RTX-treated patients. Conclusion The percentage of peripheral CD5+ B cells might reflect disease activity in RTX-treated patients. However, sole staining for CD5 as a putative surrogate marker for Breg cells did not identify a subpopulation of B cells with clear potential for meaningful clinical use. Adequate phenotyping of Breg cells is required to further explore the value of these cells as biomarkers in AAV. PMID:25332071

  15. 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 ?mol/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 ?mol/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 ?mol/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 ?mol/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 remission, with only minor persistent dysesthesia of the left foot and a persistent serum creatinine of 133 ?mol/L. PMID:26664125

  16. Genetic Susceptibility to ANCA-Associated Vasculitis: State of the Art

    PubMed Central

    Bonatti, Francesco; Reina, Michele; Neri, Tauro Maria; Martorana, Davide

    2014-01-01

    ANCA-associated vasculitis (AAV) is a group of disorders that is caused by inflammation affecting small blood vessels. Both arteries and veins are affected. AAV includes microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) renamed from Wegener’s granulomatosis, and eosinophilic granulomatosis with polyangiitis (EGPA), renamed from Churg–Strauss syndrome. AAV is primarily due to leukocyte migration and resultant damage. Despite decades of research, the mechanisms behind AAV disease etiology are still not fully understood, although it is clear that genetic and environmental factors are involved. To improve the understanding of the disease, the genetic component has been extensively studied by candidate association studies and two genome-wide association studies. The majority of the identified genetic AAV risk factors are common variants. These have uncovered information that still needs further investigation to clarify its importance. In this review, we summarize and discuss the results of the genetic studies in AAV. We also present the novel approaches to identifying the causal variants in complex susceptibility loci and disease mechanisms. Finally, we discuss the limitations of current methods and the challenges that we still have to face in order to incorporate genomic and epigenomic data into clinical practice. PMID:25452756

  17. Experience with rituximab in the treatment of antineutrophil cytoplasmic antibody associated vasculitis

    PubMed Central

    Clain, Jeremy M.; Cartin-Ceba, Rodrigo; Fervenza, Fernando C.

    2014-01-01

    Prior to the 1970s, severe cases of antineutrophil cytoplasmic antibody associated vasculitis (AAV) were thought to be invariably fatal. However, the use of cyclophosphamide-based treatment regimens fundamentally altered disease outcomes, transforming AAV into a manageable, chronic illness. Despite the tremendous success of cyclophosphamide in the treatment of AAV, there remained a need for alternative therapies, due to high rates of treatment failures and significant toxicities. In recent years, with the introduction of targeted biologic response modifiers into clinical practice, many have hoped that the treatment options for AAV could be expanded. Rituximab, a chimeric monoclonal antibody directed against the B-lymphocyte protein CD20, has been the most successful biologic response modifier to be used in AAV. Following the first report of its use in AAV in 2001, experience with rituximab for treatment of AAV has rapidly expanded. Rituximab, in combination with glucocorticosteroids, is now well established as a safe and effective alternative to cyclophosphamide for remission induction for severe manifestations of granulomatosis with polyangiitis and microscopic polyangiitis. In addition, initial experiences with rituximab for remission maintenance in these diseases have been favorable, as have experiences for remission induction in eosinophilic granulomatosis with polyangiitis. PMID:24688606

  18. Rapidly progressive IgA nephropathy: a form of vasculitis or a complement-mediated disease?

    PubMed Central

    Rojas-Rivera, Jorge; Fernández-Juárez, Gema; Praga, Manuel

    2015-01-01

    A rapidly progressive and crescentic IgA nephropathy (IgAN) is uncommon, but it has a high risk of progression to end-stage renal disease and variable response to immunosuppression. The importance of a positive anti-neutrophil cytoplasmic antibody (ANCA) serology in this group of patients is not fully understood but may have prognostic significance. On the other hand, there is growing evidence of the role of complement in the pathogenesis of IgAN, especially in cases of crescentic IgAN. Therapies directed against the complement system are a potential and rational therapeutic approach. In this issue, two clinical studies of crescentic IgAN are presented. The first work, is a retrospective case–control study describing clinical presentation, histological findings and response to treatment of crescentic IgAN/positive ANCA patients, comparing them with IgAN/negative ANCA patients and ANCA vasculitis patients. The second is a case report showing the effect of eculizumab, a humanized monoclonal antibody that is a terminal cascade complement inhibitor, as salvage therapy for crescentic IgAN resistant to conventional immunosuppression. Both studies broaden our approach to patients with aggressive forms of IgAN. PMID:26413269

  19. Neutrophil extracellular trap formation is associated with autophagy-related signalling in ANCA-associated vasculitis.

    PubMed

    Tang, S; Zhang, Y; Yin, S-W; Gao, X-J; Shi, W-W; Wang, Y; Huang, X; Wang, L; Zou, L-Y; Zhao, J-H; Huang, Y-J; Shan, L-Y; Gounni, A S; Wu, Y-Z; Zhang, J-B

    2015-06-01

    Increasing evidence indicates that aberrant neutrophil extracellular trap (NET) formation could contribute to the pathogenesis of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Recent research has provided evidence that a novel type of ANCA autoantibody, anti-lysosomal membrane protein-2 (LAMP-2) antibody, may have a pathogenic role in AAV. We have shown previously that anti-LAMP-2 antibody-stimulated NET formation contains autoantigens and anti-microbial peptides. The current study sought to determine whether LAMP-2, as a novel antigen of ANCA, was present on NETs in AAV patients, the influence of the anti-LAMP-2 antibody on the neutrophil apoptosis rate and the role of autophagy in anti-LAMP-2 antibody-induced NET formation. NET formation was assessed using immunofluorescence microscopy, scanning electron microscopy or live cell imaging. The neutrophil apoptosis rate was analysed using fluorescence activated cell sorting (FACS). Autophagy was detected using LC3B accumulation and transmission electron microscopy. The results showed that enhanced NET formation, which contains LAMP-2, was observed in kidney biopsies and neutrophils from AAV patients. The apoptosis rate decreased significantly in human neutrophils stimulated with anti-LAMP-2 antibody, and this effect was attenuated by the inhibitors of autophagy 3-methyladenine (3MA) and 2-morpholin-4-yl-8-phenylchromen-4-one (LY294002). The anti-LAMP-2 antibody-stimulated NET formation was unaffected by benzyloxycarbonyl-Val- Ala-Asp (OMe)-fluoromethylketone (zVAD-fmk) and necrostatin-1 (Nec-1), which are inhibitors of apoptosis and necrosis, respectively, but was inhibited by 3MA and LY294002. Moreover, the proportion of LC3BI that was converted to LC3BII increased significantly (P=0.0057), and massive vacuolizations that exhibited characteristics typical of autophagy were detected in neutrophils stimulated with anti-LAMP-2 antibody. Our results provide further evidence that autophagy is involved in ANCA-induced NET formation in human neutrophils. PMID:25644394

  20. Activation-induced cytidine deaminase in B cells of hepatits C virus-related cryoglobulinaemic vasculitis.

    PubMed

    Russi, S; Dammacco, F; Sansonno, S; Pavone, F; Sansonno, D

    2015-12-01

    Immunoglobulin variable region heavy chain (IgVH ) somatic gene diversification is instrumental in the transformation process that characterizes hepatitis C virus (HCV)-related B cell lymphoproliferative disorders. However, the extent to which activation-induced cytidine deaminase (AID), an enzyme essential for IgV gene somatic hypermutation (SHM), is active in cryoglobulinaemic vasculitis (CV) remains unclear. AID mRNA expression in the peripheral blood of 102 chronically hepatitis C virus (HCV)-infected patients (58 with and 44 without CV) and 26 healthy subjects was investigated using real-time reverse transcription-polymerase chain reaction (RT-PCR). The features of activation-induced cytidine deaminase (AID) protein and mRNA transcripts were explored in liver tissue biopsies and portal tracts isolated using laser capture microdissection. In chronically HCV-infected patients, AID mRNA expression was almost threefold higher in those with than in those without CV and sevenfold higher than in healthy subjects (median-fold: 6·68 versus 2·54, P?=?0·03 and versus 0·95, P?=?0·0003). AID transcript levels were significantly higher in polyclonal than in clonally restricted B cell preparations in either CV or non-CV patients (median-fold, 15·0 versus 2·70, P?=?0·009 and 3·46 versus 1·58, P?=?0·02, respectively). AID gene expression was found to be related negatively to age and virological parameters. AID protein was found in portal tracts containing inflammatory cells that, in several instances, expressed AID mRNA transcripts. Our data indicate that the aberrant expression of AID may reflect continuous B cell activation and sustained survival signals in HCV-related CV patients. PMID:26219420

  1. Nomenclature and classification of vasculitis: lessons learned from granulomatosis with polyangiitis (Wegener's granulomatosis)

    PubMed Central

    Jennette, J C

    2011-01-01

    Names influence how something is perceived. Diagnostic terms (diagnoses) are the names of diseases that are usually derived either from some distinctive characteristic of the disease or include an eponym recognizing someone who elucidated the disease. No matter how logical and appropriate a name may be, if it is not usable and used it is of no lasting value. This brief commentary focuses on the nomenclature of systemic vasculitides, and uses as a prime example Wegener's granulomatosis, which has been renamed recently ‘granulomatosis with polyangiitis’, in part because of concerns about the suitability of Friedrich Wegener as the source of an eponym. The most distinctive pathological feature of Wegener's granulomatosis is multi-focal necrotizing inflammation that has long been called granulomatosis. The systemic variant of Wegener's granulomatosis also is characterized by inflammation in many different vessels or different types, i.e. polyangiitis. Thus, granulomatosis with polyangiitis is a very appropriate alternative term for Wegener's granulomatosis. This term also is in accord with the name for a closely related vasculitis, i.e. microscopic polyangiitis. Terms that indicate aetiology and pathogenesis, when known, are useful to include in names for diseases (diagnoses). Anti-neutrophil cytoplasmic autoantibodies specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA) are implicated in the cause of granulomatosis with polyangiitis and thus also should be specified in the diagnosis (e.g. PR3-ANCA-positive granulomatosis with polyangiitis or MPO-ANCA-positive microscopic polyangiitis). As our understanding of the clinical manifestations, pathogenesis and aetiology of vasculitides change over time, the names and approaches for diagnosing these diseases will change accordingly. PMID:21447122

  2. Positron emission tomography scanning in anti-neutrophil cytoplasmic antibodies-associated vasculitis.

    PubMed

    Kemna, Michael J; Vandergheynst, Frédéric; Vöö, Stefan; Blocklet, Didier; Nguyen, Thomas; Timmermans, Sjoerd A M E G; van Paassen, Pieter; Cogan, Elie; van Kroonenburgh, Marinus J P G; Tervaert, Jan Willem Cohen

    2015-05-01

    Tools for evaluation of disease activity in patients with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) include scoring clinical manifestations, determination of biochemical parameters of inflammation, and obtaining tissue biopsies. These tools, however, are sometimes inconclusive. 2-deoxy-2-[F]-fluoro-D-glucose (FDG) positron emission tomography (PET) scans are commonly used to detect inflammatory or malignant lesions. Our objective is to explore the ability of PET scanning to assess the extent of disease activity in patients with AAV.Consecutive PET scans made between December 2006 and March 2014 in Maastricht (MUMC) and between July 2008 and June 2013 in Brussels (EUH) to assess disease activity in patients with AAV were retrospectively included. Scans were re-examined and quantitatively scored using maximum standard uptake values (SUVmax). PET findings were compared with C-reactive protein (CRP) and ANCA positivity at the time of scanning.Forty-four scans were performed in 33 patients during a period of suspected active disease. All but 2 scans showed PET-positive sites, most commonly the nasopharynx (n?=?22) and the lung (n?=?22). Forty-one clinically occult lesions were found, including the thyroid gland (n?=?4 patients), aorta (n?=?8), and bone marrow (n?=?7). The amount of hotspots, but not the highest observed SUVmax value, was higher if CRP levels were elevated. Seventeen follow-up scans were made in 13 patients and showed decreased SUVmax values.FDG PET scans in AAV patients with active disease show positive findings in multiple sites of the body even when biochemical parameters are inconclusive, including sites clinically unsuspected and difficult to assess otherwise. PMID:25997040

  3. Abnormal Expression Pattern of the IL-2 Receptor ?-Chain on CD4+ T Cells in ANCA-Associated Vasculitis

    PubMed Central

    Hoerning, André; Kribben, Andreas; Witzke, Oliver; Dolff, Sebastian

    2014-01-01

    Background/Aim. ANCA-associated vasculitis (AAV) is a small-vessel vasculitis of autoimmune origin. In addition to autoantibodies, T cells have a pivotal pathophysiological role in this disease. T-cell homeostasis and immune tolerance critically depend on IL-2 and its receptor expressed by T cells. In this study, we investigated the IL-2 receptor (IL-2r) expression on CD4+ T cells in AAV. Methods. Thirty patients with AAV and 15 age-matched healthy controls (HC) were enrolled. T cells from peripheral blood were analysed by flow cytometry for expression of the IL-2r ?- and ?-chain. Results. The IL-2r ?-chain was overexpressed in AAV as compared to HC (36 ± 16% versus 20 ± 9%, P < 0.005). The IL-2r-?-chain expression was significantly reduced on CD25+ CD4+ T-cells and CD4+CD25+FoxP3pos regulatory T-cells (Tregs; AAV versus HC: 48 ± 14% versus 62 ± 9%, P = 0.002 and 38 ± 18% versus 68 ± 5%, P = 0.002). Low ?-chain expression in AAV was associated with relapsing disease and systemic vasculitis with renal involvement. Conclusion. The IL-2r expression pattern is abnormal in AAV. To our knowledge, we are the first to show that the ?-chain expression is drastically diminished on T cells in AAV and related to a less favorable disease course. Given the indispensable function of the ?-chain in IL-2 signaling of T cells, diminished expression may contribute to disturbed immune homeostasis in AAV. PMID:24648606

  4. T cells in ANCA-associated vasculitis: what can we learn from lesional versus circulating T cells?

    PubMed Central

    2010-01-01

    Anti-neutrophil cytoplasmic antibody (ANCA) - associated vasculitis (AAV) is a life-threatening autoimmune disease characterized by an antibody-mediated glomerulonephritis and necrotizing vasculitis. Apart from antibodies, T cells are also involved in disease pathogenesis. This review stresses the hallmarks of T cell-mediated pathology in AAV and highlights the characteristics of lesional and circulating T cells in the immune response in AAV. Circulating effector T-cell populations are expanded and are in a persistent state of activation. Circulating regulatory T-cell subsets are less well characterized but seem to be impaired in function. Lesional effector T cells are present in granulomas, vasculitic lesions, and nephritis. Lesional T cells usually show pro-inflammatory properties and promote granuloma formation. Apart from T cells, dendritic cells are abundantly present at the sites of inflammation and locally orchestrate the immune response. Targeting the above-mentioned T cell-mediated disease mechanisms will potentially provide powerful therapeutic tools for AAV. PMID:20236453

  5. Incidence of Cancer in ANCA-Associated Vasculitis: A Meta-Analysis of Observational Studies

    PubMed Central

    Shang, Weifeng; Ning, Yong; Xu, Xiu; Li, Menglan; Guo, Shuiming; Han, Min; Zeng, Rui; Ge, Shuwang; Xu, Gang

    2015-01-01

    Objective The purpose of this paper is to examine cancer incidence in patients with ANCA-associated vasculitis (AASV) derived from population-based cohort studies by means of meta-analysis. Methods Relevant electronic databases were searched for studies characterizing the associated risk of overall malignancy in patients with AASV. Standardized incidence rates (SIRs) with 95% confidence intervals (CIs) were used to evaluate the strength of association. We tested for publication bias and heterogeneity and stratified for site-specific cancers. Results Six studies (n = 2,578) were eventually identified, of which six provided the SIR for overall malignancy, five reported the SIR for non-melanoma skin cancer (NMSC), four for leukemia, five for bladder cancer, three for lymphoma, three for liver cancer, four for lung cancer, three for kidney cancer, four for prostate cancer, four for colon cancer and four for breast cancer. Overall, the pooled SIR of cancer in AASV patients was 1.74 (95%CI = 1.37–2.21), with moderate heterogeneity among these studies (I2 = 65.8%, P = 0.012). In sub-analyses for site-specific cancers, NMSC, leukemia and bladder cancer were more frequently observed in patients with AASV with SIR of 5.18 (95%CI = 3.47–7.73), 4.89 (95%CI = 2.93–8.16) and 3.84 (95%CI = 2.72–5.42) respectively. There was no significant increase in the risk of kidney cancer (SIR = 2.12, 95%CI = 0.66–6.85), prostate cancer (SIR = 1.45, 95%CI = 0.87–2.42), colon cancer (SIR = 1.26, 95%CI = 0.70–2.27), and breast cancer (SIR = 0.95, 95%CI = 0.50–1.79). Among these site-specific cancers, only NMSC showed moderate heterogeneity (I2 = 55.8%, P = 0.06). No publication bias was found by using the Begg’s test and Egger's test. Conclusions This meta-analysis shows that AASV patients treatment with cyclophosphamide (CYC) are at increased risk of late-occurring malignancies, particularly of the NMSC, leukemia and bladder cancer. However, there is no significant association between AASV and kidney cancer, prostate cancer, colon cancer and breast cancer. These findings emphasize monitoring and preventative management in AASV patients after cessation of CYC therapy is momentous. PMID:25973882

  6. 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 cerebral amyloid angiopathy. The annual incidence rate of PCNSV was estimated at 2.4 cases per 1,000,000 person-years. PCNSV appears to consist of several subsets defined by the size of the vessels involved, the clinical characteristics at presentation, MRI findings, and histopathological patterns on biopsy. Early recognition and treatment may reduce poor outcomes. PMID:26020379

  7. Fibroblasts express OvHV-2 capsid protein in vasculitis lesions of American bison (Bison bison) with experimental sheep-associated malignant catarrhal fever

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Sheep-associated malignant catarrhal fever (SA-MCF) caused by ovine herpesvirus-2 (OvHV-2), a '-herpesvirus, is an often fatal disease characterized by lymphoproliferation, vasculitis, and mucosal ulceration in American bison (Bison bison), cattle (Bos taurus), and other clinically susceptible speci...

  8. CD4+ T Cells Sensitized by Vascular Smooth Muscle Induce Vasculitis, and Interferon Gamma Is Critical for the Initiation of Vascular Pathology

    PubMed Central

    Baiu, Dana Carina; Sandor, Matyas; Hart, Michael

    2010-01-01

    Primary vasculitis is the result of idiopathic inflammation in blood vessel walls. T cells are believed to play a critical role, but the nature of the pathological T-cell response remains obscure. In this study, we provide evidence that CD4+ T lymphocytes, activated in the presence of syngeneic vascular smooth muscle cells, were sufficient to induce vasculitic lesions after adoptive transfer to recipient mice. Additionally, the disease is triggered in the absence of antibodies in experiments in which both the donors of stimulated lymphocytes and the transfer recipients were mice that were deficient in B cells. Tracking and proliferation of the transferred cells and their cytokine profiles were assessed by fluorescence tagging and flow cytometry. Proliferating CD4+ T cells were evident 3 days after transfer, corresponding to the occurrence of vasculitic lesions in mouse lungs. The transferred T lymphocytes exhibited Th1 and Th17 cytokine profiles and minimal Th2. However, 1 week after vasculitis induction, effector functions could be successfully recalled in Th1 cells, but not in Th17 cells. Additionally, in the absence of constitutive interferon-? expression, T cells sensitized by vascular smooth muscle cells failed to induce vasculitis. In conclusion, our results show that Th1 cells play a key role in eliciting vasculitis in this murine model and that induction of the disease is possible in the absence of pathogenic antibodies. PMID:20971729

  9. Is there a role for TNF-alpha in anti-neutrophil cytoplasmic antibody-associated vasculitis? Lessons from other chronic inflammatory diseases.

    PubMed

    Feldmann, Marc; Pusey, Charles D

    2006-05-01

    Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is the most common cause of rapidly progressive glomerulonephritis and immune-mediated pulmonary renal syndrome. Now that the acute manifestations of the disease generally can be controlled with immunosuppressive drugs, ANCA-associated vasculitis has become a chronic and relapsing inflammatory disorder. The need to develop safer and more effective treatment has led to great interest in the mediators of chronic inflammation. There are many lessons to be learned from studies of other chronic inflammatory diseases, particularly rheumatoid arthritis (RA). The identification of a TNF-alpha-dependent cytokine cascade in the in vitro cultures of synovium in joints of patients with RA led to studies of TNF blockade in experimental models of arthritis and subsequently to clinical trials. These have culminated in the widespread introduction of anti-TNF therapy not only in RA but also in Crohn disease, ankylosing spondylitis, and several other chronic inflammatory disorders. Following a similar investigative pathway, studies that show the importance of TNF production by leukocytes and intrinsic renal cells in glomerulonephritis have been followed by the demonstration of the effectiveness of TNF blockade in several experimental models of glomerulonephritis and vasculitis. In experimental autoimmune vasculitis, improvement in disease was paralleled by a reduction in leukocyte transmigration, as demonstrated by intravital microscopy. The benefit of infliximab (a mAb to TNF) in ANCA-associated vasculitis was recently reported in a prospective open-label study. However, the use of etanercept (a soluble TNF receptor fusion protein) was not found to be of significant benefit in a randomized, controlled trial in patients with Wegener granulomatosis. Therefore, there is a need for further evaluation of the use of anti-TNF antibodies in patients with ANCA-associated glomerulonephritis. PMID:16624928

  10. 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

  11. Current and emerging treatment options for ANCA-associated vasculitis: potential role of belimumab and other BAFF/APRIL targeting agents

    PubMed Central

    Lenert, Aleksander; Lenert, Petar

    2015-01-01

    Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) comprises several clinical entities with diverse clinical presentations, outcomes, and nonunifying pathogenesis. AAV has a clear potential for relapses, and shows unpredictable response to treatment. Cyclophosphamide-based therapies have remained the hallmark of induction therapy protocols for more than four decades. Recently, B-cell depleting therapy with the anti-CD20 antibody rituximab has proved beneficial in AAV, leading to Food and Drug Administration approval of rituximab in combination with corticosteroids for the treatment of AAV in adults. Rituximab for ANCA-associated vasculitis and other clinical trials provided clear evidence that rituximab was not inferior to cyclophosphamide for remission induction, and rituximab appeared even more beneficial in patients with relapsing disease. This raised hopes that other B-cell-targeted therapies directed either against CD19, CD20, CD22, or B-cell survival factors, B-cell activating factor of the tumor necrosis factor family (BAFF) and a proliferation-inducing ligand could also be beneficial for the management of AAV. BAFF neutralization with the fully humanized monoclonal antibody belimumab has already shown success in human systemic lupus erythematosus and, along with another anti-BAFF reagent blisibimod, is currently undergoing Phase II and III clinical trials in AAV. Local production of BAFF in granulomatous lesions and elevated levels of serum BAFF in AAV provide a rationale for BAFF-targeted therapies not only in AAV but also in other forms of vasculitis such as Behcet’s disease, large-vessel vasculitis, or cryoglobulinemic vasculitis secondary to chronic hepatitis C infection. BAFF-targeted therapies have a very solid safety profile, and may have an additional benefit of preferentially targeting newly arising autoreactive B cells over non-self-reactive B cells. PMID:25609919

  12. 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

  13. Long-Term Follow-Up of Cyclophosphamide Compared with Azathioprine for Initial Maintenance Therapy in ANCA-Associated Vasculitis

    PubMed Central

    Faurschou, Mikkel; Berden, Annelies; Flossmann, Oliver; Bajema, Ingeborg; Hoglund, Peter; Smith, Rona; Szpirt, Wladimir; Westman, Kerstin; Pusey, Charles D.; Jayne, David R.W.

    2014-01-01

    Background and objectives Treatment with azathioprine within 3 months of remission induction with cyclophosphamide is a common treatment strategy for patients with ANCA-associated vasculitis. This study comprised patients undergoing long-term follow-up who were randomly allocated to azathioprine after 3–6 months or after 12 months of cyclophosphamide treatment. Design, setting, participants, & measurements Patients from 39 European centers between 1995 and 1997 with a new diagnosis of ANCA-associated vasculitis that involved the kidneys or another vital organ were eligible. At the time of diagnosis, participants were randomly allocated to convert to azathioprine after 3–6 months (the azathioprine group) or after 12 months of cyclophosphamide (the cyclophosphamide group). Patients who did not achieve a remission within 6 months were excluded. This study assessed relapses, ESRD, and death during long-term follow-up. Results Patients were allocated to the azathioprine group (n=71) and the cyclophosphamide group (n=73). Of these patients, 63 (43.8%) developed a relapse, 35 (24.3%) developed a renal relapse, 13 (9.0%) developed ESRD, and 21 (14.6%) died. Although there were worse outcomes in the azathioprine group, none were statistically significant. The subdistribution hazard ratio [sHR] for relapse was 1.63 (95% confidence interval [95% CI], 0.99 to 2.71), the composite of relapse or death hazard ratio [HR] was 1.59 (95% CI, 1.00 to 2.54), the ESRD sHR was 1.71 (95% CI, 0.56 to 5.19), and the death HR was 0.75 (95% CI, 0.32 to 1.79). Conclusions It remains uncertain whether converting to azathioprine after 3–6 months of induction cyclophosphamide therapy is as effective as converting after 12 months. Outcomes are still poor for this group of patients and further research is required to determine the optimal timing of maintenance therapy. PMID:24970876

  14. Diagnostic Value of Procalcitonin in ANCA-Associated Vasculitis (AAV) to Differentiate Between Disease Activity, Infection and Drug Hypersensitivity

    PubMed Central

    Herrmann, K; Schinke, S; Csernok, E; Moosig, F; Holle, J.U

    2015-01-01

    Objective: Procalcitonin (PCT) is considered to be a specific marker for severe bacterial infections and sepsis. Elevated PCT levels have been reported in active autoimmune diseases without infection. The aim of this study was to assess the diagnostic value of PCT serum levels in ANCA-associated vasculitis (AAV) patients with respect to infection, disease activity and drug fever using a high sensitive PCT detection method. Methods: In 53 AAV patients with elevated C-reactive protein (CRP) PCT was determined by the Thermo Scientific BRAHMS PCT sensitive KRYPTOR assay. Patients underwent standardized diagnostic procedures for evaluation of disease activity and infection. Results: 53 patients with AAV and elevated CRP (7.7±6.9 mg/dl, PCT 0.34±1.02 ng/ml) were assessed, 10 had infection with elevated CRP levels of 11.2±10.2 mg/dl and PCT levels of 1.06±2.07 ng/dl. 43 patients had no evidence of infection, 36 of them were presented with AAV with normal or only slightly positive PCT levels in active disease (n=36) (PCT 0.06±0.06 ng/ml). 7 patients had increased PCT levels due to azathioprine hypersensitivity (0.76±1.01 ng/ml). For discrimination between infection and vasculitis activity PCT was more useful than CRP with the best cut-off at 0.1 ng/ml (sensitivity 60%, specificity 92%). Conclusion: In contrast to previous studies using semiquantitative PCT assays, the KRYPTOR performs better with respect to discrimination of infection from active AAV. In all patients assessed with active AAV (and without infection) PCT levels remained below the PCT reference limit (0.5 ng/ml) for infections. Drug hypersensitivity seems to be an important differential diagnosis in the setting of elevated CRP and PCT in patients who receive azathioprine. PMID:26535070

  15. Persistent fever, neck swelling, and small vessel vasculitis following tonsillectomy in a patient with Behçet’s disease: a case report

    PubMed Central

    2012-01-01

    Introduction Behçet’s disease commonly presents with recurrent oral and genital mucocutaneous ulcerations, uveitis and various skin manifestations. Other clinical symptoms include gastrointestinal ulcerations, arthritis, venous thrombosis, arterial aneurysms and central nervous system affection. Vasculitis underlies most clinical symptoms of Behçet’s disease. Case presentation We report the case of a 62-year-old European Caucasian woman with Behçet’s disease who presented with persistent fever and neck soft-tissue swelling, despite broad antibiotic treatment, two weeks after acute tonsillitis and a tonsillectomy. Diffuse epi- and mesopharyngeal swelling shown on a computed tomography scan of her neck and persistently elevated serum markers of inflammation initially prompted suspicion of an infectious etiology. Magnet resonance imaging of her neck and a neck tissue biopsy finally confirmed small vessel vasculitis involving skin, subcutaneous tissue and muscle. Considering the clinical presentation, past medical history and histological findings, we interpreted our patient’s symptoms as a flare of Behçet’s disease. Immunosuppressive treatment led to rapid clinical improvement. Conclusion A patient with Behçet’s disease developed small vessel vasculitis of the soft tissue of her neck after tonsillitis and a tonsillectomy. Infection and surgery probably triggered a flare of Behçet’s disease. PMID:23110825

  16. TWEAK Enhances E-selectin and ICAM-1 Expression, and May Contribute to the Development of Cutaneous Vasculitis

    PubMed Central

    Chen, Tao; Guo, Zai-pei; Li, Li; Li, Meng-meng; Wang, Ting-ting; Jia, Rui-zhen; Cao, Na; Li, Jing-yi

    2013-01-01

    Our previous work indicated that TWEAK is associated with various types of cutaneous vasculitis (CV). Herein, we investigate the effects of TWEAK on vascular injury and adhesion molecule expression in CV mice. We showed that TWEAK priming in mice induced a local CV. Furthermore, TWEAK priming also increased the extravasation of FITC-BSA, myeloperoxidase activity and the expression of E-selectin and ICAM-1. Conversely, TWEAK blockade ameliorated the LPS-induced vascular damage, leukocyte infiltrates and adhesion molecules expression in LPS-induced CV. In addition, TWEAK treatment of HDMECs up-regulated E-selectin and ICAM-1 expression at both mRNA and protein levels. TWEAK also enhanced the adhesion of PMNs to HDMECs. Finally, western blot data revealed that TWEAK can induce phosphorylation of p38, JNK and ERK in HDMECs. These data suggest that TWEAK acted as an inducer of E-selectin and ICAM-1 expression in CV mice and HDMECs, may contribute to the development of CV. PMID:23457623

  17. Autoantibodies developing to myeloperoxidase and proteinase 3 in systemic vasculitis stimulate neutrophil cytotoxicity toward cultured endothelial cells.

    PubMed Central

    Savage, C. O.; Pottinger, B. E.; Gaskin, G.; Pusey, C. D.; Pearson, J. D.

    1992-01-01

    The ability of vasculitis-associated anti-neutrophil cytoplasm antibodies (ANCA) to activate neutrophils and mediate release of radiolabel from 111Indium-labeled cultured human umbilical vein endothelial cells (HUVEC) was determined as a measure of the potential cytotoxicity of ANCA-activated neutrophils against vascular endothelium. Priming of neutrophils with low doses of phorbol 12-myristate 13-acetate (PMA) (1 ng/ml) and ionomycin (0.1 mumol/1) was required, together with pretreatment of endothelial cells with BCNU (1,3-bis-[2-chloroethyl]-1-nitrosourea; 0.26 mmol/l). Under these conditions and using a 4-hour serum-free assay system, mouse monoclonal antibodies (MAb) to the target autoantigens proteinase-3 (Pr-3) and myeloperoxidase (MPO) mediated enhanced release of 111Indium from HUVEC compared with control MAb. Human IgG Fab2 C-ANCA (recognizing Pr-3) and P-ANCA (recognizing MPO) did likewise. Preactivation of HUVEC with TNF (50 U/ml, 4 hr) enhanced the release of 111Indium from HUVEC generated by neutrophils activated with anti-Pr-3 and anti-MPO MAb. These data support the suggestion that activation of neutrophils by ANCA within the vascular lumen may contribute to endothelial cell injury. PMID:1323218

  18. ANCA-Associated Systemic Vasculitis Presenting With Hypertrophic Spinal Pachymeningitis: A Report of 2 Cases and Review of Literature.

    PubMed

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

    2015-11-01

    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

  19. Hydralazine Induces Myeloperoxidase and Proteinase 3 Anti-Neutrophil Cytoplasmic Antibody Vasculitis and Leads to Pulmonary Renal Syndrome

    PubMed Central

    Sultan, Ghayyath; Werner, Sherry L.; Hura, Claudia

    2014-01-01

    We report a case of hydralazine-induced ANCA-associated glomerulonephritis with pulmonary hemorrhage. A 62-year-old Hispanic man with hypertension, who was being treated with hydralazine 100?mg three times a day for four and half years, presented to the hospital with severe anemia. He had acute kidney injury and urinalysis showed proteinuria, dysmorphic RBCs, and rare RBC cast. CT scan of the chest revealed bilateral pulmonary ground-glass infiltrates. Transbronchial biopsy was consistent with pulmonary hemorrhage. Serologic tests showed high titer PR3 ANCA and, to a lesser extent, MPO ANCA. Kidney biopsy revealed focal segmental necrotizing glomerulonephritis with crescents, without evidence of immune complex deposits. Hydralazine was discontinued and the patient was treated with corticosteroids and intravenous cyclophosphamide. At one-year follow-up, he had no symptoms and anemia had resolved. Kidney function improved dramatically. Serology showed undetectable PR3 ANCA and minimally elevated MPO ANCA. To our knowledge, hydralazine-associated PR3 ANCA has not been previously reported. The possibility of ANCA systemic vasculitis should be included in the differential diagnosis of any patient with hydralazine use and pulmonary renal syndrome. This is a potentially life threatening condition requiring prompt cessation of the drug and treatment with glucocorticoids and immunosuppression. PMID:25210633

  20. Mycophenolate mofetil in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis: a prospective pharmacokinetics and clinical study

    PubMed Central

    Chaigne, B; Gatault, P; Darrouzain, F; Barbet, C; Degenne, D; François, M; Szymanski, P; Rabot, N; Golea, G; Diot, E; Maillot, F; Lebranchu, Y; Nivet, H; Paintaud, G; Halimi, J-M; Guillevin, L; Büchler, M

    2014-01-01

    Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) treatment strategy is based on immunosuppressive agents. Little information is available concerning mycophenolic acid (MPA) and the area under the curve (AUC) in patients treated for AAV. We evaluated the variations in pharmacokinetics for MPA in patients with AAV and the relationship between MPA–AUC and markers of the disease. MPA blood concentrations were measured through the enzyme-multiplied immunotechnique (C0, C30, C1, C2, C3, C4, C6 and C9) to determine the AUC. Eighteen patients were included in the study. The median (range) MPA AUC0–12 was 50·55 (30·9–105·4) mg/h/l. The highest coefficient of determination between MPA AUC and single concentrations was observed with C3 (P?

  1. Mapping of the Outcome Measures in Rheumatology Core Set for Antineutrophil Cytoplasmic Antibody–Associated Vasculitis to the International Classification of Function, Disability and Health

    PubMed Central

    MILMAN, NATALIYA; BOONEN, ANNELIES; MERKEL, PETER A.; TUGWELL, PETER

    2015-01-01

    Objective The International Classification of Functioning, Disability and Health (ICF) is a framework and classification of health that describes health along 4 components: body functions, body structures, activities and participation, and contextual factors. This study examined the content of instruments that constitute the Outcome Measures in Rheumatology (OMERACT) core set of outcome measures for antineutrophil cytoplasmic antibody–associated vasculitis (AAV) by “mapping” them to the ICF. Methods The content of the instruments included in the AAV core set were linked to the ICF by 2 independent investigators according to previously established ICF linkage rules. Results The AAV core set includes 3 measures of disease activity (3 versions of the Birmingham Vasculitis Activity Score), 1 damage measure (Vasculitis Damage Index), 1 patient-reported outcome (Short Form 36 health survey), and death. Linking these instruments to the ICF revealed comprehensive coverage of the ICF components body functions and body structures, limited coverage of the ICF component activities and participation, and complete absence of coverage of contextual factors. Conclusion ICF was found to be useful for thematic characterization of a heterogeneous group of outcome measures for AAV, i.e., a group of complex medical conditions. Linking of the instruments selected for the OMERACT AAV core set of outcome measures to the ICF classification revealed limitations in the representation of constructs related to life impact of AAV, represented by the ICF components activities and participation and contextual factors. Further research and methods development are needed to better incorporate important aspects of functioning and health relevant to patients into clinical trials of AAV. PMID:25048363

  2. Lung involvement at presentation predicts disease activity and permanent organ damage at 6, 12 and 24 months follow?-?up in ANCA?-?associated vasculitis

    PubMed Central

    2014-01-01

    Background Antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV) may present with pulmonary involvement ranging from mild to life-threatening disease such as diffuse alveolar hemorrhage. There is a paucity of information regarding morbidity outcomes for AAV subjects presenting with lung involvement. This study determines the relationship between disease activity and damage in these subjects using the Birmingham Vasculitis Activity Score v 3 (BVAS 3) and Vasculitis Damage Index (VDI) respectively. Results 151 patients with AAV were included with 59 presenting initially with pulmonary involvement. The initial BVAS scores recorded at time of diagnosis were positively correlated with the final VDI scores at 24 months (p?

  3. Clozapine-Induced Late Agranulocytosis and Severe Neutropenia Complicated with Streptococcus pneumonia, Venous Thromboembolism, and Allergic Vasculitis in Treatment-Resistant Female Psychosis

    PubMed Central

    Voulgari, Christina; Giannas, Raphael; Paterakis, Georgios; Kanellou, Anna; Anagnostopoulos, Nikolaos; Pagoni, Stamata

    2015-01-01

    Clozapine is a second-generation antipsychotic agent from the benzodiazepine group indicated for treatment-resistant schizophrenia and other psychotic conditions. Using clozapine earlier on once a case appears to be refractory limits both social and personal morbidity of chronic psychosis. However treatment with second-generation antipsychotics is often complicated by adverse effects. We present a case of a 33-year-old Caucasian woman with a 25-year history of refractory psychotic mania after switching to a 2-year clozapine therapy. She presented clozapine-induced absolute neutropenia, agranulocytosis, which were complicated by Streptococcus pneumonia and sepsis. Clozapine-induced thromboembolism of the common femoral and right proximal iliac vein, as well as allergic vasculitis, was diagnosed. She achieved full remission on granulocyte-colony stimulating factor and specific antibiotic treatment. Early detection of severe clozapine-induced absolute neutropenia and agranulocytosis enabled the effective treatment of two among its most severe complications. Additional evidence to the previously reported possible causal relation between clozapine and venous thromboembolism is offered. Finally, clozapine-induced allergic vasculitis is confirmed as a late adverse effect of clozapine therapy. PMID:25755670

  4. CD8+/perforin+/WC1- gammadelta T cells, CD8+ alphabeta T cells, infiltrate vasculitis lesions of American bison (Bison bison) with experimental sheep-associated malignant catarrhal fever

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Sheep-associated malignant catarrhal fever (SA-MCF) caused by ovine herpesvirus-2 (OvHV-2) is a fatal disease associated with lymphoproliferation, lymphocytic vasculitis, and mucosal ulceration in clinically susceptible species. SA-MCF is an important threat to American bison (Bison bison) due to th...

  5. How Is Vasculitis Diagnosed?

    MedlinePLUS

    ... metabolism leading to problems in the blood vessels. Angiography Angiography (an-jee-OG-ra-fee) is a test ... vessels on the x-ray pictures. Doctors use angiography to help find out whether blood vessels are ...

  6. Living with Vasculitis

    MedlinePLUS

    ... Guidelines Resources Continuing Education Researchers Funding Training & Career Development Division of Intramural Research Research Resources Research Meeting Summaries Technology Transfer Clinical Trials What Are Clinical Trials? Children & Clinical Studies NHLBI Trials Clinical Trial Websites News & ...

  7. Central Nervous System Vasculitis

    MedlinePLUS

    ... cell count) high white blood cell count high platelet count kidney or liver problems allergic reactions finding immune complexes in the circulating blood identifying abnormal antibodies in the blood an increase in blood markers ...

  8. Types of Vasculitis

    MedlinePLUS

    ... usually, but not always, affect the body’s larger blood vessels. Behçet's Disease Behçet's (beh-CHET'S) disease can cause recurrent, ... HLA-B51 gene may play a role in Behçet's disease. However, not everyone who has ... the large blood vessels, especially the aorta and aortic valve. The aorta ...

  9. [A case of Churg-Strauss syndrome in which positive rheumatoid factor and MPO-ANCA preceded the development of clinical symptoms of vasculitis].

    PubMed

    Furuiye, M; Konno, K; Sumi, Y; Ohtani, Y; Sawada, M; Umino, T; Inase, N; Miyake, S; Yoshizawa, Y

    2001-06-01

    A 52-year-old man in whom bronchial asthma had been diagnosed in 1995 was admitted for the treatment of Churg-Strauss syndrome in June 1997. Positive tests MPO-ANCA and rheumatoid factor preceded the symptoms of vasculitis for several months. A skin biopsy revealed infiltration of eosinophils in the vessel walls, and the diagnosis of Churg-Strauss syndrome was confirmed. After systemic administration of corticosteroids, the symptoms other than mononeuritis improved markedly, and his MPO-ANCA and rheumatoid factor became negative. Rheumatoid factor and MPO-ANCA may be useful for the early diagnosis of Churg-Strauss syndrome in patients with bronchial asthma in which a well-controlled disease develops into an intractable condition. PMID:11530389

  10. Induction of remission in active anti?neutrophil cytoplasmic antibody?associated vasculitis with mycophenolate mofetil in patients who cannot be treated with cyclophosphamide

    PubMed Central

    Stassen, Patricia M; Tervaert, Jan Willem Cohen; Stegeman, Coen A

    2007-01-01

    Background Active anti?neutrophil cytoplasmic antibody?associated vasculitis (AAV) is commonly treated with cyclophosphamide, a drug with serious side effects, and with corticosteroids. Objective To determine the efficacy of a possible alternative drug for cyclophosphamide, oral mycophenolate mofetil (MMF) 1000?mg twice daily and oral prednisolone 1?mg/kg once daily as remission induction treatment. Methods 32 consecutive patients with 34 episodes of active vasculitis who could not be treated with cyclophosphamide were diagnosed for a median (range) of 6.0 (0.3–22)?years and experienced 4 (0–14) relapses prior to the current episode. Treatment response and relapse?free survival were analysed. Results Complete remission (CR) was obtained in 25 (78%) patients, partial remission (PR) in 6 (19%), whereas 1 (3%) patient did not respond. 19 patients relapsed, 13 (52%) after CR, 14 (3–58)?months after starting the treatment and 6 (100%) after PR, 6 (2–10)?months after starting the treatment. The median relapse?free survival was 16?months, comparable with the interval between the previous relapse and the current MMF?treated relapse (17 (3–134)?months). Relapse?free survival at 1, 3, and 5?years was 63%, 38% and 27%, respectively. Patients who had been treated successfully with cyclophosphamide before responded better (CR 84%, relapse 50%) than those who had not (CR 50%, relapse 100%). Minor gastrointestinal side effects and infections occurred frequently. MMF was prematurely discontinued due to adverse effects in two patients. Conclusion MMF, in combination with prednisolone, can induce remission in patients with relapses of AAV intolerant to cyclophosphamide. PMID:17179175

  11. Non HCV-related infectious cryoglobulinemia vasculitis: Results from the French nationwide CryoVas survey and systematic review of the literature.

    PubMed

    Terrier, Benjamin; Marie, Isabelle; Lacraz, Adeline; Belenotti, Pauline; Bonnet, Fabrice; Chiche, Laurent; Graffin, Bruno; Hot, Arnaud; Kahn, Jean-Emmanuel; Michel, Catherine; Quemeneur, Thomas; de Saint-Martin, Luc; Hermine, Olivier; Léger, Jean-Marc; Mariette, Xavier; Senet, Patricia; Plaisier, Emmanuelle; Cacoub, Patrice

    2015-12-01

    In patients with infectious cryoglobulinemia vasculitis (CryoVas) in the absence of hepatitis C virus infection, data on presentation, therapeutic management and outcome are lacking. We conducted a nationwide survey that included patients with HCV-negative CryoVas. We describe here the presentation, therapeutic management and outcome of 18 patients with non-HCV infectious CryoVas and 27 additional patients identified form a systematic review of the literature. We included 18 patients, mean age 57.9±13.5 years. Infectious causes were viral infections in 8 patients [hepatitis B virus (HBV) in 4, and cytomegalovirus, Epstein Barr virus, parvovirus B19 and human immunodeficiency virus in one case each], pyogenic bacterial infection in 6 patients, parasitic infection in 2 patients, and leprosy and candidiasis in one case each. Baseline manifestations were purpura (78%), glomerulonephritis (28%), arthralgia (28%), peripheral neuropathy (22%), skin necrosis (22%), cutaneous ulcers (17%), and myalgia (11%). Cryoglobulinemia was type II in 2/3 of cases. Most cases received specific anti-infectious therapy as first-line therapy, sometimes associated with corticosteroids, achieving sustained remission in the majority of cases. Refractory or relapsing patients, frequently related to HBV infection, showed a complete remission after rituximab in addition to antiviral therapy. In contrast, corticosteroids and/or immunosuppressive agents used in the absence of anti-infectious agents were frequently associated with refractory CryoVas. Viral and pyogenic bacterial infections represent the main causes of non-HCV infectious CryoVas. Antimicrobial therapy is commonly associated with sustained remission. Immunosuppressive agents should be considered only as a second-line option in patients with refractory vasculitis. PMID:26320984

  12. [A case of slowly progressive type 1 diabetes mellitus developing myeloperoxidase-specific anti-neutrophil cytoplasmic antibody-associated vasculitis with hypertrophic pachymeningitis manifesting as multiple cranial nerve palsy].

    PubMed

    Kurihara, Yuko; Oku, Kayo; Suzuki, Atsushi; Ohsone, Yasuo; Handa, Michiko; Okano, Yutaka

    2011-01-01

    We report a 63-year-old man with a 35-year history of slowly progressive type 1 diabetes mellitus (SPIDDM), complicated with myeloperoxidase-specific anti-neutrophil cytoplasmic antibody (MPO-ANCA)-associated vasculitis presenting alveolar hemorrhage and pachymeningitis. The patient was first diagnosed as having DM at age of 28 years old and deteriorated secretion of insulin and the typical clinical course led us to the diagnosis of SPIDDM. When he was 58 years old, he suffered from fever, headache, and alveolar hemorrhage. He was diagnosed as having MPO-ANCA associated vasculitis based on a high titer of MPO-ANCA and histological findings of lung biopsy. Treatment with steroid pulse therapy, followed by oral prednisolone and oral cyclophosohamide, resulted in clinical improvement. Five years later, he complained of double vision. A gadolinium-enhanced magnetic resonance imaging (MRI) study of the brain showed normal. Two months later, he developed right cranial nerve V~XII palsy. A second MRI study revealed thickening of the right temporal region and cerebellar dura mater, leading us to the diagnosis of hypertrophic pachymeningitis. He responded well to oral prednisolone (50 mg/day) and intravenous cyclophosohamide (500 mg). This is the first case report of SPIDDM complicated with MPO-ANCA-associated vasculitis, manifesting as alveolar hemorrhage and hypertrophic pachymeningitis. PMID:22214812

  13. Concurrent pulmonary hemorrhage and deep vein thrombosis in a child with ANCA-associated vasculitis: case report and review of literature.

    PubMed

    Tseng, Shi-Ting; Tseng, Min-Hua; Huang, Jing-Long

    2015-01-01

    Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is an uncommon but potentially life threatening disease in children. Pulmonary hemorrhage (PH) is a well recognized but lethal complication. The incidence of venous thromboembolism (VTE) is higher in patients with AAV, especially in those with active disease. However, the simultaneous occurrence of both PH and VTE has rarely been reported. Herein, we describe a 14-year-old female with AAV who developed concomitant deep vein thrombosis (DVT) and PH within 3 days after hospitalization. She was successfully treated with timely plasmapheresis and methylprednisolone pulse therapy. VTE did not occur during discontinuation of anticoagulant. On reviewing the English literature, 5 AAV patients with coexisting VTE and PH have been reported. When faced with PH, whether or not to keep anti-coagulation treatment is a dilemma. Some of the patients kept receiving anti-coagulation treatment, whereas others undergoing inferior vena cava filter implantation. Glucocorticoids and cyclophosphamide or other immunosuppressant agents were prescribed in all patients. All of the cases survived after treatment for concurrent VTE and PH, and received short- or long-term anticoagulation treatment after discharge. To the best of our knowledge, this is the first report of a pediatric patient with AAV presenting with coexistent VTE and PH. VTE should be considered to be a sign of disease flare-up, and early plasmapheresis with immunosuppressant therapy can rescue this fatal complication. PMID:26058459

  14. What Everybody is Doing but No One is Talking About: Use of Complementary and Alternative Medicine in the ANCA Associated Vasculitis Population

    PubMed Central

    Berg, Elisabeth A.B.; McGregor, JulieAnne G.; Burkart, Madelyn E.; Poulton, Caroline J.; Hu, Yichun; Falk, Ronald J.; Hogan, Susan L.

    2014-01-01

    The use and impact of complementary and alternative medicine (CAM) for anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) has not been reported. AAV patients seeking care at our center inquired about CAM, prompting a formal study. Study objectives were to discern how many AAV patients used CAM and its perceived helpfulness in disease management. Methods AAV patients completed a CAM questionnaire between July 2011 and May 2012. Patients were 18 years or older and had biopsy proven and/or clinical evidence of AAV. Medical record abstraction supplemented data. Classification detailed CAM type including “Mind” or “Mind-Body”. Perceived helpfulness of CAM was assessed as “very”, “somewhat” or “not at all/don’t know”. Results A total of 107 patients participated and were a mean age of 53 (range: 18–85), 62% female; 48% proteinase 3 (PR3)-ANCA, 44% myeloperoxidase (MPO)-ANCA and 8% ANCA-negative. Top organs involved included kidney (87%), joints (55%), lung (53%) and upper respiratory (53%). At least one type of CAM treatment or self-help practice was reported by 81% of study participants, with the most frequent being prayer (64%), exercise (27%) and massage therapy (19%). Mind-based practices were used by 28% (excluding prayer) and Mind-Body practices by 14%. Most practices were used to improve wellbeing, and Mind and Mind-Body were deemed very helpful by 83% and 87% respectively. Only 24% of study participants discussed CAM with their physician. Conclusion CAM practices were commonly used to improve well-being and found to be beneficial among AAV patients, but more open discussion is needed about CAM between physicians and patients. PMID:25599078

  15. CD14 expression is increased on monocytes in patients with anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis and correlates with the expression of ANCA autoantigens.

    PubMed

    Tarzi, R M; Liu, J; Schneiter, S; Hill, N R; Page, T H; Cook, H T; Pusey, C D; Woollard, K J

    2015-07-01

    Monocyte subsets with differing functional properties have been defined by their expression of CD14 and CD16. We investigated these subsets in anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) and determined their surface expression of ANCA autoantigens. Flow cytometry was performed on blood from 14 patients with active AAV, 46 patients with AAV in remission and 21 controls. The proportion of classical (CD14(high) CD16(neg/low)), intermediate (CD14(high) CD16(high)) and non-classical (CD14(low) CD16(high)) monocytes and surface expression levels of CD14 and CD16 were determined, as well as surface expression of proteinase 3 (PR3) and myeloperoxidase (MPO) on monocyte subsets. There was no change in the proportion of monocytes in each subset in patients with AAV compared with healthy controls. The expression of CD14 on monocytes from patients with active AAV was increased, compared with patients in remission and healthy controls (P < 0.01). Patients with PR3-ANCA disease in remission also had increased monocyte expression of CD14 compared with controls (P < 0.01); however, levels in patients with MPO-ANCA disease in remission were lower than active MPO-ANCA patients, and not significantly different from controls. There was a correlation between CD14 and both PR3 and MPO expression on classical monocytes in AAV patients (r = 0.79, P < 0.0001 and r = 0.42, P < 0.005, respectively). In conclusion, there was an increase in monocyte CD14 expression in active AAV and PR3-ANCA disease in remission. The correlation of CD14 expression with ANCA autoantigen expression in AAV may reflect cell activation, and warrants further investigation into the potential for increased CD14 expression to trigger disease induction or relapse. PMID:25766482

  16. Small-vessel vasculitis with prominent IgG4 positive plasma cell infiltrates as potential part of the spectrum of IgG4-related disease: a case report.

    PubMed

    Alba, M A; Milisenda, J; Fernández, S; García-Herrera, Ad; Hernández-Rodríguez, J; Grau, J M; Campo, E; Cid, M C

    2015-01-01

    IgG4-related disease (IgG4-RD) is a systemic entity characterised by multiorgan inflammatory lesions with abundant IgG4+ plasma cells, obliterative phlebitis, and storiform fibrosis. Involvement of several organs such as the pancreas, gastrointestinal tract, salivary glands, periorbital tissue and lymph nodes has been described. Up to now, vascular involvement by IgG4-RD has been thought to be essentially confined to large vessels. We present a patient with small-vessel systemic vasculitis involving muscle, peripheral nerve and kidney (glomerulonephritis) in the context of IgG4-RD diagnosed on the basis of elevated serum IgG4+ concentrations and histologically consistent signs in all biopsied tissues. Thoracic and abdominal aortic aneurysms in addition to aortitis, suggestive of large-vessel involvement, were also present. This observation expands the spectrum of vascular involvement in the context of IgG4-RD and supports the inclusion of IgG4-RD in the category of vasculitis associated with systemic disorder. PMID:26016765

  17. Reduced CD5(+) CD24(hi) CD38(hi) and interleukin-10(+) regulatory B cells in active anti-neutrophil cytoplasmic autoantibody-associated vasculitis permit increased circulating autoantibodies.

    PubMed

    Aybar, L T; McGregor, J G; Hogan, S L; Hu, Y; Mendoza, C E; Brant, E J; Poulton, C J; Henderson, C D; Falk, R J; Bunch, D O

    2015-05-01

    Pathogenesis of anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis is B cell-dependent, although how particular B cell subsets modulate immunopathogenesis remains unknown. Although their phenotype remains controversial, regulatory B cells (Bregs ), play a role in immunological tolerance via interleukin (IL)-10. Putative CD19(+) CD24(hi) CD38(hi) and CD19(+) CD24(hi) CD27(+) Bregs were evaluated in addition to their CD5(+) subsets in 69 patients with ANCA-associated vasculitis (AAV). B cell IL-10 was verified by flow cytometry following culture with CD40 ligand and cytosine-phosphate-guanosine (CpG) DNA. Patients with active disease had decreased levels of CD5(+) CD24(hi) CD38(hi) B cells and IL-10(+) B cells compared to patients in remission and healthy controls (HCs). As IL-10(+) and CD5(+) CD24(hi) CD38(hi) B cells normalized in remission within an individual, ANCA titres decreased. The CD5(+) subset of CD24(hi) CD38(hi) B cells decreases in active disease and rebounds during remission similarly to IL-10-producing B cells. Moreover, CD5(+) B cells are enriched in the ability to produce IL-10 compared to CD5(neg) B cells. Together these results suggest that CD5 may identify functional IL-10-producing Bregs . The malfunction of Bregs during active disease due to reduced IL-10 expression may thus permit ANCA production. PMID:25376552

  18. The CD4 Lymphocyte Count is a Better Predictor of Overall Infection Than the Total Lymphocyte Count in ANCA-Associated Vasculitis Under a Corticosteroid and Cyclophosphamide Regimen

    PubMed Central

    Shi, Yi-Yun; Li, Zhi-Ying; Zhao, Ming-Hui; Chen, Min

    2015-01-01

    Abstract Patients with antineutrophil cytoplasmic autoantibody associated vasculitis (AAV) have a high prevalence of infection during immunosuppressive therapy, and the total lymphocyte count (TLC) has been demonstrated to be an independent predictor of infection. The current study investigated the value of the TLC and its subsets, particularly the CD4 count, for predicting infections of AAV in a single Chinese cohort. A total of 124 AAV patients were retrospectively recruited in our department from December 1997 to October 2013. Multivariate Cox models with the CD4 count or TLC measured at three typical time points, that is, at baseline, at the beginning of immunosuppressant dose reduction, and at the last visit before infection or censoring, or with the measurements included as time-varying covariates, were compared to select the most predictive time point for infection. A time-dependent area under the receiver operating characteristic curve (AUC(t)) for the TLC (AUC(t)TLC) and the CD4 count (AUC(t)CD4count) measured at the most predictive time point were calculated and compared. During an average follow-up of 11.5 (range 0.5–142) months, 55 of the 124 patients (44.3%) experienced a microbiologically confirmed infection. Independent predictors of overall infection were initial creatinine clearance (P?=?0.02 and 0.04), pulmonary interstitial fibrosis (P?=?.04 and .05), pulmonary nodule or cavity (P?=?0.002 and .002), CD4 count (P??0.1). The TLC and CD4 count were both independent risk factors of overall infection and non-bacterial infection in AAV patients. The CD4 count had a higher predictive value than the TLC for overall infections, particularly during the first 2 years of immunosuppressive therapy. PMID:25950695

  19. The CD4 Lymphocyte Count is a Better Predictor of Overall Infection Than the Total Lymphocyte Count in ANCA-Associated Vasculitis Under a Corticosteroid and Cyclophosphamide Regimen: A Retrospective Cohort.

    PubMed

    Shi, Yi-Yun; Li, Zhi-Ying; Zhao, Ming-Hui; Chen, Min

    2015-05-01

    Patients with antineutrophil cytoplasmic autoantibody associated vasculitis (AAV) have a high prevalence of infection during immunosuppressive therapy, and the total lymphocyte count (TLC) has been demonstrated to be an independent predictor of infection. The current study investigated the value of the TLC and its subsets, particularly the CD4 count, for predicting infections of AAV in a single Chinese cohort.A total of 124 AAV patients were retrospectively recruited in our department from December 1997 to October 2013. Multivariate Cox models with the CD4 count or TLC measured at three typical time points, that is, at baseline, at the beginning of immunosuppressant dose reduction, and at the last visit before infection or censoring, or with the measurements included as time-varying covariates, were compared to select the most predictive time point for infection. A time-dependent area under the receiver operating characteristic curve (AUC(t)) for the TLC (AUC(t)TLC) and the CD4 count (AUC(t)CD4count) measured at the most predictive time point were calculated and compared.During an average follow-up of 11.5 (range 0.5-142) months, 55 of the 124 patients (44.3%) experienced a microbiologically confirmed infection. Independent predictors of overall infection were initial creatinine clearance (P?=?0.02 and 0.04), pulmonary interstitial fibrosis (P?=?.04 and .05), pulmonary nodule or cavity (P?=?0.002 and .002), CD4 count (P??0.1).The TLC and CD4 count were both independent risk factors of overall infection and non-bacterial infection in AAV patients. The CD4 count had a higher predictive value than the TLC for overall infections, particularly during the first 2 years of immunosuppressive therapy. PMID:25950695

  20. Vasculitis Syndromes of the Central and Peripheral Nervous Systems

    MedlinePLUS

    ... may require brain biopsy. Treatment includes steroid and immunosuppressive drugs, such as prednisolone and cyclophosphamide. It is fatal ... treated aggressively with high doses of steroids and immunosuppressive drugs such as cyclophosphamide. Eighty percent of individuals who ...

  1. Bullous reactions to bed bug bites reflect cutaneous vasculitis

    Technology Transfer Automated Retrieval System (TEKTRAN)

    This study evaluates bullous cutaneous reactions and sequential histopathology in an individual sensitized to bed bug bites in an effort to better understand the allergic response and histology associated with these bites. There was a progression of the inflammatory response across time ranging from...

  2. Confirmation of the genetic association of CTLA4 and PTPN22 with ANCA-associated vasculitis

    E-print Network

    Carr, Edward J.; Niederer, Heather A.; Williams, Julie; Harper, Lorraine; Watts, Richard A.; Lyons, Paul A.; Smith, Kenneth G. C.

    2009-12-01

    , are frequently associated with multiple diseases, whereas specific HLA associations, such as HLA-B27, tend to be disease restricted. We studied ten candidate loci on the basis of their immunoregulatory role and prior associations with type 1 diabetes (T1D...

  3. Circulating Angiopoietin-2 as a Biomarker in ANCA-Associated Vasculitis

    PubMed Central

    Monach, Paul A.; Kümpers, Philipp; Lukasz, Alexander; Tomasson, Gunnar; Specks, Ulrich; Stone, John H.; Cuthbertson, David; Krischer, Jeffrey; Carette, Simon; Ding, Linna; Hoffman, Gary S.; Iklé, David; Kallenberg, Cees G. M.; Khalidi, Nader A.; Langford, Carol A.; Seo, Philip; St. Clair, E. William; Spiera, Robert; Tchao, Nadia; Ytterberg, Steven R.; Haubitz, Marion; Merkel, Peter A.

    2012-01-01

    The endothelial-specific Angiopoietin-Tie2 ligand-receptor system is an important regulator of endothelial activation. Binding of angiopoietin-2 (Ang-2) to Tie2 receptor renders the endothelial barrier responsive to pro-inflammatory cytokines. We previously showed that circulating Ang-2 correlated with disease severity in a small cohort of critically ill patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis. The current study reassessed Ang-2 as a biomarker of disease activity and relapse in AAV. Circulating Ang-2 was measured in 162 patients with severe AAV (BVAS/WG?3, with or without glomerulonephritis) in a clinical trial. Ang-2 levels during active AAV were compared to levels in the same patients during remission (BVAS/WG?=?0). Levels in clinical subsets of AAV were compared, and association with future disease course was assessed. Ang-2 levels were elevated in severe disease (median 3.0 ng/ml, interquartile range 1.9–4.4) compared to healthy controls (1.2, 0.9–1.5). However, they did not reliably decline with successful treatment (median 2.6 ng/ml, interquartile range 1.9–3.8, median change ?0.1). Ang-2 correlated weakly with BVAS/WG score (r?=?0.17), moderately with markers of systemic inflammation (r?=?0.25–0.41), and inversely with renal function (r?=??0.36). Levels were higher in patients with glomerulonephritis, but levels adjusted for renal dysfunction were no different in patients with or without glomerulonephritis. Levels were higher in patients with newly diagnosed AAV and lower in patients in whom treatment had recently been started. Ang-2 levels during active disease did not predict response to treatment, and Ang-2 levels in remission did not predict time to flare. Thus, Ang-2 appears to have limited practical value in AAV as a biomarker of disease activity at time of measurement or for predicting future activity. PMID:22279570

  4. [Renal microaneurysms in necrotizing vasculitis--incidence in autopsy and clinical value].

    PubMed

    Mundinger, A; Pumpe, K; Beck, A; Dinkel, E; Geissler, A; Kröpelin, T; Peter, H H

    1990-01-01

    Histological renal sections of 24 autopsied patients were evaluated for ectasias with greater than or equal to 2 mm diameter that corresponded to "microaneurysms" of radiologic nomenclature. Such renal "microaneurysms" of smaller and medium sized arteries were seen in 7/9 patients with periarteritis nodosa, 6/10 patients with secondary vasculitides and 1/5 patients with Wegener's Granulomatosis. Lumen ectasias in acute or subacute lesions of smaller and medium sized arteries were caused by fibrinoid necrosis of the arterial walls. Destruction of intimal elastic fibres and scar tissue within the arterial wall resulted in real aneurysmatic ectasias. Generalised, aggressive, necrotizing vasculitides show the highest frequency of microaneurysms. In that cases angiography can frequently establish the definite diagnosis by demonstration of microaneurysms. PMID:1978449

  5. Intestinal Behçet's Disease: A True Inflammatory Bowel Disease or Merely an Intestinal Complication of Systemic Vasculitis?

    PubMed Central

    Kim, Duk Hwan

    2016-01-01

    Behçet's disease (BD) is a multi-systemic inflammatory disorder of an unknown etiology and shows a chronic recurrent clinical course. When the disease involves the alimentary tract, it is called intestinal BD because of its clinical importance. Intestinal BD is more frequently reported in East Asian countries than in Western or Middle Eastern countries. While any part of the gastrointestinal tract can be involved, the most common location of intestinal BD is the ileocecal area. A few, large, deep ulcerations with discrete border are characteristic endoscopic findings of intestinal BD. Currently, there is no single gold standard test or pathognomonic finding of intestinal BD. However, recently developed novel diagnostic criteria and a disease activity index have helped in assessing intestinal BD. As intestinal BD shares a lot of characteristics with inflammatory bowel disease, including genetic background, clinical manifestations, and therapeutic strategies, distinguishing between the two diseases in clinical practice is quite difficult. However, biologic agents such as anti-tumor necrosis factor ? antibody shows a considerable efficacy similar to inflammatory bowel disease cases. It is important to distinguish and treat those two disease entities separately from the standpoint of precise medicine. Clinicians should require comprehensive knowledge regarding the similarities and differences between intestinal BD and inflammatory bowel disease for making an accurate clinical decision. PMID:26632379

  6. Vasculitis with renal involvement in essential mixed cryoglobulinemia: Case report and mini-review

    PubMed Central

    Anis, Sabiha; Abbas, Khawar; Mubarak, Mohammad; Ahmed, Ejaz; Bhatti, Sajid; Muzaffar, Rana

    2014-01-01

    The discovery of a strong association between hepatitis C virus (HCV) infection and mixed cryoglobulinemia (MC) has led to an increasingly rare diagnosis of idiopathic essential MC (EMC). The incidence of EMC is high in regions where there is a comparatively low HCV infection burden and low in areas of high infection prevalence, including HCV. The diagnosis of EMC requires an extensive laboratory investigation to exclude all possible causes of cryoglobulin formation. In addition, although cryoglobulin testing is simple, improper testing conditions will result in false negative results. Here, we present a 46-year-old female patient with a case of EMC with dermatological and renal manifestations, highlighting the importance of extensive investigation to reach a proper diagnosis. We review the need for appropriate laboratory testing, which is often neglected in clinical practice and which can result in false negative results. This review also emphasizes the significance of an extended testing repertoire necessary for better patient management. Despite a strong association of MC with HCV infection and other causes that lead to cryoglobulin formation, EMC remains a separate entity. Correct diagnosis requires proper temperature regulation during sample handling, as well as characterization and quantification of the cryoprecipitate. Inclusion of rheumatoid factor activity and complement levels in the cryoglobulin test-panel promotes better patient management and monitoring. Consensus guidelines should be developed and implemented for cryoglobulin detection and the diagnosis of cryoglobulinemic syndrome, which will reduce variability in inter-laboratory reporting. PMID:24868518

  7. Acute necrotising pulmonary vasculitis and pulmonary hypertension in a juvenile dog.

    PubMed

    Russell, N J; Irwin, P J; Hopper, B J; Olivry, T; Nicholls, P K

    2008-07-01

    A five-month-old female Jack Russell terrier was presented for investigation of acute lethargy, anorexia, coughing, respiratory distress and weakness. Examination findings included cyanosis, a grade 3 of 6 systolic heart murmur and prolonged capillary refill time. Radiography and echocardiography revealed severe pulmonary hypertension, cor pulmonale and right-sided heart failure. Indirect measurement of the systolic pulmonary artery pressure estimated pressures over 100 mmHg. Despite treatment the patient died. Post-mortem examination did not identify a congenital cardiovascular anomaly. Histopathology confirmed acute necrotising pulmonary arteritis and immunohistochemistry failed to identify any immune complex or complement deposition. PMID:18638059

  8. [Renal damage caused by Rhupus syndrome associated with anti-neutrophil cytoplasmic antibodies vasculitis and cryoglobulinemia].

    PubMed

    Zhao, X J; Wei, T; Dong, B; Jia, Y; Wang, M

    2015-10-18

    We analyzed the clinicopathological characteristics of one patient with Rhupus syndrome associated nephropathy in Peking University People's Hospital, and reviewed the related literature. The patient was a middle aged female. She developed rheumatoid arthritis first, and then manifested mild systemic lupus erythematosus together with positive anti-neutrophil cytoplasmic antibodies (ANCA) and cryoglobulinemia several years later. The renal biopsy was performed and manifested as lupus nephritis. The transmission electron microscopy revealed cryoglobulinemia associated renal damage. This report shows that the clinicopathological characteristics in patients with Rhupus syndrome associated nephropathy are complicated. The renal pathology can be used as a diagnostic tool. PMID:26474633

  9. The kidneys and ANCA-associated vasculitis: from pathogenesis to diagnosis.

    PubMed

    Rowaiye, Olumide Olatubosun; Kusztal, Mariusz; Klinger, Marian

    2015-06-01

    Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of pauci-immune small vessel vasculitides that often affect the kidneys manifesting as rapidly progressive glomerulonephritis. Although the exact pathogenesis of AAV is not fully known, evidence from in vitro, in vivo and clinical studies all point to the involvement of ANCA in the pathogenesis of AAV. In this review, we highlight the contributory roles played by various factors (e.g. genetics, environment, B and T-regulatory cells, toll-like receptors, etc.) in the pathogenesis of AAV. Furthermore, we discuss renal involvement in AAV in terms of clinical features and the various histopathological classification patterns, which are also known to be of prognostic importance. We also present information on useful imaging techniques for localizing kidney and other organ system involvement in AAV, and also on novel laboratory methods and assays useful for rapid and more specific determination of patients' ANCA status. Finally, we demonstrate evidence on novel serum biomarkers that have been shown to correlate with disease activity in AAV. PMID:26034600

  10. Digital Vasculitis in a Patient with Rheumatoid Arthritis Responded Well to Adalimumab

    PubMed Central

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

    2014-01-01

    42-year-old old female patient, followed up with diagnosis of rheumatoid arthritis for 15 years, was admitted with necrotising ulcer of left hand 1st and 2nd fingertips and pain, swelling, limitation of movement, and morning stiffness at bilateral wrist, and metacarpophalangeal and proximal interphalangeal joints. Laboratory tests revealed elevated acute phase reactants. Radial and ulnar arteries were clear in upper extremity Doppler ultrasound. The patient was diagnosed as RA activation and digital ulcer and administered iloprost infusion for five days and 1?mg/kg corticosteroid and 20?mg/week methotrexate (MTX). After one month, a partial regression of clinical and laboratory findings was observed. However, 6 months later, due to relapsed and increased complaints and findings, adalimumab 40?mg was administered. Two months later, clinical and laboratory findings apparently decreased. PMID:25133007

  11. Contrasting genetic association of IL2RA with SLE and ANCA - associated vasculitis

    E-print Network

    Carr, Edward J.; Clatworthy, Menna R.; Lowe, Christopher E.; Todd, John A.; Wong, Andrew; Vyse, Timothy J.; Kamesh, Lavanya; Watts, Richard A.; Lyons, Paul A.; Smith, Kenneth G. C.

    2009-03-05

    evidence for association [9]. Soluble IL-2RA (sIL-2RA) is shed from human T cells following in vitro activation in response to a variety of polyclonal stimuli [5]. Significantly elevated serum sIL-2RA has been described in infection, malig- nancy... to correlate with serum sIL-2RA concentrations. We therefore genotyped autoimmune disease-prone vari- ants of the IL2RA region (rs11594656, rs41595061 and rs2104286) in two distinct autoimmune diseases associ- multi-system disease characterised by immune...

  12. Vasculitis resulting from a superficial femoral artery angioplasty with a paclitaxel-eluting balloon.

    PubMed

    Thomas, Shannon D; McDonald, Robert R A; Varcoe, Ramon L

    2014-02-01

    Drug-eluting balloons (DEBs) coated with the antiproliferative agent paclitaxel may improve primary patency by reducing recurrent luminal stenosis. A proportion of the active drug and excipient coating are known to embolize distally, but until now, there have been no reports of adverse events resulting from their use. We report an unusual case of a painful nodular, biopsy specimen-proven vasculitic rash that afflicted the ipsilateral lower limb of a patient after superficial femoral artery treatment with a DEB. This adverse event may have implications for the use of DEB in this and other vascular territories. PMID:23642919

  13. The Contribution of Transcriptomics to Biomarker Development in Systemic Vasculitis and SLE

    E-print Network

    Flint, Shaun M.; McKinney, Eoin F.; Lyons, Paul A; Smith, Kenneth G. C.

    2015-04-15

    inflammation. Many of these cells are readily sampled in the peripheral blood as they traffic between lymph node and tissues, providing a rationale that gene expression profiling of blood is as likely to yield relevant patterns as the more technically... between neutrophils and interferon-producing plasmacytoid dendritic cells [67]. 19 Gene%expression%profiling%in%ankylosing%spondylitis% The two major studies of peripheral blood gene expression in ankylosing spondylitis (AS) have conflicting...

  14. The Association between Silica Exposure and Development of ANCA-Associated Vasculitis: Systematic Review and Meta-analysis

    PubMed Central

    Gómez-Puerta, Jose A.; Gedmintas, Lydia; Costenbader, Karen H.

    2014-01-01

    Background Crystalline silica is among the environmental exposures associated with increased risk of autoimmune diseases, including rheumatoid arthritis, systemic sclerosis and systemic lupus erythematosus. Silica exposure has also been related to the development of ANCA-associated vasculitides (AAV), but past studies appear to conflict as to the presence and magnitude of the associated risks of disease. We aimed to conduct a systematic review of the existing studies and meta-analysis of their results. Methods We searched EMBASE, MEDLINE and international scientific conference abstract databases for studies examining the association of silica exposure with AAV. Studies in English, French, or Spanish were included and those examining the association of silica with ANCA-positivity alone were excluded. We assessed study quality using the Newcastle-Ottawa scale. We meta-analyzed the results using random effects models and tested for heterogeneity. We performed sensitivity and subgroup analyses, examining studies that adjusted for smoking and occupational risk factors as well as studies that analyzed by subtypes of AAV. Results We identified 158 potential manuscripts and 3 abstracts related to silica exposure and risk of AAV. 147 were excluded after abstract review and 14 underwent detailed evaluation of full manuscript/abstract. After further application of exclusion criteria, 6 studies (all case-controls) remained. The studies had moderate heterogeneity in selection of cases and controls, exposure assessment, disease definition and controlling for potential confounders. We found an overall significant summary effect estimate of silica “ever exposure” with development of AAV (summary OR 2.56, 95% CI 1.51- 4.36), with moderate heterogeneity (I2=48.40%). ORs were similar for studies examining only MPA (OR 3.95, CI 95% 1.89-8.24), compared to those only studying GPA (OR 3.56, CI 95% 1.85-6.82). Conclusion Despite moderate heterogeneity among studies, the totality of the evidence after meta-analysis points to an association between silica exposure and risk for developing AAV. PMID:23820041

  15. Wet Shape Memory Alloy Actuators for Active Vasculated Robotic Flesh Stephen A. Mascaro and H. Harry Asada

    E-print Network

    Mascaro, Stephen A.

    , for regulation of temperature, pH, and fluid volume, and for defense against disease [2]. Using for the purpose of temperature regulation and/or nutrition. It is estimated that in the human body, 90% of the blood flow to the skin occurs for the purpose of temperature regulation, while only 10% is required

  16. Plasma exchange and glucocorticoid dosing in the treatment of anti-neutrophil cytoplasm antibody associated vasculitis (PEXIVAS): protocol for a randomized controlled trial

    E-print Network

    Walsh, Michael; Merkel, Peter A.; Peh, Chen Au; Szpirt, Wladimir; Guillevin, Loïc; Pusey, Charles D.; deZoysa, Janak; Ives, Natalie; Clark, William F.; Quillen, Karen; Winters, Jeffrey L.; Wheatley, Keith; Jayne, David; PEXIVAS Investigators

    2013-03-14

    (ESRD) [2,3]. Poor survival and ESRD in AAV are attributed both to ineffective therapies and treatment toxicity [2]. At least 20% of patients do not achieve disease control or are in- tolerant of initial treatment, and an additional 50% will relapse by 5... 2013, 14:73 Page 3 of 7 http://www.trialsjournal.com/content/14/1/73cardiovascular disease, and gastrointestinal bleeding [22]. In renal transplantation, concern over GC toxicity prompted early GC withdrawal and even GC avoidance regimens. Meta...

  17. Edinburgh Research Explorer Novel expression signatures identified by transcriptional

    E-print Network

    MacDonald, Andrew

    of separated leucocyte subsets in systemic lupus erythematosus and vasculitis Citation for published version of separated leucocyte subsets in systemic lupus erythematosus and vasculitis' Annals of the Rheumatic Diseases in processes underlying disease pathogenesis. Conclusion Analysis of leucocyte subsets enabled

  18. Polyarteritis Nodosa

    MedlinePLUS

    ... Arteritis Wegener’s Granulomatosis All information contained within the Johns Hopkins Vasculitis Center website is intended for educational ... read on this website. Patient Privacy · Copyright © 2015 Johns Hopkins Vasculitis Center · Log in

  19. Eosinophilic Granulomatosis with Polyangiitis, formerly Churg-Strauss Syndrome (EGPA)

    MedlinePLUS

    ... Arteritis Wegener’s Granulomatosis All information contained within the Johns Hopkins Vasculitis Center website is intended for educational ... read on this website. Patient Privacy · Copyright © 2015 Johns Hopkins Vasculitis Center · Log in

  20. Overview of the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides.

    PubMed

    Jennette, J Charles

    2013-10-01

    The nomenclature and classification of vasculitis has been difficult and controversial for many decades. This is problematic both for research on vasculitis as well as clinical care of patients with vasculitis. The first (1994) International Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitides (CHCC) proposed names and definitions for the most common forms of vasculitis. Since then, there have been substantial advances in our understanding of vasculitis and changes in medical terminology. In addition, CHCC 1994 did not propose a nomenclature for some relatively common forms of vasculitis, such as vasculitis secondary to other diseases. To address these issues, a second International Chapel Hill Consensus Conference was held in 2012. The goals were to change names and definitions as appropriate, and add important categories of vasculitis not included in CHCC 1994. This overview summarizes the 2012 CHCC and points out the changes compared to the 1994 CHCC. Notable changes include the introduction of new terms such as granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and immunoglobulin A vasculitis and the inclusion of categories for variable vessel vasculitis and secondary forms of vasculitis. PMID:24072416

  1. Comparison of Salivary and Serum Levels of Soluble Toll-Like Receptor 3 (TLR3) in Patients with Sjögren's Syndrome (SS) Compared to Age- and Sex-Matched Controls 

    E-print Network

    Parks, Harrison Lee

    2014-04-28

    SS 1. Cutaneous manifestations of SS include xerosis, angular cheilitis, eyelid dermatitis, prutitus, cutaneous vasculitis (often as palpable purpura), and erythema annulare 17. Cutaneous rashes of SS often cannot be distinguished clinically... and hypergammaglobulinemia are often concomitant, while palpable purpura is associated with dermal vasculitis. Dermal vasculitis is linked with SS-related extraglandular manifestations and lymphoma 20. In a recent study of pSS, 89 of 559 patients (16%) presented...

  2. Diagnosis and therapeutic options for peripheral vasculitic neuropathy

    PubMed Central

    2015-01-01

    Vasculitis can affect the peripheral nervous system alone (nonsystemic vasculitic neuropathy) or can be a part of primary or secondary systemic vasculitis. In cases of pre-existing systemic vasculitis, the diagnosis can easily be made, whereas suspected vasculitic neuropathy as initial or only manifestation of vasculitis requires careful clinical, neurophysiological, laboratory and histopathological workout. The typical clinical syndrome is mononeuropathia multiplex or asymmetric neuropathy, but distal-symmetric neuropathy can frequently be seen. Standard treatments include steroids, azathioprine, methotrexate and cyclophosphamide. More recently the B-cell antibody rituximab and intravenous immunoglobulins have shown to be effective in some vasculitic neuropathy types. PMID:25829955

  3. Pregnancy and Medically Assisted Conception in Rare Diseases

    ClinicalTrials.gov

    2015-05-20

    Rheumatoid Arthritis; Spondyloarthritis; Psoriatic Arthritis; Systemic Lupus Erythematosus; Antiphospholipid Syndrome; Sjogren Syndrome; Scleroderma; Myositis; Vasculitis; Mastocytosis; Various Autoimmune and/or Systemic and/or Rare Diseases

  4. Observational Study of the Genetic Architecture of Neutrophil-Mediated Inflammatory Skin Diseases

    ClinicalTrials.gov

    2014-06-11

    Other Specified Inflammatory Disorders of Skin or Subcutaneous Tissue; Pyoderma Gangrenosum; Erosive Pustular Dermatosis of the Scalp; Sweet's Syndrome; Behcet's Disease; Bowel-associated Dermatosis-arthritis Syndrome; Pustular Psoriasis; Acute Generalized Exanthematous Pustulosis; Keratoderma Blenorrhagicum; Sneddon-Wilkinson Disease; IgA Pemphigus; Amicrobial Pustulosis of the Folds; Infantile Acropustulosis; Transient Neonatal Pustulosis; Neutrophilic Eccrine Hidradenitis; Rheumatoid Neutrophilic Dermatitis; Neutrophilic Urticaria; Still's Disease; Erythema Marginatum; Unclassified Periodic Fever Syndromes / Autoinflammatory Syndromes; Dermatitis Herpetiformis; Linear IgA Bullous Dermatosis; Bullous Systemic Lupus Erythematosus; Inflammatory Epidermolysis Bullosa Aquisita; Neutrophilic Dermatosis of the Dorsal Hands (Pustular Vasculitis); Small Vessel Vasculitis Including Urticarial Vasculitis; Erythema Elevatum Diutinum; Medium Vessel Vasculitis

  5. Histology Self-Study Presentation Ellen O'Dea

    E-print Network

    Gleeson, Joseph G.

    . Systemic lupus erythematosus (SLE) 3. Scleroderma (Systemic sclerosis) 4. Crystal induced disease (Gout: Rheumatoid Arthritis Systemic Lupus Erythematosus Sclerosis Gout Vasculitis #12;Lymphoid System Cells

  6. 38 CFR Appendix C to Part 4 - Alphabetical Index of Disabilities

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 6847 Soft tissue sarcoma: Muscle, fat, or fibrous connected 5329 Neurogenic origin 8540 Vascular origin... Varicose veins 7120 Vasculitis, primary cutaneous 7826 Vertebral fracture or dislocation 5235...

  7. 38 CFR Appendix C to Part 4 - Alphabetical Index of Disabilities

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 6847 Soft tissue sarcoma: Muscle, fat, or fibrous connected 5329 Neurogenic origin 8540 Vascular origin... Varicose veins 7120 Vasculitis, primary cutaneous 7826 Vertebral fracture or dislocation 5235...

  8. Diagnostic relevance of fibronectin in cryoprecipitates.

    PubMed

    Robinson, M W; Walton, K W; Scott, D G; Scott, D L

    1990-01-01

    Fibronectin is a known component of plasma cryoprecipitates. It is seen in cryoglobulins from patients with monoclonal gammopathies and also from rheumatoid arthritis, and patients with systemic lupus erythematosus and other connective tissue diseases. We evaluated the clinical relevance of measures of cryoprecipitable fibronectin from the sera of 88 patients with rheumatic diseases and 27 healthy controls. There were 28 patients with rheumatoid arthritis, 19 with systemic vasculitis, 5 with cutaneous vasculitis, and 36 with a systemic connective tissue disorder. We measured total and cryoprecipitable fibronectin and for comparison immunoglobulins G, A, and M and complement C3 and C4. Cryoprecipitable fibronectin was detected in 33% control sera and 42% patient sera. The mean levels were higher in the sera of patients in all diagnostic groups. The highest levels were seen in rheumatoid patients with systemic disease, systemic vasculitis, and connective tissue diseases. The presence of cryoprecipitable fibronectin was related to the clinical activity of systemic vasculitis; none of the 6 patients with clinically inactive vasculitis had detectable fibronectin in their cryoprecipitates; but it was seen in 7 to 13 cases with active vasculitis. There were only weak relationships between cryoprecipitable fibronectin and immunoglobulin and complement levels in cryoprecipitates. We conclude that routinely measuring fibronectin levels in cryoprecipitates is generally of doubtful diagnostic value. However, it appears to be a useful marker of the clinical activity of systemic vasculitis and we recommend its use in the laboratory assessment of vasculitis. PMID:2349438

  9. Cryoglobulinemia

    MedlinePLUS

    ... through education, awareness and research. A A A Youtube LinkedIn Twitter Facebook Giving Tubesday 2015 + Vasculitis Behcet’s ... Book Vasculitis Terms A – Z VF Medical Consultants YouTube Education Videos YouTube Education Videos produced by Alliance ...

  10. Vasculitic neuropathy following exposure to minocycline

    PubMed Central

    Baratta, John M.; Dyck, P. James B.; Brand, Patricio; Thaisetthawatkul, Pariwat; Dyck, Peter J.; Engelstad, JaNean K.; Goodman, Brent

    2015-01-01

    Objective: To report 3 patients with minocycline-induced autoimmunity resulting in peripheral nerve vasculitis. Methods: We report 3 patients who, during minocycline treatment for acne vulgaris, developed subacute onset of pain and weakness caused by vasculitis in single and multiple mononeuropathy patterns. Results: Each patient underwent either a nerve or muscle biopsy that confirmed vasculitis. One patient additionally developed systemic symptoms (including fever, fatigue, and night sweats) and another had a posterior circulation stroke. Symptoms developed with either early or prolonged use of minocycline. Despite withdrawal of minocycline, patients needed long-term immunotherapy to gain neurologic improvement. Conclusions: Our findings suggest that the typical neuropathy associated with minocycline use is painful single or multiple mononeuropathy due to peripheral nerve vasculitis, which may also be accompanied by presumed CNS vasculitis (presenting as stroke). PMID:26601119

  11. Characterization and analysis of carbon fibre-reinforced polymer composite laminates with embedded circular vasculature

    PubMed Central

    Huang, C.-Y.; Trask, R. S.; Bond, I. P.

    2010-01-01

    A study of the influence of embedded circular hollow vascules on structural performance of a fibre-reinforced polymer (FRP) composite laminate is presented. Incorporating such vascules will lead to multi-functional composites by bestowing functions such as self-healing and active thermal management. However, the presence of off-axis vascules leads to localized disruption to the fibre architecture, i.e. resin-rich pockets, which are regarded as internal defects and may cause stress concentrations within the structure. Engineering approaches for creating these simple vascule geometries in conventional FRP laminates are proposed and demonstrated. This study includes development of a manufacturing method for forming vascules, microscopic characterization of their effect on the laminate, finite element (FE) analysis of crack initiation and failure under load, and validation of the FE results via mechanical testing observed using high-speed photography. The failure behaviour predicted by FE modelling is in good agreement with experimental results. The reduction in compressive strength owing to the embedding of circular vascules ranges from 13 to 70 per cent, which correlates with vascule dimension. PMID:20150337

  12. Biologics in vasculitides: Where do we stand, where do we go from now?

    PubMed

    Pazzola, Giulia; Muratore, Francesco; Pipitone, Nicolò; Salvarani, Carlo

    2015-06-01

    Biological agents represent a valid therapeutic option in patients with severe and/or relapsing vasculitis. Over the last years, some of these agents have become an established therapy (such as RTX in AAV or IFX for ocular BD), and some appear to hold promise to become so. In addition, there are a number of drugs in the pipeline that may contribute to further improve the prognosis of vasculitis. As the range of medications available for vasculitis widens, the need will also increase to define the best treatment schemes as well as to identify those patients that may benefit most from biological agents. PMID:26024760

  13. Vasculitic neuropathies.

    PubMed

    Gwathmey, Kelly Graham; Burns, Ted Michael; Collins, Michael Paul; Dyck, P James Bonham

    2014-01-01

    The vasculitic neuropathies are a diverse group of disorders characterised by the acute-to-subacute onset of painful sensory and motor deficits that result from inflammatory destruction of nerve blood vessels and subsequent ischaemic injury. They are common in patients with primary systemic vasculitis and are seen in vasculitis secondary to disorders such as rheumatoid arthritis, viral infections, and diabetic inflammatory neuropathies. It is imperative that neurologists recognise these disorders to initiate treatment promptly and thereby prevent morbidity and mortality. To simplify the approach to patients with vasculitis of the peripheral nerves, a straightforward, dichotomous classification scheme can be used in which the vasculitic neuropathies are divided into two groups-nerve large arteriole vasculitis and nerve microvasculitis-on the basis of the size of the involved vessels. The size of the affected blood vessels correlates with the clinical course and prognosis in patients with vasculitic neuropathy. PMID:24331794

  14. 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.

  15. West Nile Virus Infection in Killer

    E-print Network

    Wang, David

    , Les Dalton, Erika Nilson, and David Wang In 2007, nonsuppurative encephalitis was identified demonstrated WNV antigen within neurons. WNV should be considered in cases of encephalitis in cetaceans. West. Histologic review demonstrated moderate multifocal subacute vasculitis and nonsuppurative encephalitis

  16. Behcet's Disease

    MedlinePLUS

    You are here: Home / Types of Vasculitis / Behcet’s Disease Behcet’s Disease First Description Who gets Behcet’s Disease (the “typical” patients)? Classic symptoms of Behcet’s Disease What causes Behcet’s Disease? ...

  17. The CARRA Registry

    ClinicalTrials.gov

    2015-11-16

    Juvenile Idiopathic Arthritis; Systemic Lupus Erythematosus; Mixed Connective Tissue Disease; Juvenile Ankylosing Spondylitis; Juvenile Dermatomyositis; Localized Scleroderma; Systemic Sclerosis; Vasculitis; Sarcoid; Fibromyalgia, Primary; Auto-inflammatory Disease; Idiopathic Uveitis Idiopathic

  18. Safety and Efficacy of Anti-Pandemic H1N1 Vaccination in Rheumatic Diseases

    ClinicalTrials.gov

    2010-06-25

    Rheumatoid Arthritis; Spondyloarthritis; Systemic Lupus Erythematosus (SLE); Dermatomyositis (DM); DMixed Connective Tissue Disease; Systemic Vasculitis; Systemic Sclerosis (SSc); Sjögren's Syndrome; Antiphospholipid Syndrome; Juvenile Idiopathic Arthritis; Juvenile SLE; Juvenile DM

  19. A Real World, Observational Registry of Chronic Wounds and Ulcers

    ClinicalTrials.gov

    2014-10-29

    Diabetic Foot; Varicose Ulcer; Pressure Ulcer; Surgical Wound Dehiscence; Vasculitis; Skin Ulcer; Leg Ulcer; Wounds and Injuries; Pyoderma; Peripheral Arterial Disease; Diabetic Neuropathies; Lymphedema; Venous Insufficiency; Diabetes Complications; Amputation Stump

  20. 21 CFR 312.32 - IND safety reporting.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ...would be unexpected (by virtue of greater severity) if the investigator brochure referred only to elevated hepatic enzymes or hepatitis. Similarly, cerebral thromboembolism and cerebral vasculitis would be unexpected (by virtue of greater specificity)...

  1. 21 CFR 600.80 - Postmarketing reporting of adverse experiences.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ...necrosis would be unexpected (by virtue of greater severity) if the labeling only referred to elevated hepatic enzymes or hepatitis. Similarly, cerebral thromboembolism and cerebral vasculitis would be unexpected (by virtue of greater specificity)...

  2. 21 CFR 312.32 - IND safety reporting.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ...would be unexpected (by virtue of greater severity) if the investigator brochure referred only to elevated hepatic enzymes or hepatitis. Similarly, cerebral thromboembolism and cerebral vasculitis would be unexpected (by virtue of greater specificity)...

  3. 21 CFR 600.80 - Postmarketing reporting of adverse experiences.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ...necrosis would be unexpected (by virtue of greater severity) if the labeling only referred to elevated hepatic enzymes or hepatitis. Similarly, cerebral thromboembolism and cerebral vasculitis would be unexpected (by virtue of greater specificity)...

  4. 21 CFR 312.32 - IND safety reporting.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ...would be unexpected (by virtue of greater severity) if the investigator brochure referred only to elevated hepatic enzymes or hepatitis. Similarly, cerebral thromboembolism and cerebral vasculitis would be unexpected (by virtue of greater specificity)...

  5. 21 CFR 310.305 - Records and reports concerning adverse drug experiences on marketed prescription drugs for human...

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ...necrosis would be unexpected (by virtue of greater severity) if the labeling only referred to elevated hepatic enzymes or hepatitis. Similarly, cerebral thromboembolism and cerebral vasculitis would be unexpected (by virtue of greater specificity) if...

  6. 21 CFR 600.80 - Postmarketing reporting of adverse experiences.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ...necrosis would be unexpected (by virtue of greater severity) if the labeling only referred to elevated hepatic enzymes or hepatitis. Similarly, cerebral thromboembolism and cerebral vasculitis would be unexpected (by virtue of greater specificity)...

  7. 21 CFR 314.80 - Postmarketing reporting of adverse drug experiences.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ...necrosis would be unexpected (by virtue of greater severity) if the labeling only referred to elevated hepatic enzymes or hepatitis. Similarly, cerebral thromboembolism and cerebral vasculitis would be unexpected (by virtue of greater specificity) if...

  8. 21 CFR 310.305 - Records and reports concerning adverse drug experiences on marketed prescription drugs for human...

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ...necrosis would be unexpected (by virtue of greater severity) if the labeling only referred to elevated hepatic enzymes or hepatitis. Similarly, cerebral thromboembolism and cerebral vasculitis would be unexpected (by virtue of greater specificity) if...

  9. 21 CFR 310.305 - Records and reports concerning adverse drug experiences on marketed prescription drugs for human...

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ...necrosis would be unexpected (by virtue of greater severity) if the labeling only referred to elevated hepatic enzymes or hepatitis. Similarly, cerebral thromboembolism and cerebral vasculitis would be unexpected (by virtue of greater specificity) if...

  10. 21 CFR 314.80 - Postmarketing reporting of adverse drug experiences.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ...necrosis would be unexpected (by virtue of greater severity) if the labeling only referred to elevated hepatic enzymes or hepatitis. Similarly, cerebral thromboembolism and cerebral vasculitis would be unexpected (by virtue of greater specificity) if...

  11. 21 CFR 312.32 - IND safety reporting.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ...would be unexpected (by virtue of greater severity) if the investigator brochure referred only to elevated hepatic enzymes or hepatitis. Similarly, cerebral thromboembolism and cerebral vasculitis would be unexpected (by virtue of greater specificity)...

  12. 21 CFR 310.305 - Records and reports concerning adverse drug experiences on marketed prescription drugs for human...

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ...necrosis would be unexpected (by virtue of greater severity) if the labeling only referred to elevated hepatic enzymes or hepatitis. Similarly, cerebral thromboembolism and cerebral vasculitis would be unexpected (by virtue of greater specificity) if...

  13. 21 CFR 314.80 - Postmarketing reporting of adverse drug experiences.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ...necrosis would be unexpected (by virtue of greater severity) if the labeling only referred to elevated hepatic enzymes or hepatitis. Similarly, cerebral thromboembolism and cerebral vasculitis would be unexpected (by virtue of greater specificity) if...

  14. 21 CFR 310.305 - Records and reports concerning adverse drug experiences on marketed prescription drugs for human...

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ...necrosis would be unexpected (by virtue of greater severity) if the labeling only referred to elevated hepatic enzymes or hepatitis. Similarly, cerebral thromboembolism and cerebral vasculitis would be unexpected (by virtue of greater specificity) if...

  15. ANCA / MPO / PR3 Antibodies Test

    MedlinePLUS

    ... other autoimmune disorders , such as systemic lupus erythematosus , rheumatoid arthritis , and Sjögren syndrome . The following table shows results that may be seen in some vasculitis conditions. Condition % of patients with cANCA pattern (PR3 antibodies) % of patients with ...

  16. Vasculitides of the gastrointestinal tract.

    PubMed

    Ahn, Eric; Luk, Adriana; Chetty, Runjan; Butany, Jagdish

    2009-05-01

    Systemic vasculitis is often not considered as a possible diagnosis by clinicians because of its low prevalence compared with other more common diseases. Vasculitis can affect any end organ, and it is therefore often missed early on in disease progression. Gastrointestinal (GI) manifestations of vasculitis are considered rare and the presentation is often nonspecific. However, if there is significant involvement of the major vessels of the gastrointestinal system, life-threatening sequelae, including perforation and bowel ischemia, may occur. This makes early and immediate management crucial to improve long-term morbidity and mortality. Diagnosis of various GI vasculitides often relies on correlation of clinical manifestations with pathology and additional investigations. This paper reviews the various vasculitides that affect the GI tract, including systemic lupus erythematosus, mixed connective tissue disease, Henoch Schönlein purpura, polyarteritis nodosa, Churg-Strauss syndrome, Wegener's granulomatosis, microscopic polyangiitis, enterocolic lymphocytic phlebitis, and Behcet's disease. Segmental arterial mediolysis, mistakenly believed to be a vasculitis, is also discussed. PMID:19891302

  17. Methotrexate

    MedlinePLUS

    ... you have these tests performed outside of the Johns Hopkins System please have a copy of these ... t feel well. All information contained within the Johns Hopkins Vasculitis Center website is intended for educational ...

  18. Comparison of Salivary and Serum Levels of Soluble Toll-Like Receptor 4 (TLR4) in Patients with Sjögren's Syndrome (SS), A Case:Control Study 

    E-print Network

    Blansett, Jonathan Andrew

    2014-05-01

    , and myostitis due to medications should also be considered and treated appropriately. Neurologic problems including vasculitis, thrombitic and/or paraneoplastic processes can also cause symptoms of weakness and generalized fatigue. Analysis of acute.... The skin may be affected and display palpable or non-palpable purpura associated with cryoglobulianemia and/or hyperglobulinaemia.(6) Also, some patients may display medium vessel pathology and present with urticarial vasculitis, necrosing 3...

  19. Intestinal infarction and portal vein thrombosis in a patient with henoch schonlein purpura.

    PubMed

    Abebe, Mekdess; Patnaik, Asha; Miller, Frederick; Roppelt, Heidi; Wadhwa, Nand K; Abate, Mersema; Nord, Edward P

    2012-01-01

    Henoch Schonlein purpura is a systemic vasculitis that commonly affects children and teenagers but also affects adults of all ages. In most instances it has a benign course. Organ involvement, particularly in adults, and notably the kidneys and gastrointestinal tract may require therapeutic intervention and may have a less favorable outcome. We report a case of a 58-year-old man who presented with purpura and who rapidly developed catastrophic intestinal vasculitis, leading to his demise. PMID:23097737

  20. A young boy with affected heart and mind: arterial involvement in a patient with undiagnosed Behçet's disease.

    PubMed

    Yalcin, Ulvi; Gurses, Kadri Murat; Kocyigit, Duygu; Isik, Metin; Ozer, Necla

    2014-01-01

    Behçet's disesase is a vasculitis that manifests with oral and genital ulcerations, skin lesions, uveitis, vascular, central nervous system and gastrointestinal involvement. While most patients develop mucocutaneous and genital ulcers along with eye disease, other patients may also present with arthritis, frank vasculitis, thrombophlebitis and central nervous system disease. This is the first case in the literature, where the diagnosis of Behçet's disease was made secondary to concomitant involvement of coronary and carotid arteries. PMID:25005759

  1. Epidemiology of Vasculitides in Khorasan Province, Iran

    PubMed Central

    Jokar, Mohammadhassan; Mirfeizi, Zahra

    2015-01-01

    Vasculitides are a heterogeneous group of more than 20 diseases defined by inflammation and destruction of blood vessels. We aimed to study the demographic characteristics of the primary vasculitides in the North East of Iran. We retrospectively studied the medical records of patients diagnosed with any kind of vasculitis at the Clinic and Department of Rheumatology of the Imam Reza Hospital, Mashhad, Iran between January 1, 2002, and December 31, 2012. Patients were classified according to the American College of Rheumatology 1990 criteria for the classification of vasculitis and the 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. A total of 721 patients (51.5% male, 48.5% female) with a diagnosis of primary vasculitis was identified. The frequency distributions of vasculitic disorders were as follows: Behcet’s disease, 63.6%; cutaneous leukocytoclastic angiitis, 8.2%; granulomatosis with polyangiitis (Wegener’s), 6.8%; Takayasu’s arteritis. 6%; giant cell arteritis, 4%; polyarteritis nodosa, 2.1%; microscopic polyangiitis, 0.6%; eosinophilic granulomatosis with polyangiitis (Churg-Strauss), 1.8%; cryoglobulinemic vasculitis, 0.3%; and IgA vasculitis (Henoch-Schonlein purpura), 3.5%. In our population, the most common forms of vasculitis are Behcet’s disease, cutaneous leukocytoclastic angiitis, and granulomatosis with polyangiitis (Wegener’s). PMID:26170524

  2. Spontaneous closure of patent ductus arteriosus after an episode of Kawasaki disease: a case report

    PubMed Central

    2012-01-01

    Introduction Kawasaki disease is regarded as systemic vasculitis. Many experts believe that not only coronary arteries but also other small arteries are involved during the period of systemic inflammation. However, the evidence to support this point view is limited. Case presentation We report the case of a one-year-four-month-old Taiwanese girl whose patent ductus arteriosus was incidentally found during an episode of Kawasaki disease. The ductus closed spontaneously after the acute phase of Kawasaki disease. Conclusions In this patient, the patent ductus arteriosus may have closed spontaneously after Kawasaki disease due to its involvement in the generalized vasculitis that this disease incurs. This would support the theory that the vasculitis of Kawasaki disease is limited not only to coronary arteries but also to all medium- sized arteries. PMID:22277763

  3. Cutaneous vasculitidis: histology and immunofluorescence.

    PubMed

    Filosa, A; Verdelli, A; Bianchi, B; Del Bianco, E; Bugatti, L; Filosa, G; Caproni, M

    2015-04-01

    Cutaneous vasculitis comprises a wide spectrum of diseases that involve predominantly the blood vessels and surrounding tissues of the skin. Few vasculitic syndromes have pathognomonic clinical, radiographic and/or laboratory findings; thus, confident and accurate diagnosis of vasculitis requires histological confirmation. Skin biopsy should be done, optimally within 24 to 48 hours after vasculitic lesions appear. Deep excision biopsy must be preferred. Direct immunofluorescence of lesional skin is helpful in the diagnosis of vasculitides in the light of a proper clinico-pathological setting and diagnostic in some peculiarly forms. Cutaneous histological patterns can be used to generate relevant clinical differential diagnoses, and, when coupled with patient's history, clinical and laboratory data, allow more precise and accurate diagnosis of vasculitic syndromes. This review will focus on histopathological and immunologic pattern of the more common cutaneous vasculitis syndromes, based on the 2012 Revised International CHCC. PMID:25776140

  4. Budd-chiari syndrome and renal arterial neurysms due to Behcet disease: a rare association.

    PubMed

    Batur, Abdussamet; Dorum, Meltem; Yüksekkaya, Hasan Ali; Koc, Osman

    2015-01-01

    Behcet's disease is a multisystemic vasculitis of unknown etiology with a chronic relapsing course. Vasculitis in Behcet's disease with predominant vascular involvement is the only vasculitis that affects both arteries and veins of any size. Involvement of the renal artery and inferior vena cava is rare among the arteries and veins, respectively. When disease affect the veins, it is in the form of thrombosis. Arterial complications include aneurysms, stenosis and occlusions. Both rupture of arterial aneurysm and occlusion of suprahepatic veins, causing Budd-Chiari syndrome, are associated with a high mortality rate. Vascular involvement is more common in male patients than in female patients. Men and patients with a younger age of onset present with a more severe prognosis. In this case report, we describe a very rare cause of intrarenal arterial aneurysm's rupture with previous Budd-Chiari syndrome due to Behcet's disease and successful angiographic embolization of actively bleeding aneurysm. PMID:26491527

  5. Budd-chiari syndrome and renal arterial neurysms due to behcet disease: a rare association

    PubMed Central

    Batur, Abdussamet; Dorum, Meltem; Yüksekkaya, Hasan Ali; Koc, Osman

    2015-01-01

    Behcet's disease is a multisystemic vasculitis of unknown etiology with a chronic relapsing course. Vasculitis in Behcet's disease with predominant vascular involvement is the only vasculitis that affects both arteries and veins of any size. Involvement of the renal artery and inferior vena cava is rare among the arteries and veins, respectively. When disease affect the veins, it is in the form of thrombosis. Arterial complications include aneurysms, stenosis and occlusions. Both rupture of arterial aneurysm and occlusion of suprahepatic veins, causing Budd-Chiari syndrome, are associated with a high mortality rate. Vascular involvement is more common in male patients than in female patients. Men and patients with a younger age of onset present with a more severe prognosis. In this case report, we describe a very rare cause of intrarenal arterial aneurysm's rupture with previous Budd-Chiari syndrome due to Behcet's disease and successful angiographic embolization of actively bleeding aneurysm. PMID:26491527

  6. Severe Henoch-Schönlein purpura with infliximab for ulcerative colitis

    PubMed Central

    Song, Yang; Shi, Yan-Hong; He, Chong; Liu, Chang-Qin; Wang, Jun-Shan; Zhao, Yu-Jie; Guo, Yan-Min; Wu, Rui-Jin; Feng, Xiao-Yue; Liu, Zhan-Ju

    2015-01-01

    Infliximab (IFX) is an anti-tumor necrosis factor chimeric antibody that is effective for treatment of autoimmune disorders such as Crohn’s disease and ulcerative colitis (UC). IFX is well tolerated with a low incidence of adverse effects such as infections, skin reactions, autoimmunity, and malignancy. Dermatological manifestations can appear as infusion reaction, vasculitis, cutaneous infections, psoriasis, eczema, and skin cancer. Here, we present an unusual case of extensive and sporadic subcutaneous ecchymosis in a 69-year-old woman with severe UC, partial colectomy and cecostomy, following her initial dose of IFX. The reaction occurred during infliximab infusion, and withdrawal of IFX led to gradual alleviation of her symptoms. We concluded that Henoch-Schönlein purpura, a kind of leukocytoclastic vasculitis, might have contributed to the development of the bruising. Although the precise mechanisms of the vasculitis are still controversial, such a case highlights the importance of subcutaneous adverse effects in the management of UC with IFX. PMID:26019477

  7. Severe Henoch-Schönlein purpura with infliximab for ulcerative colitis.

    PubMed

    Song, Yang; Shi, Yan-Hong; He, Chong; Liu, Chang-Qin; Wang, Jun-Shan; Zhao, Yu-Jie; Guo, Yan-Min; Wu, Rui-Jin; Feng, Xiao-Yue; Liu, Zhan-Ju

    2015-05-21

    Infliximab (IFX) is an anti-tumor necrosis factor chimeric antibody that is effective for treatment of autoimmune disorders such as Crohn's disease and ulcerative colitis (UC). IFX is well tolerated with a low incidence of adverse effects such as infections, skin reactions, autoimmunity, and malignancy. Dermatological manifestations can appear as infusion reaction, vasculitis, cutaneous infections, psoriasis, eczema, and skin cancer. Here, we present an unusual case of extensive and sporadic subcutaneous ecchymosis in a 69-year-old woman with severe UC, partial colectomy and cecostomy, following her initial dose of IFX. The reaction occurred during infliximab infusion, and withdrawal of IFX led to gradual alleviation of her symptoms. We concluded that Henoch-Schönlein purpura, a kind of leukocytoclastic vasculitis, might have contributed to the development of the bruising. Although the precise mechanisms of the vasculitis are still controversial, such a case highlights the importance of subcutaneous adverse effects in the management of UC with IFX. PMID:26019477

  8. A rare case of Behçet disease with generalised myositis, cardiomyositis and necrotising fasciitis.

    PubMed

    Ng, Felix; Chiong, Fabian Joon Kiong; Buchanan, Russell; Burrell, Louise M

    2016-01-01

    Behçet disease (BD) is a rare relapsing, multisystem vasculitis characterised by recurrent oral and genital ulcers, and uveitis. As an autoimmune small vessel vasculitis, BD can involve other organs including the skin, joints, nervous system, kidney and the gastrointestinal tract. This report describes a 40-year-old woman who presented with an uncommon feature of BD, namely myositis, and who went on to develop myocarditis and polymicrobial necrotising fasciitis. To the best of our knowledge, this is the first reported case of an immunocompromised-associated infection occurring in BD without concurrent immunosuppressive therapy. PMID:26740268

  9. A hidden giant: Wallenberg syndrome and aortal wall thickening as an atypical presentation of a giant cell arteritis

    PubMed Central

    Stengl, Katharina Luisa; Buchert, Ralph; Bauknecht, Hans; Sobesky, Jan

    2013-01-01

    We report a case of a 73-year-old woman with a brainstem stroke presenting as Wallenberg syndrome. By transoesophageal echocardiography and combined 18F-fluordeoxyglucose positron emission and CT (18F-FDG PET/CT), the diagnosis of large artery vasculitis owing to giant cell arteritis was confirmed. In the absence of classical clinical signs, the examination of the large extracranial vessels by ultrasound and 18F-FDG PET/CT played the key role in detecting a widespread vasculitis. PMID:23456154

  10. A hidden giant: Wallenberg syndrome and aortal wall thickening as an atypical presentation of a giant cell arteritis.

    PubMed

    Stengl, Katharina Luisa; Buchert, Ralph; Bauknecht, Hans; Sobesky, Jan

    2013-01-01

    We report a case of a 73-year-old woman with a brainstem stroke presenting as Wallenberg syndrome. By transoesophageal echocardiography and combined 18F-fluordeoxyglucose positron emission and CT (18F-FDG PET/CT), the diagnosis of large artery vasculitis owing to giant cell arteritis was confirmed. In the absence of classical clinical signs, the examination of the large extracranial vessels by ultrasound and 18F-FDG PET/CT played the key role in detecting a widespread vasculitis. PMID:23456154

  11. Necrotizing arteritis and spinal subarachnoid hemorrhage in Sjögren syndrome.

    PubMed

    Alexander, E L; Craft, C; Dorsch, C; Moser, R L; Provost, T T; Alexander, G E

    1982-06-01

    A 37-year-old woman with primary Sjögren syndrome developed mixed cryoglobulinemia and systemic vasculitis. Subarachnoid hemorrhage occurred as a result of necrotizing anterior spinal arteritis. Although rarely seen in mixed cryoglobulinemia, central nervous system complications have recently been documented in Sjögren syndrome. The patient's serum contained antibodies to the Ro(SSA) cytoplasmic antigen, and these antibodies were concentrated in the cryoglobulin fraction. Anti-Ro(SSA) antibodies are associated with the occurrence of vasculitis in patients with Sjögren syndrome, which suggests that the spinal arteritis and subarachnoid hemorrhage in this patient may have been directly related to the underlying connective tissue disorder. PMID:6126150

  12. Successful treatment of hemorrhagic bullous henoch-schönlein purpura with oral corticosteroid: a case report.

    PubMed

    Kocaoglu, Celebi; Ozturk, Ramazan; Unlu, Yasar; Akyurek, Fatma Tuncez; Arslan, Sukru

    2013-01-01

    Henoch-Schönlein purpura (HSP) is a vasculitis of small-sized blood vessels, resulting from immunoglobulin-A-mediated inflammation. It is the most common acute systemic vasculitis in childhood and mainly affects skin, gastrointestinal tract, joints, and kidneys. The characteristic rash of HSP consists of palpable purpuric lesions 2 to 10?mm in diameter concentrating in the buttocks and lower extremities. The occurrence of hemorrhagic bullae in children with HSP is rarely encountered. This report describes a 4.5-year-old female patient with HSP associated with hemorrhagic bullous lesions. PMID:23691409

  13. Churg-Strauss syndrome presenting with conjunctival and eyelid masses: a case report.

    PubMed

    Ameli, F; Phang, K S; Masir, N

    2011-12-01

    Churg-Strauss syndrome, a small and medium vessel vasculitis, was first described by Churg and Strauss in 1951. It is characterised by the presence of asthma, prominent tissue and blood eosinophilia, systemic vasculitis, and pulmonary and systemic necrotising allergic granulomas. Involvement of the skin, heart and gastrointestinal tract is well documented, but ocular presentation is unusual. We describe a 40-year-old lady who presented with recurrent upper eyelid swelling due to conjunctival lesions. Although she has chronic asthma, Churg-Strauss syndrome was never suspected. The diagnosis of Churg-Strauss syndrome was only made following histological examination of the conjunctival lesions. PMID:22390118

  14. Management of IRVAN syndrome with observation.

    PubMed

    Basha, Madhi; Brown, Gary C; Palombaro, Geremie; Shields, Carol L; Shields, Jerry A

    2014-01-01

    A 7-year-old girl with IRVAN (idiopathic retinal vasculitis, aneurysms, and neuroretinitis) syndrome was monitored for 9 years. The patient had symmetric multiple aneurysmal dilations, retinal vasculitis, and severe hard exudation in the macula bilaterally. Long-term visual acuity in her untreated right eye was 20/25. In the left eye, treated with laser panretinal photocoagulation and intravitreal vascular endothelial growth factor inhibitor therapy, visual acuity decreased to 20/200 from macular hard exudates and central fibrovascular tissue. Observation is a consideration in IRVAN syndrome if the vision remains good with hard exudation. PMID:24766157

  15. CanVasc recommendations for the management of antineutrophil cytoplasm antibody (ANCA)-associated vasculitides - Executive summary.

    PubMed

    McGeoch, Lucy; Twilt, Marinka; Famorca, Leilani; Bakowsky, Volodko; Barra, Lillian; Benseler, Susan; Cabral, David A; Carette, Simon; Cox, Gerald P; Dhindsa, Navjot; Dipchand, Christine; Fifi-Mah, Aurore; Goulet, Michele; Khalidi, Nader; Khraishi, Majed M; Liang, Patrick; Milman, Nataliya; Pineau, Christian A; Reich, Heather; Samadi, Nooshin; Shojania, Kam; Taylor-Gjevre, Regina; Towheed, Tanveer E; Trudeau, Judith; Walsh, Michael; Yacyshyn, Elaine; Pagnoux, Christian

    2015-01-01

    The Canadian Vasculitis research network (CanVasc) is composed of physicians from different medical specialties, including rheumatology and nephrology and researchers with expertise in vasculitis. One of its aims was to develop recommendations for the diagnosis and management of antineutrophil cytoplasm antibody (ANCA)-associated vasculitides in Canada. This executive summary features the 19 recommendations and 17 statements addressing general AAV diagnosis and management, developed by CanVasc group based on a synthesis of existing international guidelines, other published supporting evidence and expert consensus considering the Canadian healthcare context. PMID:26557369

  16. Behcet's Disease: New Concepts in Cardiovascular Involvements and Future Direction for Treatment

    PubMed Central

    Owlia, M. B.; Mehrpoor, G.

    2012-01-01

    Behcet's disease (BD) is the only systemic vasculitis involving both arteries and vein in any sizes. It frequently encounters in rheumatology clinics. It has some major morbidities and even fatal outcomes in some cases. The aim of this paper is to analyze the main concepts on pathophysiology and treatment options in BD, focusing on cardiovascular aspects, thrombosis, and potential future treatment. PMID:22530146

  17. Confusing presentation of chaetomium brain abscess.

    PubMed

    Waqas, Muhammad; Waheed, Shahan; Mangrio, Salman Ahmed; Rashid, Sana; Qadeer, Mohsin; Bari, Ehsan

    2014-12-01

    We put forth the case of a young immunocompetent female who presented with fever and left basal ganglia bleed secondary to vasculitis. After 2 weeks of prednisolone therapy, she continued to deteriorate. MRI scan revealed enhancement of previous hematoma, pus was aspirated surgically and fungal culture grew Chaetomium. PMID:24977715

  18. BRIEF REPORT: ODOCOILEUS HEMIONUS DEER ADENOVIRUS IS RELATED TO THE MEMBERS OF ATADENOVIRUS GENUS

    Technology Transfer Automated Retrieval System (TEKTRAN)

    The Odocoileus hemionus deer adenovirus (OdAdV-1) causes systemic and local vasculitis and proves extremely lethal for mule deer. To characterize the virus, part of the genome flanking the fiber gene was cloned and sequenced. The sequence revealed two open-reading frames that mapped to pVIII hexon...

  19. Autologous Peripheral Blood Stem Cell Transplantation in Patients With Life Threatening Autoimmune Diseases

    ClinicalTrials.gov

    2005-06-23

    Purpura, Schoenlein-Henoch; Graft Versus Host Disease; Anemia, Hemolytic, Autoimmune; Rheumatoid Arthritis; Churg-Strauss Syndrome; Hypersensitivity Vasculitis; Wegener's Granulomatosis; Systemic Lupus Erythematosus; Giant Cell Arteritis; Pure Red Cell Aplasia; Juvenile Rheumatoid Arthritis; Polyarteritis Nodosa; Autoimmune Thrombocytopenic Purpura; Takayasu Arteritis

  20. Multiorgan dysfunction syndrome secondary to joint supplement overdosage in a dog

    PubMed Central

    Nobles, Irma J.; Khan, Safdar

    2015-01-01

    A 5-year-old spayed female Bernese mountain dog, with a chief complaint of vomiting and melena ingested approximately 200 nutritional joint supplement tablets. Despite aggressive therapy, the patient developed a coagulopathy, pancreatitis, peritonitis, acute kidney injury, and was euthanized. Postmortem examination revealed myocardial necrosis, pneumonia, centrilobular hemorrhage and necrosis of the liver, vasculitis, and acute tubular necrosis. PMID:25829554

  1. Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with Polyangiitis.

    PubMed

    Ali, Robert; Baldeo, Candice; Onyenekwe, Jesse; Lala, Roshan; Landa, Cristian; Siddiqi, Anwer

    2015-01-01

    Granulomatosis with polyangiitis (GPA), previously termed Wegener's Granulomatosis, is an autoimmune small vessel vasculitis which is highly associated with antineutrophil cytoplasmic antibodies (ANCA) and has varied clinical manifestations. Diagnosis hinges on identifying a combination of clinical features of systemic vasculitis, positive ANCA serology, and histological evidence of necrotizing vasculitis, necrotizing glomerulonephritis, or granulomatous inflammation from a relevant organ biopsy. The American College of Rheumatology has also developed a classification criteria focusing specifically on nasal or oral inflammation, abnormal chest radiograph, and abnormal urinary sediment, along with granulomatous inflammation, which helps to distinguish GPA from other forms of systemic vasculitis. In the case presented below, the diagnosis of GPA was delayed as the patient had a concomitant atypical endobronchial carcinoid which predisposed to postobstructive pneumonia. Fortunately, the papular lesions that developed across her lower limbs prompted further investigations. The return of appropriate serology coincided with progression to alveolar hemorrhage, offering a more complete clinical picture, and when she responded to the combination of steroid, cyclophosphamide, and plasma exchange, the diagnosis of GPA was cinched. PMID:26347844

  2. [A cause of palatal necrosis not to ignore].

    PubMed

    Boulagnon, Camille; Kovacs, Ovidiu-Bujor; Patey, Martine

    2015-04-01

    We report a case of pseudotumoral nasal septum and hard palate perforation in a 42-years-old man. The diagnosis retained after differential diagnosis exclusion was necrotic midfacial lesion due to chronic inhalation of cocaine. This condition can mimic vasculitis, primary tumors and granulomatous infections. Differential diagnosis and pathophysiology of this condition will be discussed in this anatomo-clinical case. PMID:25778762

  3. Comparison of PR3-ANCA and MPO-ANCA Epitope Specificity upon Disease Relapse

    EPA Science Inventory

    BACKGROUND Relapse is a major clinical problem in ANCA vasculitis that causes increased morbidity and mortality. Compared to MPO-ANCA patients, patients with PR3-ANCA run a significantly increased risk of experiencing relapses. We hypothesized that a relapsing patient is produ...

  4. POLYNEUROPATHY + NEURONOPATHY: Points in Differential Diagnosis 1. Selective Functional Involvement

    E-print Network

    Baloh, Bob

    Toxic: Pb; Dapsone; Botulism; Tick Acute: Porphyria; Axonal GBS (AMAN); Polio; West Nile Diabetic distally in the lower extremities Extraocular muscle Botulism Diabetes Miller-Fisher Diphtheria Rule out Acute Immune: GBS; Vasculitis Toxic: Botulism; Thallium; Vacor; Drugs; Org-phos Infections: Tick; Lyme

  5. Emphysema in nonsmokers: alpha 1-antitrypsin deficiency and other causes.

    PubMed

    Lee, Pyng; Gildea, Thomas R; Stoller, James K

    2002-12-01

    Nonsmokers with signs of emphysema at an earlier age than is typical for emphysema deserve a workup for one of the less common causes of emphysema, which include alpha 1-antitrypsin deficiency, connective tissue diseases, hypocomplementemic urticarial vasculitis syndrome, intravenous drug use, human immunodeficiency virus infection, and several rare metabolic disorders. PMID:12546267

  6. Polyarteritis nodosa presented as a dilatation of the intrahepatic bile duct.

    PubMed

    Choi, Han Lim; Sung, Ro Hyun; Kang, Min Ho; Jeon, Hyun Jeong; Yun, Hyo Yung; Jang, Lee Chan; Choi, Jae Woon; Song, Young Jin; Ryu, Dong Hee

    2014-11-01

    Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis of small- and medium-sized arteries in multiorgan systems. PAN may affect the gastrointestinal tract in 14%-65% of patients, but rarely involves the biliary tract and liver. We describe a patient without underlying disease who was diagnosed with PAN during resection of the gallbladder and liver. PMID:25368854

  7. Diagnosis and classification of eosinophilic granulomatosis with polyangiitis (formerly named Churg-Strauss syndrome).

    PubMed

    Mouthon, Luc; Dunogue, Bertrand; Guillevin, Loïc

    2014-01-01

    Recently, a group of experts in the field suggested to rename Churg-Strauss syndrome as eosinophilic granulomatosis with polyangiitis (EGPA). This condition, first described in 1951, is a rare small- and medium-sized-vessel vasculitis characterized by an almost constant association with asthma and eosinophilia, and, by the presence of anti-myeloperoxidase (MPO) antineutrophil cytoplasm antibodies (ANCA) in 30-38% of the patients. Vasculitis typically develops in a previously asthmatic and eosinophilic middle-aged patient. Asthma is severe, associated with eosinophilia and extrapulmonary symptoms. Most frequently EGPA involves the peripheral nerves and skin. Other organs, however, may be affected and must be screened for vasculitis, especially those associated with a poorer prognosis, such as the heart, kidney and gastrointestinal tract, as assessed by the recently revised Five-Factor Score (FFS). Recent insights, particularly concerning clinical differences associated with ANCA status, showed that EGPA patients might constitute a heterogeneous group. Thus, EGPA patients with anti-MPO ANCA suffered more, albeit not exclusively, from vasculitis symptoms, such as glomerulonephritis, mononeuritis multiplex and alveolar hemorrhage, whereas ANCA-negative patients more frequently develop heart involvement. This observation led to the hypothesis that EGPA might be divided into different clinical and pathophysiological subtypes, which could be managed better with more specifically adapted therapies. For now, EGPA treatment still relies mainly on corticosteroids and, when necessary for patients with poorer prognoses, combined immunosuppressant drugs, especially cyclophosphamide. Overall survival of EGPA patients is good, despite not uncommon relapses. PMID:24530234

  8. Wegener’s granulomatosis mimicking inflammatory bowel disease and presenting with chronic enteritis

    PubMed Central

    Shahedi, Kamyar; Hanna, Ramy Magdy; Melamed, Oleg; Wilson, James

    2013-01-01

    Wegener’s granulomatosis, also known as anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, is a small vessel vasculitis with primarily pulmonary, renal, and sinus disease manifestations. The prevalence of Wegener’s granulomatosis is three cases per 100,000 patients. Cardiovascular, neurologic, cutaneous, and joint manifestations have been reported in many case reports and case series. Gastrointestinal manifestations are less noted in Wegener’s granulomatosis, although they have been previously reported in the form of intestinal perforation and intestinal ischemia. Additionally, there are characteristic findings of vasculitis that are noted with active Wegener’s granulomatosis of the small bowel. We report a case of an elderly patient who presented with weight loss, diarrhea, and hematochezia. His symptoms were chronic and had lasted for more than 1 year before diagnosis. Inflammatory bowel disease or chronic enteritis due to Salmonella arizonae because of reptile exposure originally were suspected as etiologies of his presentation. The findings of proteinuria, renal failure, and pauci-immune glomerulonephritis on renal biopsy, in conjunction with an elevated c-ANCA titer, confirmed the diagnosis of Wegener’s granulomatosis with associated intestinal vasculitis. This case demonstrates an atypical presentation of chronic duodenitis and jejunitis secondary to Wegener’s granulomatosis, which mimicked inflammatory bowel disease. PMID:24124396

  9. Phenomics in Autoimmune and Inflammatory Diseases

    ClinicalTrials.gov

    2015-09-04

    Healthy Volunteer; Rheumatoid Arthritis; Ankylosing Spondylitis; Systemic Lupus Erythematosus/Antiphospholipid Syndrome; FMF; Cryopyrin-Associated Periodic Syndromes /TNF-receptor Associated Periodic Syndrome; Vasculitis; Uveitis; Myositis; Crohn's Disease; Ulcerative Rectocolitis; Type 1 Diabetes; Unclassified IAD Knee and/or Hip Arthritis, Muscular Dystrophy

  10. Cutaneous zygomycosis caused by Saksenaea vasiformis in a diabetic patient.

    PubMed Central

    Bearer, E A; Nelson, P R; Chowers, M Y; Davis, C E

    1994-01-01

    A 49-year-old woman with diabetes mellitus rapidly developed necrotizing cellulitis with fat necrosis and vasculitis after minor trauma to the right arm. Zygomycosis was diagnosed histologically. The lesion responded to aggressive debridement, amphotericin B, and normalization of blood glucose. Cultures yielded structures characteristic of Saksenaea vasiformis only after transfer to saline agar. Images PMID:7929783

  11. OVINE HERPESVIRUS 2 MRNA EXPRESSION IN CATTLE AND BISON WITH MALIGNANT CATARRHAL FEVER

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Malignant catarrhal fever (MCF) is a fulminant disease of certain susceptible ruminants caused by ovine herpesvirus 2 (OvHV-2). The characteristic lesions of MCF include lymphoproliferation, vasculitis and mucosal ulceration. The pathogenesis of these lesions is very poorly understood, but is most ...

  12. Spontaneous Superficial Femoral Artery Pseudoaneurysm in Behcet's Disease

    PubMed Central

    Sendil, Selin; Sayin, Omer Ali; Barburoglu, Mehmet; Gok, Emre; Turkyilmaz, Gulsum; Basaran, Murat; Alpagut, Ufuk; Dayioglu, Enver

    2014-01-01

    Behcet's disease is an autoimmune multisystemic disorder on vasculitis base. Cardiovascular involvement is the most important predictor of morbidity and mortality. The treatment should be planned carefully for pathologies requiring interventions. In our report, we present a 45-year-old patient with spontaneous superficial femoral artery pseudoaneurysm, our treatment strategy, and circumstances we faced. PMID:24822070

  13. Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with Polyangiitis

    PubMed Central

    Ali, Robert; Baldeo, Candice; Onyenekwe, Jesse; Lala, Roshan; Landa, Cristian; Siddiqi, Anwer

    2015-01-01

    Granulomatosis with polyangiitis (GPA), previously termed Wegener's Granulomatosis, is an autoimmune small vessel vasculitis which is highly associated with antineutrophil cytoplasmic antibodies (ANCA) and has varied clinical manifestations. Diagnosis hinges on identifying a combination of clinical features of systemic vasculitis, positive ANCA serology, and histological evidence of necrotizing vasculitis, necrotizing glomerulonephritis, or granulomatous inflammation from a relevant organ biopsy. The American College of Rheumatology has also developed a classification criteria focusing specifically on nasal or oral inflammation, abnormal chest radiograph, and abnormal urinary sediment, along with granulomatous inflammation, which helps to distinguish GPA from other forms of systemic vasculitis. In the case presented below, the diagnosis of GPA was delayed as the patient had a concomitant atypical endobronchial carcinoid which predisposed to postobstructive pneumonia. Fortunately, the papular lesions that developed across her lower limbs prompted further investigations. The return of appropriate serology coincided with progression to alveolar hemorrhage, offering a more complete clinical picture, and when she responded to the combination of steroid, cyclophosphamide, and plasma exchange, the diagnosis of GPA was cinched. PMID:26347844

  14. Naturally occurring sheep-associated malignant catarrhal fever in North American pigs

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Two cases of sheep-associated malignant catarrhal fever (MCF) in pigs were diagnosed on a small farm in New York State, and in Kentucky, U.S.A. In both cases initial diagnosis was based on histopathological changes representing typical lymphoproliferative vasculitis in multiple tissues of the affect...

  15. Giant cell arteritis - a series of cases and review of literature.

    PubMed

    Sehra, Shiv Tej; Setty, Arathi

    2013-01-01

    Giant cell arteritis is the most frequently occurring primary vasculitis in European and North American adults over the age of 50 years. It usually presents with constitutional symptoms like fatigue, malaise and fevers with a temporal headache. We present a series of three interesting cases of the disease, discuss the differential diagnosis and review the literature on the same. PMID:25409672

  16. Colonic localization of indium-111 labeled leukocytes in active Behcet's disease

    SciTech Connect

    Harre, R.G.; Conrad, G.R.; Seabold, J.E.

    1988-06-01

    A patient with known Behcet's disease demonstrated intense colonic localization of In-111 labeled leukocytes. Gastrointestinal involvement had not been previously manifested, but extensive colonic inflammation was documented by endoscopy. This case illustrates the utility of In-111 labeled leukocyte imaging for detecting active bowel disease in a debilitated patient with documented Behcet's vasculitis.

  17. Pancreatic mass as an initial manifestation of polyarteritis nodosa: A case report and review of the literature

    PubMed Central

    Yokoi, Yoshihiro; Nakamura, Ippei; Kaneko, Takeshi; Sawayanagi, Tomoki; Watahiki, Youichi; Kuroda, Makoto

    2015-01-01

    Classic polyarteritis nodosa (PAN) that targets medium-sized muscular arteries and microscopic polyangiitis (MPA), characterized by inflammation of small-caliber vessels and the presence of circulating myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA), are distinct clinicopathological entities of systemic vasculitis. A 66-year-old woman presented with fever, cholestasis and positive MPO-ANCA. Radiological examination showed a pancreatic mass compressing the bile duct. Therefore, we performed pancreatoduodenectomy. Histopathological examination revealed that necrotizing vasculitis predominantly affecting the medium-sized vessels, spared arterioles or capillaries in the pancreas, a finding consistent with PAN. Unexpectedly, renal biopsy revealed small-caliber vasculitis and glomerulonephritis, supporting MPA. The initial manifestation of a pancreatic mass associated with vasculitis has only been reported in 7 articles. Its diagnosis is challenging because no reliable clinico-radiological findings have been observed. Clinicians should be aware of such cases and early diagnosis followed by immunosuppression is mandatory. Our findings may reflect a polyangiitis overlap syndrome coexisting between pancreatic PAN and renal MPA. PMID:25624739

  18. State of the Art in the Treatment of Systemic Vasculitides

    PubMed Central

    Luqmani, Raashid Ahmed

    2014-01-01

    Anti-neutrophil cytoplasm antibodies (ANCA) are associated with small vessel vasculitides (AASV) affecting the lungs and kidneys. Structured clinical assessment using the Birmingham Vasculitis Activity Score and Vasculitis Damage Index should form the basis of a treatment plan and be used to document progress, including relapse. Severe disease with organ or life threatening manifestations needs cyclophosphamide or rituximab, plus high dose glucocorticoids, followed by lower dose steroid plus azathioprine, or methotrexate. Additional plasmapheresis is effective for very severe disease, reducing dialysis dependence from 60 to 40% in the first year, but with no effect on mortality or long-term renal function, probably due to established renal damage. In milder forms of ANCA-associated vasculitis, methotrexate, leflunomide, or mycophenolate mofetil are effective. Mortality depends on initial severity: 25% in patients with renal failure or severe lung hemorrhage; 6% for generalized non-life threatening AASV but rising to 30–40% at 5?years. Mortality from GPA is four times higher than the background population. Early deaths are due to active vasculitis and infection. Subsequent deaths are more often due to cardiovascular events, infection, and cancer. We need to improve the long-term outcome, by controlling disease activity but also preventing damage and drug toxicity. By contrast, in large vessel vasculitis where mortality is much less but morbidity potentially greater, such as giant cell arteritis (GCA) and Takayasu arteritis, therapeutic options are limited. High dose glucocorticoid results in significant toxicity in over 80%. Advances in understanding the biology of the vasculitides are improving therapies. Novel, mechanism based therapies such as rituximab in AASV, mepolizumab in eosinophilic granulomatosis with polyangiitis, and tocilizumab in GCA, but the lack of reliable biomarkers remains a challenge to progress in these chronic relapsing diseases. PMID:25352843

  19. Churg-Strauss syndrome.

    PubMed

    Greco, Antonio; Rizzo, Maria Ida; De Virgilio, Armando; Gallo, Andrea; Fusconi, Massimo; Ruoppolo, Giovanni; Altissimi, Giancarlo; De Vincentiis, Marco

    2015-04-01

    Churg-Strauss syndrome (CSS), alternatively known as eosinophilic granulomatosis with polyangiitis (EGPA), was first described in 1951 by Churg and Strauss as a rare disease characterized by disseminated necrotizing vasculitis with extravascular granulomas occurring exclusively among patients with asthma and tissue eosinophilia. EGPA is classified as a small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs) and the hypereosinophilic syndromes (HESs) in which vessel inflammation and eosinophilic proliferation are thought to contribute to organ damage. Although still considered an idiopathic condition, EGPA is classically considered a Th2-mediated disease. Emerging clinical observations provide compelling evidence that ANCAs are primarily and directly involved in the pathogenesis of AASVs, although recent evidence implicates B cells and the humoral response as further contributors to EGPA pathogenesis. EGPA has traditionally been described as evolving through a prodromic phase characterized by asthma and rhino-sinusitis, an eosinophilic phase marked by peripheral eosinophilia and organ involvement, and a vasculitic phase with clinical manifestations due to small-vessel vasculitis. The American College of Rheumatology defined the classification criteria to distinguish the different types of vasculitides and identified six criteria for EGPA. When four or more of these criteria are met, vasculitis can be classified as EGPA. The French Vasculitis Study Group has identified five prognostic factors that make up the so-called five-factor score (FFS). Patients without poor prognosis factors (FFS=0) have better survival rates than patients with poor prognosis factors (FFS?1). The treatment of patients with CSS must be tailored to individual patients according to the presence of poor prognostic factors. A combination of high-dose corticosteroids and cyclophosphamide is still the gold standard for the treatment of severe cases, but the use of biological agents such as rituximab or mepolizumab seems to be a promising therapeutic alternative. PMID:25500434

  20. Alternative Complement Pathway Activation Products in Urine and Kidneys of Patients with ANCA-Associated GN

    PubMed Central

    Gou, Shen-Ju; Yuan, Jun; Wang, Chen; Zhao, Ming-Hui

    2013-01-01

    Summary Background and objectives Previous study revealed that complement activation products of the alternative pathway could be detected in renal specimens of human ANCA-associated vasculitis. The current study aimed to investigate the clinical and pathologic significance of complement activation products in the urine and kidneys of patients with ANCA-associated vasculitis. Design, setting, participants, & measurements Renal biopsy specimens from 29 patients with ANCA-associated vasculitis diagnosed at Peking University First Hospital from January of 2008 to December of 2010 were randomly collected. Urine samples from 27 of 29 patients in active stage and 22 ANCA-associated vasculitis patients in complete remission who were independent of the above-mentioned 29 patients were collected. Urine samples from 28 patients with lupus nephritis and 25 healthy individuals were also collected. The renal deposition of Bb, C3d, and C5b-9 were detected by immunohistochemistry. The urinary levels of Bb, C3a, C5a, and soluble C5b-9 were determined by ELISA. Results The deposition, measured by the mean optical density of Bb, which is an alternative complement pathway marker, in glomeruli correlated with the proportion of total crescents (r=0.50, P=0.006), the extent of interstitial infiltrate (r=0.59, P=0.001), interstitial fibrosis (r=0.45, P=0.01), and tubular atrophy (r=0.55, P=0.002), whereas it correlated inversely with the proportion of normal glomeruli (r=?0.49, P=0.008). The urinary levels of Bb, C3a, C5a, and soluble C5b-9 were all significantly higher in active compared with remission stage. The urinary levels of Bb in patients with active ANCA-associated vasculitis correlated with the serum creatinine (r=0.56, P=0.002) and correlated inversely with the proportion of normal glomeruli in renal specimens (r=?0.49, P=0.009). Conclusions The present study provides additional evidence that complement activation through the alternative pathway occurred in the development of ANCA-associated vasculitis. The renal deposition of Bb and urinary Bb levels were associated with the severity of renal injury. PMID:24115193

  1. Comparative aspects of murine proteinase 3.

    PubMed

    Relle, Manfred; Thomaidis, Thomas; Galle, Peter R; Schwarting, Andreas

    2011-08-01

    The neutrophilic granule protein proteinase 3 (PR3) is the preferred target antigen of anti-neutrophil cytoplasmic antibodies (ANCA) found in the serum of patients with Wegener's granulomatosis, a systemic small-vessel vasculitis. Due to the lack of data concerning the murine homologue of human PR3, we assessed the neutrophil marker polypeptide PR3 in mice by generating a murine-specific PR3 antibody. In contrast to humans, peripheral blood leukocytes are not the main resource of murine PR3. Interestingly, we could show that the mouse bone marrow is the main PR3 source, indicating that it is a large reservoir for functional neutrophils. This pool of neutrophils could be rapidly mobilized after injection of IL-8. The development of the new PR3 antibody provides a new tool for studying the maturation processes of the murine hematopoietic system and will also support the generation of infectious disease or vasculitis mouse models. PMID:21120490

  2. The spectrum of lung involvement in collagen vascular-like diseases following allogeneic hematopoietic stem cell transplantation: report of 6 cases and review of the literature.

    PubMed

    Bergeron, Anne; Bengoufa, Djaouida; Feuillet, Séverine; Meignin, Véronique; Peffault de Latour, Régis; Rybojad, Michel; Gossot, Dominique; Azoulay, Elie; Socié, Gérard; Tazi, Abdellatif

    2011-03-01

    Multisystem autoimmune diseases occurring after allogeneic hematopoietic stem cell transplantation are infrequent, late-onset manifestations that resemble well-defined collagen vascular disorders. Because the lung is frequently involved in the course of connective tissue disorders, we focused on lung manifestations occurring in autoimmune diseases following allogeneic stem cell transplantation. In the present series, we report 6 patients with systemic lupus erythematous, mixed connective tissue disease, Sjögren syndrome, polymyositis, and ANCA-positive vasculitis who presented with a spectrum of pulmonary manifestations affecting the airways, lung parenchyma, and probably respiratory muscles. We identified 3 different histopathologic patterns of interstitial pneumonia consistent with the underlying autoimmune disorder: lymphocytic interstitial pneumonia and non-specific interstitial pneumonia in 2 patients with Sjögren syndrome and diffuse alveolar damage in 1 patient with ANCA-positive vasculitis. These lung manifestations had poor prognoses. Further studies are needed to determine the optimal therapy for these complications. PMID:21358437

  3. Progressive Hemifacial Atrophy With Contralateral Uveitis: A Case Report

    PubMed Central

    Ayyildiz, Onder; Ayyildiz, Simel; Durukan, Ali Hakan; Sobaci, Gungor

    2015-01-01

    Introduction: Progressive hemifacial atrophy, known as Parry-Romberg syndrome (PRS), was first described by Parry in 1825. There is a progressive atrophy of facial tissues including skin, bones and muscles. Ophthalmic disorders are common and include keratitis, uveitis, cataract, ipsilateral enophthalmos, optic neuritis, retinal vasculitis and scleral melting. Case Presentation: We describe a patient with progressive hemifacial atrophy at right facial side who developed granulomatous uveitis and periferic retinal vasculitis in his left eye. We started topical and systemic steroid therapy. Uveitic reaction had regressed almost entirely after a 3-month steroid treatment. Conclusions: The individuals should have multidisciplinary approach for the variety of disorders to maintain the appropriate treatment for a better appearance of the patients. PMID:26473067

  4. [Primary manifestation of Wegener's granulomatosis of the prostate].

    PubMed

    Mundinger, A; Pumpe, K; Grosser, G; Herbst, E W; Grotz, W; Kröpelin, T

    1989-07-01

    In approximately 2-7% of patients with Wegener's granulomatosis involvement of the prostate has been demonstrated histologically. This usually comes about quite late in the course of disease, after generalization of the granulomatous vasculitis from respiratory tract to kidneys and other organs. The patient we present had a highly atypical first manifestation of Wegener's granulomatosis in the prostate, generalized vasculitis not developing until later. When a biopsy shows unclear granulomatous changes in the prostate, a test for anticytoplasmatic antibodies should be performed in addition to the conventional serologic and cultural examinations. Anticytoplasmatic antibodies are highly specific for the diagnosis of active Wegener's granulomatosis. Early diagnosis and immediate initiation of immunosuppressive therapy with cyclophosphamide and corticosteroids can prevent or limit organ damage and improve the prognosis in Wegener's granulomatosis. PMID:2474881

  5. A Clinical Picture of the Visual Outcome in Adamantiades-Behçet's Disease

    PubMed Central

    Figus, Michele; Posarelli, Chiara; Albert, Timothy G.; Talarico, Rosaria; Nardi, Marco

    2015-01-01

    Adamantiades-Behçet's disease is a multisystemic vasculitis with multiorgan involvement. Ocular disorders occur often in this syndrome typically in the form of a relapsing-remitting panuveitis and vasculitis and can lead to blindness as one of its most disabling complications if left untreated. There are known risk factors related with the worst visual prognosis, which require early and intensive treatment in order to obtain a rapid suppression of inflammation and to prevent future relapses. The management strategy to avoid vision loss and blindness currently involves the use of local and systemic drugs including steroids and immunosuppressive and biologic agents. This review aims to demonstrate how the introduction and the use of biologic agents improves the visual outcome of patients with Adamantiades-Behçet's disease. PMID:26558256

  6. Adult dermatomyositis with bleeding ulcer in the pharynx.

    PubMed

    Kusano, Junko; Takahashi, Yuka; Misaki, Yoshikata; Murai, Norihiko

    2014-01-01

    Dermatomyositis (DM) is one of the idiopathic inflammatory myopathies caused by complement-mediated vasculopathy or vasculitis in the muscle. Although the gastrointestinal (GI) mucosa has been reported to be involved as a result of vasculitis or vasculopathy, ulceration in the pharynx is a rare manifestation of DM. A 54-year-old woman complaining of muscle weakness in the extremities, low-grade fever, and dysphagia was diagnosed as having DM. Despite medical treatment with corticosteroids and immunosuppressive agents, her DM progressed rapidly, leading to exacerbation of the dysphagia. About 3 weeks after undergoing tracheostomy as a preventive measure against aspiration, the patient developed intractable respiratory tract hemorrhage. Repeated laryngoendoscopy revealed a bleeding ulceration in the pharynx that required hemostasis with electric cautery under general anesthesia. No bleeding recurred thereafter. Histopathologically, the pharynx exhibited nonspecific inflammatory cell infiltration in the muscle tissue. This rare manifestation may be considered in cases of DM with unexplainable airway bleeding. PMID:25328739

  7. [Skin diseases associated with chronic hepatitis C].

    PubMed

    Podányi, B; Lengyel, G; Hársing, J; Becker, K; Horváth, A

    1998-11-01

    The authors are discussing hepatic and extrahepatic pathologic processes caused by hepatitis C virus (HCV) infection and they focus their interest to the skin disorders appearing in the presence of chronic, active HCV infections. The trigger of the immunologic processes leading to dermatologic manifestations are the activated T cells (CD8 + cytotoxic T lymphocytes), cytokins, and also the expansion of certain B cells. Pathologic immunologic phenomena may initiate various dermatologic manifestations. Immunoglobulins, immuncomplexes generated by the disease itself are manifested as various forms of cutan vasculitis. In the present series of patients (pts), HCV related skin disorders known from the literature were diagnosed in eleven cases and they were representing 7 different disease entities. These were palpable purpura (3 pts), urticaria, prurigo and alopecia areata (2-2 pts), lichen ruber planus, pruritus and vitiligo (1-1 patient respectively). The case reports of 2 pts, one with palpable purpura (vasculitis purpurica), one with prurigo and vitiligo are presented in details. PMID:9842236

  8. Biotherapies in Behçet's disease.

    PubMed

    Comarmond, Cloé; Wechsler, Bertrand; Bodaghi, Bahram; Cacoub, Patrice; Saadoun, David

    2014-07-01

    Behçet's disease (BD) is a systemic large-vessel vasculitis characterized by a wide clinical spectrum including recurrent oral and genital ulcerations, uveitis, vascular, neurological, articular, renal and gastrointestinal manifestations. Therapeutic management of BD depends on the clinical presentation and organ involved. Although colchicine, nonsteroidal antiinflammatory agents and topical treatments with corticosteroids are often sufficient for mucocutaneous and joint involvements, more aggressive approach with immunosuppressive agents is warranted for severe manifestations such as posterior uveitis, retinal vasculitis, vascular, and neurological and gastrointestinal involvements. However, some patients still have refractory disease, relapse, sight threatening eye disease, or irreversible organ damage. Recent improvements in the understanding of the pathogenic mechanisms have led to the identification of potential targets and future biological therapies for BD. In contrast to current non-specific immunosuppressive agents, the emergence of biotherapies provides the possibility of interfering with specific pathogenic pathways. Novel targeted biotherapies might be used in the future for BD. PMID:24473176

  9. Cardiac manifestations in Behcet's disease

    PubMed Central

    Demirelli, Selami; Degirmenci, Husnu; Inci, Sinan; Arisoy, Arif

    2015-01-01

    Summary Behcet's disease (BD) is a chronic inflammatory disorder, with vasculitis underlying the pathophysiology of its multisystemic effects. Venous pathology and thrombotic complications are hallmarks of BD. However, it has been increasingly recognised that cardiac involvement and arterial complications are also important aspects of the course of the disease. Cardiac lesions include pericarditis, endocarditis, intracardiac thrombosis, myocardial infarction, endomyocardial fibrosis, and myocardial aneurysm. Treatment of cardiovascular involvement in BD is largely empirical, and is aimed towards suppressing the vasculitis. The most challenging aspect seems to be the treatment of arterial aneurysms and thromboses due to the associated risk of bleeding. When the prognosis of cardiac involvement in BD is not good, recovery can be achieved through oral anticoagulation, immunosuppressive therapy, and colchicine use. In this review, we summarise the cardiovascular involvement, different manifestations, and treatment of BD. PMID:25984424

  10. Acute Hemorrhagic Edema of Infancy; a Report of Five Iranian Infants and Review of the Literature

    PubMed Central

    Moradinejad, Mohammad-Hassan; Entezari, Pegah; Mahjoub, Fatemeh; Ziaee, Vahid

    2011-01-01

    Background Acute hemorrhagic edema of infancy (AHEI) is a benign self limiting leukocytoclastic vasculitis in young children. Serious complications, e.g. renal and gastrointestinal involvement, are not usually detected in AHEI patients. Case Presentation We report five patients with AHEI. Our patients were 17 to 21 months old. One patient presented with gastrointestinal bleeding due to this syndrome, the other one experienced second attack and scrotal edema due to epididymo-orchitis, while the third patient had renal involvement as hematuria and the other one had bilateral auricular chondritis. One of our cases was a typical case of AHEI without any complications, so a skin biopsy was not necessary. In this study, we describe the symptoms, probable triggering factors and treatment of choice for each patient. Conclusion Although AHEI is a childhood vasculitis with no impairment of the general condition, some organ involvements such as gastrointestinal, renal or scrotal lesions and rarely chondritis are probable in these patients. PMID:23056774

  11. Evaluation of 10-Year Experience of Wegener's Granulomatosis in Iranian Children

    PubMed Central

    Tahghighi, Fatemeh; Moradinejad, Mohamad-Hassan; Aghighi, Yahya; Shiari, Reza; Raeeskarami, Seyed-Reza; Salehzadeh, Farhad; Javadi, Vadood

    2013-01-01

    Wegener's granulomatosis or granulomatosis polyangiitis (GPA) is an uncommon chronic systemic vasculitis in children. The aim of this study was to describe pediatric patients with GPA in Iran. We studied records of all patients with GPA diagnosis who were referred to all Iranian Pediatric Rheumatology Division from 2002 to 2011. A total of 11 patients (5 females and 6 males) enrolled in this study. In children less than 15 years old, the prevalence of GPA is 0.6 per million. The mean age of GPA diagnosis was 11 years and average delay diagnosis was 20 months. Mortality rate was 18.1% due to pulmonary vasculitis and infection. The most common organ system involvement was upper and lower respiratory tract involvement (81.8% and 63.9%, resp.). Other common manifestations were renal (36.3), skin (27.2%), and eye involvement (18.2%). PMID:23956873

  12. [Panuveitis associated with papillary carcinoma of the thyroid].

    PubMed

    Pierru, A; Tieulie, N; Gastaud, P; Baillif, S

    2013-12-01

    Ocular involvement secondary to thyroid carcinomas is uncommon. Uveal metastasis may occur. More rarely, they can be responsible for paraneoplastic syndromes. We report the case of a 64-year-old woman who presented with a severe bilateral panuveitis with venous vasculitis associated with hyperthyroidism from a multinodular goiter, complicated by papillary carcinoma. Systemic steroid therapy was initiated; ocular symptoms resolved completely after total thyroidectomy. Other causes of panuveitis with venous vasculitis were ruled out. This is the first reported case of panuveitis associated with papillary thyroid carcinoma. The occurrence of the ocular symptoms with hyperthyroidism and their remission after surgery supports the possibility that this association may not be coincidental. A paraneoplastic phenomenon is suspected. PMID:24211307

  13. F-18 FDG PET/CT Findings of a Patient with Takayasu Arteritis Before and After Therapy

    PubMed Central

    Sa?er, Sait; Y?lmaz, Sabire; Özhan, Meftune; Halaç, Metin; Ergül, Nurhan; Çiftci, Hediye; Çermik, T. Fikret

    2012-01-01

    Vasculitis is defined as inflammation and necrosis with leukocytic infiltration of the blood vessel wall. Takayasu arteritis is a chronic inflammatory arteritis that primarily involves the aorta and its main branches. A 64-year-old female patient with a 2-month history of fever of unknown origin was presented to our clinic for F-18 FDG PET/CT imaging. Baseline PET/CT images demonstrated intense F-18 FDG uptake in the aorta, bilateral subclavian and brachiocephalic arteries consistent with Takayasu arteritis. After 2 months of immunosuppressive therapy, she was asymptomatic and follow-up FDG PET/CT scan showed almost complete disappearance of large vessels’ F-18 FDG uptake. FDG PET/CT is a sensitive technique for assessing presence of large-vessel vasculitis such as Takayasu arteritis, extent of large-vessel inflammation and disease activity after therapy. Conflict of interest:None declared. PMID:23486556

  14. Structural brain lesions in inflammatory bowel disease

    PubMed Central

    Dolapcioglu, Can; Dolapcioglu, Hatice

    2015-01-01

    Central nervous system (CNS) complications or manifestations of inflammatory bowel disease deserve particular attention because symptomatic conditions can require early diagnosis and treatment, whereas unexplained manifestations might be linked with pathogenic mechanisms. This review focuses on both symptomatic and asymptomatic brain lesions detectable on imaging studies, as well as their frequency and potential mechanisms. A direct causal relationship between inflammatory bowel disease (IBD) and asymptomatic structural brain changes has not been demonstrated, but several possible explanations, including vasculitis, thromboembolism and malnutrition, have been proposed. IBD is associated with a tendency for thromboembolisms; therefore, cerebrovascular thromboembolism represents the most frequent and grave CNS complication. Vasculitis, demyelinating conditions and CNS infections are among the other CNS manifestations of the disease. Biological agents also represent a risk factor, particularly for demyelination. Identification of the nature and potential mechanisms of brain lesions detectable on imaging studies would shed further light on the disease process and could improve patient care through early diagnosis and treatment. PMID:26600970

  15. Do neutrophil extracellular traps contribute to the heightened risk of thrombosis in inflammatory diseases?

    PubMed Central

    Rao, Ashish N; Kazzaz, Nayef M; Knight, Jason S

    2015-01-01

    Thrombotic events, both arterial and venous, are a major health concern worldwide. Further, autoimmune diseases, such as systemic lupus erythematosus, anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, and antiphospholipid syndrome, predispose to thrombosis, and thereby push the risk for these morbid events even higher. In recent years, neutrophils have been identified as important players in both arterial and venous thrombosis. Specifically, chromatin-based structures called neutrophil extracellular traps (NETs) play a key role in activating the coagulation cascade, recruiting platelets, and serving as scaffolding upon which the thrombus can be assembled. At the same time, neutrophils and NETs are emerging as important mediators of pathogenic inflammation in the aforementioned autoimmune diseases. Here, we first review the general role of NETs in thrombosis. We then posit that exaggerated NET release contributes to the prothrombotic diatheses of systemic lupus erythematosus, ANCA-associated vasculitis, and antiphospholipid syndrome.

  16. [Gastroenterologic aspects of connective tissue diseases].

    PubMed

    Altomonte, L; Zoli, A; Alessi, F; Ghirlanda, G; Greco, A V; Magarò, M

    1985-07-14

    The connective tissue disorders are a protean group of acquired diseases which have in common widespread immunologic and inflammatory alterations of connective tissue. The acquired connective tissue diseases generally include the following clinical entities: rheumatoid arthritis, systemic lupus erythematosus, polymyositis, polyarteritis nodosa, scleroderma, mixed connective tissue disease, Sjögren's and Behcet's sindromes. These entities have certain features in common which include sinovitis, pleuritis, myocarditis, endocarditis, pericarditis, peritonitis, vasculitis, myositis, changes in skin, alteration of connective tissue and nephritis. Gastrointestinal and hepatic involvement in connective tissue disorders are not the most important features, nevertheless appear almost regularly. Anorexia, nausea, vomiting, abdominal pain, malabsorption may affect patients suffering by rheumatoid arthritis, systemic lupus erythematosus and other collagenophaties. In some cases mesenteric vasculitis may cause intestinal ischemia which may result in bowel infarction, mucosal ulceration, hemorrhage, perforation. After an extensive review of the existing literature the Authors make an accurate evaluation of gastrointestinal and hepatic alterations in connective tissue diseases. PMID:2862608

  17. Concurrent Churg-Strauss syndrome and temporal arteritis in a young patient with pulmonary nodules.

    PubMed

    Amato, M B; Barbas, C S; Delmonte, V C; Carvalho, C R

    1989-06-01

    Very few cases of pulmonary vasculitis that cannot be classified into a single category of vasculitis have been described. We report the first case of a vasculitic process in which pulmonary involvement with asthma, eosinophilic interstitial infiltrates, and small nodules were seen in association with jaw claudication and temporal arteritis with giant cells found on biopsy. Other signs of systemic involvement were also present such as peripheral neuropathy, hematuria with erythrocytic casts and proteinuria, pericardial effusion, and a dilated cardiomyopathy. The histopathologic picture was complex and unique. The early age of onset, the multisystemic involvement, and the prompt response to cyclophosphamide pointed to a diagnosis of "polyangiitis overlap syndrome," with some aspects of Churg-Strauss syndrome and also temporal arteritis. Physicians should be aware of these polymorphous and life-threatening pulmonary vasculitic syndromes, which require aggressive immunosuppressor therapy. PMID:2729757

  18. Adult Dermatomyositis with Bleeding Ulcer in the Pharynx

    PubMed Central

    Kusano, Junko; Takahashi, Yuka

    2014-01-01

    Dermatomyositis (DM) is one of the idiopathic inflammatory myopathies caused by complement-mediated vasculopathy or vasculitis in the muscle. Although the gastrointestinal (GI) mucosa has been reported to be involved as a result of vasculitis or vasculopathy, ulceration in the pharynx is a rare manifestation of DM. A 54-year-old woman complaining of muscle weakness in the extremities, low-grade fever, and dysphagia was diagnosed as having DM. Despite medical treatment with corticosteroids and immunosuppressive agents, her DM progressed rapidly, leading to exacerbation of the dysphagia. About 3 weeks after undergoing tracheostomy as a preventive measure against aspiration, the patient developed intractable respiratory tract hemorrhage. Repeated laryngoendoscopy revealed a bleeding ulceration in the pharynx that required hemostasis with electric cautery under general anesthesia. No bleeding recurred thereafter. Histopathologically, the pharynx exhibited nonspecific inflammatory cell infiltration in the muscle tissue. This rare manifestation may be considered in cases of DM with unexplainable airway bleeding. PMID:25328739

  19. Detection of circulating immune complexes by Raji cell assay: comparison of flow cytometric and radiometric methods

    SciTech Connect

    Kingsmore, S.F.; Crockard, A.D.; Fay, A.C.; McNeill, T.A.; Roberts, S.D.; Thompson, J.M.

    1988-01-01

    Several flow cytometric methods for the measurement of circulating immune complexes (CIC) have recently become available. We report a Raji cell flow cytometric assay (FCMA) that uses aggregated human globulin (AHG) as primary calibrator. Technical advantages of the Raji cell flow cytometric assay are discussed, and its clinical usefulness is evaluated in a method comparison study with the widely used Raji cell immunoradiometric assay. FCMA is more precise and has greater analytic sensitivity for AHG. Diagnostic sensitivity by the flow cytometric method is superior in systemic lupus erythematosus (SLE), rheumatoid arthritis, and vasculitis patients: however, diagnostic specificity is similar for both assays, but the reference interval of FCMA is narrower. Significant correlations were found between CIC levels obtained with both methods in SLE, rheumatoid arthritis, and vasculitis patients and in longitudinal studies of two patients with cerebral SLE. The Raji cell FCMA is recommended for measurement of CIC levels to clinical laboratories with access to a flow cytometer.

  20. What's in a name?

    PubMed

    Chu, Chia-Kai; Morgan, Michael L; Almarzouqi, Sumayya; Lee, Andrew G; Newman, Steven A

    2016-01-01

    A 51-year-old man with rheumatoid arthritis and diabetes mellitus presented with new onset left-sided hemiparesis, left-sided neglect, and left-sided incongruous, denser inferiorly, homonymous hemianopsia. Magnetic resonance image of the brain showed prominent swelling of the right frontal, parietal, and occipital lobes greater than on the left with significant change in the fluid-attenuated inversion recovery signal in the gray matter. Perinuclear antineutrophil cytoplasmic antibodies titers were elevated, and skin biopsy demonstrating leukocytoclastic vasculitis. He showed marked clinical and radiographic improvement in association with recovery of vascular abnormalities after the initiation of prednisone, pulse therapy with methylprednisolone, and methotrexate. Clinicians should be aware of the possibility of neuro-ophthalmic manifestations of rheumatoid arthritis, including rheumatoid arthritis-related vasculitis causing homonymous hemianopsia. PMID:26212152

  1. Nontraumatic hepatic hematoma caused by Wegener's granulomatosis: an unusual cause of abdominal pain.

    PubMed

    Doganay, Selim; Kocakoc, Ercan; Balaban, Mehtap

    2010-07-16

    Wegener's granulomatosis (WG) is a vasculitis of unknown origin characterised by prominent involvement of upper and lower respiratory tract and kidney. There are only a handful of reported cases in the literature about hepatic involvement of WG. This report shows a patient with WG whose main complaint was severe abdominal pain due to nontraumatic subcapsular hepatic hematoma. To our knowledge, this is the first reported case of WG with hepatic hematoma depicted by US and CT in the English literature. PMID:20651870

  2. A rare presentation of cerebral venous sinus thrombosis associated with tubercular meningitis

    PubMed Central

    Verma, Rajesh; Lalla, Rakesh; Patil, Tushar B; Tiwari, Navin

    2013-01-01

    Central nervous system (CNS) tuberculosis may manifest as meningitis, meningoencephalitis, tuberculoma, tubercular abscess, stroke due to tuberculous vasculitis and tuberculous encephalopathy. Occasionally, tubercular meningitis (TBM) can predispose to cerebral venous sinus thrombosis (CVST). We report a young man, who developed CVST as a complication of TBM. Worsening of pre-existing headache, impairment of consciousness and seizures should raise suspicion of CVST in any patient with CNS infection. Early diagnosis and appropriate clinical management are important for good outcome. PMID:23917359

  3. Rare refractory Kawasaki disease in an adolescent boy with cardiac and diffuse coronary artery involvement.

    PubMed

    Šileikien?, Rima; Kudzyt?, Jolanta; Jankauskas, Antanas; Labanauskas, Liutauras; Rakauskien?, Vilma; Jurkien?, Nemira; K?valas, Rimantas

    2013-01-01

    Kawasaki disease is an acute multisystemic vasculitis occurring predominantly in infants and young children and rarely in adolescents and adults. At elderly age, Kawasaki disease may remain unrecognized with a subsequent delay in appropriate therapy and an increased risk of coronary artery aneurysms. We report a case of intravenous immunoglobulin- and aspirin-resistant Kawasaki disease and severe cardiovascular damage in an adolescent boy. The article discusses major issues associated with the management of refractory Kawasaki disease. PMID:24375247

  4. Single Bilateral Dexamethasone Implant in Addition to Panretinal Photocoagulation and Oral Azathioprine Treatment in IRVAN Syndrome.

    PubMed

    Saatci, Ali Osman; Ayhan, Ziya; Take?, Ömer; Yaman, Aylin; Bajin, F Meltem Söylev

    2015-01-01

    The idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome is a disease characterized by multiple retinal macroaneurysms, neuroretinitis and peripheral capillary nonperfusion. Visual loss may result from either ischemia-related complications or macular involvement. Treatment is not always rewarding. We report a case with stage 2 IRVAN syndrome who was successfully treated with a single bilateral intravitreal dexamethasone implant in addition to panretinal photocoagulation and systemic azathioprine treatment. PMID:25802506

  5. Single Bilateral Dexamethasone Implant in Addition to Panretinal Photocoagulation and Oral Azathioprine Treatment in IRVAN Syndrome

    PubMed Central

    Saatci, Ali Osman; Ayhan, Ziya; Take?, Ömer; Yaman, Aylin; Bajin, F. Meltem Söylev

    2015-01-01

    The idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome is a disease characterized by multiple retinal macroaneurysms, neuroretinitis and peripheral capillary nonperfusion. Visual loss may result from either ischemia-related complications or macular involvement. Treatment is not always rewarding. We report a case with stage 2 IRVAN syndrome who was successfully treated with a single bilateral intravitreal dexamethasone implant in addition to panretinal photocoagulation and systemic azathioprine treatment. PMID:25802506

  6. Henoch-Schönlein Purpura with Posterior Reversible Encephalopathy Syndrome.

    PubMed

    Stefek, Bryan; Beck, Michael; Ioffreda, Michael; Gardner, Lyndsay; Stefanski, Michael

    2015-11-01

    Henoch-Schönlein purpura is a common small-vessel vasculitis that presents in children as nonthrombocytopenic, nontender, and palpable purpura, arthritis/arthralgia, and abdominal pain. Central nervous system involvement is a reported, but likely, under-recognized complication. We present an 8-year-old boy with Henoch-Schönlein purpura and posterior reversible encephalopathy syndrome, after proposed mechanism and treatment considerations. PMID:26323197

  7. [Non-clostridial anaerobic bacteria can cause acute pyelonephritis (an experimental trial)].

    PubMed

    Kogan, M I; Pasechnik, D G; Naboka, Iu L; Ibishev, Kh S; Gazaev, Z I; Gudima, I A

    2012-01-01

    Acute obstructive pyelonephritis (PN) caused by Peptococcus niger (Pn), E. coli and mixed infection (Pn + E. coli) was modeled in rabbits. Morphologic examination showed that in animals with peptococcal infection renal lesions were identical to those in escherichial PN. Hemorrhagic infarctions and vasculitis develop in animals with mixed infection. These morphological findings were confirmed bacteriologically. The experimental trial confirms the role of Pn in development of acute obstructive PN in animals. PMID:22876624

  8. [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. PMID:25627304

  9. Fatal neurotoxicity in a patient with down syndrome treated with chemotherapy, irradiation, stem cell transplant, and clofarabine.

    PubMed

    Johnston, Donna L; Bains, Tejinder; Mandel, Karen; Klaassen, Robert; Halton, Jacqueline

    2010-04-01

    Clofarabine is an effective therapy of pediatric patients with relapsed acute lymphoblastic leukemia (ALL). We present a child with Down syndrome who had received previous chemotherapy, cranial radiation, and a stem cell transplant with total body irradiation for her acute lymphoblastic leukemia. She subsequently relapsed and was treated with clofarabine. After her third course, she had a stroke that was felt to be secondary to dehydration and radiation vasculitis. After her subsequent course of clofarabine, she developed fatal neurotoxicity. PMID:20216234

  10. Varicella associated acute respiratory distress syndrome in an adult patient: an example for extracorporeal respiratory support in Brazilian endemic diseases

    PubMed Central

    Mendes, Marcela da Silva; Yeh-Li, Ho; Romano, Thiago Gomes; Santos, Edzangela Vasconcelos; Hirota, Adriana Sayuri; Kono, Bruna Mitiyo; Felicio, Marilia Francesconi; Park, Marcelo

    2014-01-01

    A case of a 30 year-old man presenting with severe systemic chickenpox with refractory hypoxemia, central nervous system vasculitis and anuric renal failure is described. Ambulance transportation and support using veno-venous extracorporeal membrane oxygenation were necessary until the patient recovered. Ultimately, the potential use of extracorporeal membrane oxygenation support in low-middle income countries to manage common diseases is discussed. PMID:25607272

  11. Microscopic polyangiitis causing diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis

    PubMed Central

    Lababidi, Mohamad Hani; Okolo, Chukwuka; Elhassan, Ahmed; Iroegbu, Nkemakolam

    2015-01-01

    Diffuse alveolar hemorrhage complicating small vessel vasculitis is a life-threatening emergency and should be considered in the differential diagnosis of patients who develop rapidly progressive dyspnea with alveolar opacities on chest imaging. In these patients, the coexistence of pulmonary and renal involvement suggests a multisystem disease. We present a case of a man who presented to our hospital with diffuse alveolar hemorrhage, severe anemia, and rapidly progressive glomerulonephritis. PMID:26424944

  12. Anesthetic management of a patient with Henoch-Schonlein purpura for drainage of cervical lymphadenitis: A case report.

    PubMed

    Hasija, Neha; Taxak, Susheela; Bhardwaj, Mamta; Vashist, Kirti

    2014-04-01

    Henoch-Schonlein purpura (HSP) is a multisystem disease and immunoglobulin A-mediated vasculitis with a self-limited course affecting the skin, joints, gastrointestinal tract, and kidneys. Severe renal and central nervous system disease may lead to life-threatening conditions, and immunosuppressive agents and plasmapheresis may be needed. We report successful management of a 6-year-old patient with HSP for drainage of cervical lymphadenitis. PMID:24843348

  13. Granulomatosis with polyangiitis presenting as a parotid gland abscess.

    PubMed

    Dias, Blenda; Soares, Daniela; Sampaio, Patrick; Santiago, Mittermayer

    2015-01-01

    Granulomatosis with polyangiitis (GPA) is a small-vessel vasculitis consisting of necrotizing granulomatous lesions in airways and focal necrotizing glomerulonephritis. However, it may affect other sites such as the skin, central nervous system, eyes, heart, gastrointestinal tract, and liver. We describe a rare case of GPA in which the initial manifestation was the involvement of the parotid gland mimicking a pyogenic abscess. PMID:25815014

  14. In situ thrombosis in pulmonary arterial aneurysms due to Behçet's disease and efficacy of ?mmunosuppressive therapy.

    PubMed

    Ozkaya, Sevket; Sahin, Unal; Gumus, Aziz; Ta?ç?, Filiz; C?narka, Halit; Yavuz, Asiye

    2012-01-01

    BehçetDisease (BD) is a systemic vasculitis characterized by recurrent oral and genital ulcers and uveitis, arthritis, and involvement of the gastrointestinal tract, central nervous system and blood vessels. The aneurysms of the pulmonary arteries, with or without thrombosis, are typical manifestation of BD. We report a case with BD, pulmonary arterial aneurysms(PAA) and in situ thrombosis. We aimed to show the effectiveness of immunosuppressive treatment on in situ thrombosis in a case with PAA and BD. PMID:23078955

  15. Susac syndrome: a case report and PET imaging findings.

    PubMed

    Dielman, Charlotte; Laureys, Guy; Meurs, Alfred; Bissay, Veronique; Ebinger, Guy

    2009-09-01

    We describe the case of a twenty-year-old woman with subacute encephalopathy, who subsequently developed hearing loss and ophtalmopathy. The clinical triad and typical findings on magnetic resonance imaging and cerebrospinal fluid analysis led to the diagnosis of Susac syndrome. Brain positron emission tomography showed abnormalities which are comparable with other types of central nervous system vasculitis, and distinct from those found in multiple sclerosis. PMID:19902818

  16. Skin diseases as extrahepatic manifestations of HCV. Review of some clinical cases.

    PubMed

    Paoletti, V; Parlapiano, C; Labbadia, G; Cavina, G; Marziali, M; Donnarumma, A; Paoletti, F

    2002-09-01

    Among extra-hepatic manifestations of hepatitis C virus (HCV) infection particular interest is focused on some dermatological diseases such as: leukocytoclastic vasculitis, oral lichen planus, pruritus-urticaria, psoriasis. Aim of this paper is to analyze these typical dermatoses in a population of patients with HCV infection and describe the characteristic clinical pictures. These clinical pictures confirm the importance of liver examination in presence of skin diseases not related to other pathogenetic mechanisms. PMID:16491052

  17. Small bowel involvement documented by capsule endoscopy in Churg-Strauss syndrome

    PubMed Central

    Beye, Birane; Lesur, Gilles; Claude, Pierre; Martzolf, Lionel; Kieffer, Pierre; Sondag, Daniel

    2015-01-01

    Churg-Strauss syndrome is a small and medium vessel vasculitis and is also known as allergic granulomatous angiitis. Gastrointestinal involvement is common in patients with Churg-Strauss syndrome (20-50%). The most common symptoms are abdominal pain, diarrhoea and occasionally gastrointestinal bleeding and perforation. We present a case of Churg-Strauss syndrome with small bowel lesions documented by video capsule endoscopy. PMID:26664542

  18. Histopathologic Study of Pathergy Test in Behçet's Disease

    PubMed Central

    Ozluk, Ekin; Balta, Ilknur; Akoguz, Ozlem; Kalkan, Goknur; Astarci, Muzeyyen; Akbay, Gulfer; Eksioglu, Meral

    2014-01-01

    Background: The pathergy test (PT) is important in the diagnosis of Behçet's disease (BD). However, misinterpretation of the test might cause false-positive or false-negative results. Therefore, immunopathologic and histopathologic tests are recommended with PT. Aims and Objectives: The aim of this study is to determine histopathologic findings of positive pathergy reaction at BD. Materials and Methods: This study was performed on 23 patients with BD. All patients were in active period of the disease. After 48 h from the injection, biopsy was performed on positive pathergy lesions. Results: Of the specimen from positive PT lesions of patients with BD, nine revealed mixed type inflammatory cell infiltration (39.1%), two revealed lobular panniculitis without vasculitis (8.7%), two revealed neutrophil rich infiltration (8.7%), and five revealed lymphocyte rich infiltration (21.7%) at the subcutaneous tissue. Mixed type inflammatory cell infiltration (43.4%), endothelial swelling and thickening (17.3%), erythrocyte extravasation (26.0%), perivascular cell infiltration (13.0%), lymphocytic vascular reaction (8.6%), lymphocytic vasculitis (13.0%), and leukocytoclastic vasculitis (21.7%) were detected in dermis by histopathologic examinations. There was no statistically significant difference between histopathologic findings and sex, family history, and systemic involvement except uveitis. Conclusion: This is an exceptional study since it is the first study that determines subcutaneous tissue findings of positive pathergy reaction in Behçet patients. In our study, uveitis was found to be statistically significant in the patients who had vasculitis in dermis. It can be a clue for prediction of disease severity and course. Further, studies that include wide number of patients will better illuminate the correlation between subcutaneous tissue findings and disease severity and clinical course. PMID:25484413

  19. Acute hemorrhagic edema of infancy: report of 4 cases and review of the current literature.

    PubMed

    Karremann, Michael; Jordan, Alexander J; Bell, Nellie; Witsch, Michael; Dürken, Matthias

    2009-04-01

    Acute hemorrhagic edema of infancy (AHEI) is a cutaneous leukocytoclastic vasculitis that usually occurs in children younger than 2 years of age. It is a rare disease characterized by mild fever, a violent onset of hemorrhagic skin lesions, and edema usually followed by a spontaneous and complete recovery. Although the etiology is unknown, AHEI often follows infections, drug treatment, or vaccination. In the present report, the authors describe 4 cases of AHEI and review the relevant literature. PMID:18772356

  20. Juvenile dermatomyositis in South African children is characterised by frequent dystropic calcification: a cross sectional study

    PubMed Central

    2014-01-01

    Background To describe Juvenile dermatomyositis (JDM) that has rarely been reported in Sub-Saharan Africa in children. Methods Retrospective record review of children with JDM attending a tertiary hospital in South Africa. Results Twenty-one children (16 female, five male) with JDM had a mean (SD) age at presentation of 9.8 (3.3) years. Mean follow-up period was 2.6 (2.2) years. The commonest presenting features were skin rash (71%), muscle weakness (71%), inflammatory arthritis (42%) and calcinosis (29%). The cumulative frequency of calcinosis was 71%. Skin vasculitis was present in 9(43%), and 7 (33%) had Staphylococcus aureus infections. Calcinosis was strongly associated with vasculitis; 11/15 (73.3%) with calcinosis had vasculitis versus 0/6 without vasculitis (p?=?0.003). Patients with calcinosis had significantly lower creatinine kinase (CK) levels compared to those without calcinosis [mean (SD) 272 U/L (401) vs. 2414 U/L (3201), respectively, p?=?0.016]. All children with calcinosis had Staphylococcus aureus infection, but there was no significant difference in their duration of symptoms to presentation. Joint contractures, occurring in eight patients (38%), were associated with a significantly lower age at presentation [mean (SD) 6.8(2.8) vs. 11.6(2.1) years (no contractures) p?=?0.0003], and significantly higher CRP and ESR levels. Three patients were lost to follow-up, two died. In the remaining 16 patients: 10 (47%) experienced remission, 2 relapsed and 4 persistent active disease. Conclusion African children with JDM have increased vasculitic disease and high levels of calcinosis with low muscle enzymes, particularly CK. Younger children are at higher risk of contractures and disability. Patients are at high risk of developing Staphylococcus aureus infection. Rapid and aggressive therapy is necessary. PMID:24397895

  1. [Cases of atypical pulmonary diseases under influence of industrial aerosols].

    PubMed

    Liubchenko, P N; Stashuk, G A; Terpigorev, S A; Atamanchuk, A A; Massarygin, V V

    2014-01-01

    The article includes discharge epicrises of patients working in contact with quartz-containing dust, welding aerosol, cotton dust, irritant gases emitted in wood burning. Description covers cases of lung fibrosis, idiopathic interstitial pneumonia, systemic vasculitis with lungs involvement, sarcoidosis. The authors suggest that further accumulation of materials, further investigations of lung tissue state, epidemiologic studies will help to widen the list of occupational pulmonary diseases. PMID:25881397

  2. Henipavirus Encephalitis: Recent Developments and Advances.

    PubMed

    Ong, Kien Chai; Wong, Kum Thong

    2015-09-01

    The genus Henipavirus within the family Paramyxoviridae includes the Hendra virus (HeV) and Nipah virus (NiV) which were discovered in the 1990s in Australia and Malaysia, respectively, after emerging to cause severe and often fatal outbreaks in humans and animals. While HeV is confined to Australia, more recent NiV outbreaks have been reported in Bangladesh, India and the Philippines. The clinical manifestations of both henipaviruses in humans appear similar, with a predominance of an acute encephalitic syndrome. Likewise, the pathological features are similar and characterized by disseminated, multi-organ vasculopathy comprising endothelial infection/ulceration, vasculitis, vasculitis-induced thrombosis/occlusion, parenchymal ischemia/microinfarction, and parenchymal cell infection in the central nervous system (CNS), lung, kidney and other major organs. This unique dual pathogenetic mechanism of vasculitis-induced microinfarction and neuronal infection causes severe tissue damage in the CNS. Both viruses can also cause relapsing encephalitis months and years after the acute infection. Many animal models studied to date have largely confirmed the pathology of henipavirus infection, and provided the means to test new therapeutic agents and vaccines. As the bat is the natural host of henipaviruses and has worldwide distribution, spillover events into human populations are expected to occur in the future. PMID:26276024

  3. The Influence and Role of Microbial Factors in Autoimmune Kidney Diseases: A Systematic Review

    PubMed Central

    2015-01-01

    A better understanding of the pathophysiology of autoimmune disorders is desired to allow tailored interventions. Despite increased scientific interest a direct pathogenic factor in autoimmune renal disease has been described only in a minority like membranous nephropathy or ANCA-associated vasculitis. Nonetheless the initial step leading to the formation of these antibodies is still obscure. In this review we will focus on the possible role of microbial factors in this context. Staphylococcus aureus may be a direct pathogenetic factor in granulomatosis with polyangiitis (GPA). Chronic bacterial colonization or chronic infections of the upper respiratory tract have been proposed as trigger of IgA vasculitis and IgA nephropathy. Interventions to remove major lymphoid organs, such as tonsillectomy, have shown conflicting results but may be an option in IgA vasculitis. Interestingly no clear clinical benefit despite similar local colonization with bacterial strains has been detected in patients with IgA nephropathy. In systemic lupus erythematosus injection of bacterial lipopolysaccharide induced progressive lupus nephritis in mouse models. The aim of this review is to discuss and summarize the knowledge of microbial antigens in autoimmune renal disease. Novel methods may provide insight into the involvement of microbial antigens in the onset, progression, and prognosis of autoimmune kidney disorders. PMID:26078982

  4. Limited form of Wegener's granulomatosis in a patient with Crohn's disease. A case report.

    PubMed

    Ben Salah, Raida; Frikha, Faten; Snoussi, Mouna; Abderrahmen, Masmoudi; Hentati, Yosr; Mnif, Zeineb; Turki, Hamida; Bahloul, Zouhir

    2014-12-01

    Wegener's granulomatosis (WG) is a multisystemic disease of unknown etiology characterized by necrotizing vasculitis and granulomatous inflammation. Although, the gastrointestinal tract can be involved in this vasculitis, the association between WG and Crohn's disease (CD) exists and was previously described. We report the case of a 29 year-old Tunisian white patient previously diagnosed with CD, presenting 3 years later with a perforated nasal septum, mucosal ulcers and sinusitis. He had also a skin ulcerative eruption of the trunk. There was no evidence of a flare of his CD. A computed tomography scan of the head showed nasal septal perforation, and maxillary pansinusitis. A biopsy from the nasal septum demonstrated nasal mucosa with extensive necrotizing granulomatous reaction associated with vasculitis. The patient was diagnosed as having limited form of Wegener's granulomatosis. He was treated with prednisone 60 mg/day and methotrexate 15 mg weekly. After Two months of treatment, he had no complaints, skin eruption and orofacial lesions were improved. Although, the gastrointestinal tract can be involved in patients with WG, the association with a CD exists and was previously described. The distinction between the two conditions and their possible coexistence is very important and requires histological examination of involved tissue. PMID:25910303

  5. Erythema nodosum-like lesions in Behçet's disease: a clinicopathological study of 26 cases.

    PubMed

    Misago, Noriyuki; Tada, Yoshifumi; Koarada, Syuichi; Narisawa, Yutaka

    2012-11-01

    The histopathological features of erythema nodosum-like lesions remain controversial with regard to whether they resemble those of conventional erythema nodosum. We reviewed the clinicopathological features of erythema nodosum-like lesions in 26 patients with Behçet's disease and evaluated the clinical characteristics of Behçet's disease in these patients. The results suggest that: (i) the clinico-pathological features of 27% of the erythema nodosum-like lesions in Behçet's disease are indistinguishable from those of conventional erythema nodosum; (ii) the other 73% of the erythema nodosum-like lesions are histopathologically characterized by the presence of vasculitis (venulitis or phlebitis); (iii) the clinical features of erythema nodosum-like lesions with vasculitis show heterogeneity; (iv) the presence of the erythema nodosum type lesion may be an indicator of the mildness of Behçet's disease; and (v) the presence of severe vasculitis, especially phlebitis, in erythema nodosum-like lesions may be an indicator of the involvement of the gastrointestinal tract in Behçet's disease. PMID:22504656

  6. Sudden death due to polyarteritis nodosa.

    PubMed

    Shields, Lisa B E; Burge, Meredith; Hunsaker, John C

    2012-09-01

    Classical polyarteritis nodosa (cPAN) refers to a rare, potentially fatal systemic transmural necrotizing vasculitis that usually affects medium-sized, and occasionally small, muscular arteries, primarily involves the kidneys, gastrointestinal tract, skin, nervous system, joints, and muscles, and is rarely, if ever, expressed in the lungs. The incidence of mortality has significantly decreased with recently developed treatment modalities, in particular antiviral medications. Sudden death due to previously undiagnosed cPAN is rarely encountered. We report a case of a young man who had been evaluated on three occasions by medical personnel in the 3 weeks prior to his death. He had complained of nonspecific symptoms of abdominal and perineal/suprapubic pain, nausea, vomiting, sensation of chilling, and constipation. The spectrum of diagnoses included "gastroenteritis," enteric infection, and prostatitis. Found agonal at home and dying despite immediate cardiopulmonary resuscitation (CPR), he underwent a medicolegal autopsy, which revealed vasculitis of various organs, including heart (myocardium and epicardium) and extramural coronary arteries, liver, spleen, kidneys, adrenal glands, stomach and bowel, omentum, gallbladder, and pancreas. His sudden death was cardiac in nature due to PAN associated clinically with hepatitis B surface antigen positivity (hepatitis B virus-associated PAN [HBV-PAN]). A complete autopsy with thorough histopathological examination is necessary to diagnose this uncommon yet potentially fatal vasculitis. PMID:21983834

  7. Colon Perforation and Budd-Chiari Syndrome in Behçet’s Disease

    PubMed Central

    Ba?, Y?lmaz; Güney, Güven; Uzbay, P?nar; Zobac?, Ethem; Ardal?, Selin; Özkan, Ay?egül Taylan

    2015-01-01

    Patient: Female, 38 Final Diagnosis: Behçet’s disease Symptoms: Severe abdominal pain • fever Medication: — Clinical Procedure: Parsiyel colectomy Specialty: Surgery Objective: Unusual clinical course Background: Behçet’s disease is a chronic inflammatory disease involving multiple systems, with vasculitis being the most important pathological feature. Multiple colon perforations are thought to be secondary to vasculitis and they occur in patients with ulcers. These may be encountered within the entire colon but most commonly in the ileocecal region. Intestinal perforation and Budd-Chiari syndrome are infrequent in Behçet’s disease, and are associated with high mortality and morbidity. Budd-Chiari syndrome results from occlusion of either hepatic veins or adjacent inferior vena cava, or both. Case Report: We report a patient with Behçet’s disease having multiple perforations in the transverse colon, descending colon, and sigmoid colon. The patient also had Budd-Chiari syndrome due to inferior vena cava thrombosis extending into the right and middle hepatic vein. Our observations are presented with a review of the literature. Conclusions: In Behçet’s disease, treatment of colon perforation necessitates urgent surgery, whereas management of Budd-Chiari syndrome is directed towards the underlying cause. Behçet’s disease, as a chronic multisystemic disease with various forms of vasculitis, is resistant to medical and surgical treatment. Prognosis is worse in Behçet’s disease with colon perforation than that in Budd-Chiari syndrome alone. PMID:25934795

  8. Nitric oxide as an inflammatory mediator in autoimmune MRL-lpr/lpr mice.

    PubMed Central

    Weinberg, J B

    1998-01-01

    Nitric oxide (.NO) may exhibit proinflammatory features. .NO synthase type 2 (NOS2) is overexpressed and .NO overproduced in rodent models of induced inflammation. Blockage of .NO production by administration of NOS inhibitors prevents or reduces various types of induced inflammation in mice and rats. We have shown that autoimmune MRL-lpr/lpr mice overexpress NOS2 and overproduce .NO in an age-dependent fashion that parallels expression of arthritis, glomerulonephritis, and vasculitis. Blocking .NO production by oral administration of the NOS inhibitor NG-monomethyl-L-arginine reduced the arthritis, glomerulonephritis, and vasculitis, but it did not modify serum anti-DNA antibody levels or glomerular deposition of immune complexes. When mice with genetically disrupted NOS2 were backcrossed to MRL-lpr/lpr mice, the resultant (-/-) mice expressed no NOS2 and produced no .NO, the wild-type (+/+) mice overexpressed NOS2 and overproduced .NO (in comparison to normal, control mice), and the heterozygous (+/-) mice expressed and produced intermediate levels. Nephritis and arthritis in the (-/-) mice were comparable to that in MRL-lpr/lpr mice, but vasculitis was markedly decreased. Levels of anti-DNA antibodies were comparable in all mice, but IgG rheumatoid factor production was markedly reduced in the (-/-) mice. These results of studies in MRL-lpr/lpr mice with genetically disrupted NOS2 highlight the heterogeneity and complexity of the role of NOS2 and .NO in inflammation. PMID:9788887

  9. Development of Canadian Recommendations for the Management of ANCA-Associated Vasculitides: Results of the National Needs Assessment Questionnaire

    PubMed Central

    Famorca, Leilani; Twilt, Marinka; Barra, Lillian; Bakowsky, Volodko; Benseler, Susanne; Cabral, David; Carette, Simon; Dhindsa, Navjot; Fifi-Mah, Aurore; Goulet, Michelle; Khalidi, Nader; Khraishi, Majed; McGeoch, Lucy; Milman, Nataliya; Pineau, Christian; Shojania, Kam; Taylor-Gjevre, Regina; Towheed, Tanveer; Trudeau, Judith; Yacyshyn, Elaine; Liang, Patrick; Pagnoux, Christian

    2015-01-01

    Objectives : To study variations in Canadian clinical practice patterns for the management of ANCA-associated vasculitis (AAV) and identify points to consider for the development of national recommendations. Material and Methodology : A 30-item needs assessment questionnaire was sent to all members of the Canadian Vasculitis network (CanVasc), Canadian Rheumatology Association (CRA), Canadian Thoracic Society (CTS) and Canadian Society of Nephrology (CSN). Respondent characteristics, practice patterns, concerns and expectations were analyzed. Results : Among 132 physicians who followed at least 1 vasculitis patient and responded to the survey, 39% stated that they felt confident in their management of AAV. Several variations in practice were observed regarding diagnostic procedure, induction and maintenance treatments and use of biologics; some were due to logistic constraints (difficulties in access to some specific tests, drugs or care; lack of health care coverage for the costs). The top 5 topics for which recommendations are expected involve treatment for remission induction, maintenance, refractory disease, and relapse as well as biologics. Conclusion : Practice variations identified in this needs assessment survey will serve to formulate key questions for the development of CanVasc recommendations. PMID:25893028

  10. Eosinophilic Granulomatosis With Polyangiitis With Thrombotic Microangiopathy: Is Simultaneous Systemic Lupus Erythematosus Associated With Clinical Manifestations?: A Case Report and Review of the Literature.

    PubMed

    Fukui, Shoichi; Iwamoto, Naoki; Tsuji, Sosuke; Umeda, Masataka; Nishino, Ayako; Nakashima, Yoshikazu; Suzuki, Takahisa; Horai, Yoshiro; Koga, Tomohiro; Kawashiri, Shin-Ya; Ichinose, Kunihiro; Hirai, Yasuko; Tamai, Mami; Nakamura, Hideki; Origuchi, Tomoki; Kinoshita, Naóe; Kawakami, Atsushi

    2015-11-01

    Eosinophilic granulomatosis with polyangiitis (EGPA) is one of the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, which is characterized by vasculitis of the small to medium-sized vessels. On the contrary, thrombotic microangiopathy (TMA) is a life-threatening condition which can cause ischemic organ injury. Although several case reports have described patients with TMA associated with ANCA-associated vasculitis except for EGPA, there are no previous case reports of EGPA associated with TMA.A 71-year-old Japanese man was diagnosed with EGPA based on his asthma, eosinophilia, lung opacity, refractory sinusitis, and positive myeloperoxidase-ANCA. He was also diagnosed with TMA based on peripheral schizocytes and hemolytic anemia. We performed plasmapheresis and started high-dose corticosteroid therapy; thereafter, he improved promptly. His case also fulfilled the classification criteria of systemic lupus erythematosus (SLE) based on the pleural effusion, renal disorder, anemia, thrombocytopenia, positive antidouble-stranded DNA antibody, and low complement. Elements of SLE were thought to affect his clinical course.We reviewed 11 patients with EGPA or hypereosinophilic syndrome (HES) associated with SLE, including our case. Patients with EGPA or HES associated with SLE had more heart complications than patients with simple EGPA or simple HES did. Patients with EGPA or HES associated with SLE had more pleural effusion than patients with simple SLE did.Clinical manifestations of eosinophilia with SLE or SLE with eosinophilia may differ from simple SLE or simple eosinophilia. PMID:26559264

  11. Effects of nutrition on disease and life span. I. Immune responses, cardiovascular pathology, and life span in MRL mice.

    PubMed Central

    Mark, D. A.; Alonso, D. R.; Quimby, F.; Thaler, H. T.; Kim, Y. T.; Fernandes, G.; Good, R. A.; Weksler, M. E.

    1984-01-01

    Mice of the autoimmune, lymphoproliferative strain MRL/lpr and the congenic, nonlymphoproliferative strain MRL/n were fed one of six diets from weaning on-ward. These mice were sacrificed at 3 or 5 months of age. Low fat diets resulted in lower cholesterol and higher triglyceride levels than did cholesterol-containing high-fat diets. Caloric restriction of MRL/lpr mice was associated with an increased plaque-forming cell response to trinitrophenylated polyacrylamide beads, less lymphoproliferation, and less severe glomerulonephritis. Diet did not affect the incidence of autoimmune vasculitis in MRL/lpr mice sacrificed at 5 months. MRL/lpr mice fed a low-fat, calorically restricted diet from 5 months of age to death lived longer than mice which were fed ad libitum a cholesterol-containing, high-fat diet. At death, MRL/lpr mice fed the former diet had the autoimmune vasculitis which had been evident in mice killed at 5 months, whereas mice fed the latter diet, in addition to the vasculitis, had a high incidence of atherosclerotic lesions of intrarenal and aortic branch arteries. Images Figure 5 Figure 6 Figure 7 Figure 8 Figure 9 Figure 10 PMID:6333184

  12. P-ANCA cranial pachymeningitis: a case report.

    PubMed

    Jacobi, D; Maillot, F; Hommet, C; Arsène, S; Cottier, J P; Lamisse, F; Guillevin, L

    2005-04-01

    Pachymeningitis is an inflammatory process that thickens the dura mater. This disease has various etiologies including infectious, neoplastic, or autoimmune diseases. We present the case of a patient who developed cranial pachymeningitis with a clinical and biological picture suggestive of a neurological form of vasculitis. A 51-year-old woman developed rhinitis, otitis media, headaches, and deterioration of her condition after a course of recombinant hepatitis B vaccine. After a booster dose of the vaccine, she developed unilateral visual loss and impairment of multiple cranial nerves. Blood analysis showed inflammation and presence of antimyeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA). Cranial magnetic resonance imaging (MRI) showed pachymeningitis. A complete remission was obtained with immunosuppressive therapy. The initial clinical presentation and subsequent remission under immunosuppressive therapy were suggestive of a vasculitis with nervous system involvement. Though vasculitis was not proven histologically in this patient, we believe that MPO-ANCA-related autoimmunity provoked the patient's disease as already reported in similar cases. As pachymeningitis is a fibrosing process, early recognition and treatment of an autoimmune etiology, even in the absence of previous pulmonary or renal involvement, is required to prevent definitive neurological impairment. PMID:15578248

  13. [Infective endocarditis due to high level aminoglycoside resistant Enterococcus faecalis and methicillin resistant coagulase-negative staphylococci presenting with rheumatic manifestations].

    PubMed

    Pi?kin, Nihal; Akduman, Deniz; Aydemir, Hande; Celebi, Güven; Oztoprak, Nefise; Akta?, Elif

    2008-07-01

    Infective endocarditis has variable clinical presentations and may present with rheumatologic manifestations. Infective endocarditis due to high level aminoglycoside resistant enterococci represents a severe therapeutic challenge as none of the currently recommended treatment regimens are bactericidal against these isolates. In this report, a case of infective endocarditis with double aetiology, high level aminoglycoside resistant Enterococcus faecalis together with methicillin-resistant coagulase-negative staphylococci (MR-CNS), presenting with leukocytoclastic vasculitis and rapidly progressive glomerulonephritis, has been presented. A 48-years-old woman was admitted to our hospital with malaise and non-pruritic purpural rush on her lower extremities. On admission she had no fever or leukocytosis. Skin biopsy showed leukocytoclastic vasculitis and steroid therapy was started. On 12th day of admission rapidly progressive glomerulonephritis was diagnosed and she received plasmapheresis and haemodialysis support. Transthoracic echocardiography (TTE) demonstrated 1 x 1.5 cm vegetation on the mitral valve. An initial diagnosis of infective endocarditis was made and empirical treatment with vancomycin and gentamicin was started. All blood cultures yielded high level aminoglycoside resistant E. faecalis and additionally two of them yielded MR-CNS. Vancomycin was administered in combination with high dose ampicillin and repeated blood cultures taken after administration of ampicillin, revealed no growth. The patient remained afebrile, renal functions improved and a repeat TTE done on 20th day of ampicillin therapy showed waning of the vegetation. On 42nd day of treatment repeat TTE showed new vegetation on the mitral valve and severe valve insufficiency, so the patient was scheduled for mitral valve replacement. She was treated for 12 weeks with vancomycin and ampicillin and recovered successfully. In conclusion; infective endocarditis should be considered in the differential diagnosis of leukocytoclastic vasculitis and rapidly progressive glomerulonephritis. Physicians should document their treatment outcomes and experience with high level aminoglycoside resistant enterococcal infective endocarditis, which is a therapeutic challenge, so that the best therapeutic options can be identified. PMID:18822897

  14. Relationship among antineutrophil cytoplasmic antibody, blood urea nitrogen and complement in patients with eosinophilic granulomatosis polyangiitis (Churg-Strauss syndrome).

    PubMed

    Kawakami, Tamihiro; Kimura, Satoko; Takeuchi, Sora; Soma, Yoshinao

    2013-07-01

    Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis characterized by a history of asthma, hypereosinophilia. The prevalence of ANCA in EGPA is less common than in other ANCA-associated vasculitis. Increasing evidence of complement activation in the pathogenesis of ANCA-associated vasculitis has been provided by studies in animal models. We examined EGPA patients with cutaneous manifestations as an initial sign and investigated the correlations among clinical, serological and histopathological findings. We focused on differences among ANCA, blood urea nitrogen and complement levels such as complement 3 (C3), C4 and total complement hemolytic activity (CH50). We retrospectively investigated the records of 22 patients (11 male and 11 female) with EGPA admitted to our hospital from 1997-2012. Ten of the 22 patients (46%) were positive for serum myeloperoxidase (MPO)-ANCA. In contrast, all the patients were negative for serum proteinase 3 ANCA. There was a significantly positive correlation between serum CH50 and C4 levels in patients with EGPA. Serum blood urea nitrogen (BUN) levels differed significantly between MPO-ANCA-positive and -negative patients. Serum CH50 levels were higher in MPO-ANCA-positive patients compared to negative patients. Serum BUN levels were higher in elevated CH50 patients compared to normal and low CH50-negative patients. We propose that positive findings for MPO-ANCA with CH50 high activity may be a risk factor for developing renal insufficiency. Assuming there are correlations between the presence of ANCA and complements, earlier diagnosis based on initial efficacious treatment for EGPA. PMID:23594281

  15. Cardiac tamponade leading to the diagnosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome): a case report and review of the literature.

    PubMed

    Yano, Toshiyuki; Ishimura, Shutaro; Furukawa, Tetsuaki; Koyama, Masayuki; Tanaka, Marenao; Shimoshige, Shinya; Hashimoto, Akiyoshi; Miura, Tetsuji

    2015-11-01

    Eosinophilic granulomatosis with polyangiitis (EGPA), which was previously called Churg-Strauss syndrome, is a necrotizing systemic vasculitis of unknown cause accompanied by prominent eosinophilia. Cardiovascular complications, including eosinophilic myocarditis, are a major cause of mortality in this disorder. Acute pericarditis with slight pericardial effusion is a typical manifestation in EGPA, though hemodynamically significant pericardial effusion has been reported in a few cases. We report a case that initially presented with isolated cardiac tamponade, which was followed by systemic manifestations of EGPA over 3 weeks. Including the present case, previous EGPA cases with cardiac tamponade are reviewed to delineate its clinical characteristics. PMID:25070496

  16. Membranous glomerulonephritis and cellular crescents induced by levamisole-adulterated cocaine abuse: a case report

    PubMed Central

    Moll-Guillen, Jose-Luis; Espí-Reig, Jordi; Blanes-Julia, Marino; García-Martínez, Ana-María; Pujol-Marco, Conrad; Hernández-Jaras, Julio

    2015-01-01

    Levamisole is illicitly employed as a cocaine adulterant. The consumption of levamisole-adulterated cocaine can provoke anti-neutrophil cytoplasmic antibody (ANCA)-associated syndromes. Patients carrying an HLAB27 allele are known to be at higher risk of developing agranulocytosis when treated with levamisole. Likewise, patients with ANCA-associated vasculitis (AAV) and internal organ involvement have typically been exposed to offending agents for prolonged periods of time, often on the order of years. Here, we report an unusual case of a patient in which kidney biopsy showed membranous glomerulonephritis with cellular crescents associated with levamisole-contaminated cocaine use. PMID:26605317

  17. Endovascular Treatment of Left Subclavian Artery Pseudoaneurysm after Clavicle Fracture in an Elderly Adult with a 40-Year History of Behçet’s Disease

    PubMed Central

    Hamasaki, Takafumi; Yamamoto, Shuichi; Kawai, Tsuyoshi; Sugiura, Kimihiko

    2015-01-01

    Behçet’s disease (BD) is a rare chronic systemic vasculitis of unknown etiology that presents in young adults. We report a very rare case of a left subclavian artery pseudoaneurysm identified after a left clavicle fracture in a 70-year-old man with a 40-year history of intestinal BD. The patient received fixation by banding to treat a fracture after falling from a bed about 5 months previously, and he was referred to our hospital due to the finding of a left subclavian pulsatile mass. As enhanced computed tomography showed a left subclavian artery pseudoaneurysm, it was repaired using an endovascular stent graft.

  18. Polyarteritis Nodosa

    PubMed Central

    Howard, Travis; Ahmad, Kinza; Swanson, Jerome (Allen) A.; Misra, Sanjay

    2015-01-01

    The first description of polyarteritis nodosa (PAN) was in 1852 by Karl Rokitansky, a Pathologist at the University of Vienna.(1) The initial report describes a 23 year-old man who had a five-day history of fever and diarrhea. Since then, the definition of PAN has evolved. The currently accepted definition of PAN comes from the 2012 Chapel Hill Conference which classified PAN as a necrotizing arteritis not associated with anti-neutrophil cytoplasmic antibodies (ANCAs) of medium or small arteries without glomerulonephritis or vasculitis in arterioles, capillaries, or venules.(2) PMID:25770638

  19. HELLP Syndrome Complicated with Postpartum Subcapsular Ruptured Liver Hematoma and Purtscher-Like Retinopathy.

    PubMed

    Cernea, Daniela; Dragoescu, Alice; Novac, Marius

    2012-01-01

    Purtscher's retinopathy is usually associated with trauma, acute pancreatitis, vasculitis, lupus, and bone fractures. It was rarely described postpartum in patients with preeclampsia as well as associated with HELLP syndrome. We present a case of a multiparous patient aged 44 with severe preeclampsia and postpartum HELLP syndrome complicated with Purtscher-like retinopathy and large ruptured subcapsular liver hematoma that required emergency abdominal surgery after premature delivery of a dead fetus. Postsurgical outcome was favorable regarding both liver function and visual acuity. PMID:22852104

  20. 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. PMID:26504868

  1. Subdural Empyema Complicating Bacterial Meningitis: A Challenging Diagnosis in a Patient with Polysubstance Abuse

    PubMed Central

    Dakkak, Melissa; Cullinane, William Russell; Ramoutar, Virin Rajiv Neil

    2015-01-01

    Subdural empyema (SDE) and cerebrovascular accident (CVA) are uncommon life-threatening complications of bacterial meningitis, which require urgent neurosurgical intervention to prevent adverse outcomes. Clinicians must be vigilant of the onset of focal neurologic deficits or seizure activity to establish the diagnosis of SDE. Streptococcus pneumoniae accounts for <1% of pyogenic brain abscesses. This case describes a presentation of community acquired pneumococcal pneumonia in which the diagnosis of SDE with vasculitis induced CVA was confounded by concomitant substance abuse and sedation. PMID:26543484

  2. Microscopic polyangiitis associated with pleuropericarditis, pulmonary embolism and pulmonary hemorrhage as a complication of silicosis

    PubMed Central

    Al-Rajhi, Amjad; Brega, Elisa Ferreira; Colman, Neil C.

    2015-01-01

    Silica (silicon dioxide) occupational exposure has been linked to both pulmonary and extra-pulmonary toxicity. Silicosis is the major pulmonary toxicity, which has also been associated with the development of collagen-vascular disease and with anti-neutrophil cytoplasmic antibody (ANCA)-positive vasculitis, especially perinuclear anti-neutrophil cytoplasmic antibodies (P-ANCA). The most common pulmonary manifestations of microscopic polyangitis (MPA) are interstitial fibrosis and alveolar hemorrhage. We describe a patient who had unusual presentation of microscopic polyangitis, characterized by lung hemorrhage, rapidly progressive glomerulonephritis, pleuropericarditis and pulmonary embolism that was associated with a history of silica exposure and radiologic evidence for silicosis. PMID:26236617

  3. Cutaneous Manifestations in POEMS Syndrome: Case Report and Review.

    PubMed

    Marinho, Flauberto Sousa; Pirmez, Rodrigo; Nogueira, Renata; Cuzzi, Tullia; Sodré, Celso Tavares; Silva, Marcia

    2015-01-01

    The authors report a case of sensorimotor polyneuropathy, diffuse cutaneous hyperpigmentation, skin sclerodermiform thickening and papular lesions in the infraclavicular and abdominal region. Besides weight loss, there were diabetes mellitus and hypothyroidism. The alterations were consistent with POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes) syndrome, which is a rare systemic disease with monoclonal proliferation of plasmacytes and slow progression. Cutaneous alterations are present in 68% of patients with diffuse cutaneous hyperpigmentation, plethora and acrocyanosis. Leukonychia, necrotizing vasculitis, hypertrichosis and cutaneous thickening of sclerodermiform type are also cited. The onset of multiple cutaneous angiomas in this syndrome has been observed in 24-44% of patients. PMID:26034475

  4. Cutaneous Manifestations in POEMS Syndrome: Case Report and Review

    PubMed Central

    Marinho, Flauberto Sousa; Pirmez, Rodrigo; Nogueira, Renata; Cuzzi, Tullia; Sodré, Celso Tavares; Silva, Marcia

    2015-01-01

    The authors report a case of sensorimotor polyneuropathy, diffuse cutaneous hyperpigmentation, skin sclerodermiform thickening and papular lesions in the infraclavicular and abdominal region. Besides weight loss, there were diabetes mellitus and hypothyroidism. The alterations were consistent with POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes) syndrome, which is a rare systemic disease with monoclonal proliferation of plasmacytes and slow progression. Cutaneous alterations are present in 68% of patients with diffuse cutaneous hyperpigmentation, plethora and acrocyanosis. Leukonychia, necrotizing vasculitis, hypertrichosis and cutaneous thickening of sclerodermiform type are also cited. The onset of multiple cutaneous angiomas in this syndrome has been observed in 24–44% of patients. PMID:26034475

  5. Antineutrophil Cytoplasmic Antibody–associated Glomerulonephritis Complicated by Pneumatosis Intestinalis

    PubMed Central

    Nakagawa, Saki; Akimoto, Tetsu; Takeda, Shin-ichi; Okada, Mari; Miki, Atsushi; Yamamoto, Hisashi; Muto, Shigeaki; Kusano, Eiji; Nagata, Daisuke

    2015-01-01

    Pneumatosis intestinalis is a characteristic imaging phenomenon indicating the presence of gas in the bowel wall. The link between pneumatosis intestinalis and various kinds of autoimmune diseases has been reported anecdotally, while information regarding the cases with antineutrophil cytoplasmic antibodies (ANCA)–associated vasculitis complicated by concurrent pneumatosis intestinalis is lacking. In this report, we describe our serendipitous experience with one such case of pneumatosis intestinalis in a patient with ANCA-associated glomerulonephritis. We also discuss several therapeutic concerns that arose in the current case, which had an impact on the pathogenesis of the disease. PMID:26309421

  6. Eosinophilic granulomatosis with polyangiitis and diffuse gastrointestinal involvement.

    PubMed

    Franco, Diana L; Ruff, Kevin; Mertz, Lester; Lam-Himlin, Dora M; Heigh, Russell

    2014-09-01

    Eosinophilic granulomatosis with polyangiitis (EGPA), formerly named Churg-Strauss syndrome, is a rare systemic small- and medium-sized-vessel vasculitis, characterized by the presence of severe asthma as well as blood and tissue eosinophilia. Gastrointestinal (GI) symptoms, like diarrhea and abdominal pain, are common; however, there are few reports of histologic evidence of GI involvement. We report the case of a patient on treatment for EGPA who presented with recurrent small bowel obstruction and choledocholithiasis. Biopsies of the esophagus, small bowel and common bile duct showed diffuse eosinophilia, with clear EGPA in the GI tract. Improved awareness of GI EGPA may allow for timely management of this disorder. PMID:25473392

  7. Concurrence of malignant fibrohistiocytoma and Takayasu arteritis: a case report.

    PubMed

    Du, Jin-Feng; Chen, Jin-Wei; Li, Fen; Tian, Jing; Xie, Xi; Mao, Ni

    2012-10-01

    Takayasu arteritis (TA) is a type of systemic large-vessel vasculitis that usually affects the aorta and its major branches. It remains unrecognized owing to delayed diagnosis (Boltin et al. in Rheumatol Int 27(10):985-987, 2007) and non-characteristic clinical features. It has been described in association with many autoimmune diseases, such as inflammatory digestive tract diseases. However, report of TA associated with tumors, especially malignant tumors, are rare. We here presented a case diagnosed by both Takayasu arteritis and malignant fibrous histiocytoma, from which we learned not only clinical lessons, but also consensus of relationships between these two diseases. PMID:21969061

  8. Acute penile pain and swelling in a 4-year-old child with Henoch-Schönlein purpura.

    PubMed

    Tewary, Kishor Kumar; Khodaghalian, Bernadette; Narchi, Hassib

    2015-01-01

    Henoch-Schönlein purpura (HSP) is the most common vasculitis found in children. It usually affects the small vessels of the skin, joints, gastrointestinal tract and, more rarely, kidneys, brain, lungs and genitalia. Apart from classical presentation with purpuric rashes around buttocks and lower extremities, features such as arthralgia, abdominal pain, haematuria and proteinuria as well as scrotal swelling have been described in the literature. Penile involvement is rare and is not commonly described. We describe a child with HSP who developed penile involvement. We review the literature of all the cases reported in detail, in order to highlight useful clinical presentation, management and prognosis of this rare manifestation. PMID:25858918

  9. Duodenal Perforation Precipitated by Scrub Typhus.

    PubMed

    Rajat, Raghunath; Deepu, David; Jonathan, Arul Jeevan; Prabhakar, Abhilash Kundavaram Paul

    2015-01-01

    Scrub typhus is an acute febrile illness usually presenting with fever, myalgia, headache, and a pathognomonic eschar. Severe infection may lead to multiple organ failure and death. Gastrointestinal tract involvement in the form of gastric mucosal erosions and ulcerations owing to vasculitis resulting in gastrointestinal bleeding is common. This process may worsen a pre-existent asymptomatic peptic ulcer, causing duodenal perforation, and present as an acute abdomen requiring surgical exploration. We report the case of a patient with no previous symptoms or risk factors for a duodenal ulcer, who presented with an acute duodenal perforation, probably precipitated by scrub typhus infection. PMID:26069430

  10. Subarachnoid and intracerebral hemorrhage in patients with churg-strauss syndrome: two case reports.

    PubMed

    Go, Myeong Hoon; Park, Jeong Un; Kang, Jae Gyu; Lim, Yong Cheol

    2012-09-01

    Churg-Strauss syndrome (CSS) is a systemic necrotizing vasculitis of the small and medium vessels, associated with extravascular eosinophilic granulomas, peripheral eosinophilia, and asthma. The exact etiology of CSS is unknown. This syndrome commonly affects the lungs, peripheral nerves, skin, heart, and gastrointestinal tract, but rarely the central nervous system. Subarachnoid and intracerebral hemorrhage in CSS patients is extremely rare; however, clinicians should consider that CSS may be a cause of intracranial hemorrhage and its high rate of mortality and morbidity. The authors report on two cases of subarachnoid and intracerebral hemorrhage with CSS and discuss a brief review of CSS. PMID:23210058

  11. [Skin manifestations related to hepatitis C viruses].

    PubMed

    Doutre, M S; Beylot, C; Beylot-Barry, M; Couzigou, P; Beylot, J

    1995-01-01

    The hepatitis C virus causes both hepatic and extrahepatic disorders, particularly as regards dermatology. The link between essential mixed cryoglobulinemias and the C virus infection has been clearly evidenced., whereas its frequency seems low in other systemic vasculitis such as polyarteritis nodosa. Similarly, the link between C virus hepatopathy and porphyria cutanea tarda is now proven. Lichen planus is also described as being associated with this virus, but further epidemiological studies are required to determine the exact prevalence of lichen in C virus hepatopathy cases. Finally, various cutaneous disorders, such as urticaria, erythema multiforme, dermo-hypodermitis, etc, occasionally arise during acute or chronic hepatitis C. PMID:7481154

  12. Behcet's Disease with Intracardiac Thrombus Presenting with Fever of Unknown Etiology

    PubMed Central

    Ajmani, Sajal; Misra, Durga Prasanna; Raja, Deep Chandh; Mohindra, Namita; Agarwal, Vikas

    2015-01-01

    A young male was referred to us for evaluation of fever of unknown origin (FUO). He had history of recurrent painful oral ulcers for one year and moderate to high grade fever, pustulopapular rash, and recurrent genital ulcers for 6 months and hemoptysis for 3 days. He was detected to have intracardiac thrombi and pulmonary arterial thrombosis along with underlying Behcet's disease (BD). Patient responded to high dose prednisolone (1?mg/Kg/day) along with monthly parenteral cyclophosphamide therapy. This case highlights the fact that BD is an important cause for pulmonary artery vasculitis with intracardiac thrombus formation, and such patients can present with FUO. PMID:26425377

  13. [An abdominal aortic aneurysm revealing Behçet's disease].

    PubMed

    Naouli, H; Zrihni, Y; Jiber, H; Bouarhroum, A

    2014-12-01

    Behçet's disease is a vasculitis of unknown origin. Vascular lesions predominantly affect veins. Arterial involvement is rare but usually associated with poor prognosis. Aortic syndromes are usually aneurysmal and occasionally reveal Behçet's disease. We report the case of a 46-year-old man whose Behçet's disease was revealed by a sub-renal abdominal aortic aneurysm in pre-rupture state. The diagnosis of this disease was retained based on clinical, biological and radiological criteria. The surgical procedure consisted in the resection of the aneurysmal sac and the interposition of a prosthetic PTFE tube. PMID:25457357

  14. Acute mental status change as the presenting feature of adrenal insufficiency in a patient with autoimmune polyglandular syndrome type II and stroke.

    PubMed

    Watson, Sara; Raj, Shekar; Eugster, Erica; Sanchez, Juan

    2014-05-01

    Primary adrenal insufficiency (AI) in children usually presents with non-specific symptoms such as fatigue, nausea, vomiting, and anorexia. Here, we report an unusual case of a 15 year old girl who presented with acute mental status change and was ultimately diagnosed with AI due to autoimmune polyglandular syndrome type II (APS2). Central nervous system imaging revealed a cerebral infarction. To our knowledge, the constellation of APS2, stroke and acute mental status change has not been previously reported. We review the literature with regard to the presentation of AI as well as the association between vasculitis and APS2. PMID:24259239

  15. Hypereosinophilic syndrome as a cause of fatal thrombosis: two case reports with histological study.

    PubMed

    Fujita, Kumi; Ishimaru, Hiroyasu; Hatta, Kazuhiro; Kobashi, Yoichiro

    2015-08-01

    Herein we present two cases of hypereosinophilic syndrome with a unique clinical presentation. One patient showed severe systemic thrombosis with splenic rupture and the other patient showed finger gangrene with various systemic symptoms. Both patients were examined histologically, and several characteristics were noted. First, fresh or organized thrombosis with marked eosinophilic infiltration was observed in the cavity and walls of the thrombosed vessels. Second, many eosinophils showed degranulation and were positive for eosinophilic cationic protein on immunohistological examination. Third, the structures of thrombosed vessels were well preserved, which is not observed in systemic vasculitis. These patients exhibited no neoplastic features and were treated with prednisolone with excellent therapeutic results. PMID:25388084

  16. IgG4-Related Tubulointerstitial Nephritis Pattern in 18F-FDG PET/CT.

    PubMed

    Bélissant, Ophélie; Guernou, Mohamed; Rouvier, Philippe; Compain, Caroline; Bonardel, Gérald

    2015-10-01

    A 17-year-old adolescent girl was admitted with chronic arthralgia, Raynaud phenomenon, pericarditis, and evidences of chronic diffuse inflammation. F-FDG PET/CT scan was performed to search systemic vasculitis and showed diffuse moderate uptake in the kidneys. We suggested the existence of a nephritis, but the ultrasonography result was normal, and no treatment was introduced. Another F-FDG PET/CT scan was performed 7 months later to explore abdominal pain. It showed again diffuse intense uptake in both kidneys. A proteinuria was highlighted, and renal biopsy allowed to diagnose IgG4-related disease. PMID:26204208

  17. FDG PET-CT in the Diagnosis of Takayasu Arteritis Presenting as Fever of Unknown Origin: A Case Report

    PubMed Central

    Kim, Joohae

    2015-01-01

    Takayasu arteritis is a chronic vasculitis involving the large vessels. At diagnosis, ischemic symptom are usually present in the affected vessels. However, fever of unknown origin (FUO) is rare as an initial presentation and renders the condition difficult to diagnose. In this case report, we describe a patient who presented with a fever of unknown origin. A 68-year-old female was diagnosed with Takayasu arteritis after fluorodeoxyglucose (FDG) positron-emission tomography-computed tomography (PET-CT) was performed at the prepulseless stage. FDG PET-CT can assist in the early diagnosis of Takayasu arteritis patients with FUO and can improve the prognosis of such patients. PMID:26483994

  18. Endovascular Treatment of a Superior Mesenteric Artery Aneurysm Secondary to Behcet's Disease with Onyx (Ethylene Vinyl Alcohol Copolymer)

    SciTech Connect

    Gueven, Koray Rozanes, Izzet; Kayabali, Murat; Minareci, Ozenc

    2009-01-15

    Behcet's disease is a complex multisystemic chronic inflammatory disease that is characterized by oral and genital aphtous ulcers and vasculitis. Aneurysms of major arteries are the most important cause of mortality in Behcet's disease. Four patients with superior mesenteric artery (SMA) aneurysms related to Behcet's disease have been reported in the literature. We report here the first successful endovascular treatment of a giant, wide-necked SMA aneurysm secondary to Behcet's disease. We performed a balloon-assisted embolization technique using ethylene vinyl alcohol copolymer (Onyx, ev3, Irvine, CA, USA). There were no signs of recurrence during 2-year follow-up.

  19. Thromboangiitis obliterans (Buerger disease)

    PubMed Central

    Seebald, Jessica; Gritters, Lyndon

    2015-01-01

    Thromboangiitis obliterans (Buerger disease) is an occlusive, nonatherosclerotic, inflammatory vasculitis that causes ischemia in small and medium vessels. Most commonly, Buerger disease is diagnosed in 40- to 45-year-old men with a heavy smoking history. Our case exemplifies the most common presentation, diagnosis, and treatment in a 53-year-old male smoker who presents with arm pain and dusky cool fingers. A Buerger diagnosis requires exclusion of autoimmune, diabetic, and embolic causes. The only recognized treatment for this disease is smoking cessation. PMID:26649109

  20. Polyarteritis nodosa presenting as massive upper gastrointestinal hemorrhage.

    PubMed

    Perez, R A; Silver, D; Banerjee, B

    2000-01-01

    We report the first case of massive upper gastrointestinal hemorrhage as the initial presentation of polyarteritis nodosa (PAN), which is an uncommon form of systemic necrotizing vasculitis that may involve many organ systems and could affect any age group. Abdominal pain is the most common sign of gastrointestinal involvement. Gastrointestinal bleeding occurs less frequently in approximately 6% of cases. Reported cases of gastrointestinal hemorrhage have been in the form of coffee ground emesis, melena, or hematochezia. Such bleeding complications have resulted from ischemic ulceration or perforation of the small or large intestine. However, we are unaware of previous reports showing massive hematemesis to be the initial presentation of PAN. PMID:10854517

  1. Behçet's disease

    PubMed Central

    Kontogiannis, V; Powell, R

    2000-01-01

    Behçet's disease is a systemic vasculitis of unknown aetiology characteristically affecting venules. Onset is typically in young adults with recurrent oral and genital ulceration, uveitis, skin manifestations, arthritis, neurological involvement, and a tendency to thrombosis. It has a worldwide distribution but is prevalent in Japan, the Middle East, and some Mediterranean countries. International diagnostic criteria have been proposed, however diagnosis can be problematical, particularly if the typical ulcers are not obvious at presentation. Treatment is challenging, must be tailored to the pattern of organ involvement for each patient and often requires combination therapies.???Keywords: Behçet's disease; oral ulcers; uveitis; immunosuppressants PMID:11009577

  2. What is myeloperoxidase doing in ANCA-associated glomerulonephritis?

    PubMed

    Couser, William G; Johnson, Richard J

    2015-11-01

    O'Sullivan et al. describe glomerular localization of myeloperoxidase (MPO) in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and correlate the amount of deposition with severity of injury. MPO is the antigen against which anti-MPO ANCAs are directed, the antigen to which pathogenic T cells that can induce antibody-independent AAV are directed, and MPO can induce glomerular injury directly by interacting with H2O2 and a halide to halogenate glomerular structures. All three of these roles are likely involved in human disease. PMID:26579678

  3. Technetium scanning in Kaposi's sarcoma and its simulators

    SciTech Connect

    Gunnoe, R.; Kalivas, J.

    1982-04-01

    The clinical picture of ulcerated purple plaques on the legs often suggests several diagnoses: Kaposi's sarcoma, stasis dermatitis, atrophie blanche (livedoid vasculitis), and a poorly understood condition called acroangiodermatitis of Favre-Chaix (pseudo-Kaposi's sarcoma). Even the skin biopsy may not always be conclusive. We describe our experience with three patients with pseudo-Kaposi's sarcoma, one with true Kaposi's sarcoma and two with atrophie blanche. Clinical and histopathologic similarities among these three conditions pointed up the need for additional confirmatory studies, i.e., isotope scanning. The technetium scan was positive in both Kaposi's sarcoma and pseudo-Kaposi's sarcoma but negative in atrophie blanche.

  4. Computed Tomography Angiography of the Lower Extremities.

    PubMed

    Cook, Tessa Sundaram

    2016-01-01

    CT angiography (CTA) of the lower extremities is an important and versatile, noninvasive tool for diagnosis as well as surgical or endovascular interventional planning. Although lower extremity CTA is most commonly performed in patients with peripheral artery disease or trauma affecting the lower extremities, it also plays a role in the workup of nonischemic etiologies such as vasculitis, aneurysms, and congenital vascular malformations. CT scan protocols should adjust bolus timing and multiphasic imaging to account for the clinical question of interest, and 3-dimensional postprocessing plays an important role in the visualization and interpretation of these high-resolution imaging examinations. PMID:26654395

  5. Retiform purpura: a new stigmata of illicit drug use?

    PubMed

    Geller, Lauren; Whang, Talley B; Mercer, Stephen E; Phelps, Robert

    2011-01-01

    We report a 50-year-old woman who presented with a six-month history of recurrent retiform purpura of uncertain etiology. Laboratory findings included neutropenia, positive anticardiolipin IgM antibody, and a weakly positive p-ANCA. Histopathology revealed a leukocytoclastic vasculitis with intravascular thrombi. Urine toxicology screen was positive for cocaine. These findings are similar to recent reports of agranulocytosis and purpura induced by levamisole-tainted cocaine. A review of the clinical and histopathological findings associated with levamisole-induced purpura will be discussed. PMID:21382290

  6. Splenic Infarct Due to Scrub Typhus

    PubMed Central

    Raj, Selvin Sundar; Krishnamoorthy, Adhiti; Jagannati, Manjeera; Abhilash, Kundavaram PP

    2014-01-01

    Scrub typhus is a mite borne infectious disease that has the potential to involve multiple organs and can be fatal. Involvement of the abdomen in the form of hepatitis, gastric ulcerations and pancreatitis are well-documented, the pathology being disseminated vasculitis. However involvement of the spleen in scrub typhus is extremely rare and is reported only in a few autopsy studies. We report the case of a 50-year-old lady who presented with fever and left upper quadrant abdominal pain due to a splenic infarct due to scrub typhus. PMID:24926171

  7. 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

  8. Can sulfasalazine therapy induce or exacerbate Wegener's granulomatosis?

    PubMed

    Denissen, N H A M; Peters, J G P; Masereeuw, R; Barrera, P

    2008-01-01

    Sulfasalazine (SSZ) can induce serological and clinical autoimmune reactions but the occurrence of SSZ-related Wegener's granulomatosis (WG) has not been reported before. We describe two patients with rheumatoid factor (RF)-positive rheumatoid arthritis (RA) who developed biopsy-proven WG with serious organ involvement during SSZ therapy. The pathogenetic mechanism that explains the relationship between SSZ and the occurrence of a de novo anti-neutrophil cytoplasmic antibody (ANCA)-related vasculitis or a flare is discussed. We propose that WG can be a rare complication of SSZ therapy and that this, like other autoimmune adverse events of this drug, is mediated by SSZ-induced apoptosis. PMID:18189198

  9. Overlap between dermatomyositis and ANCA vasculitides

    PubMed Central

    Yuste, Claudia; Rapalai, Molefe; Pritchard, Benjamin A.; Jones, Terence J.; Amoasii, Constanza; Al-Ansari, Atheer; Ramakrishna, Satish B.

    2014-01-01

    We present the second report of the association between antineutrophil cytoplasm antibodies (ANCA)-associated vasculitis with dermatomyositis (DM). A 47-year-old woman suddenly developed rapidly progressive renal failure in the context of (DM). The kidney biopsy showed focal and segmental necrotizing glomerulonephritis with crescent formation. Cyclophosphamide treatment was commenced resulting in a significant recovery of kidney function and maintenance of recovery at 6 months. Although the pathophysiology is unknown, we hypothesize that CD8-T-deficient cells and MPO+ neutrophils in the DM lesions play an important role in the disease process. PMID:25859353

  10. Sildenafil in severe peripheral ischemia induced by terlipressin. A case report.

    PubMed

    Bañuelos Ramírez, David D; Sánchez Alonso, Silvia; Ramírez Palma, María Magdalena

    2011-01-01

    A female patient in the sixth decade of life developed renal failure secondary to gastrointestinal bleeding, which was non-responsive to conventional therapy. She was treated with terlipressin iv; showing improvement but developing, over the next few days, ischemia and necrosis of the toes in the lower extremities, simulating necrotizing vasculitis, without response to regular management. An alternative therapy, oral sildenafil 50mg BID, was used, with a rapid reversal of the clinical picture. The vasodilator action of sildenafil is useful in cases of ischemia induced by some drugs. The objective this report is to warm about the utility of sildenafil in some potentially severe cases, like the one above. PMID:21794781

  11. Behçet's disease with a large intracardiac thrombus: a case report

    PubMed Central

    Baykan, M; Celik, S; Erdol, C; Baykan, E; Durmus, I; Bahadir, S; Erdol, H; Orem, C; Cakirbay, H

    2001-01-01

    Behçet's disease is recognised as a chronic multisystem disorder with vasculitis as its underlying pathological process. Cardiac involvement is rare and often associated with poor prognosis. A case of a 33 year old man with Behçet's disease, presenting with a large right ventricle and right atrial thrombus, is reported. Two dimensional (cross sectional), colour Doppler, and transoesophageal echocardiography, angiography, computed tomography, and magnetic resonance imaging were used to diagnose the disease. With cyclophosphamide and dexamethasone treatment, the cardiac lesions progressively resolved.???Keywords: Behçet's disease; intracardiac thrombus PMID:11250984

  12. Sacral myeloradiculitis (Elsberg syndrome) secondary to eosinophilic meningitis caused by Angiostrongylus cantonensis

    PubMed Central

    Hsu, Jui-Jen; Chuang, Shin-Hung; Chen, Chia-Hsin; Huang, Mao-Hsiung

    2009-01-01

    Elsberg syndrome secondary to eosinophilic meningitis caused by Angiostrongylus cantonensis is uncommon. Clinicians should consider a wide differential diagnosis including tumour, spinal cord infarction, necrosis, vasculitis, drug induced or other sources of infection. In addition, acute urinary retention is a urological emergency and clinicians should keep in mind the prevention of bladder overdistension. The intervention of rehabilitation programmes and clean intermittent catheterisation education for bladder management, in accordance with the patient’s condition, is also important. Earlier rehabilitation is important to ensure a speedy recovery and to prevent further complications. PMID:21811516

  13. Late-appearing brachiocephalic aneurysm: an atypical vascular sequella of Kawasaki disease.

    PubMed

    Yang, Gaby; Thompson, Deborah; Warren, Andrew

    2009-02-01

    Kawasaki disease (KD) is an acute vasculitis that can result in cardiovascular sequelae. One of the most serious consequences of KD is the development of arterial aneurysms that can lead to thrombosis and ischemia. Commonly, these lesions, occurring early in the course of KD, are found in the coronary arteries. However, this location is not exclusive. This report presents a case of KD with atypical findings of a late-appearing brachiocephalic artery aneurysm. The authors suggest that for patients with complicated KD, surveillance should include a careful vascular survey to discover aneurysms distant from the heart. PMID:18704549

  14. Experimental adenovirus hemorrhagic disease in white-tailed deer fawns.

    PubMed

    Woods, L W; Lehmkuhl, H D; Swift, P K; Chiu, P H; Hanley, R S; Nordhausen, R W; Stillian, M H; Drew, M L

    2001-01-01

    Infection with a newly described endotheliotropic adenovirus was the cause of a 1993 epizootic reminiscent of hemorrhagic disease in California mule deer (Odocoileus hemionus columbianus and O. hemionus hemionus). Pulmonary edema and intestinal luminal hemorrhage, or necrotizing stomatitis associated with systemic or localized vasculitis, respectively, were common lesions seen in animals that died during the epizootic. In order to determine if white-tailed deer (Odocoileus virginianus) also are susceptible to infection and fatal disease with the deer adenovirus, eight white-tailed deer fawns (4- to 6-mo-old) were inoculated with purified deer adenovirus. Four were inoculated intravenously and four were inoculated through the mucous membranes. Seven days post-inoculation, one of the fawns inoculated intravenously died. Pulmonary edema and hemorrhagic enteropathy were associated with pulmonary and intestinal vasculitis with systemic multiorgan distribution of endotheliotropic adenovirus as demonstrated by transmission electron microscopy and immunohistochemistry. Adenovirus was reisolated from lung homogenates of the fawn that died of adenovirus hemorrhagic disease. PMID:11272490

  15. Cavia porcellus as a Model for Experimental Infection by Trypanosoma cruzi

    PubMed Central

    Castro-Sesquen, Yagahira E.; Gilman, Robert H.; Yauri, Verónica; Angulo, Noelia; Verastegui, Manuela; Velásquez, Daniel E.; Sterling, Charles R.; Martin, Diana; Bern, Caryn

    2011-01-01

    The guinea pig (Cavia porcellus) is a natural reservoir for Trypanosoma cruzi but has seldom been used as an experimental infection model. We developed a guinea pig infection model for acute and chronic Chagas disease. Seventy-two guinea pigs were inoculated intradermally with 104 trypomastigotes of T. cruzi strain Y (experimental group); 18 guinea pigs were used as control group. Eight animals from the experimental group and two from the control group were sacrificed 5, 15, 20, 25, 40, 55, 115, 165, and 365 days after inoculation. During the acute phase (15 to 55 days), we observed parasitemia (with a peak on day 20) and positive IgM and IgG Western blots with anti-shed acute-phase antigen bands. The cardiac tissue showed vasculitis, necrosis (on days 40 to 55), moderate to severe inflammation, and abundant amastigote nests. Smaller numbers of amastigote nests were also present in kidney, brain, and other organs. In the early chronic phase (115 to 165 days), parasitemia disappeared and anti–T. cruzi IgG antibodies were still detectable. In cardiac tissue, the number of amastigote nests and the grade of inflammation decreased. In the chronic phase (365 days), the cardiac tissue showed vasculitis and fibrosis; detectable parasite DNA was associated with higher grades of inflammation. The experimental T. cruzi infection model in guinea pigs shows kinetics and pathologic changes similar to those of the human disease. PMID:21703410

  16. Donor-Specific Antibodies Accelerate Arteriosclerosis after Kidney Transplantation

    PubMed Central

    Nochy, Dominique; Bruneval, Patrick; van Huyen, J. P. Duong; Glotz, Denis; Suberbielle, Caroline; Zuber, Julien; Anglicheau, Dany; Empana, Jean-Philippe; Legendre, Christophe; Loupy, Alexandre

    2011-01-01

    In biopsies of renal allografts, arteriosclerosis is often more severe than expected based on the age of the donor, even without a history of rejection vasculitis. To determine whether preformed donor-specific antibodies (DSAs) may contribute to the severity of arteriosclerosis, we examined protocol biopsies from patients with (n = 40) or without (n = 59) DSA after excluding those with any evidence of vasculitis. Among DSA-positive patients, arteriosclerosis significantly progressed between month 3 and month 12 after transplant (mean Banff cv score 0.65 ± 0.11 to 1.12 ± 0.10, P = 0.014); in contrast, among DSA-negative patients, we did not detect a statistically significant progression during the same timeframe (mean Banff cv score 0.65 ± 0.11 to 0.81 ± 0.10, P = not significant). Available biopsies at later time points supported a rate of progression of arteriosclerosis in DSA-negative patients that was approximately one third that in DSA-positive patients. Accelerated arteriosclerosis was significantly associated with peritubular capillary leukocytic infiltration, glomerulitis, subclinical antibody-mediated rejection, and interstitial inflammation. In conclusion, these data support the hypothesis that donor-specific antibodies dramatically accelerate post-transplant progression of arteriosclerosis. PMID:21493773

  17. Autoantibodies in Renal Diseases – Clinical Significance and Recent Developments in Serological Detection

    PubMed Central

    Mastroianni-Kirsztajn, Gianna; Hornig, Nora; Schlumberger, Wolfgang

    2015-01-01

    Autoimmune dysfunctions are the “bête noire” in a range of debilitating nephropathies. Autoimmune-mediated damage to the kidneys can be triggered by autoantibodies directed against specific proteins or renal structures, for example, the phospholipase A2 receptor or the glomerular basement membrane, resulting in glomerular diseases such as primary membranous nephropathy or Goodpasture’s disease. Moreover, secondary damage to the kidney can be part of the wide-reaching effects of systemic autoimmune diseases such as vasculitis or systemic lupus erythematosus (SLE) – the latter counts lupus nephritis among its most severe manifestations. Systemic autoimmune diseases are characterized by non-organ-specific autoantibodies, directed for example against neutrophil cytoplasmic antigens in systemic vasculitis and against double-stranded DNA and nucleosomes in SLE. A large variety of innovative and highly specific and sensitive autoantibody tests have been developed in the last years that are available to identify autoimmune kidney diseases at an early stage. Thus, serological in vitro diagnostics allow for appropriate interventional therapy in order to prevent disease progression often resulting in need of dialysis and transplantation. PMID:26029207

  18. Granulomatosis with polyangiitis and intravenous immunoglobulins: a case series and review of the literature.

    PubMed

    Guidelli, Giacomo Maria; Tenti, Sara; Pascarelli, Nicola Antonio; Galeazzi, Mauro; Fioravanti, Antonella

    2015-08-01

    Granulomatosis with polyangiitis, formerly known as Wegener's granulomatosis or disease, is a systemic, necrotizing small-vessel vasculitis, belonging to the group of anti-neutrophil cytoplasm antibody vasculitis. The therapeutic strategy includes, in most cases, corticosteroids associated, at least in severe forms of the disease, with immunosuppressive agents: cyclophosphamide and rituximab to induce remission, methotrexate, azathioprine and mycophenolate mofetil to prevent relapses. Intravenous immunoglobulins represent an alternative adjuvant therapy. We described 5 cases of patients with granulomatosis with polyangiitis treated with monthly high-dose intravenous immunoglobulins (500mg/kg/daily for 3 consecutive days for 9months). No patients experienced adverse reactions, and 4 patients (80%) achieved a complete remission after 9 courses of this therapy, which was maintained also 3months later, although we are unable to determine whether improvement in outcomes was a direct result of the IVIG. We also discussed the beneficial effects of intravenous immunoglobulins in patients suffering from granulomatosis with polyangiitis, reporting the previously published data. PMID:25816995

  19. Microscopic polyangiitis: Advances in diagnostic and therapeutic approaches.

    PubMed

    Greco, Antonio; De Virgilio, Armando; Rizzo, Maria Ida; Gallo, Andrea; Magliulo, Giuseppe; Fusconi, Massimo; Ruoppolo, Giovanni; Tombolini, Mario; Turchetta, Rosaria; de Vincentiis, Marco

    2015-09-01

    Microscopic polyangiitis (MPA) is an idiopathic autoimmune disease characterized by systemic vasculitis. The disease predominantly affects small-calibre blood vessels and is associated with the presence of antineutrophil cytoplasmic autoantibodies (ANCA). Microscopic polyangiitis was considered to be a disease entity by Savage et al. in 1985. Microscopic polyangiitis has a reported low incidence and a slight male predominance. The aetiology of MPA remains unknown. There is, however, increased evidence that MPA is an autoimmune disease in which ANCAs, particularly those reacting with MPO, are pathogenic. MPA belongs to the systemic vasculitides, indicating that multiple organs can be affected. The major organs involved in MPA are the kidneys and the lungs. As expected for an illness that affects multiple organ systems, patients with MPA can present with a myriad of different symptoms. Ear, nose and throat (ENT) manifestations are not considered to be clinical symptoms of MPA, but in the majority of populations described, ENT involvement was found in surprisingly high percentages. MPA is part of the ANCA-associated vasculitides, which are characterized by necrotizing vasculitis of small vessels. Diagnosis is mainly established by clinical manifestations, computed tomography (TC), ANCA antibody detection and renal and pulmonary biopsy. The introduction of aggressive immunosuppressive treatment has substantially improved the prognosis. The standardized therapeutic regimen is based on cyclophosphamide and corticosteroids. Using this regimen, remission can be achieved in most of the patients. Rituximab may represent an important alternative to cyclophosphamide for patients who may not respond adequately to antimetabolite therapies. PMID:25992801

  20. Vitreous hemorrhage secondary to retinal vasculopathy in a patient with dyskeratosis congenita.

    PubMed

    Finzi, Alessandro; Morara, Mariachiara; Pichi, Francesco; Veronese, Chiara; Ciardella, Antonio P

    2014-08-01

    The aim of this study was to report a case of vitreous hemorrhage secondary to retinal vasculitis in a patient with dyskeratosis congenita. A 16-year-old white male was referred to the Ophthalmology Clinic due to deterioration of vision in his left eye. Medical history was significant for dyskeratosis congenita associated with thrombocytopenia. General physical examination revealed reticular pigmentation on the upper half of the chest, vertical ridges and splitting of finger nails, and oral mucosal leukoplakia. Ophthalmological examination of the anterior segment was unremarkable. Retinal examination revealed vitreous hemorrhage in the left eye veiling the retinal details. A possible history of trauma was denied. Fundus examination of the right eye showed retinal vascular sheathing with a few dot and blot retinal hemorrhages. Fluorescein angiography revealed extensive areas of non-perfusion beyond the equator in the right eye, later treated with scatter laser photocoagulation. We performed a 23-gauge vitrectomy with endolaser treatment of the new vascularization areas in the left eye. After 6 months, best-corrected visual acuity in the right and left eye was 20/20 and 20/25, respectively. Rather than being confined to anterior segment abnormalities like conjunctivitis, blepharitis and nasolacrimal duct obstruction which are reported in the literature, dyskeratosis congenita can cause significant visual loss due to retinal vasculitis and vitreous hemorrhage. Therefore physicians and ophthalmologists should be aware of this possibility and prompt diagnosis and treatment could prevent further visual loss in such patients. PMID:24114504

  1. Hamsi scoring in the prediction of unfavorable outcomes from tuberculous meningitis: results of Haydarpasa-II study.

    PubMed

    Erdem, Hakan; Ozturk-Engin, Derya; Tireli, Hulya; Kilicoglu, Gamze; Defres, Sylviane; Gulsun, Serda; Sengoz, Gonul; Crisan, Alexandru; Johansen, Isik Somuncu; Inan, Asuman; Nechifor, Mihai; Al-Mahdawi, Akram; Civljak, Rok; Ozguler, Muge; Savic, Branislava; Ceran, Nurgul; Cacopardo, Bruno; Inal, Ayse Seza; Namiduru, Mustafa; Dayan, Saim; Kayabas, Uner; Parlak, Emine; Khalifa, Ahmad; Kursun, Ebru; Sipahi, Oguz Resat; Yemisen, Mucahit; Akbulut, Ayhan; Bitirgen, Mehmet; Popovic, Natasa; Kandemir, Bahar; Luca, Catalina; Parlak, Mehmet; Stahl, Jean Paul; Pehlivanoglu, Filiz; Simeon, Soline; Ulu-Kilic, Aysegul; Yasar, Kadriye; Yilmaz, Gulden; Yilmaz, Emel; Beovic, Bojana; Catroux, Melanie; Lakatos, Botond; Sunbul, Mustafa; Oncul, Oral; Alabay, Selma; Sahin-Horasan, Elif; Kose, Sukran; Shehata, Ghaydaa; Andre, Katell; Dragovac, Gorana; Gul, Hanefi Cem; Karakas, Ahmet; Chadapaud, Stéphane; Hansmann, Yves; Harxhi, Arjan; Kirova, Valerija; Masse-Chabredier, Isabelle; Oncu, Serkan; Sener, Alper; Tekin, Recep; Elaldi, Nazif; Deveci, Ozcan; Ozkaya, Hacer Deniz; Karabay, Oguz; Senbayrak, Seniha; Agalar, Canan; Vahaboglu, Haluk

    2015-01-01

    Predicting unfavorable outcome is of paramount importance in clinical decision making. Accordingly, we designed this multinational study, which provided the largest case series of tuberculous meningitis (TBM). 43 centers from 14 countries (Albania, Croatia, Denmark, Egypt, France, Hungary, Iraq, Italy, Macedonia, Romania, Serbia, Slovenia, Syria, Turkey) submitted data of microbiologically confirmed TBM patients hospitalized between 2000 and 2012. Unfavorable outcome was defined as survival with significant sequela or death. In developing our index, binary logistic regression models were constructed via 200 replicates of database by bootstrap resampling methodology. The final model was built according to the selection frequencies of variables. The severity scale included variables with arbitrary scores proportional to predictive powers of terms in the final model. The final model was internally validated by bootstrap resampling. A total of 507 patients' data were submitted among which 165 had unfavorable outcome. Eighty-six patients died while 119 had different neurological sequelae in 79 (16%) patients. The full model included 13 variables. Age, nausea, vomiting, altered consciousness, hydrocephalus, vasculitis, immunosuppression, diabetes mellitus and neurological deficit remained in the final model. Scores 1-3 were assigned to the variables in the severity scale, which included scores of 1-6. The distribution of mortality for the scores 1-6 was 3.4, 8.2, 20.6, 31, 30 and 40.1%, respectively. Altered consciousness, diabetes mellitus, immunosuppression, neurological deficits, hydrocephalus, and vasculitis predicted the unfavorable outcome in the scoring and the cumulative score provided a linear estimation of prognosis. PMID:25634680

  2. A review on the vascular features of the hyperimmunoglobulin E syndrome

    PubMed Central

    Yavuz, H; Chee, R

    2010-01-01

    Autosomal recessive, autosomal dominant and the sporadic forms of hyperimmunoglobulin E syndrome (HIES) are multi-system disorders. Although HIES patients may present with cold abscesses, the vascular features of HIES are not well recognized. The objective of this review is to characterize the nature and spectrum of vascular abnormalities in HIES patients. Vascular abnormalities in HIES patients were reviewed with Medline and Google Scholar-based searches. In brief, the searches combined terms related to HIES with the terms related to vasculature. Furthermore, reference lists from the original studies and review papers identified were screened. There were vascular abnormalities in 25 patients with HIES. These abnormalities were identified as aneurysms (coronary, aortic, carotid and cerebral), pseudoaneurysms, congenital patent ductus venosus, superior vena cava syndrome, vasculitides, vascular ectasia, thrombosis and others. They may be congenital or acquired, in the veins and arteries, affecting both sexes. These abnormalities can be seen in all subtypes of HIES. They could be also fatal in children and adults. Limited pathological investigations revealed the presence of vasculitis. Three of the patients were found to have overlap diseases. In this review, the spectrum of vascular abnormalities in HIES are documented and discussed in detail for the first time. They highlight a previously under-recognized and potentially devastating complication of these disorders. These vascular abnormalities constitute one of the major clinical characteristics in HIES. The presence of hypereosinophilia, vasculitis and defective angiogenesis in HIES may contribute to the formation of vascular abnormalities in HIES. PMID:19912258

  3. Neurological manifestations of gastrointestinal disorders, with particular reference to the differential diagnosis of multiple sclerosis.

    PubMed

    Ghezzi, A; Zaffaroni, M

    2001-11-01

    Neurological manifestations of gastrointestinal disorders are described, with particular reference to those resembling multiple sclerosis (MS) on clinical or MRI grounds. Patients with celiac disease can present cerebellar ataxia, progressive myoclonic ataxia, myelopathy, or cerebral, brainstem and peripheral nerve involvement. Antigliadin antibodies can be found in subjects with neurological dysfunction of unknown cause, particularly in sporadic cerebellar ataxia ("gluten ataxia"). Patients with Whipple's disease can develop mental and psychiatric changes, supranuclear gaze palsy, upper motoneuron signs, hypothalamic dysfunction, cranial nerve abnormalities, seizures, ataxia, myorhythmia and sensory deficits. Neurological manifestations can complicate inflammatory bowel disease (e.g. ulcerative colitis and Crohn's disease) due to vascular or vasculitic mechanisms. Cases with both Crohn's disease and MS or cerebral vasculitis are described. Epilepsy, chronic inflammatory polyneuropathy, muscle involvement and myasthenia gravis are also reported. The central nervous system can be affected in patients with hepatitis C virus (HCV) infection because of vasculitis associated with HCV-related cryoglobulinemia. Mitochondrial neurogastrointestinal encephalopathy (MNGIE) is a disease caused by multiple deletions of mitochondrial DNA. It is characterized by peripheral neuropathy, ophthalmoplegia, deafness, leukoencephalopathy, and gastrointestinal symptoms due to visceral neuropathy. Neurological manifestations can be the consequence of vitamin B1, nicotinamide, vitamin B12, vitamin D, or vitamin E deficiency and from nutritional deficiency states following gastric surgery. PMID:11794474

  4. Successful management of Churg-Strauss syndrome using omalizumab as adjuvant immunomodulatory therapy: first documented pediatric case.

    PubMed

    Iglesias, E; Camacho Lovillo, M; Delgado Pecellín, I; Lirola Cruz, M J; Falcón Neyra, M D; Salazar Quero, J C; Bernabeu-Wittel, J; González Valencia, J P; Neth, O

    2014-03-01

    Churg-Strauss syndrome (CSS) is an anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis; it is extremely rare in childhood and defined according to the Chapel-Hill Consensus as an eosinophil-rich and granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small to medium-sized vessels. Children commonly have a history of asthma and sinusitis whilst clinical presentation typically involves pulmonary tract and less frequently skin, heart, gastrointestinal tract, and peripheral nerves. Cardiopulmonary disease is higher in children and prognosis is worse. It is associated with significant eosinophilia and raised serum IgE-levels. ANCA are only found in 25% of childhood cases. Here we report the case of a 10-year-old girl who presented to us with vomiting, abdominal pain, and weight loss, paresthesias of lower extremities and breathlessness as well as a history of asthma, sinusitis and allergic rhinitis. She was treated with corticosteroids, cyclophosphamide, intravenous immunoglobulin, mycophenolate mofetil (MMF), and rituximab. However, remission was only achieved after initiation of omalizumab therapy, a recombinant humanized anti-IgE antibody. To the best of our knowledge this is the first pediatric patient suffering from CSS successfully managed with adjuvant anti-IgE therapy resulting in the control of respiratory as well as gastrointestinal symptoms. PMID:24136903

  5. Pneumatosis intestinalis associated with Henoch-Schönlein purpura.

    PubMed

    Fatima, Ayesha; Gibson, Donald Paul

    2014-09-01

    Henoch-Schönlein purpura (HSP) is the most common vasculitis in children. It is a disorder of the inflammatory cascade leading to immunoglobulin A deposition and leukocytoclastic vasculitis of small vessels of skin, kidneys, joints, and gastrointestinal (GI) tract. A wide variety of GI manifestations are seen in ?50% to 75% of patients with HSP. Diffuse colicky abdominal pain is the most common GI symptom. The small bowel is the most frequently involved GI site. Intussusception is rare but is the most common surgical complication. We report the case of a 2-year-old girl with a 5-day history of abdominal pain followed by a palpable purpuric rash. Her urinalysis, complete blood cell count, and tests of renal function were normal. An acute abdominal series was unremarkable initially, and abdominal ultrasound imaging showed ascites and thickened small bowel loops. She was diagnosed with HSP. The abdominal pain worsened, and an abdominal computed tomography scan demonstrated distal small bowel wall thickening and pneumatosis intestinalis in the descending colon. She was started on total parenteral nutrition and antibiotics and placed on bowel rest. She was given 2 mg/kg of intravenous immunoglobulin. Her abdominal pain gradually improved over the next week, and a repeat computed tomography scan showed significant improvement of the small bowel wall thickening and pneumatosis. The purpuric rash improved, and her abdominal pain resolved. We report a case of HSP and pneumatosis intestinalis, an association that has not been reported previously. PMID:25157006

  6. [Symptomatic diseases in pneumology].

    PubMed

    Behr, J; Avsar, K

    2013-10-01

    The diagnosis and treatment of granulomatous lung diseases is challenging. This article describes two of these entities: (1) eosinophilic vasculitis with polyangiitis which is clinically characterized as a combination of bronchial asthma and eosinophilic granulomatous vasculitis. Antoneutrophil cytoplasmic antibodies are present in approximately 40?% of patients. Treatment with steroids is sufficient in patients with isolated pulmonary manifestation but extrapulmonary manifestations, e.g. heart, central nervous system (CNS), kidneys and gastrointestinal tract warrant combined immunosuppression with prednisolone and cyclophosphamide. (2) In Germany tuberculosis is an orphan disease with an incidence of 5.3/100,000 in the year 2011. Prolonged cough, night sweats and weight loss should be considered suspicious of tuberculosis. Microbiological diagnosis has been improved by gene and PCR technology. The traditional Mendel-Mantoux skin test has widely been replaced by the interferon gamma release assay (IGRA). Standard treatment of non-resistant mycobacterium tuberculosis is based on a combination of isonizide, rifampicine, pyrazinamide and ethambutol for 2 months followed by 4 months of isoniazide plus rifampicine. Therapy resistant, multiple drug resistant (MDR) and extensively drug resistant (XDR) tuberculosis bacteria should be treated by experienced specialists. PMID:23989402

  7. Spontaneous Bacterial Peritonitis and Henoch-Schönlein Purpura in a Patient with Liver Cirrhosis

    PubMed Central

    Gupta, Neil; Kim, Joyce; Njei, Basile

    2015-01-01

    Henoch-Schönlein purpura (HSP) is a small vessel systemic vasculitis, predominantly affecting children, characterized by a tetrad of manifestations, specifically palpable purpura, arthralgia, abdominal pain, and renal disease. HSP in the adult population is rare, and no case has been described of HSP in liver cirrhosis with spontaneous bacterial peritonitis (SBP). We present the case of a 58-year-old male with liver cirrhosis, who was subsequently diagnosed with SBP and later HSP. In this patient, the diagnosis of HSP was demonstrated clinically by his palpable purpura, diarrhea, hematuria, and abdominal pain and confirmed pathologically by his renal and skin biopsies demonstrating leukocytoclastic vasculitis and IgA complexes. We believe that this is an example of altered IgA processing in cirrhosis leading to the development of IgA immune complexes and ultimately HSP. The patient additionally had SBP, which may have increased his risk for developing HSP given antigen processing by mucosa-associated lymphoid tissues leading to immune complex deposition, which may not have been effectively cleared in the context of his liver disease. The patient unfortunately died of gastrointestinal hemorrhage, which is unclear to be due to his underlying cirrhosis or a gastrointestinal manifestation of HSP itself. PMID:26064127

  8. Severe congestive heart failure as the main symptom of eosinophilic granulomatosis and polyangiitis (Churg-Strauss syndrome).

    PubMed

    Za??ska, Jolanta; Wiatr, El?bieta; Zych, Jacek; Szopi?ski, Janusz; Oniszh, Karina; Kober, Jaros?aw; Piotrowska-Kownacka, Dorota; Roszkowski-?li?, Kazimierz

    2014-01-01

    Patients with cardiovascular symptoms are mainly diagnosed in cardiological wards. However, sometimes the other reasons for acute coronary syndrome and heart failure are found. One of such reasons is hypereosinophilia which can be recognized if number of blood eosinophils exceeds 1500/mm3. High eosinophilia is connected with production of cytotoxic eosinophilic proteins which can cause eosinophilic vasculitis or eosinophilic myocarditis. One of the better known hypereosinophilic syndromes is EGPA described by the pathomorphologists Churg and Strauss. The further research works allowed for the clinical characteristics of patients with EGPA. In the course of this disease the following three phases were recognized : prodromal-allergic, eosinophilic, vasculitic. The definitive diagnosis can be established only in the third phase, when vasculitis causes organ involvement. Besides symptoms of the respiratory tract (asthma, nasal polyps, eosinophilic lung infiltrations) also cardiovascular symptoms, gastrointestinal tract symptoms, as well as skin lesions and kidneys involvement can appear. The most dangerous for patients is involvement of the nervous and cardiovascular systems. We present a patient with asthma and eosinophilia in whom EGPA was diagnosed in the course of acute recurrent substernal chest pain, with subsequent signs of cardiac insufficiency. PMID:25339570

  9. Case of Behçet's disease complicated by oculomotor nerve palsy associated with internal carotid artery-posterior communicating artery aneurysm.

    PubMed

    Yamaoka, Toshifumi; Murota, Hiroyuki; Katayama, Ichiro

    2015-03-01

    Behçet's disease (BD) is a relapsing systemic inflammatory disorder of unknown etiology involving systemic vasculitis. Vasculitis in BD results from the involvement of arteries, veins and blood vessels of all sizes, which leads to the three major manifestations of this condition: venous occlusion, arterial occlusion and aneurysm formation. Therefore, whole-body vascular involvement should always be considered in BD patients. Here, we describe the first appearance of an internal carotid-posterior communicating artery aneurysm, resulting in complete oculomotor nerve palsy in a BD patient. A 44-year-old Japanese man suffered from recurrent episodes of erythema nodosum that had presented on the lower extremities for the past 2 years. His condition was diagnosed as an incomplete type of BD based on relapsing oral and genital ulcers, skin eruptions, such as erythema nodosum and folliculitis, a positive pathergy test and systemic arthralgia. Ten years after his initial clinical presentation, he had manifestations of right-sided ptosis and cyclic dull pain in his right temporal region. Magnetic resonance imaging and angiography revealed a right internal carotid artery-posterior communicating artery aneurysm. Although oculomotor nerve palsy associated with internal carotid artery-posterior communicating artery aneurysm in a BD patient has not been reported previously, our report highlights the fact that this abnormal manifestation should be considered in those with vasculo-BD. PMID:25573207

  10. Clinical Experience of Interferon Alfa-2a Treatment for Refractory Uveitis in Behçet's Disease

    PubMed Central

    Park, Ji-Youn; Chung, Yoo-Ri; Lee, Kihwang; Song, Ji Hun

    2015-01-01

    Behçet's disease (BD) involves multisystem vasculitis of unknown origin. Ocular manifestations of BD mostly include bilateral panuveitis and retinal vasculitis, which are very challenging to treat. Interferon alfa-2a (IFN) has been recently introduced for treating refractory Behçet uveitis, mainly in Germany and Turkey. Nonetheless, there is so far no consensus about the ideal treatment regimen of IFN for Behçet uveitis. We report our experience of IFN treatment in five Korean BD patients with refractory uveitis. All patients complained of oral ulcers; one patient had a positive pathergy test and 2 showed the presence of HLA-B51. Immunosuppressive agents used prior to IFN treatment included cyclosporine and methotrexate. The IFN treatment was commenced with a dose of 6-9 MIU/day for 7 days, adjusted according to individual ocular manifestations, tapered down to 3 MIU three times in a week, and then discontinued. All patients showed positive response to IFN treatment; 50% of them showed complete response without additional major ocular inflammation during the follow-up period. Other BD symptoms also improved after IFN treatment in most cases. After treatment, the relapse rate and the required dose of oral corticosteroid were decreased in most cases, showing a significant steroid-sparing effect. However, the visual acuity was not improved in most cases due to irreversible macular sequelae. Despite the small sample size of this study, we suggest that, in Korean patients, IFN is an effective treatment modality for BD uveitis as was observed in German and Turkish patients. PMID:26069144

  11. Cutaneous Ulcers as Initial Presentation of Localized Granulomatosis with Polyangiitis: A Case Report and Review of the Literature

    PubMed Central

    Nasir, Noreen; Ali, Syed Ahsan; Mehmood Riaz, Hafiz Mohammed

    2015-01-01

    Background. Granulomatosis with polyangiitis (GPA) is an ANCA associated small vessel vasculitis characterized by necrotizing granulomatous inflammation involving the upper and the lower respiratory tract and the kidneys. The disease has a broad clinical spectrum that ranges from limited/localized involvement of a single organ system to a generalized systemic vasculitis that affects several organs with evidence of end organ damage. Atypical forms of the disease have been recognized with or without respiratory tract involvement with a long protracted course before manifesting as generalized disease. Case Presentation. We describe a 57-year-old woman who presented with recurrent fever and cutaneous ulcers on her legs who was diagnosed to have granulomatosis with polyangiitis (GPA) after an extensive evaluation which excluded infectious, other vasculitides, connective tissue disease and malignant etiologies. Conclusion. In the absence of typical manifestations, granulomatosis with polyangiitis (GPA) is indeed a diagnostic challenge to the physician. Atypical manifestations like unexplained recurrent fever and cutaneous ulcers nevertheless call for keeping a low threshold for the diagnosis of GPA as the disease can initially present in localized form before heralding into a generalized disease. PMID:26664797

  12. Ocular Involvement of Behçet's Syndrome: a Comprehensive Review.

    PubMed

    Ozyazgan, Yilmaz; Ucar, Didar; Hatemi, Gulen; Yazici, Yusuf

    2015-12-01

    Behçet's syndrome (BS) is a vasculitis involving several organ systems including the eyes. Ocular involvement is one of the most disabling complications of BS, causing loss of vision that may progress to blindness if left untreated. The typical form of ocular involvement is a relapsing and remitting panuveitis and retinal vasculitis. Initial attacks may spontaneously improve and subsequently disappear in a few weeks but tend to recur if left untreated. Destructive and recurrent attacks, especially with posterior segment and retina involvement, may cause irreversible ocular structural changes and permanent damage in sensory retina, resulting in loss of vision. The risk of irreversible damage to ocular tissue which may result in loss of vision warrants early and intensive treatment especially in patients at high risk such as young men who tend to follow an aggressive disease course. The management strategy involves rapid suppression of inflammation during the attacks and prevention of recurrent attacks. Local and systemic measures including immunosuppressives, corticosteroids, and biologic agents are used for this purpose. Surgery may be required in selected cases. The prognosis of eye involvement has greatly improved over the last decades with the effective use of immunosuppressives. PMID:24828904

  13. Early and irreversible recurrence MPO-ANCA-positive glomerulonephritis after renal transplantation.

    PubMed

    Le Berre, Ludmilla; Dufay, Alexandre; Cantarovich, Diego; Meurette, Aurélie; Audrain, Marie; Giral, Magali; Blancho, Gilles; Fakhouri, Fadi; Hourmant, Maryvonne; Dantal, Jacques

    2015-06-01

    Kidney transplantation (KTx) is the treatment of choice for eligible patients suffering from anti-neutrophil cytoplasmic antibody(ANCA)-associated vasculitis (AAV) who are in clinical remission, regardless of ANCA status. With current immunosuppressive protocols, the recurrence rate of this primary disease in the kidney graft is low and is generally observed after the 1st year of transplantation, with a favorable outcome following conventional treatment. We report here two unusual observations of early (diagnosed within 2 weeks) and aggressive (graft failure despite therapy) recurrences in the kidney graft. These observations suggest that systematic induction by depleting antibodies and antibiotic prophylaxis may help prevent this rare but severe condition. In addition, we monitored these patients for the anti- lysosomal membrane protein-2 antibody (LAMP2ab) titers, but we found that LAMP2ab titers were not a surrogate marker of early recurrence if the LAMP2ab concentration was higher in AVV recipients before KTx. Finally, we must keep in mind that rare cases of early and aggressive recurrence ANCA-associated vasculitis on kidney graft are a challenge for early diagnosis and treatment. PMID:25345380

  14. Diagnostic potential of antibody titres against Candida cell wall ?-glucan in Kawasaki disease

    PubMed Central

    Ishibashi, K; Fukazawa, R; Miura, N N; Adachi, Y; Ogawa, S; Ohno, N

    2014-01-01

    Kawasaki disease (KD) is an acute vasculitis syndrome of unknown aetiology in children. The administration of Candida cell wall antigens induced KD-like coronary vasculitis in mice. However, the responses of KD patients to Candida cell wall antigen are unknown. In this study, we examined the response of KD patients to ?-glucan (BG), one of the major fungal cell wall antigens, by measuring the anti-BG titre. In KD patients, the anti-C. albicans cell wall BG titre was higher than that in normal children. The anti-BG titre was also higher in KD patients compared to children who served as control subjects. The efficacy of intravenous immunoglobulin (IVIG) therapy in KD is well established. We categorized the KD patients into three groups according to the therapeutic efficacy of intravenous immunoglobulin (IVIG) and compared the anti-BG titre among these groups. Anti-BG titres were similar in the control group and the non-responsive group. In the fully responsive group, the anti-BG titre showed higher values than those in the normal children. This study demonstrated clinically that KD patients have high antibody titres to Candida cell wall BG, and suggested the involvement of Candida cell wall BG in the pathogenesis of KD. The relationship between IVIG therapy and anti-BG titre was also shown. These results provide valuable insights into the therapy and diagnosis of KD. PMID:24635107

  15. Noninvasive investigation of the gastrointestinal tract in collagen-vascular disease.

    PubMed

    Keshavarzian, A; Saverymuttu, S H; Chadwick, V S; Lavender, J P; Hodgson, H J

    1984-11-01

    We have investigated the use of 111Indium granulocyte scanning, which would be expected to identify areas of perivascular or generalized neutrophil infiltration, in patients with collagen-vascular diseases suffering from either gastrointestinal symptoms (diarrhea or abdominal pain in 15 patients) or otherwise unexplained fever (six patients). Among patients with gastrointestinal symptoms, seven of 15 had positive scans suggesting large or small bowel involvement--three of three patients with Behcet's syndrome, four of five with vasculitis, no patient in six with systemic lupus erythematosus, and no patient with Churg-Strauss syndrome. Among patients without gastrointestinal symptoms, only one patient with polyarteritis nodosa had a positive scan, showing both large and small intestinal involvement. White cell scanning offers a noninvasive, readily tolerated technique for identifying inflammatory involvement of the intestine in patients with collagen-vascular disorders. Gastrointestinal involvement is rare in the absence of symptoms; and among symptomatic patients, inflammatory involvement of the gut is more likely to be found in patients with vasculitis or Behcet's. PMID:6507410

  16. Successful open abdomen treatment for multiple ischemic duodenal perforated ulcers in dermatomyositis

    PubMed Central

    2014-01-01

    Introduction Dermatomyositis is an autoimmune disease characterized by proximal myopathy, cutaneous Gottron papules and heliotrope rash; intestinal involvement associated to acute vasculitis is less common but could be a life-threatening condition. Methods A 21-year-old woman, affected by dermatomyositis, presented to our attention with a three-day story of severe abdominal pain, no bowel movement and biliary vomit. She was diagnosed with acute abdomen. A CT scan with bowel contrast demonstrated the presence of a leakage from the retroperitoneal aspect of duodenum. The surgical and clinical management in the light of literature review is presented. Results Our first approach consisted in primary repair of the duodenal perforation with omentopexy. Post-operative course was complicated by hemorrhage. A reintervention showed a new perforation associated with multiple ischemic intestinal areas. We performed a gastroenteric anastomosis with functional exclusion of the damaged duodenum and positioning of drainages to create a biliary fistula. A nutritional enteric tube and an open abdomen vacuum-assisted closure system to monitor the fistula creation and to prevent abdominal contamination and collections were positioned. To reduce the amount of biliary leakage, a percutaneous transhepatic biliary drainage was placed, with progressive fistula flow disappearance in four months. Conclusions In patients with dermatomyositis, when clinical findings and symptoms suggest abdominal vasculitis, it is very important to be aware of the risk of bowel and particularly duodenal perforations. Open abdomen treatment favors control of contamination by gastrointestinal contents, offers temporary abdominal closure, helps ICU care and delays definitive surgery. PMID:26085838

  17. Glomerulopathy in Kuwait: the spectrum over the past 7 years.

    PubMed

    El-Reshaid, W; El-Reshaid, K; Kapoor, M M; Madda, J P

    2003-07-01

    There are few studies that examine, prospectively, the epidemiological profile of glomerulopathy (GP) and its clinicopathological correlation. All patients referred to Al-Amiri renal center in Kuwait from January 1st, 1995 to December 31st, 2001 were screened for GP. Detailed clinical data were collected and serological markers were done. Renal biopsy was performed whenever indicated. During those 7 years, a total of 584 patients were diagnosed, on histological basis, to have GP, 315 of whom were Kuwaiti nationals. During the same period of the study, 26 patients presented with bilateral small kidneys, history of proteinuria > 2 g/day and lacked systemic manifestations of autoimmune disease. Furthermore, 164 patients with clinical manifestations of diabetic glomerulosclerosis were not subjected to kidney biopsy. Hence, the calculated annual incidence rate of GP in Kuwaiti nationals was 34.5 per 100,000 population (PTP). The calculated rate of diabetic glomerulosclerosis was 13.4 PTP and that of nondiabetic 21.1 PTP. The calculated incidence rates of GP increased with age and were twice as high in males compared to females. Vasculitis was more common in elderly males while SLE nephritis was a disease of adults, 88.7% of whom were females. In the subgroup of primary GP, focal segmental glomerulosclerosis was the most common histological lesion accounting for 18.0% of the total biopsies in Kuwaiti patients, yet only 36.8% of those who fulfilled the criteria of primary type. Minimal change disease was the second primary GP (13.0%), followed by immunoglobulin A deposition disease (7.9%) and membranous glomerulonephritis (5%). Autoimmune diseases such as systemic lupus erythematosus (SLE) and vasculitis were common. Interestingly, only 44 of 72 (61.1%) of patients with SLE and 11 of the 62 (17.7%) of patients with vasculitis presented with rapidly progressive glomerulonephritis. On the other hand, 10 of 58 (17.2%) patients with nephroangiosclerosis presented with renal failure and protein excretion > 2 g/day simulating primary GP. Furthermore, only 21 of 40 (52.5%) patients with IgA nephropathy presented with "benign disease". Prospective studies are essential to ascertain the actual incidence and etiology of GP. The loose clinicopathological correlation in GP dictates an aggressive diagnostic approach in its study and management. PMID:12911167

  18. Henoch-Schönlein purpura in northern Spain: clinical spectrum of the disease in 417 patients from a single center.

    PubMed

    Calvo-Río, Vanesa; Loricera, Javier; Mata, Cristina; Martín, Luis; Ortiz-Sanjuán, Francisco; Alvarez, Lino; González-Vela, M Carmen; González-Lamuño, Domingo; Rueda-Gotor, Javier; Fernández-Llaca, Héctor; González-López, Marcos A; Armesto, Susana; Peiró, Enriqueta; Arias, Manuel; González-Gay, Miguel A; Blanco, Ricardo

    2014-03-01

    The severity of clinical features and the outcomes in previous series of patients reported with Henoch-Schönlein purpura (HSP) vary greatly, probably due to selection bias. To establish the actual clinical spectrum of HSP in all age groups using an unselected and wide series of patients diagnosed at a single center, we performed a retrospective review of 417 patients classified as having HSP according to the criteria proposed by Michel et al. Of 417 patients, 240 were male and 177 female, with a median age at the time of disease diagnosis of 7.5 years (interquartile range [IQR], 5.3-20.1 yr). Three-quarters of the patients were children or young people aged 20 years or younger (n = 315), and one-quarter were adults (n = 102). The most frequent precipitating events were a previous infection (38%), usually an upper respiratory tract infection, and/or drug intake (18.5%) shortly before the onset of the vasculitis. At disease onset the most common manifestations were skin lesions (55.9%), nephropathy (24%), gastrointestinal involvement (13.7%), joint symptoms (9.1%), and fever (6.2%). Cutaneous involvement occurring in all patients, mainly purpuric skin lesion, was the most common manifestation when the vasculitis was fully established, followed by gastrointestinal (64.5%), joint (63.1%), and renal involvement (41.2%). The main laboratory findings were leukocytosis (36.7%), anemia (8.9%), and increased serum IgA levels (31.7%). The most frequent therapies used were corticosteroids (35%), nonsteroidal antiinflammatory drugs (14%), and cytotoxic agents (5%). After a median follow-up of 12 months (IQR, 2-38 mo), complete recovery was observed in most cases (n = 346; 83.2%), while persistent, usually mild, nephropathy was observed in only 32 (7.7%) cases. Relapses were observed in almost a third of patients (n = 133; 31.9%).In conclusion, although HSP is a typical vasculitis affecting children and young people, it is not uncommon in adults. The prognosis is favorable in most cases, depending largely on renal involvement. PMID:24646467

  19. Acute chorioamnionitis and funisitis: definition, pathologic features, and clinical significance.

    PubMed

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

    2015-10-01

    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. Intraamniotic infection generally has been considered to be the cause of acute chorioamnionitis and funisitis; however, recent evidence indicates that "sterile" intraamniotic inflammation, which occurs in the absence of demonstrable microorganisms induced by "danger signals," is frequently associated with these lesions. In the context of intraamniotic infection, chemokines (such as interleukin-8 and granulocyte chemotactic protein) establish a gradient that favors the migration of neutrophils from the maternal or fetal circulation into the chorioamniotic membranes or umbilical cord, respectively. Danger signals that are 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 present in 3-5% of term placentas and in 94% of pacentas delivered at 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 of the fetal inflammatory response syndrome, a condition characterized by an elevation in the fetal plasma concentration of interleukin-6, and associated with the impending onset of preterm labor, a higher rate of neonatal morbidity (after adjustment for gestational age), and multiorgan fetal involvement. This syndrome is the counterpart of the systemic inflammatory response syndrome in adults: a risk factor for short- and long-term complications (ie, sterile inflammation in fetuses, neonatal sepsis, bronchopulmonary dysplasia, periventricular leukomalacia, and cerebral palsy). This article reviews the definition, pathogenesis, grading and staging, and clinical significance of the most common lesions in placental disease. Illustrations of the lesions and diagrams of the mechanisms of disease are provided. PMID:26428501

  20. [Cardiac involvement in Churg-Strauss syndrome].

    PubMed

    Brucato, Antonio; Maestroni, Silvia; Masciocco, Gabriella; Ammirati, Enrico; Bonacina, Edgardo; Pedrotti, Patrizia

    2015-09-01

    Churg-Strauss syndrome, recently renamed eosinophilic granulomatosis with polyangiitis (EGPA), is a rare form of systemic vasculitis, characterized by disseminated necrotizing vasculitis with extravascular granulomas occurring among patients with asthma and tissue eosinophilia. EGPA is classified as a small and medium-sized vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA) and the hypereosinophilic syndrome. Typical clinical features include asthma, sinusitis, transient pulmonary infiltrates and neuropathy. Blood eosinophils are often >1500/µl or more than 10% on the differential leukocyte count. Blood eosinophils should always be tested in unexplained cardiac disorders, and may normalize even after low doses of corticosteroids. ANCA are positive in 40-60% of cases, mainly anti-myeloperoxidase. Heart involvement occurs in approximately 15-60% of EGPA patients, especially those who are ANCA negative. Any cardiac structure can be involved, and patients present with myocarditis, heart failure, pericarditis, arrhythmia, coronary arteritis, valvulopathy, intracavitary cardiac thrombosis. Although cardiovascular involvement is usually an early manifestation, it can also occur later in the course of the disease. A significant proportion of patients with cardiac involvement is asymptomatic. In the absence of symptoms and major ECG abnormalities, cardiac involvement may be detected in nearly 40% of the patients. All patients with EGPA should be studied not only with a detailed history of cardiac symptoms and ECG, but also with echocardiography; if abnormalities are detected, a cardiac magnetic resonance study should be performed. Coronary angiography and endomyocardial biopsy should be reserved to selected cases. Heart involvement carries a poor prognosis and causes 50% of the deaths of these patients. It is often insidious and underestimated. Optimal therapy is therefore important and based on high-dose corticosteroids plus immunosuppressive agents, particularly cyclophosphamide in case of myocardial inflammation. Thus, early diagnosis of cardiac involvement and subsequent therapy may prevent progression of cardiac disease. At present, the role of troponin and brain natriuretic peptide in monitoring and therapy remains unclear. Orthotopic heart transplantation is feasible in case of severe disease, even if the experience is limited in -EGPA, and optimal post-transplantation immunosuppressive strategy has yet to be defined. PMID:26418389

  1. New insights into the pathogenesis of giant cell arteritis and hopes for the clinic.

    PubMed

    Carmona, F David; Martín, Javier; González-Gay, Miguel A

    2016-01-01

    Giant cell arteritis is a complex immune-mediated disease that involves large blood vessels in individuals older than 50 years. Recent studies have confirmed a strong association of this form of vasculitis with the HLA region, particularly with HLA class II genes. However, other non-HLA loci, such as protein tyrosine phosphatase non-receptor type 22, may also account for the susceptibility to giant cell arteritis. In addition, genetic variants located in genes encoding proinflammatory cytokines seem to influence the phenotypic expression of the disease, including the risk of severe ischemic complications, the presence of polymyalgia rheumatica and the higher incidence of relapses observed in some patients. The identification of putative genetic markers of disease severity could have clear therapeutic implications, as it may allow us to identify patients who are potentially responders to specific treatments. PMID:26367100

  2. Pedal Gangrenous Changes in the Digits of an Adolescent With Ulcerative Colitis: A Case Report.

    PubMed

    Bhoola, Prashant H; Shtofmakher, Garry; Bahri, Ankur; Patel, Ankur A; Barlizo, Sharon R; Trepal, Michael

    2014-08-16

    Ulcerative colitis is an autoimmune inflammatory disease of the colon and is occasionally associated with thrombosis. We report the case of an adolescent with ulcerative colitis who presented with bilateral gangrenous toes without signs of ascending cellulitis. Radiographs indicated the presence of bilateral and erosive changes in the distal phalanges. The vascular team referred the patient for podiatric intervention for distal vasculitis and thrombosis of the digital vessels. Transphalangeal amputations were performed, and postoperative antibiotics were initiated. The surgical sites healed uneventfully, and the patient was able to resume daily activities. Thrombosis of the foot in the context of ulcerative colitis is a rare, but serious, complication that can lead to serious comorbidities, including amputation. PMID:25135104

  3. Life-threatening rupture of an external iliac artery pseudoaneurysm caused by necrotizing fasciitis following laparoscopic radical cystectomy: a case report

    PubMed Central

    2014-01-01

    Background Pseudoaneurysms are caused by trauma, tumors, infections, vasculitis, atherosclerosis and iatrogenic complications. In this paper, we report about a patient with rupture of an external iliac artery pseudoaneurysm, which lead to hemorrhagic shock, after undergoing laparoscopic radical cystectomy and extended pelvic lymphadenectomy. Case presentation The patient was a 68-year-old Japanese male diagnosed with invasive bladder cancer. Laparoscopic radical cystectomy and extended pelvic lymphadenectomy were performed. On postoperative day 12, he developed a high fever and an acute inflammatory response with redness and swelling in the right inguinal region. He was diagnosed with necrotizing fasciitis and underwent debridement. On postoperative day 42, a sudden hemorrhage developed from the open wound in the right inguinal region. He was diagnosed with external iliac artery pseudoaneurysm rupture by computed tomography. Conclusion These complications occur extremely rarely after cystectomy with pelvic lymphadenectomy. There are no reports to date on these complications following laparoscopic cystectomy with pelvic lymphadenectomy. PMID:24885366

  4. PAN's labyrinth: a multidisciplinary delayed diagnosis and patient's perspective.

    PubMed

    Barlow, Emma Louise; Seddon, Owen; Healy, Brendan

    2016-01-01

    Polyarteritis nodosa (PAN) is a rare, severe form of vasculitis affecting medium-sized vessels. It manifests as a multisystem syndrome, and may be associated with hepatitis B virus-associated PAN (HBV-PAN) although the incidence of this is declining with better vaccination strategies and awareness of bloodborne virus screening. We report a case in which a patient displayed many classical features of the disease, occurring separately over a period of months and leading to contact with various medical specialties. Managing each symptom in isolation led to a number of misdiagnoses (including testicular cancer) and the patient experienced considerable psychological stress and morbidity as a result. The case was complicated by acute pancreatitis developing after an initial treatment response. This may have been iatrogenic (as a consequence of either entecavir or steroids) or secondary to PAN. For our patient, this led to a protracted clinical course but eventual complete resolution of both pathologies. PMID:26733433

  5. Wiskott-Aldrich Syndrome: Description of a New Gene Mutation With Normal Platelet Volume.

    PubMed

    Yoonessi, Leila; Randhawa, Inderpal; Nussbaum, Eliezer; Saharti, Samah; Do, Paul; Chin, Terry; Zwerdling, Ted

    2015-10-01

    Wiskott-Aldrich syndrome (WAS) is a rare X-linked primary immunodeficiency characterized by an increased incidence of autoimmunity, malignancy, microthrombocytes with thrombocytopenia, eczema, and recurrent infections. In this case report, we present a novel mutation, hemizygous for c.1125_1129delTGGAC mutation in the WAS gene, and a unique clinical presentation. Our patient was initially diagnosed with a milk protein allergy after presenting with a lower gastrointestinal bleed, leukopenia, and thrombocytopenia with normal platelet volume. However, signs of vasculitis and detection of microthrombocytes required additional testing and consideration of WAS. This case report illustrates the importance of retaining a high index of clinical suspicion despite normal platelet volume, as well as adding to the growing number of known mutations associated with WAS. PMID:26241726

  6. Skin biopsy: Biopsy issues in specific diseases.

    PubMed

    Elston, Dirk M; Stratman, Erik J; Miller, Stanley J

    2016-01-01

    Misdiagnosis may result from biopsy site selection, technique, or choice of transport media. Important potential sources of error include false-negative direct immunofluorescence results based on poor site selection, uninformative biopsy specimens based on both site selection and technique, and spurious interpretations of pigmented lesions and nonmelanoma skin cancer based on biopsy technique. Part I of this 2-part continuing medical education article addresses common pitfalls involving site selection and biopsy technique in the diagnosis of bullous diseases, vasculitis, panniculitis, connective tissue diseases, drug eruptions, graft-versus-host disease, staphylococcal scalded skin syndrome, hair disorders, and neoplastic disorders. Understanding these potential pitfalls can result in improved diagnostic yield and patient outcomes. PMID:26702794

  7. A 45-Year-Old Man With Recurrent Dyspnea and Hemoptysis during Exercise: Exercise-Induced Pulmonary Hemorrhage/Edema

    PubMed Central

    Kim, Dae Sung; Lee, Minhyeok; Kwon, Oh Jung; Jeong, Inbeom; Son, Ji Woong; Na, Moon Jun

    2015-01-01

    A 45-year-old man presented with dyspnea and hemoptysis during exercise. A chest computed tomography (CT) revealed multifocal diffuse patchy ground glass opacity and interlobular septal thickening in both the lungs. Permeability pulmonary edema or pulmonary hemorrhage was suspected. Serologic studies for autoimmune disorders and vasculitis were negative. There was no laboratory evidence of coagulopathy, other hematopoietic disease or infectious disease. Considering correlation with exercise, we diagnosed exercise-induced pulmonary hemorrhage (EIPH) or exercise-induced pulmonary edema (EIPE). The patient was managed with antifibrinolytics, antibiotics, and antitussive agent. After a week, follow-up chest CT revealed completely resolved pulmonary hemorrhage. About 2 months after the first event, he visited again with dyspnea and hemoptysis during running. In the present study, we report a case of recurrent pulmonary hemorrhage after exercise. PMID:26508928

  8. Prodromal Alzheimer's disease presenting as cerebral amyloid angiopathy-related inflammation with spontaneous amyloid-related imaging abnormalities and high cerebrospinal fluid anti-A? autoantibodies.

    PubMed

    Boncoraglio, Giorgio B; Piazza, Fabrizio; Savoiardo, Mario; Farina, Laura; DiFrancesco, Jacopo C; Prioni, Sara; Tagliavini, Fabrizio; Parati, Eugenio A; Giaccone, Giorgio

    2015-01-01

    Cerebral amyloid angiopathy-related inflammation (CAA-ri), a rare form of vasculitis associated with amyloid-? (A?) deposition in vessel walls, has been proposed as a spontaneous human model of the amyloid-related imaging abnormalities (ARIA) occurring after anti-A? immunotherapy for the treatment of Alzheimer's disease (AD). We describe a case of a patient with biopsy-proven CAA-ri and prodromal AD, confirmed by means of neuropsychological examination after 20 months follow-up, presenting with ARIA and high levels of cerebrospinal fluid anti-A? autoantibodies. This case further supports the analogies between the inflammatory response driven by anti-A? immunotherapy and that spontaneously occurring in CAA-ri. PMID:25537009

  9. Chronic urticaria.

    PubMed Central

    Burrall, B. A.; Halpern, G. M.; Huntley, A. C.

    1990-01-01

    Urticaria affects 15% to 20% of the population once or more during a lifetime. Chronic urticaria is a frequent recurrent eruption over a period greater than 6 weeks; the cause remains a mystery in more than 75% of cases. Urticaria and angioedema may be produced by immunologic or nonimmunologic means. Urticarial vasculitis, contact urticaria, mastocytosis, physical urticarias, dermatographism, cholinergic urticaria, localized heat urticaria, cold urticaria, aquagenic urticaria, and vibratory angioedema all require specific evaluation and treatment. Chronic idiopathic urticaria is usually controlled by antihistamines; depending on the circadian rhythm of the eruption, sedative or nonsedative antihistamines are prescribed. Some patients will require a combination of H1 and H2 antagonists, or even parenteral corticosteroids. PMID:1970697

  10. The progressive systemic sclerosis/systemic lupus overlap: an unusual clinical progression.

    PubMed Central

    Asherson, R A; Angus, H; Mathews, J A; Meyers, O; Hughes, G R

    1991-01-01

    Three patients with the unusual combinations of discoid lupus, systemic lupus erythematosus (SLE), and progressive systemic sclerosis (PSS) are reported. The first patient developed PSS eight years after a diagnosis of discoid lupus had been made and this was complicated by myositis six years later. The second patient developed PSS more than 20 years after being diagnosed as having SLE. The third patient developed SLE with predominant features of urticarial vasculitis six years after PSS. Mild myositis also ensued. There were no antibodies to U1RNP demonstrable in any of these patients. The clinical progression of SLE to PSS or vice versa in the absence of features of mixed connective tissue disease is distinctly uncommon. Images PMID:2042989

  11. Behçet’s Disease as a Model of Venous Thrombosis

    PubMed Central

    La Regina, Micaela; Gasparyan, Armen Yuri; Orlandini, Francesco; Prisco, Domenico

    2010-01-01

    Behçet’s disease (BD) is a chronic inflammatory disease of unknown aetiology characterized by recurrent oral, genital aphthous ulcerations, uveitis, skin lesions and other multisystem affections associated with vasculitis. Different types of vessels, predominantly veins, can be affected in BD. The frequency of vascular lesions in BD, such as superficial and deep venous thromboses, arterial aneurysms and occlusions, ranges between 7-29%. In this review, various factors of thrombogenesis in BD, particularly pro- and antithrombotic endothelial and non-endothelial factors, factors of coagulation, platelet activation and rheological changes are presented and discussed from positions of Virchow’s triad of venous thrombosis. Despite advances in understanding of thrombogenesis in BD, still many issues of diagnosis and targeted preventive and therapeutic measures remain unresolved. Further studies are needed to clarify the pathobiology of BD-related thrombosis and to provide the clinicians with recommendations over the utility, safety and effectiveness of the antithrombotic therapy in BD. PMID:20360979

  12. Quantitation and characterization of soluble immune complexes precipitated from sera by polyethylene glycol (PEG).

    PubMed

    Chia, D; Barnett, E V; Yamagata, J; Knutson, D; Restivo, C; Furst, D

    1979-09-01

    Polyethylene glycol (PEG) was used to isolate immune complexes from sera. Complexes were then quantified and partially characterized by a variety of immunological techniques. Complexes were detected in rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Felty's syndrome and vasculitis, but not in polymyositis, scleroderma or Behçets syndrome. The level of immune complexes correlated with disease activity in SLE and RA patients. Rheumatoid factors and anti-DNA antibodies were enriched by PEG precipitation of RA and SLE sera, respectively, thus these antibodies appeared to be present in the form of soluble immune complexes. Immune complexes usually contained all three immunoglobulin classes, IgG, IgM and IgA. Complexes from RA patients were generally larger and often contained Clq, while C3 was found almost exclusively in SLE complexes which also tended to be smaller. Such compositional differences may one day explain the differences in clinical manifestations of various immune complex-mediated diseases. PMID:315842

  13. Quantitation and characterization of soluble immune complexes precipitated from sera by polyethylene glycol (PEG).

    PubMed Central

    Chia, D; Barnett, E V; Yamagata, J; Knutson, D; Restivo, C; Furst, D

    1979-01-01

    Polyethylene glycol (PEG) was used to isolate immune complexes from sera. Complexes were then quantified and partially characterized by a variety of immunological techniques. Complexes were detected in rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Felty's syndrome and vasculitis, but not in polymyositis, scleroderma or Behçets syndrome. The level of immune complexes correlated with disease activity in SLE and RA patients. Rheumatoid factors and anti-DNA antibodies were enriched by PEG precipitation of RA and SLE sera, respectively, thus these antibodies appeared to be present in the form of soluble immune complexes. Immune complexes usually contained all three immunoglobulin classes, IgG, IgM and IgA. Complexes from RA patients were generally larger and often contained Clq, while C3 was found almost exclusively in SLE complexes which also tended to be smaller. Such compositional differences may one day explain the differences in clinical manifestations of various immune complex-mediated diseases. PMID:315842

  14. [Recent advances in the treatment of antineutrophil cytoplasm antibody associated vasculitides].

    PubMed

    Szabó, Melinda Zsuzsanna; Pálfi, Patrícia; Bazsó, Anna; Poór, Gyula; Kiss, Emese

    2015-10-11

    The authors review the nomenclature of vasculitides and the classification of antineutrophil cytoplasm antibody associated vasculitides and present the method of measuring disease activity (Five-factor Score, Birmingham Vasculitis Activity Score) and its role in defining therapeutical needs. They discuss the treatment algorithm of antineutrophil cytoplasm antibody associated vasculitides, present the sometimes equipotential medications used during the induction therapy followed by a maintenance regimen, and outline their usage and possible side-effects that may require medical attention. They point out the importance of plasmapheresis as an adjunctive treatment in some cases, as well as indications and possible outcome of kidney transplantation in therapy-resistant cases. Finally, they review several ongoing clinical studies, as their outcome will probably influence therapeutical opportunities of antineutrophil cytoplasm antibody associated vasculitides in the next few years. PMID:26551168

  15. Orbital Granulomatosis With Polyangiitis (Wegener Granulomatosis)

    PubMed Central

    Muller, Karra; Lin, Jonathan H.

    2014-01-01

    The pathology of granulomatosis with polyangiitis (GPA), formerly Wegener granulomatosis, typically features a granulomatous and sometimes necrotizing vasculitis targeting the respiratory tract and kidneys. However, orbital involvement occurs in up to 60% of patients and is frequently the first or only clinical presentation in patients with systemic or limited forms of GPA. Orbital GPA can cause significant morbidity and potentially lead to complete loss of vision and permanent facial deformity. Fortunately, GPA is highly responsive to medical treatment with corticosteroids combined with cyclophosphamide or, more recently, rituximab. Therefore, it is imperative for this disease to be accurately diagnosed on orbital biopsy and distinguished from other histologically similar orbital lesions. Herein, we review the clinical and pathologic findings of orbital GPA, focusing on the differentiation of this disease from other inflammatory orbital lesions. PMID:25076302

  16. Linkage analysis of angiotensin-converting enzyme (ACE) insertion/deletion polymorphism and systemic lupus erythematosus.

    PubMed

    Kaufman, K M; Kelly, J; Gray-McGuire, C; Asundi, N; Yu, H; Reid, J; Baird, T; Hutchings, D; Bruner, G; Scofield, R H; Moser, K; Harley, J B

    2001-05-25

    Previous studies have suggested an association between systemic lupus erythematosus (SLE) and an insertion/deletion polymorphism in the angiotensin-converting enzyme gene (ACE). This polymorphism consists of a 250-bp insertion/deletion of an alu repeat in the 16th intron of the ACE gene. Individuals homozygous for the deletion have a higher level of circulating enzyme. Due to the important role of this enzyme in regulating the renin--angiotensin and kallikrein--kininogen systems, it is possible that the ACE insertion/deletion may play a role in SLE, which can include vasculitis and vascular changes. Using primers flanking the insertion/deletion site, we have examined the ACE gene in lupus patients and family members using genomic DNA obtained from the Lupus Multiplex Registry and Repository (LMRR). We were unable to detect significant linkage or genetic association between the ACE gene and SLE. PMID:11377823

  17. Fatal coxiellosis in Swainson's Blue Mountain Rainbow Lorikeets (Trichoglossus haematodus moluccanus).

    PubMed

    Woc-Colburn, A M; Garner, M M; Bradway, D; West, G; D'Agostino, J; Trupkiewicz, J; Barr, B; Anderson, S E; Rurangirwa, F R; Nordhausen, R W

    2008-03-01

    Three Swainson's Blue Mountain Rainbow Lorikeets (Trichoglossus haematodus moluccanus), ranging from 6 to 8 months of age, presented with lethargy, emaciation, and progressive neurologic signs. The first one died 24 hours after the onset of clinical signs, and the other two were euthanized 10 to 14 days after the onset of progressive neurologic disease. Clinical signs in these lorikeets included head pressing, hemiparesis, seizures, obtunded mentation, weakness, and lethargy. Two of the lorikeets had hepatomegaly, and one had splenomegaly on gross examination. Histopathology revealed disseminated microgranulomas in the liver, spleen, and brain, and lymphohistocytic perivascular encephalitis and cephalic vasculitis. Electron microscopic examination of macrophages in brain lesions revealed spherical to rod-shaped prokaryotic organisms with a trilaminar cell wall. Molecular analysis revealed a novel species of Coxiella. This is believed to be the first report of a Coxiella sp. causing disease in a lorikeet. PMID:18424842

  18. Gamma Delta (??) T Cells and Their Involvement in Behçet's Disease

    PubMed Central

    Hasan, Md. Samiul; Bergmeier, Lesley Ann; Petrushkin, Harry; Fortune, Farida

    2015-01-01

    Behçet's disease (BD) is a multisystem inflammatory disorder characterized by orogenital ulcerations, ocular manifestations, arthritis, and vasculitis. The disease follows a relapsing-remitting course and its pathogenesis is unknown. Genetic predisposition and immune-dysregulation involving gamma delta (??) T cells are reported to have a role. ?? T cells are atypical T cells, which represent a small proportion of total lymphocytes. They have features of both innate and adaptive immunity and express characteristics of conventional T cells, natural killer cells, and myeloid antigen presenting cells. These unconventional T cells are found in the inflammatory BD lesions and have been suggested to be responsible for inducing and/or maintaining the proinflammatory environment characteristic of the disease. Over the last 20 years there has been much interest in the role of ?? T cells in BD. We review the literature and discuss the roles that ?? T cells may play in BD pathogenesis. PMID:26539557

  19. [Emphysematous cystitis: Report of one case].

    PubMed

    Vera A, Nicolás; Zwanzger M, Christian; Troncoso C, Pablo

    2015-03-01

    Emphysematous cystitis is found in diabetic patients and in individuals with urinary stasis and immunosuppression. We report a 58-year-old male with hypertension, type 2 Diabetes on insulin treatment and central nervous system vasculitis on immunosuppressive therapy. He was admitted with weight loss and gait instability. A PET-CT showed a circumscribed image of air in the bladder contour without involving the upper urinary tract, suggesting emphysematous cystitis. Re-interrogated, the patient referred pneumaturia, dysuria and febrile sensation one week before admission. Urine culture showed Enterobacter aerogenes. He was treated with a urinary catheter, metabolic control and parenteral antimicrobials. The patient was discharged without symptoms 21 days after admission, with the bladder catheter. PMID:26005827

  20. Tryptophan-induced eosinophilia-myalgia syndrome.

    PubMed Central

    Criswell, L. A.; Sack, K. E.

    1990-01-01

    Eight patients who became ill while taking tryptophan had myalgia, fatigue, rash, fever, edema, alopecia, arthralgias, diminished joint motion, skin tightening, muscle cramping, and distal paresthesias. Three had shortness of breath, and one had pulmonary hypertension. Laboratory abnormalities included peripheral eosinophilia, leukocytosis, thrombocytosis, raised erythrocyte sedimentation rate, and elevated serum levels of aldolase, lactate dehydrogenase, and liver enzymes. Of 4 chest radiographs, 3 were abnormal. Of 5 skin and muscle biopsies, 4 showed sclerosis or mixed inflammatory cell infiltration of the dermis, subcutis, and fascia. Eosinophils were often present, but vasculitis was absent. Muscle inflammation was minimal. We conclude that the "eosinophilia-myalgia syndrome" is related to the ingestion of tryptophan and that abnormalities in the secretion of lymphokines may be important in its pathogenesis. PMID:2219890

  1. Sweet’s syndrome associated with Hashimoto’s thyroiditis

    PubMed Central

    Francisco, Christina Raissa I; Patal, Perpetua C; Cubillan, Eileen A; Isip-Tan, Iris Thiele

    2011-01-01

    Sweet’s syndrome or acute febrile neutrophilic dermatosis is characterised by the abrupt onset of painful erythematous plaques or nodules, pyrexia (>38°F) and histopathologic evidence of a dense neutrophilic infiltrate without vasculitis. It has been reported in association with many diseases, however, its association with Hashimoto’s thyroiditis is rare. A 47-year-old Filipino woman with a 30-year history of an asymptomatic anterior neck mass developed painful, erythematous annular plaques on her arms with associated fever. Skin biopsy confirmed the diagnosis of Sweet’s syndrome. The anterior neck mass was confirmed to be Hashimoto’s thyroiditis. This is a rare association with only two reported cases in the literature. There are no published cases in the Philippines on Sweet’s syndrome and Hashimoto’s thyroiditis to date. PMID:22678935

  2. Endothelium-Neutrophil Interactions in ANCA-Associated Diseases

    PubMed Central

    Halbwachs, Lise

    2012-01-01

    The two salient features of ANCA-associated vasculitis (AAV) are the restricted microvessel localization and the mechanism of inflammatory damage, independent of vascular immune deposits. The microvessel localization of the disease is due to the ANCA antigen accessibility, which is restricted to the membrane of neutrophils engaged in ?2-integrin–mediated adhesion, while these antigens are cytoplasmic and inaccessible in resting neutrophils. The inflammatory vascular damage is the consequence of maximal proinflammatory responses of neutrophils, which face cumulative stimulations by TNF-?, ?2-integrin engagement, C5a, and ANCA by the Fc?RII receptor. This results in the premature intravascular explosive release by adherent neutrophils of all of their available weapons, normally designed to kill IgG-opsonized bacteria after migration in infected tissues. PMID:22942199

  3. Vertebral artery dissection after a chiropractor neck manipulation.

    PubMed

    Jones, Jeremy; Jones, Catherine; Nugent, Kenneth

    2015-01-01

    The differential diagnosis for ischemic central nervous system infarcts in young patients includes paradoxic emboli through cardiac shunts, vasculitis, and vascular trauma. We report a young woman who developed headache, vomiting, diplopia, dizziness, and ataxia following neck manipulation by her chiropractor. A computed tomography scan of the head revealed an infarct in the inferior half of the left cerebellar hemisphere and compression of the fourth ventricle causing moderate acute obstructive hydrocephalus. Magnetic resonance angiography revealed severe narrowing and low flow in the intracranial segment of the left distal vertebral artery. The patient was treated with mannitol and a ventriculostomy and had an excellent functional recovery. This report illustrates the potential hazards associated with neck trauma, including chiropractic manipulation. The vertebral arteries are at risk for aneurysm formation and/or dissection, which can cause acute stroke. PMID:25552813

  4. Vertebral artery dissection after a chiropractor neck manipulation

    PubMed Central

    Jones, Jeremy; Nugent, Kenneth

    2015-01-01

    The differential diagnosis for ischemic central nervous system infarcts in young patients includes paradoxic emboli through cardiac shunts, vasculitis, and vascular trauma. We report a young woman who developed headache, vomiting, diplopia, dizziness, and ataxia following neck manipulation by her chiropractor. A computed tomography scan of the head revealed an infarct in the inferior half of the left cerebellar hemisphere and compression of the fourth ventricle causing moderate acute obstructive hydrocephalus. Magnetic resonance angiography revealed severe narrowing and low flow in the intracranial segment of the left distal vertebral artery. The patient was treated with mannitol and a ventriculostomy and had an excellent functional recovery. This report illustrates the potential hazards associated with neck trauma, including chiropractic manipulation. The vertebral arteries are at risk for aneurysm formation and/or dissection, which can cause acute stroke. PMID:25552813

  5. [Skin changes in rheumatic diseases].

    PubMed

    Dobri?, Ivan

    2005-01-01

    The Intruduction includes those eflorescences that might be useful for diagnostics in rheumatology. Further in the text we have described four groups of rheumatic disorders. The first group: rheumatic diseases (lupus erythematosus, dermatomyositis, systemic scleroderma, the mixed connective tissue disease, allergic vasculitis, polyarteritis) which are the most common from the dermatological point of view. The second group: rheumatic diseases (Wegener's granulomatosis, rheumatoid arthritis, Sjögren, Reiter and Behçet syndrome and Kawasaki's disease) which are rarely of interest to our dermatologists. In this group there is also psoriatic arthritis, which is not rare in dermatology but its diagnostics and treatment belong to rheumatologists' field of expertise. The third group: infections (rheumatic fever, diseminated gonococcal infection, subacute bacterial endocarditis, Lyme disesease). The fourth group: metabolic disorders (gout). The diseases of the first group are described completely. In the second, third and fourth group of the diseases we have included only skin changes. PMID:16689123

  6. [When is it a diagnosis of overlap syndrome?].

    PubMed

    Alexa, Ioana Dana; Paraschiv, Oana; Constantinescu, Gina; Panaghiu, Larisa; Palade, Florentina

    2003-01-01

    The connective tissue diseases comprise a group of syndromes of unknown etiology affecting as many as 1 person in 40, often with a predilection for the female sex. Included are: systemic lupus erytematosus (SLE), polymyositis and dermatomyositis, Sjögren syndrome, scleroderma and the vasculitis (polyarteritis nodosa, Wegener's, giant cell arteritis). There are patients who are not easily defined; having features overlapping with those of other connective tissue diseases. A variety of terms such as mixed connective tissue disease, undifferentiated connective tissue syndrome and overlap syndrome have emerged to describe such patients. Although many of these overlap syndromes are unlikely to have life-threatening consequences, they may be extremely debilitating and distressing, significantly reducing quality of life for the patient and his or her family. We present the case of a patient initially diagnosed with dermatomyositis and who eventually evolved to overlap syndrome by developing SLE. PMID:14755959

  7. Hepatitis C Virus Infection and Its Rheumatologic Implications

    PubMed Central

    Sayiner, Zeynel A.; Haque, Uzma; Malik, Mohammad U.

    2014-01-01

    Extrahepatic manifestations are frequently encountered among patients with chronic hepatitis C virus (HCV) infection. Many of these manifestations are autoimmune disorders, with added mortality and morbidity due to involvement of multiple organ systems. Symptoms of HCV infection and rheumatic diseases may be similar and include arthralgia, myalgia, arthritis, and vasculitis. Also, serologic abnormalities may be found in both cases. Some treatment modalities for HCV infection, including interferon therapy, may aggravate the symptoms of rheumatic diseases, thus confounding clinical presentation. It is imperative to distinguish whether symptoms such as arthralgia, myalgia, and arthritis occur in patients with HCV infection due to primary chronic HCV infection or to a newly developed rheumatologic disease process. PMID:24987312

  8. A case of erythema nodosum and serositis associated with myelodysplastic syndrome.

    PubMed

    Choi, Jung-Hye; Ahn, Myung-Ju; Park, Yong-Wook; Oh, Ho-Suk; Lee, Young-Yeul; Kim, In-Soon

    2005-06-01

    Myelodysplastic syndrome (MDS) is a heterogenous group of stem cell disorders usually characterized by progressive refractory cytopenias, which could progress to acute myeloid leukemia. MDS may be associated with a wide spectrum of skin lesions, including neoplastic cell infiltration, Sweet's syndrome, pyoderma gangrenosum, erythema elevatum diutinum, vasculitis, and panniculitis. However, erythema nodosum is rarely associated with MDS. Unusual rheumatologic manifestations in patients with MDS also have been reported, which range from asymptomatic serological abnormalities to classic connective tissue disorders such as Sjögren's syndrome, relapsing polychondritis, systemic lupus erythematosus, rheumatoid arthritis and mixed connective tissue disease. However, concurrent erythema nodosum and serositis has rarely been reported. We describe a case of MDS with erythema nodosum and immune-mediated pericardial effusion in a 34-year-old woman. PMID:16134776

  9. Differential Diagnosis of Necrotizing Sinonasal Lesions.

    PubMed

    Montone, Kathleen T

    2015-12-01

    Context .- A number of entities may result in necrosis in the sinonasal tract and lead to significant morbidity and mortality. These include infections, necrotizing vasculitis, neoplastic processes, and drug dependency. This review will concentrate on the differential diagnosis of sinonasal necrotizing lesions. Objective .- To review the differential diagnoses of necrotizing destructive lesions of the sinonasal tract. Data Sources .- The current literature was reviewed to provide updated information regarding the differential diagnosis of sinonasal necrotizing lesions, including infectious disease processes; antineutrophilic cytoplasmic antibody-associated vasculitides; neoplastic processes, particularly natural killer/T-cell lymphomas; and drug abuse. Conclusions .- The differential diagnosis of necrotizing sinonasal lesions is broad, with often overlapping diagnostic features that lead to diagnostic challenges. Ancillary tests such as special stains and immunohistochemical studies can offer significant assistance. PMID:26619023

  10. New animal model for human ocular toxocariasis: ophthalmoscopic observation

    PubMed Central

    Takayanagi, T. H.; Akao, N.; Suzuki, R.; Tomoda, M.; Tsukidate, S.; Fujita, K.

    1999-01-01

    BACKGROUND/AIMS—Although human ocular toxocariasis causes severe vision defect, little is known about its aetiology, diagnosis, and treatment. To develop a new animal model for human ocular toxocariasis, ophthalmological findings of fundi in Mongolian gerbils, Meriones unguiculatus, and BALB/c mice were investigated following infection with Toxocara canis.?METHODS—Using an ophthalmoscope, which was specifically developed to observe the fundi of small animals, ocular changes of fundi of 20 gerbils and 11 mice were monitored after oral infection with embryonated eggs of T canis.?RESULTS—Vitreous, choroidal, and retinal haemorrhages were consistently observed in Mongolian gerbils, but rarely in mice. Severe exudative lesions and vasculitis were often present in gerbils but not in mice. Migrating larvae were also frequently observed in gerbils.?CONCLUSION—Mongolian gerbils are more appropriate animal model for human ocular toxocariasis than previously used experimental animal such as mice, guinea pigs, rabbits, and monkeys because of its high susceptibility of ocular infection.?? PMID:10413704

  11. Clinical Immunology Review Series: An approach to the patient with recurrent superficial abscesses

    PubMed Central

    Johnston, S L

    2008-01-01

    ARTICLES PUBLISHED IN THIS CLINICAL IMMUNOLOGY REVIEW SERIES allergy in childhood, allergy diagnosis by use of the clinical immunology laboratory, anaphylaxis, angioedema, management of pulmonary disease in primary antibody deficiency, recurrent infections in childhood, recurrent infections in adulthood, recurrent oro-genital ulceration, recurrent superficial abscesses, urticaria, vasculitis/CTD Patients may be referred to the immunology clinic for investigation of recurrent superficial abscess formation. In the majority of adult patients this clinical presentation does not equate with an underlying primary immune deficiency. Nevertheless, recurrent mucocutaneous abscesses can be associated with significant morbidity and long-term complications, including scarring and fistula formation, and may be associated with underlying immune-mediated disease. This review sets out an approach to the patient with recurrent superficial abscesses, focusing on the differential diagnoses, investigation and management of both the common causes and those associated with specific immune deficiency. PMID:18422735

  12. [Renal artery aneurysm in Wegener's granulomatosis].

    PubMed

    Grosser, G; Mundinger, A; Pumpe, C; Herbst, E W; Kuhlmann, M; Kröpelin, T

    1989-08-01

    Microaneurysms of renal and visceral arteries are characteristic signs of periarteritis nodosa. Normally they are not found in Wegener's disease, where glomerulonephritis is commonly observed. We report on a patient with vasculitis of the upper and lower respiratory tract, focal glomerulonephritis, prostatic and pulmonary granulomas and anti-cytoplasm antibodies corresponding to Wegener's disease. The most striking findings in angiography were multiple small aneurysms of the peripheral branches of the renal artery. At necroscopy these angiographic findings were histologically proven as necrosis of the arterial wall with destruction of the elastic lamina, causing local vascular ectasia. These renal vascular changes are a characteristic sign of periarteritis nodosa. The combination of clinical, laboratory, radiologic and histologic findings in our patient can be explained as an overlap-syndrome of Wegener's disease and periarteritis nodosa. We assume that the combination of pathologic findings in our patient correspond to a rare atypical renal manifestation of Wegener's disease. PMID:2569753

  13. Eosinophilic myocarditis: case series and literature review.

    PubMed

    Sohn, Kyoung-Hee; Song, Woo-Jung; Kim, Byung-Keun; Kang, Min-Koo; Lee, Suh-Young; Suh, Jung-Won; Yoon, Yeonyee E; Kim, Sae-Hoon; Youn, Tae-Jin; Cho, Sang-Heon; Chang, Yoon-Seok

    2015-04-01

    Eosinophilic myocarditis is a condition resulting from various eosinophilic diseases, including helminth infection, drug hypersensitivity, systemic vasculitis or idiopathic hypereosinophilic syndromes. Clinical manifestations of eosinophilic myocarditis may vary from early necrosis to endomyocardial fibrosis. Eosinophilic myocarditis is one of the most fatal complications of hypereosinophilia. However, eosinophilic myocarditis has been rarely reported in the literature, particularly in Asia Pacific regions, reflecting the under-recognition of the disease among clinicians. Early recognition is crucial for improving clinical outcomes of eosinophilic myocarditis. Early administration of systemic corticosteroid is necessary in eosinophilic myocarditis regardless of underlying causes, as delayed treatment may result in fatal outcomes. In addition, differential diagnoses of underlying causes for eosinophilia are necessary to improve long-term outcomes. PMID:25938077

  14. Eosinophilic myocarditis: case series and literature review

    PubMed Central

    Sohn, Kyoung-Hee; Song, Woo-Jung; Kim, Byung-Keun; Kang, Min-Koo; Lee, Suh-Young; Suh, Jung-Won; Yoon, Yeonyee E; Kim, Sae-Hoon; Youn, Tae-Jin; Cho, Sang-Heon

    2015-01-01

    Eosinophilic myocarditis is a condition resulting from various eosinophilic diseases, including helminth infection, drug hypersensitivity, systemic vasculitis or idiopathic hypereosinophilic syndromes. Clinical manifestations of eosinophilic myocarditis may vary from early necrosis to endomyocardial fibrosis. Eosinophilic myocarditis is one of the most fatal complications of hypereosinophilia. However, eosinophilic myocarditis has been rarely reported in the literature, particularly in Asia Pacific regions, reflecting the under-recognition of the disease among clinicians. Early recognition is crucial for improving clinical outcomes of eosinophilic myocarditis. Early administration of systemic corticosteroid is necessary in eosinophilic myocarditis regardless of underlying causes, as delayed treatment may result in fatal outcomes. In addition, differential diagnoses of underlying causes for eosinophilia are necessary to improve long-term outcomes. PMID:25938077

  15. Respiratory Tract Amyloidosis. State-of-the-Art Review with a Focus on Pulmonary Involvement.

    PubMed

    de Almeida, Renata Rocha; Zanetti, Gláucia; Pereira E Silva, Jorge Luiz; Neto, Cesar Augusto Araujo; Gomes, Antônio Carlos Portugal; de Souza Portes Meirelles, Gustavo; da Silva, Thiago Krieger Bento; Nobre, Luiz Felipe; Hochhegger, Bruno; Escuissato, Dante Luiz; Marchiori, Edson

    2015-12-01

    Amyloidosis is a constellation of disease entities characterized by abnormal extracellular deposition and accumulation of protein and protein derivatives, which show apple-green birefringence when stained with Congo red and viewed under polarized light. Amyloid can infiltrate virtually all organ systems and can display multiple and diverse imaging findings. Pathologically, respiratory involvement occurs in 50 % of patients with amyloidosis, and its clinical signs and symptoms vary depending on whether the disease is systemic or localized. The four main patterns of respiratory tract involvement are tracheobronchial, nodular parenchymal, diffuse alveolar septal, and lymphatic. Imaging findings of amyloidosis are nonspecific and vary in each pattern; knowledge about the disease impairment type is thus very important, and amyloidosis should be considered in the differential diagnosis of other very common diseases, such as infectious diseases, neoplasms, and vasculitis. This literature review describes the main clinical and imaging manifestations of amyloidosis, focusing on respiratory tract involvement and differential diagnosis. PMID:26310967

  16. Urticaria Multiforme

    PubMed Central

    Bernardo, Sebastian G.; Kovalerchik, Olga; Ahmad, Moneeb

    2013-01-01

    Urticaria multiforme is a benign cutaneous hypersensitivity reaction seen in pediatric patients that is characterized by the acute and transient onset of blanchable, annular, polycyclic, erythematous wheals with dusky, ecchymotic centers in association with acral edema. It is most commonly misdiagnosed as erythema multiforme, a serum-sickness-like reaction, or urticarial vasculitis. Since these three diagnoses represent distinct clinical entities with unique prognoses and management strategies, it is important that physicians distinguish urticaria multiforme from its clinical mimics in order to optimize patient care. By performing a thorough history and physical examination, the astute clinician can make the correct diagnosis and develop an appropriate, effective treatment plan while avoiding unnecessary biopsies and laboratory evaluations. The authors report a case of urticaria multiforme in a four-year-old girl in order to emphasize the distinctive morphological manifestations of this rare, albeit unique, disease seen in the pediatric population. PMID:23556035

  17. PTPN22: the archetypal non-HLA autoimmunity gene

    PubMed Central

    Stanford, Stephanie M.; Bottini, Nunzio

    2015-01-01

    PTPN22 encodes a tyrosine phosphatase that is expressed by haematopoietic cells and functions as a key regulator of immune homeostasis by inhibiting T-cell receptor signalling and by selectively promoting type I interferon responses after activation of myeloid-cell pattern-recognition receptors. A single nucleotide polymorphism of PTPN22, 1858C>T (rs2476601), disrupts an interaction motif in the protein, and is the most important non-HLA genetic risk factor for rheumatoid arthritis and the second most important for juvenile idiopathic arthritis. PTPN22 exemplifies a shared autoimmunity gene, affecting the pathogenesis of systemic lupus erythematosus, vasculitis and other autoimmune diseases. In this Review, we explore the role of PTPN22 in autoimmune connective tissue disease, with particular emphasis on candidate-gene and genome-wide association studies and clinical variability of disease. We also propose a number of PTPN22-dependent functional models of the pathogenesis of autoimmune diseases. PMID:25003765

  18. A Rare Case of Zosteriform Cutaneous Metastases from a Nasopharyngeal Carcinoma

    PubMed Central

    González García, Andrés; Fernández, Emiliano Grillo; Barbolla Díaz, Ignacio; Ballester, Asunción; Pian, Héctor; Fraile, Guadalupe

    2015-01-01

    From a clinical point of view, the most common presentations of cutaneous metastatic disease are papules and nodules. However, a wide morphological spectrum of lesions has been described, including erythematous patches or plaques, inflammatory erysipelas-like lesions, diffuse sclerodermiform lesions with induration of the skin, telangiectatic papulovesicles, purpuric plaques mimicking vasculitis, and alopecia areata like scalp lesions. The so-called zosteriform pattern has been described to be in few cases and to the best of our knowledge has never been described associated with a metastasis of a nasopharyngeal carcinoma. This case highlights the relevance of including cutaneous metastases in the differential diagnosis of patients with nonhealing herpes zoster-like lesions, especially in those with underlying neoplasm recently diagnosed. PMID:26697235

  19. Clinical application of autologous technetium-99m-labelled eosinophils to detect focal eosinophilic inflammation in the lung.

    PubMed

    Loutsios, Chrystalla; Farahi, Neda; Simmonds, Rosalind; Cullum, Ian; Gillett, Daniel; Solanki, Chandra; Solanki, Kishor; Buscombe, John; Condliffe, Alison M; Peters, A Mike; Chilvers, Edwin R

    2015-11-01

    The detection of focal eosinophilic inflammation by non-invasive means may aid the diagnosis and follow-up of a variety of pulmonary pathologies. All current methods of detection involve invasive sampling, which may be contraindicated or too high-risk to be performed safely. The use of injected autologous technetium-99m (Tc-99m)-labelled eosinophils coupled to single-photon emission computed tomography (SPECT) has been demonstrated to localise eosinophilic inflammation in the lungs of a patient with antineutrophil cytoplasmic antibody-positive vasculitis. Here, we report on the utility of this technique to detect active eosinophilic inflammation in a patient with focal lung inflammation where a biopsy was contraindicated. PMID:26108571

  20. Predictive value of ANCA in atypical primary brain localization of Wegener's granulomatosis.

    PubMed

    Ossi, E; Rossanese, A; Casagrande, F; Rotilio, A; Mingrino, S; Poci, C

    2002-03-01

    Wegener's Granulomatosis is a necrotizing vasculitis generally involving upper and lower respiratory tract and kidneys. The central nervous system is involved in less than 10% of the patients during the course of the disease and primary involvement is even rarer. We present and discuss the case of a patient with primitive cerebral localization of Wegener's Granulomatosis in which the diagnosis and the beginning of correct therapy were delayed, in spite of a rising c-ANCA titer, due to a misinterpretation of a bioptic specimen. This delay caused renal damage and pulmonary cavitations which needed a long time to recover. This case report suggests that the central nervous system can be the site of a primary localization of Wegener's Granulomatosis even without any other organ involvement. The diagnosis must be made as soon as possible in order to prevent spread to other sites since the disease is usually very aggressive and severe. PMID:12002391

  1. Self-limiting recurrent bullous Henoch-Schonlein purpura with lupus anticoagulant.

    PubMed

    Gration, Betty; Osakwe, Edwin

    2015-01-01

    Henoch-Schonlein purpura (HSP) is the most common acute systemic vasculitis of childhood with an incidence of approximately 1:10,000. It commonly presents with skin, gastrointestinal tract, joints and renal system signs and symptoms. We present a case of recurrent self-limiting HSP with lupus anticoagulant presenting with haemorrhagic bullous skin lesions and scrotal swelling. The initial presentation resolved within 4 weeks but over the next 6 months, a few self-limiting but milder skin lesions occurred. Some institutions have advocated the use of steroids in these cases but our case showed that the use of steroids may not be required. This atypical presentation of HSP led to unnecessary investigations that this case report aims to prevent in the future by raising its awareness. PMID:25604501

  2. Acute abdomen in patients with systemic lupus erythematosus and antiphospholipid syndrome. Importance of early diagnosis and treatment.

    PubMed

    Titos-García, Alberto; Aranda-Narváez, José Manuel; Marín-Camero, Naira; Fernández-Burgos, Isabel Rosa; Montiel-Casado, María Custodia; González-Sánchez, Antonio Jesús; Santoyo-Santoyo, Julio

    2015-01-01

    Systemic lupus erithematosus (SLE) is an autoimmune disease with multiorgan involvement caused principally by vasculitis of small vessels. The gastrointestinal tract is one of the most frequently affected by SLE, with abdominal pain as the most common symptom. An early diagnosis and treatment of lupus enteritis is essential to avoid complications like hemorrhage or perforation, with up to 50 % of mortality rate. However, differential diagnosis sometimes is difficult, especially with other types of gastrointestinal diseases as digestive involvement of antiphospholipid syndrome (APS), moreover when both entities may coexist. We describe the case of a patient with both diseases that was diagnosed with lupus enteritis and treated with steroid therapy; the patient had an excellent response. PMID:25603332

  3. Churg-Strauss and montelukast.

    PubMed

    Carlesimo, M; Mari, E; Palese, E; Pranteda, G; Camplone, G

    2011-01-01

    Churg-Strauss syndrome (CSS) is a systemic small vessel vasculitis involving lungs, skin, heart, gastrointestinal tract and peripheral nerves. We report the case of a 36-year-old woman with a necrotic lesion on the left foot of two months duration, associated with hypereosinophilia, patchy lung infiltrates, cardiac damage and a mononeuritis. The personal history was remarkable only for an asthma, treated with Montelukast, a leukotriene receptor antagonist (LRA). Clinical symptoms, laboratory exams and instrumental examinations led us to the diagnosis of CSS. In recent years several studies have reported the possible relationship between use of leukotriene receptor antagonist (LRA) and CSS expression. We report this case to underline the possible relationship between LRA and CSS and its etiopathogenetic mechanism. PMID:22230414

  4. [Behcet's disease--ophthalmological and general aspects: Part 2: Therapy].

    PubMed

    Pleyer, U; Hazirolan, D; Winterhalter, S; Stübiger, N

    2013-03-01

    Behcet's disease (also called Admantiades-Behcet syndrome) is a chronic vasculitis. The disease is characterized by exacerbations and remissions of symptoms and organ manifestations and may produce only mild mucocutaneous lesions, whereas ocular lesions can cause blindness. In addition, involvement of the gastrointestinal tract, central nervous system (CNS) and large blood vessels is sometimes life-threatening. Cyclosporin A is the only agent for treatment of ocular lesions registered in Germany; however, the neurotoxicity and nephrotoxicity restrict usage of the drug. In patients suffering from severe uveitis, biologics have been a breakthrough. Interferon (IFN) alpha therapy has shown significant efficacy for intraocular inflammation. Monoclonal antibodies to TNF-alpha and interleukin-1 have been successful in clinical trials and are approved in some countries. This article summarizes the current state of knowledge and emphasizes the important role of the ophthalmologist in the therapy of Behcet's disease. PMID:23504099

  5. Successful use of Rituximab in a patient with recalcitrant multisystemic eosinophilic granulomatosis with polyangiitis.

    PubMed

    Najem, Catherine E; Yadav, Rajwardhan; Carlson, Elise

    2015-01-01

    Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare eosinophil-rich disorder characterised by necrotising granulomatous inflammation affecting small to medium sized vessels. Extrapulmonary manifestations can be life-threatening when heart, central nervous system (CNS), gastrointestinal tract or kidneys are affected. We describe a case of a 56-year-old woman with a long-standing history of asthma, who presented with an acute sudden painless loss of vision after she had been recently diagnosed with EGPA and induced with pulse steroids and cyclophosphamide. The patient had a complicated hospital course with multisystemic involvement of active vasculitis, involving heart, kidneys, muscles, eyes and CNS. The patient's devastating condition responded remarkably to Rituximab. The role of Rituximab in EGPA is not yet proven. Few cases are reported in the literature about the role of Rituximab in EGPA, of which only one described retinal artery occlusion as a presentation of a recently treated patient with EGPA. PMID:25979957

  6. Single organ variant of polyarteritis nodosa in epididymis.

    PubMed

    Garg, Ketan; Dawson, Leelavathi

    2015-01-01

    Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that typically affects medium-sized muscular arteries, with occasional involvement of small muscular arteries. Unlike some other vasculitides (e.g. microscopic polyarteritis, Wegener's granulomatosis) PAN is not associated with antineutrophil cytoplasmic antibodies. Patients typically present with systemic symptoms such as fever, weight loss, and malaise. The kidneys, skin, joints, muscles, nerves, and gastrointestinal tract are commonly involved, usually in some combination. PAN can affect any organ, but usually spare the lungs. Clinical variants or subsets of PAN include single-organ disease and cutaneous-only PAN. Scrotal involvement is rarely the first presenting sign. We herein report a case of 36-year-old man who presented with a swelling in the left epididymis, which was surgically removed. The swelling histopathologically showed necrotizing inflammation, fibrinoid necrosis of the medium-sized arteries of the epididymis and was diagnosed to be single organ variant of PAN. PMID:26458682

  7. Acute abdomen for lymphangioma of the small bowel mesentery: a case report and review of the literature.

    PubMed

    Botey, Mireia; Muñoz-Ramos, Carlos; Bonfill, Joan; Roura, Jordi; Torres, Mireia; Mocanu, Sorin; Arner, Toni; Pérez, Gumer; Salvans, Florenci; García-San-Pedro, Ángel

    2015-01-01

    Systemic lupus erithematosus (SLE) is an autoimmune disease with multiorgan involvement caused principally by vasculitis of small vessels. The gastrointestinal tract is one of the most frequently affected by SLE, with abdominal pain as the most common symptom. An early diagnosis and treatment of lupus enteritis is essential to avoid complications like hemorrhage or perforation, with up to 50 % of mortality rate. However, differential diagnosis sometimes is difficult, especially with other types of gastrointestinal diseases as digestive involvement of antiphospholipid syndrome (APS), moreover when both entities may coexist. We describe the case of a patient with both diseases that was diagnosed with lupus enteritis and treated with steroid therapy; the patient had an excellent response. PMID:25603331

  8. Churg-Strauss Syndrome as an Unusual Cause of Dysphagia: Case Report

    PubMed Central

    Park, Jihye; Moon, Su-Jin; Park, Geun-Young; Jang, Yongjun; Kim, Yeonjin

    2015-01-01

    Systemic vasculitis is a rare disease, and the diagnosis is very difficult when patient shows atypical symptoms. We experienced an unusual case of dysphagia caused by Churg-Strauss syndrome with lower cranial nerve involvement. A 74-year-old man, with a past history of sinusitis, asthma, and hearing deficiency, was admitted to our department for evaluation of dysphagia. He also complained of recurrent bleeding of nasal cavities and esophagus. Brain magnetic resonance imaging did not show definite abnormality, and electrophysiologic findings were suggestive of mononeuritis multiplex. Dysphagia had not improved after conventional therapy. Biopsy of the nasal cavity showed extravascular eosinophilic infiltration. All these findings suggested a rare form of Churg-Strauss syndrome involving multiple lower cranial nerves. Dysphagia improved after steroid therapy. PMID:26161355

  9. [Atypical onset of Churg and Strauss syndrome in a child].

    PubMed

    Tellier, S; Dallocchio, A; Brochard, K; Pajot, C; Garnier, A; Bandin, F; Mazereeuw, J; Vial, J; Decramer, S

    2012-06-01

    We report the case of a 13-year-old boy who had been treated since the age of 6 for moderate asthma. Except asthma, his past medical history was uneventful. The patient was referred for the sudden onset of bilateral leg edemas with peripheral purpuric lesions. Blood tests showed increased blood eosinophilia (9000/mm(3)) with no fever. The antineutrophil cytoplasmic antibodies (ANCA) were negative. The skin biopsy showed extensive ischemic subcutaneous necrosis related to necrotizing vasculitis. The general secondary symptoms occurred with multiorgan involvement (pulmonary infiltrates, peripheral neuropathy, gastrointestinal tract symptoms, and arthralgia). Genital infiltration was also noted. The child's general health was preserved. Neither cardiac nor renal involvement were found. The patient showed favorable clinical progression after oral prednisone therapy. PMID:22541509

  10. Neuro-Behçet disease and autoinflammatory disorders.

    PubMed

    Miller, Julie J; Venna, Nagagopal; Siva, Aksel

    2014-09-01

    Misregulation of innate immunity leads to autoinflammation. Behçet disease is an autoinflammatory condition involving recurrent attacks of inflammation in skin, eyes, joints, and even the nervous system. The etiology may involve vascular inflammation. Central nervous system involvement in neuro-Behçet disease (NBD) comes in the form of parenchymal NBD or nonparenchymal NBD. The parenchymal form has a predilection for the brainstem, diencephalon and cerebral hemispheres, and represents a meningoencephalitis thought to be related to small vessel vasculitis. Cerebral venous sinus thrombosis, arising from a vasculitic process of large veins, comprises the majority of vascular NBD cases. The rarer monogenetic autoinflammatory syndromes are characterized by periodic fever, and typically present in the pediatric population. Neurologic involvement in these syndromes typically presents in the form of an aseptic meningitis. Treatment of autoinflammatory disorders involves immune modulation with corticosteroids, disease-modifying antirheumatic medications, and increasingly antibodies targeting cytokines like tumor necrosis factor ? and interleukin 1. PMID:25369439

  11. Cerebral tumor or pseudotumor?

    PubMed

    Leclercq, D; Trunet, S; Bertrand, A; Galanaud, D; Lehéricy, S; Dormont, D; Drier, A

    2014-10-01

    Pseudotumoral lesions are uncommon but important to identity lesions. They can occur during inflammatory diseases (systemic diseases, vasculitis, demyelinating diseases), infectious, and vascular diseases. Also, in a patient with a treated tumor, pseudo-progression and radionecrosis must be differentiated from the tumoral development. Diagnosis can be difficult on an MRI scan, but some MRI aspects in conventional sequences, diffusion, perfusion and spectroscopy can suggest the pseudotumoral origin of a lesion. Imaging must be interpreted according to the context, the clinic and the biology. The presence of associated intracranial lesions can orientate towards a systemic or infectious disease. A T2 hyposignal lesion suggests granulomatosis or histiocytosis, especially if a meningeal or hypothalamic-pituitary involvement is associated. Non-tumoral lesions are generally not hyperperfused. In the absence of a definitive diagnosis, the evolution of these lesions, whether under treatment or spontaneous, is fundamental. PMID:25260711

  12. Behcet colitis.

    PubMed

    Chin, Anne B; Kumar, Anjali S

    2015-06-01

    Behcet disease (BD) is a chronic, multisystem, inflammatory disease characterized by variable clinical manifestations involving systemic vasculitis of both the small and large blood vessels. The majority of BD patients present with recurrent oral ulcers in combination with other manifestations of the disease, including genital ulcers, skin lesions, arthritis, uveitis, thrombophlebitis, gastrointestinal or central nervous system involvement. Gastrointestinal BD occurs in 3 to 25% of the BD patients and shares many clinical characteristics with inflammatory bowel disease (IBD). Consequently, the differentiation between IBD and gastrointestinal manifestation of BD is very difficult. Intestinal BD should be considered in patients who present with abdominal pain, diarrhea, weight loss, and rectal bleeding who are susceptible or at a risk for intestinal BD. PMID:26034406

  13. Juvenile Churg-Strauss Syndrome as an Etiology of Myocarditis and Ischemic Stroke in Adolescents; a Case Report

    PubMed Central

    Moradinejad, Mohammad-Hassan; Rezaei, Amir; Ziaee, Vahid

    2011-01-01

    Background Churg-Strauss syndrome (CSS), a systemic vasculitis accompanied by asthma and eosinophilia, almost invariably affects the lung and is frequently associated with cutaneous involvement. It rarely has cardiac involvement. We report an unusual case of CSS with myocardial involvement and stroke. Case Presentation A 16-year old female suffered of allergic asthma for 4 years. She was under treatment with oral prednisolone and seretide inhalation. After CSS diagnosis, she developed paroxysmal atrial tachycardia. Serum levels of Troponin I and Troponin T were increased indicating massive myocardial damage probably due to myocarditis. After 5 months she developed acute hemiparesis without any evidence of ischemic or hemorrhagic event. She was treated with IVIg, intravenous pulses of methylprednisone and cyclophosphamide for each complication. Conclusion Myocarditis and stroke may also complicate CSS which should be taken in consideration for better management. PMID:23056844

  14. Missed Kawasaki disease in childhood presenting as myocardial infarction in adults.

    PubMed

    Bhagwat, Ajit; Mukhedkar, Sachin; Ekbote, Shriganesh; Gordon, John B

    2015-01-01

    Kawasaki disease (KD) is an acute, self-limited vasculitis that occurs in young children and was first described by Japanese pediatrician Tomisaku Kawasaki in 1967. Although originally thought to be a rare condition, KD has become the most common cause of acquired heart disease in the pediatric population in developed countries. The majority of patients with KD appear to have a benign prognosis, but a subset of patients with coronary artery aneurysms are at risk for ischemic events and require lifelong treatment. In the 4 decades since the initial recognition of KD, the number of patients reaching adulthood has continued to grow. Adult cardiologists will be increasingly involved in the management of these patients. Currently, there are no established guidelines for the evaluation and treatment of adult patients who have had KD. We report 4 most probable cases of KD missed in childhood and presented as acute coronary syndrome in adulthood. PMID:26304576

  15. Acute Hemorrhagic Edema of Infancy: an unusual diagnosis for the general pediatrician

    PubMed Central

    Cunha, Diego Fontana Siqueira; Darcie, Ana Letícia Fornazieri; Ferronato, Angela Espósito; Hein, Noely; Lo, Denise Swei; Yoshioka, Cristina Ryoka Miyao; Hirose, Maki; Cardoso, Debora Morais; Gilio, Alfredo Elias

    2015-01-01

    Acute Hemorrhagic Edema of Infancy (AHEI) is a rare leukocytoclastic vasculitis, clinically characterized by the classical triad: palpable purpuric skin lesions, edema and fever, and is commonly misdiagnosed as Henoch-Schönlein purpura. In addition to its sudden onset, AHEI is also characterized by its self-limited course with complete and spontaneous recovery occurring between 1 and 3 weeks. Because of the scarcity of studies on therapy with corticosteroids, the conservative approach is usually recommended. The authors report an unusual case of an one-year-old boy who presented with typical cutaneous rash of AHEI and orchitis, the latter showing complete resolution after less than 24 hours of prednisolone therapy. The authors call attention to this entity mainly as a differential diagnosis of Henoch-Schönlein purpura and to the importance of new studies to establish the benefits of corticosteroid therapy for AHEI. PMID:26558246

  16. A surprise behind a case of winter vomiting virus.

    PubMed

    Panopoulou, Aikaterini; Vermaak, Erin; McHugh, Neil

    2013-01-01

    A 79-year-old man was admitted through the medical take with norovirus gastritis. Routine plain chest radiography demonstrated a right coin lesion. CT and subsequent positron emission tomography showed a right upper lobe mass consistent with primary bronchial carcinoma. The lesion was resected and histology revealed a granulomatous necrotising mass without evidence of dysplasia. Meticulous investigations for infectious and non-infectious causes of necrotising granulomatous diseases were repeatedly negative. His postoperative recovery was complicated by a hospital-acquired pneumonia and a pulmonary embolism. CT pulmonary angiography showed progression of the previously resected mass and repeat biopsy was similar to the initial. A clinical diagnosis of antineutrophil cytoplasmic antibody-negative vasculitis without extrapulmonary manifestations was made and immunosuppressive therapy was initiated with rapid clinical response. PMID:23878289

  17. Rehabilitation or the death penalty: autoimmune B cells in the dock.

    PubMed

    Dahal, Lekh N; Cragg, Mark S

    2015-03-01

    CD20-based monoclonal antibodies have become established as treatments for lymphoma, rheumatoid arthritis, systemic lupus erythematosus, vasculitis and dermatomyositis, with the principle therapeutic mechanism relating to B-cell depletion through effector cell engagement. An article by Brühl et al. in this issue of the European Journal of Immunology [Eur. J. Immunol. 2015. 45: 705-715] reveals a fundamentally distinct mechanism of silencing autoimmune B-cell responses. Rather than B-cell depletion, the authors use anti-CD79b antibodies to induce B-cell tolerance and suppress humoral immune responses against collagen to prevent the development of arthritis in mice. Here we highlight the differences in the mechanisms used by anti-CD20 and anti-CD79b Ab therapy and discuss why depletion of B cells may not be required to treat autoimmune arthritis and other B-cell-associated pathologies. PMID:25639261

  18. Frosted branch angiitis as ocular manifestation of Behçet's disease: unusual case report and literature review.

    PubMed

    Kwon, Soon Jae; Park, Dong Ho; Shin, Jae Pil

    2013-12-01

    We report an unusual case of unilateral frosted branch angiitis associated with Behçet's disease, including a review of previously reported cases. A 39-year-old male with history of recurrent oral and genital ulcers presented with visual loss in his left eye. Fundus findings demonstrated occlusive retinal vasculitis resembling acute frosted branch angiitis. Laboratory examinations including viral markers revealed no abnormal findings except positive HLA-B51. The patient was treated with systemic steroid and cyclosporine. Six months after presentation, new oral ulcers and pseudofolliculitis appeared, and he was diagnosed with Behçet's disease following rheumatology consultation. During follow-up, there was no change in visual acuity of hand movement, and disc neovascularization developed even after complete panretinal photocoagulation. Ocular manifestations of Behçet's disease can present as unilateral frosted branch angiitis, and may consecutively involve in both eyes. Early immunosuppressive treatment is recommended. PMID:24311935

  19. Cold urticaria associated with C4 deficiency and elevated IgM.

    PubMed

    Stafford, C T; Jamieson, D M

    1986-04-01

    Various immunologic abnormalities have been implicated in cold urticaria. This is the first report of cold urticaria associated with C4 deficiency and elevated IgM. A 12-year-old male developed urticaria upon exposure to cold. He denied fever, purpura, hemoglobinuria, Raynaud's disease, or arthralgias. Family history was negative for cold urticaria. Immunologic studies revealed elevated IgM (186 mg/dL) as well as decreased CH100 and C4 (8.0 mg/dL). C1, C2, and C3 were normal. Ice cube skin test was positive, but passive transfer tests were negative. Biopsy was not diagnostic for vasculitis, although it revealed a few immunofluorescent deposits of IgM and C4. Complement genetic studies revealed deficiency of two half-null C4 haplotypes expressed as C4A*3QO and B*2QO. PMID:3963523

  20. [Wegener's disease and exposure to silica. Study of the physiopathological mechanisms].

    PubMed

    Moulin, Philippe; Lehucher-Michel, Marie-Pascale

    2004-11-01

    CRYSTALLINE SILICA: Crystalline silica is an abundant mineral found in multiple occupational exposures. It is a recognised occupational risk factor for some systemic auto-immune diseases such as progressive systemic scleroderma and Caplan-Colinet's syndrome, or just suspected in Goujerot-Sjögren's syndrome, periarteritis nodosa and systemic lupus erythematosus. WEGENER'S GRANULOMATOSIS: A rare disease of unknown of unknown aetiology, the disease is characterised by granulomatous vasculitis affecting the upper respiratory tracks, lungs and kidneys, associated with glomerulonephropathy. THE ROLE OF SILICA PARTICLES: Studies have shown that crystalline silica may be implied in Wegener's disease. Associated with other factors, notably with a genetic predisposal, silica particles might, through the release of reactive oxygen radicals and inflammatory cytokines, trigger-off immunopathological mechanisms. The latter would be manifested by exocytosis of the proteinase 3 and myeloperoxidase antigens on the surface of the polynuclear neutrophils. PMID:15615243

  1. Idiopathic pulmonary hemosiderosis presenting in an adult: A case report and review of the literature

    PubMed Central

    Sherani, Khalid M.; Upadhyay, Hinesh N.; Sherani, Farha K.; Vakil, Abhay P.; Sarkar, Samir S.

    2015-01-01

    Diffuse alveolar hemorrhage (DAH) is characterized by the presence of hemoptysis, anemia, and the presence of diffuse parenchymal infiltrates on imaging studies. Idiopathic pulmonary hemosiderosis (IPH) is an uncommon cause of diffuse alveolar hemorrhage (DAH) and is classically known to present in childhood. Adult-onset IPH is extremely rare. We report the case of a 48-year-old female patient who presented with hemoptysis and acute hypoxic respiratory failure, requiring intubation and mechanical ventilation. Imaging studies showed diffuse bilateral patchy infiltrates. Bronchoalveolar lavage (BAL) confirmed the diagnosis of DAH. Extensive workup including video-assisted thoracoscopic surgical lung biopsy (VATS) failed to reveal any vasculitis, infectious, immunological or connective tissue disorder, as the underlying cause for DAH. The patient was successfully treated with high-dose steroid therapy. PMID:26180395

  2. Toxocara canis infection: Unusual trigger of systemic lupus erythematosus.

    PubMed

    Levy, Michaël; Bourrat, Emmanuelle; Baudouin, Véronique; Guillem, Colette; Peuchmaur, Michel; Deschênes, Georges; Fila, Marc

    2015-08-01

    Infection by Toxocara canis can cause systemic vasculitis. We report here a unique case of systemic lupus erythematosus (SLE) triggered by T. canis infection. An 8-year-old girl was treated with albendazole therapy for common toxocariasis, but she developed two weeks later, asthenia, fever, infiltrated maculopapular eruption of the face, peripheral vascular disease with necrosis of the fingers and inflammatory anemia with proteinuria. Anti-nuclear, anti-DNA and anti-Sm antibodies positivity, together with minimal change nephritis with mesangial exclusive IgM deposit on renal biopsy and clinical relapse after initially successful steroid therapy, led to the diagnosis of SLE. T. canis infection can trigger systemic lupus but must also be ruled out of the differential diagnosis given its association with autoimmunity. PMID:26147636

  3. Recent Advances in Kawasaki Disease

    PubMed Central

    Kim, Kyu Yeun

    2016-01-01

    Kawasaki disease (KD) is characterized with acute systemic vasculitis, occurs predominantly in children between 6 months to 5 years of age. Patients with this disease recover well and the disease is self-limited in most cases. Since it can lead to devastating cardiovascular complications, KD needs special attention. Recent reports show steady increases in the prevalence of KD in both Japan and Korea. However, specific pathogens have yet to be found. Recent advances in research on KD include searches for genetic susceptibility related to KD and research on immunopathogenesis based on innate and acquired immunity. Also, search for etiopathogenesis and treatment of KD has been actively sought after using animal models. In this paper, the recent progress of research on KD was discussed. PMID:26632378

  4. An unusual case of acute kidney injury - idiopathic granulomatous tubulointerstitial nephritis.

    PubMed

    Balafa, Olga; Liapis, Georgios; Duni, Anila; Xiromeriti, Sophia; Kalaitzidis, Rigas; Elisaf, Moses

    2015-10-01

    We present a case which emphasizes the importance of performing a kidney biopsy in each case of acute kidney injury (AKI) of unknown etiology. The unexpected histological diagnosis of granulomatous interstitial nephritis (GIN) is a rare cause of AKI. The main causes of GIN include drugs (NSAIDs, antibiotics), sarcoidosis, and infections (mycobacterial and fungal). In our case, a 68-year-old woman was admitted with AKI, absence of symptoms and unremarkable history, apart from coronary heart disease. Renal biopsy was performed, since history as well as clinical and laboratory data could not define a cause of AKI. A more meticulous clinical and laboratory investigation followed the histological diagnosis in order to rule out sarcoidosis, vasculitis or any other known causes of GIN. Finally the diagnosis was characterized as AKI due to idiopathic GIN. The patient responded well to corticosteroids. PMID:25707458

  5. A Rare Case of Zosteriform Cutaneous Metastases from a Nasopharyngeal Carcinoma.

    PubMed

    González García, Andrés; Fernández, Emiliano Grillo; Barbolla Díaz, Ignacio; Ballester, Asunción; Pian, Héctor; Fraile, Guadalupe

    2015-01-01

    From a clinical point of view, the most common presentations of cutaneous metastatic disease are papules and nodules. However, a wide morphological spectrum of lesions has been described, including erythematous patches or plaques, inflammatory erysipelas-like lesions, diffuse sclerodermiform lesions with induration of the skin, telangiectatic papulovesicles, purpuric plaques mimicking vasculitis, and alopecia areata like scalp lesions. The so-called zosteriform pattern has been described to be in few cases and to the best of our knowledge has never been described associated with a metastasis of a nasopharyngeal carcinoma. This case highlights the relevance of including cutaneous metastases in the differential diagnosis of patients with nonhealing herpes zoster-like lesions, especially in those with underlying neoplasm recently diagnosed. PMID:26697235

  6. Update on the therapy of Behçet disease

    PubMed Central

    Saleh, Zeinab

    2014-01-01

    Behçet disease is a chronic inflammatory systemic disorder, characterized by a relapsing and remitting course. It manifests with oral and genital ulcerations, skin lesions, uveitis, and vascular, central nervous system and gastrointestinal involvement. The main histopathological finding is a widespread vasculitis of the arteries and veins of any size. The cause of this disease is presumed to be multifactorial involving infectious triggers, genetic predisposition, and dysregulation of the immune system. As the clinical expression of Behçet disease is heterogeneous, pharmacological therapy is variable and depends largely on the severity of the disease and organ involvement. Treatment of Behçet disease continues to be based largely on anecdotal case reports, case series, and a few randomized clinical trials. PMID:24790727

  7. Anaphylactoid Purpura Manifested after Acute Gastroenteritis with Severe Dehydration in an 8-Year-Old Male Child: A Case Report.

    PubMed

    Thakkar, Umang G; Vanikar, Aruna V; Trivedi, Hargovind L

    2015-12-01

    Anaphylactoid purpura, also known as Henoch-Schönleinpurpura (HSP), is an IgA-mediated vasculitis that tends to be a benign disease of childhood. Up to 50% of cases are preceded by an upper tract respiratory infection caused by group-A beta-hemolytic streptococcus and present with the common tetrad of abdominal pain, arthritis, purpuric rash, and renal involvement. The majority of patients recover completely. Here we document a rare case of anaphylactoid purpura which manifested with skin lesions in the form of palpable purpura following about of acute gastroenteritis with severe dehydration; it was treated with a short regimen of steroid therapy, which resulted in the complete remission of the disease. We conclude that prompt diagnosis and multidisciplinary intervention will lead to appropriate management-consisting of the installation of early short-course steroid therapy and thus, prevent further complications and the recurrence of the disease. PMID:26602584

  8. Systemic lupus erythaematosus presenting as spontaneous splenic rupture.

    PubMed

    Cruz, António José; Castro, Alexandra

    2015-01-01

    Systemic lupus erythaematosus (SLE) is known to involve the reticuloendothelial system, but spontaneous splenic rupture (SSR) in the context of the disease is a very rare complication. We observed a 61-year-old woman with an unremarkable previous medical history who presented with SSR and underwent an emergency splenectomy. The histopathological analysis of the specimen revealed signs of vasculitis. On review of symptoms with the patient, a history of oligoarthralgia, photosensitivity, xerostomia and Raynaud phenomenon was elicited. Laboratory investigations revealed lymphopaenia, mild proteinuria and positive antinuclear and anti-dsDNA antibodies. The patient was started on hydroxychloroquine and the disease has since remained silent. This article addresses the rare association between SLE and SSR. PMID:26621866

  9. The Use of 18F-FDG-PET/CT for Diagnosis and Treatment Monitoring of Inflammatory and Infectious Diseases

    PubMed Central

    Glaudemans, Andor W. J. M.; de Vries, Erik F. J.; Dierckx, Rudi A. J. O.; Slart, Riemer H. J. A.; Signore, Alberto

    2013-01-01

    FDG-PET, combined with CT, is nowadays getting more and more relevant for the diagnosis of several infectious and inflammatory diseases and particularly for therapy monitoring. Thus, this paper gives special attention to the role of FDG-PET/CT in the diagnosis and therapy monitoring of infectious and inflammatory diseases. Enough evidence in the literature already exists about the usefulness of FDG-PET/CT in the diagnosis, management, and followup of patients with sarcoidosis, spondylodiscitis, and vasculitis. For other diseases, such as inflammatory bowel diseases, rheumatoid arthritis, autoimmune pancreatitis, and fungal infections, hard evidence is lacking, but studies also point out that FDG-PET/CT could be useful. It is of invaluable importance to have large prospective multicenter studies in this field to provide clear answers, not only for the status of nuclear medicine in general but also to reduce high costs of treatment. PMID:24027590

  10. Pathogenesis and treatment of ANCA-associated vasculitides.

    PubMed

    Kallenberg, Cees G M

    2015-01-01

    Recent studies have increased our insight into the pathogenesis of ANCA-associated vasculitis (AAV). Although many data from in vitro and in vivo experimental studies support the pathogenic role of the autoantibodies, cellular immunity seems involved as well. Besides, an amplification loop via the alternative pathway of complement is apparent. These new insights make a more targeted therapeutic approach possible. In particular, the B-cell depleting antibody rituximab has been shown non-inferior to cyclophosphamide for induction of remission, and even superior in patients with relapsing disease being positive for PR3-ANCA. Rituximab is also superior to cyclophosphamide for maintaining remission. Blocking the C5a-receptor seems promising as well as an alternative for high dose corticosteroids during induction of remission. PMID:26457917

  11. Cardiogenic shock, asthma, and hypereosinophilia.

    PubMed

    Bouabdallaoui, Nadia; Arlet, Jean-Benoît; Hagege, Albert A

    2015-02-01

    Eosinophilic granulomatosis with polyangiitis (EGPA) (formerly Churg-Strauss) is a multisystem syndrome associating asthma, hypereosinophilia,and signs of peripheral vasculitis. We report the case of a 21-year-old man admitted for cardiogenic shock revealing a severe left and right ventricular dysfunction. Hypereosinophilia, history of asthma, and peripheral neuropathy strongly suggested the diagnosis of EGPA. Cardiac magnetic resonance imaging confirmed heart involvement with a diffuse subendocardial late gadolinium enhancement. The patient was successfully treated with systemic corticosteroids, intravenous cyclophosphamide, and inotropes. At 6-month follow-up, the patient is doing well. Cardiac involvement is rare in EGPA and often carries poor prognosis. Specific early steroid therapy may improve hemodynamic at short term, allowing postponing the need for circulatory mechanical support or heart transplantation. PMID:25195047

  12. Eosinophilic ascites secondary to toxocariasis.

    PubMed

    Tau, A H; Vizcaychipi, K A; Cabral, D H; Maradei, J L; Ferreyra, A H; San Sebastián, E I

    2015-01-01

    Eosinophilic ascites is a very rare disorder. It can be a manifestation of the eosinophilic gastroenteritis in its serosal form or it can be secondary to infections, malignancies, vasculitis or hypereosinophilic syndrome. Among all infections, the ones produced by invasive helminth parasites should be initially suspected and ruled out. We report the case of a patient with eosinophilic ascites associated with diarrhea, abdominal pain and eosinophilia in peripheral blood. Eosinophilic colitis was also demonstrated in a colonic biopsy and empirical steroid treatment was started for suspected eosinophilic gastroenteritis. Later on, the patient improved; the ascites disappeared and the eosinophil blood count returned to normal. Subsequently, serologic testing for toxocariasis was received positive and therefore, the diagnosis of eosinophilic gastroenteritis was discarded; albendazole was also added to treatment. The patient remained asymptomatic on follow-up. We emphasize the need to rule out parasitic infections in all patients with gastrointestinal symptoms, eosinophilia and eosinophilic infiltration of gastrointestinal tissues. PMID:26448417

  13. Anti-inflammatory actions of acupuncture.

    PubMed Central

    Zijlstra, Freek J; van den Berg-de Lange, Ineke; Huygen, Frank J P M; Klein, Jan

    2003-01-01

    Acupuncture has a beneficial effect when treating many diseases and painful conditions, and therefore is thought to be useful as a complementary therapy or to replace generally accepted pharmacological intervention. The attributive effect of acupuncture has been investigated in inflammatory diseases, including asthma, rhinitis, inflammatory bowel disease, rheumatoid arthritis, epicondylitis, complex regional pain syndrome type 1 and vasculitis. Large randomised trials demonstrating the immediate and sustained effect of acupuncture are missing. Mechanisms underlying the ascribed immunosuppressive actions of acupuncture are reviewed in this communication. The acupuncture-controlled release of neuropeptides from nerve endings and subsequent vasodilative and anti-inflammatory effects through calcitonine gene-related peptide is hypothesised. The complex interactions with substance P, the analgesic contribution of beta-endorphin and the balance between cell-specific pro-inflammatory and anti-inflammatory cytokines tumour necrosis factor-alpha and interleukin-10 are discussed. PMID:12775355

  14. Angiography-negative primary angiitis of the central nervous system in childhood.

    PubMed

    Park, Michael S; Marlin, Arthur E; Gaskill, Sarah J

    2014-01-01

    Childhood primary angiitis of the CNS is a recently characterized, potentially reversible disease process. A favorable outcome requires early diagnosis and appropriate treatment. The histological findings of childhood primary angiitis of the CNS are characterized by a lymphocytic, nongranulomatous vasculitis. This disorder can lead to neurological deficits, seizures, and strokes. Laboratory and radiographic investigation are part of the evaluation, but are often nonspecific. Conventional angiography can fail to show any abnormality, and biopsy may ultimately be required for diagnosis. Although there can be significant rates of morbidity and mortality if untreated, patients who receive appropriate therapy can experience excellent outcomes, and in many cases will demonstrate near-complete or total clinical and radiographic resolution. The case of a previously healthy 13-year-old girl with new-onset generalized tonic-clonic seizures is presented, with a review of the literature. PMID:24160668

  15. Recurrent episodes of hemorrhagic alveolitis in relapsing catastrophic antiphospholipid syndrome: the same side of the dark moon.

    PubMed

    Pieralli, Filippo; Grazzini, Maddalena; Vannucchi, Vieri; Mancini, Antonio; Cammelli, Daniele; Nozzoli, Carlo

    2014-03-01

    Catastrophic antiphospholipid syndrome (CAPS) is a rare variant of antiphospholipid syndrome characterized by widespread thrombotic microangiopathy and multiorgan failure. Clinically, CAPS signs and symptoms can mimic vasculitis of systemic lupus erythematosus, disseminated intravascular coagulation, and thrombotic thrombocytopenic purpura. CAPS is burdened by high mortality, nearly 50% in most series. However, patients surviving the acute phase rarely suffer of CAPS relapses. Moreover, concomitant pulmonary hemorrhagic alveolitis is a very rare complication warranting an ominous prognosis. Only few reports of relapsing CAPS are described in literature, and pathogenetic mechanisms are poorly understood and the optimal treatment is yet unknown. We report a case of a young man suffering from multiple relapses of CAPS and recurrent hemorrhagic pulmonary alveolitis refractory to aggressive combination treatment. PMID:24362787

  16. Severe Eosinophilic Syndrome Associated with the Use of Probiotic Supplements: A New Entity?

    PubMed Central

    Mendoza, Fabian A.; Purohit, Shivani; Kenyon, Lawrence; Jimenez, Sergio A.

    2012-01-01

    Severe eosinophilic syndromes related to the administration or use of unsuspected immunogenic substances have been described previously. Many of these diseases presented initially as clusters or isolated cases. The spanish toxic oil syndrome, the eosinophilia myalgia syndrome, and nephrogenic systemic fibrosis are examples of such diseases. We describe 2 cases of a severe eosinophilic syndrome characterized by marked peripheral blood eosinophilia (>15,000?cells/ml), mononeuritis multiplex, and necrotizing vasculitis which developed in a close temporal association with the recent onset use of nonprescription probiotics. There was no history of a prior autoimmune disease. Although both cases had prompt response to immunosuppression with rapid resolution of peripheral blood eosinophilia and accompanying constitutional symptoms, they remained with permanent neurological deficits. PMID:23259129

  17. Henipavirus pathogenesis and antiviral approaches.

    PubMed

    Mathieu, Cyrille; Horvat, Branka

    2015-03-01

    Hendra virus and Nipah virus are closely related, recently emerged zoonotic paramyxoviruses, belonging to the Henipavirus genus. Both viruses induce generalized vasculitis affecting particularly the respiratory tract and CNS. The exceptionally broad species tropism of Henipavirus, the high case fatality rate and person-to-person transmission associated with Nipah virus outbreaks emphasize the necessity of effective antiviral strategies for these intriguing threatening pathogens. Current therapeutic approaches, validated in animal models, target early steps in viral infection; they include the use of neutralizing virus-specific antibodies and blocking membrane fusion with peptides that bind the viral fusion protein. A better understanding of Henipavirus pathogenesis is critical for the further advancement of antiviral treatment, and we summarize here the recent progress in the field. PMID:25634624

  18. [Mild hemophilia A fortuitously discovered during Henoch-Schönlein purpura].

    PubMed

    Joly, B; d'Oiron, R; Desconclois, C; Bendelac, L; Rafowicz, A; Meyzer, C; Labrune, P; Veyradier, A

    2015-11-01

    Henoch-Schönlein purpura is a common form of immunological vasculitis in children. Hemophilia A is a genetic disorder, inherited in a X-linked recessive pattern, and characterized by spontaneous hemorrhage or prolonged bleeding due to factor VIII deficiency. The clinical signs depend on the severity of factor VIII deficiency. We herein report the case of a 4-year-old boy admitted to the emergency room for typical rheumatoid purpura, associated with a lengthening of aPTT, whose exploration had uncovered mild hemophilia A. Laboratory assays should explore lengthening of aPTT: firstly the presence of lupus anticoagulant without bleeding risk, in an inflammatory context; secondly a deficiency of VWF and one of the factors involved in the extrinsic coagulation pathway associated with bleeding risk. PMID:26412326

  19. Rheumatologic emergencies.

    PubMed

    Gutiérrez-González, Luis Arturo

    2015-12-01

    Rheumatological conditions can sometimes present as emergencies. These can occur due to the disease process or infection; contrary to what many people think, rheumatologic emergencies like a pain, rheumatic crisis, or attack gout do not compromise the patient's life. This article mentioned only true emergencies: catastrophic antiphospholipid syndrome (cAPS), kidney-lung syndrome, central nervous system (CNS) vasculitis, anti-Ro syndrome (neonatal lupus), and macrophage activation syndrome (MAS). The management of above emergencies includes critical care, immunosuppression when indicated, and use of a diagnostic flowchart as well as fast laboratory profile for making decisions. Anticoagulants have to be used in the management of antiphospholipid syndrome. A good understanding of these conditions is of paramount importance for proper management. PMID:26099604

  20. Low-dose cyclosporin for multiple colonic ulcers associated with mixed connective tissue disease.

    PubMed

    Maeda, N; Abe, S; Yoshizawa, T; Ogawa, K; Sekigawa, I; Iida, N; Eguchi, M; Matsumoto, M; Hashimoto, H; Hirose, S

    1999-01-01

    This report describes a patient with multiple colonic ulcers and mixed connective tissue disease. The histological findings of the colonic lesions showed vasculitis with T-cell infiltration, and the peripheral T cells were frequently in the activated phase of the cell cycle. In this patient, low-dose cyclosporin treatment (2.5 mg/kg/day) inhibited the T-cell activation in the peripheral lymphocytes and was very effective in the gastrointestinal disorder, which might be related to T-cell activation. This case suggests the possibility that even low-dose cyclosporin can exert a great influence on peripheral T cells and directly inhibit T-cell activation, thereby improving symptoms related to T-cell activation. PMID:10524557

  1. Ulcerative colitis and steroid-responsive, diffuse interstitial lung disease

    SciTech Connect

    Balestra, D.J.; Balestra, S.T.; Wasson, J.H.

    1988-07-01

    The authors describe a patient with ulcerative colitis and extracolonic manifestations in whom diffuse interstitial pulmonary disease developed that was responsive to glucocorticoid therapy one year after total proctocolectomy. The patient presented in December 1983 with a subacute course marked by cough and progressive exertional dyspnea, abnormal chest examination results, and a chest roentgenogram that revealed diffuse interstitital and alveolar infiltrates. A transbronchial biopsy specimen revealed a polymorphic interstitial infiltrate, mild interstitial fibrosis without apparent intraluminal fibrosis, and no vasculitis, granulomas, or significant eosinophilic infiltration. Within one week of the initiation of daily high-dose steroid therapy, the patient's symptoms dramatically improved; chest roentgenogram and forced vital capacity (60%) improved at a slower rate. All three measures deteriorated when alternate-day prednisone therapy was started but once again improved until the patient was totally asymptomatic, chest roentgenograms were normal, and forced vital capacity was 80% of the predicted value 2 1/2 years later.

  2. Total lymphoid irradiation therapy in refractory rheumatoid arthritis. Fifteen- to forty-month followup

    SciTech Connect

    Brahn, E.; Helfgott, S.M.; Belli, J.A.; Anderson, R.J.; Reinherz, E.L.; Schlossman, S.F.; Austen, K.F.; Trentham, D.E.

    1984-05-01

    Twelve patients with refractory rheumatoid arthritis were treated with total lymphoid irradiation (TLI) to a total cumulative dose of 3,000 rads. Post-TLI morbidity/mortality included 8 patients with xerostomia, 4 with weight loss of greater than 10 kg, 3 with loss of 4 or more teeth, 3 with herpes zoster, 4 with bacterial infection that was fatal in 2, 3 with hypothyroidism, 1 with cutaneous vasculitis, and death from myocardial infarction in 1 patient and cardiorespiratory arrest in another. Ten of the patients were reevaluated 15-40 months (mean +/- SE, 30 +/- 2) after completion of TLI, and significant improvement was noted in several disease parameters including number of swollen joints, duration of morning stiffness, and 50-foot walking time. Blood lymphopenia and a decrease in helper T cells (T4) were also noted. These data suggest that changes in immunoregulation induced by TLI can produce longlasting alterations in rheumatoid arthritis, although adverse effects may limit its efficacy.

  3. Recent Advances in Kawasaki Disease.

    PubMed

    Kim, Kyu Yeun; Kim, Dong Soo

    2016-01-01

    Kawasaki disease (KD) is characterized with acute systemic vasculitis, occurs predominantly in children between 6 months to 5 years of age. Patients with this disease recover well and the disease is self-limited in most cases. Since it can lead to devastating cardiovascular complications, KD needs special attention. Recent reports show steady increases in the prevalence of KD in both Japan and Korea. However, specific pathogens have yet to be found. Recent advances in research on KD include searches for genetic susceptibility related to KD and research on immunopathogenesis based on innate and acquired immunity. Also, search for etiopathogenesis and treatment of KD has been actively sought after using animal models. In this paper, the recent progress of research on KD was discussed. PMID:26632378

  4. Biologic therapy for refractory scleritis: a new treatment perspective.

    PubMed

    de Fidelix, Tania Sales de Alencar; Vieira, Luis Antonio; de Freitas, Denise; Trevisani, Virginia Fernandes Moça

    2015-12-01

    Scleritis is an umbrella term for a heterogeneous group of ocular diseases that can be associated with autoimmune or systemic disorders. The purpose of this article was to review the literature regarding the use of biologic drugs to treat refractory scleritis. A search of the MEDLINE, Embase, and LILACS electronic databases was conducted, and the reference lists of published articles were hand-searched. No language filters were used. This search strategy yielded no randomized trials of the use of biologic therapies to treat scleritis; only case reports and retrospective studies were retrieved. These studies suggest that monoclonal antibodies (infliximab and adalimumab) are superior to the soluble TNF receptor fusion protein etanercept for the treatment of scleritis in patients that do not respond to corticosteroids and/or to immunosuppressive treatment. Rituximab seems to be the best option for scleritis associated with vasculitis. PMID:26319144

  5. Mouse interstitial lung disease and pleuritis induction by human Mollicute-like organisms.

    PubMed Central

    Wirostko, E.; Johnson, L. A.; Wirostko, W. J.

    1988-01-01

    Mollicute-Like Organisms (MLO) are cell-wall deficient intracellular bacterial pathogens. As MLO are non-cultivable, detection is based on finding typical Mollicute bodies within the host cell using a transmission electron microscope. Extracellular Mollicutes cause disease by a variety of mechanisms. MLO cause disease by similar mechanisms, and in addition directly alter the host cell nucleus, replace the cytoplasm, and destroy the organelles. MLO parasitization of plant cells causes a well studied chronic vascular disease reversible by tetracycline antibiotics. Recently similar MLO were reported to cause human chronic ocular vasculitis. As it parasitizes, lyses, and destroys leucocytes, it has been termed Leucocytoclastic MLO. Inoculation of this MLO into mouse eyelids produced delayed onset chronic ocular and lethal cardiac vasculitis. All lesions demonstrated tissue lysis with leucocytic infiltrates and MLO parasitized leucocytes. MLO-caused human and mouse disease responds to Rifampin. This report describes the 40 interstitial lung disease lesions in 21 of 100 of those MLO inoculated mice vs 0 in 200 controls (P less than 0.05) and 27 pleuritis lesions in 17 mice vs 0 control mice (P less than 0.05). The lung and pleural disease were associated in 13 lesions and unassociated in 41 lesions. MLO parasitized leucocytes were found in both the lung and pleural lesions from six of six MLO inoculated mice versus none of six controls. As most human interstitial lung and pleural diseases are idiopathic and closely resemble this mouse disease, they may be induced by MLO and treatable by Rifampin. Images Fig. 1 Fig. 2 Fig. 3 Fig. 4 Fig. 5 Fig. 6 Fig. 7 Fig. 8 PMID:3219289

  6. The interface of inflammation and subclinical atherosclerosis in granulomatosis with polyangiitis (Wegener's): a preliminary study.

    PubMed

    Hajj-Ali, Rula A; Major, Jennifer; Langford, Carol; Hoffman, Garry S; Clark, Tiffany; Zhang, Li; Sun, Zhiyuan; Silverstein, Roy L

    2015-10-01

    The objective of this study is to assess the relationship between inflammatory disease in granulomatosis with polyangiitis (GPA, Wegener's) and the development of subclinical atherosclerosis. A total of 46 adult patients with GPA were enrolled. Disease status was measured by Birmingham vasculitis assessment scores as modified for GPA, vasculitis damage index, disease duration, and number of relapses. Classic atherosclerotic risk factors, platelet aggregation responses, and circulating microparticle (MP) levels were recorded. All patients underwent carotid artery intima-media thickness (IMT) measurement as outcome for subclinical atherosclerosis. In univariate analyses, systolic and diastolic blood pressure, creatinine, and age were significantly associated with higher IMT (? values 0.37, 0.38, 0.35, and 0.054, respectively [P < 0.02 for all]). In a multiple regression model, greater number of relapses, older age at the onset of disease, and higher diastolic blood pressure were found to be associated with higher IMT (P values 0.003, <0.001, and 0.031, respectively). MP counts and platelet reactivity correlated well with disease activity in GPA. Furthermore, MPs were found to activate vascular endothelial cells and platelets in vitro. The cumulative burden of systemic inflammation in GPA correlated with the development of subclinical atherosclerosis. The correlation with subclinical atherosclerosis could be because of glucocorticoid use and not the inflammatory process in GPA, giving the inherent bias that exits with the use of glucocorticoid with each relapse. The findings of increased levels of circulating leukocyte-derived MPs and enhanced platelet reactivity during relapse suggest possible roles for MPs and platelets in disease pathogenesis and support a growing literature that links inflammation, atherosclerosis, and platelet activation. This hypothesis is further substantiated by our demonstration that MPs isolated from plasma of GPA patients can activate platelets and vascular endothelial cells. PMID:26024800

  7. Autoimmunity and the outer retina.

    PubMed

    Rahi, A H; Addison, D J

    1983-01-01

    Structurally and therefore antigenically the retina is a complex tissue. Since it develops as an extension from the neural tube it shares with the brain several cell membranes and cytoplasm associated antigens including those present in neurofilaments of the various neurones and the glial filaments of the astrocytes. The advent of monoclonal antibodies has helped to dissect, in detail, the antigenic makeup of the retina. Nervous system antigens (NS-3, 4 and 7) are generously represented in the retina. At least in the chick eye there seems to be a concentration gradient of retinal antigens along a dorsoventral axis which is believed to provide means by which neurones of developing retinal signal and receive the positional information necessary for the formation of specific synapses. It now seems certain that organ-specific antigens are presented not only in the photoreceptors and the retinal pigment epithelium but also in the retinal ganglion cells and the astrocytes. Photoreceptor outer-segment contains soluble antigens which when injected in rats, rabbits, guinea-pigs or monkeys produce varying degrees of intraocular inflammation leading to uveitis, retinal detachment, photoreceptor degeneration and occasionally retinal vasculitis. Both cell-mediated and humoral immunity to photoreceptor antigen has been demonstrated in various types of uveitis (including toxoplasmosis and sarcoidosis), pars planitis, vitriitis, Behçets disease, sympathetic ophthalmitis, Vogt-Koyanagi-Harada syndrome, birdshot retinopathy, retinitis pigmentosa and retinal vasculitis. Retinal autoimmunity is also found in retinal detachment and diabetic retinopathy, particularly after Argon laser photocoagulation. Antibodies to retinal antigens are also found in patients with systemic lupus erythematosus and other systemic immune disorders without ocular involvement. The precise pathogenetic role of retinal autoimmunity in eye disease is therefore uncertain. It may simply represent an epiphenomenon which develops afer retinal damage due to physical, micro-organismal or immunological insult. Alternatively it is possible that although autoimmunity does not initiate ocular inflammation it perpetuates and maintains the inflammatory state and produces further damage to ocular tissues. PMID:6611615

  8. Eosinophilic Granulomatosis with Polyangiitis: An Overview

    PubMed Central

    Gioffredi, Andrea; Maritati, Federica; Oliva, Elena; Buzio, Carlo

    2014-01-01

    Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystemic disorder, belonging to the small vessel anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, defined as an eosinophil-rich and necrotizing granulomatous inflammation often involving the respiratory tract, and necrotizing vasculitis predominantly affecting small to medium-sized vessels, associated with asthma and eosinophilia. EGPA pathogenesis is not well known: HLA-DRB1*04 and *07, HLA-DRB4 and IL10.2 haplotype of the IL-10 promoter gene are the most studied genetic determinants. Among the acquired pathogenetic factors, the exposure to different allergens, infections, vaccinations, drugs, and silica exposure have been involved. Eosinophils are the most characteristic cells in EGPA and different studies have demonstrated their role as effector and immunoregulatory cells. EGPA is considered as a disease with a prevalent activation of the Th-2 cellular-mediated inflammatory response and also humoral immunity plays an important role. A link between B and T inflammatory responses may explain different disease features. EGPA typically develops into three sequential phases: the allergic phase, distinguished by the occurrence of asthma, allergic rhinitis, and sinusitis, the eosinophilic phase, in which the main pathological finding is the eosinophilic organ infiltrations (e.g., lungs, heart, and gastrointestinal system), and the vasculitic phase, characterized by purpura, peripheral neuropathy, and constitutional symptoms. ANCA (especially pANCA anti-myeloperoxidase) are present in 40–60% of the patients. An elevation of IgG4 is frequently found. Corticosteroids and cyclophosphamide are classically used for remission induction, while azathioprine and methotrexate are the therapeutic options for remission maintenance. B-cell depletion with rituximab has shown promising results for remission induction. PMID:25404930

  9. Interstitial nephritis with moderate-to-heavy proteinuria: an unusual combination.

    PubMed

    Ghosh, Biplab; Singh, Rana Gopal; Behura, Sanjeev Kumar; Soni, Ashutosh; Sharatchandra, Lou Krakpam; Singh, Shivendra

    2012-03-01

    Interstitial nephritis with proteinuria >1 g/day is uncommon and almost always the result of drug-induced ATIN with an associated minimal change glomerulonephritis (GN). Here, we present a series of five unusual cases of interstitial nephritis without GN but with proteinuria >1 g/day, and they were identified from renal biopsies done from February 2008 to March 2009. Out of 236 patients who underwent renal biopsy, only five met the inclusion criteria. Three patients presented with edema and two with oliguria, while none had frank hematuria, fever, arthralgia, skin rash or history of exposure to nonsteroidal antiinflamatory drugs, analgesics, antibiotics, allopurinol, or Chinese herb before presentation. Urinalysis revealed hematuria in two patients, pyuria in three and nephrotic range proteinuria in two. All had normal complement levels and were negative for antinuclear antibodies, Anti-dsDNA antibody, and antineutrophil cyto-plasmic antibodies. Clinical diagnosis was nephrotic syndrome in two patients, the third had diagnosis of rapidly progressive GN, the fourth had HIV associated nephropathy, and the fifth had unexplained advanced renal failure. Though three patients had renal dysfunction only one required dialysis. Light microscopy of renal biopsies revealed granulomatous interstitial nephritis in three patients and small vessel vasculitis in two of them. One patient had nongranulomatous interstitial nephritis along with vasculitis. Acute interstitial nephritis was the only finding in one patient. In conclusion, patients with interstitial nephritis can present with moderate-to-heavy proteinuria probably due to cytokine-like permeability increasing factor secreted by inflammatory cells in the interstitium. PMID:22382248

  10. Diagnosis and classification of polyarteritis nodosa.

    PubMed

    Hernández-Rodríguez, José; Alba, Marco A; Prieto-González, Sergio; Cid, Maria C

    2014-01-01

    Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis preferentially targeting medium-sized arteries and not associated with glomerulonephritis or small vessel involvement. Anti-neutrophil cytoplasmic antibodies are typically negative. PAN may be triggered by viral infections, particularly hepatitis B virus, but remains idiopathic in most cases. Clinical manifestations of PAN are multisystemic. Peripheral nerve and skin are the most frequently affected tissues. Involvement of the gastrointestinal tract, kidneys, heart, and central nervous system is associated with higher mortality. Laboratory abnormalities reveal a prominent acute phase response but are non-specific. Histologic confirmation of vasculitis in medium sized arteries is desirable and biopsies must be obtained from symptomatic organs if feasible. Skin or muscle and nerve are preferred because of higher diagnostic yield and safety. If biopsies are negative or cannot be obtained, visceral angiography, may reveal multiple micro-aneurysms supporting the diagnosis of PAN. Current treatment policy includes high-dose corticosteroids, which are combined with immunosuppressive agents when critical organ involvement or life-threatening complications occur. IV pulse cyclophosphamide in the remission induction phase, later switched to a safer immunosuppressant for remission maintenance is a frequently used therapeutic approach. A recent consensus algorithm for the classification of PAN has attempted to overcome some of the caveats of the 1990 American College of Rheumatology (ACR) classification criteria which have proven to be unsatisfactory, and has also confirmed the low prevalence of PAN compared to other systemic necrotizing vasculitides. European league against rheumatic diseases (EULAR)/ACR endorsed international cooperation to establish new diagnostic/classification criteria is currently under way. PMID:24485157

  11. Eosinophilic granulomatosis with polyangiitis: an overview.

    PubMed

    Gioffredi, Andrea; Maritati, Federica; Oliva, Elena; Buzio, Carlo

    2014-01-01

    Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystemic disorder, belonging to the small vessel anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, defined as an eosinophil-rich and necrotizing granulomatous inflammation often involving the respiratory tract, and necrotizing vasculitis predominantly affecting small to medium-sized vessels, associated with asthma and eosinophilia. EGPA pathogenesis is not well known: HLA-DRB1*04 and *07, HLA-DRB4 and IL10.2 haplotype of the IL-10 promoter gene are the most studied genetic determinants. Among the acquired pathogenetic factors, the exposure to different allergens, infections, vaccinations, drugs, and silica exposure have been involved. Eosinophils are the most characteristic cells in EGPA and different studies have demonstrated their role as effector and immunoregulatory cells. EGPA is considered as a disease with a prevalent activation of the Th-2 cellular-mediated inflammatory response and also humoral immunity plays an important role. A link between B and T inflammatory responses may explain different disease features. EGPA typically develops into three sequential phases: the allergic phase, distinguished by the occurrence of asthma, allergic rhinitis, and sinusitis, the eosinophilic phase, in which the main pathological finding is the eosinophilic organ infiltrations (e.g., lungs, heart, and gastrointestinal system), and the vasculitic phase, characterized by purpura, peripheral neuropathy, and constitutional symptoms. ANCA (especially pANCA anti-myeloperoxidase) are present in 40-60% of the patients. An elevation of IgG4 is frequently found. Corticosteroids and cyclophosphamide are classically used for remission induction, while azathioprine and methotrexate are the therapeutic options for remission maintenance. B-cell depletion with rituximab has shown promising results for remission induction. PMID:25404930

  12. 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

  13. Peripheral Endothelial (Dys)Function, Arterial Stiffness and Carotid Intima-Media Thickness in Patients after Kawasaki Disease: A Systematic Review and Meta-Analyses

    PubMed Central

    Dietz, Sanne M.; Tacke, Carline E. A.; Hutten, Barbara A.; Kuijpers, Taco W.

    2015-01-01

    Background Kawasaki disease (KD) is a systemic pediatric vasculitis. Its main complication is the development of coronary arterial aneurysms (CAA), causing an increased risk for ischemia and myocardial infarction. It is unclear whether KD patients, apart from the presence of CAA, have an increased cardiovascular disease (CVD) risk due to the previous systemic vasculitis. The aim of this study was to systematically review and meta-analyse the literature regarding surrogate markers for CVD risk in KD patients. Methods Medline and Embase were searched for articles comparing endothelial dysfunction (flow-mediated dilation, nitroglycerin-mediated dilation and peripheral arterial tonometry), vascular stiffness (stiffness index, pulse wave velocity) and carotid intima-media thickness (cIMT) between patients and controls. Two investigators assessed the articles for eligibility and evaluated quality. Results Thirty studies were included. For all outcomes, moderate to high heterogeneity between studies was found. Most studies reported a decreased flow-mediated dilation in the whole KD- and CAA-positive group compared to controls, while data on CAA-negative patients were conflicting. The stiffness index was increased in the majority of studies evaluating the whole KD- and CAA-positive group, but not in most studies on CAA-negative patients. Mean cIMT was neither significantly increased in the whole KD-group nor in the CAA-positive group nor in most studies studying CAA-negative patients. Studies measuring maximum cIMT were conflicting. Conclusion Literature suggests that surrogate markers for CVD risk in KD patients are increased in CAA-positive but not in CAA-negative patients. This may indicate that CAA-positive patients should be monitored for CVD in later life. The results of this review have to be interpreted with care due to substantial heterogeneity between studies and methodological limitations, as well as the lack of long-term follow-up studies. PMID:26161871

  14. Production of anti-endothelial cell antibodies by coculture of EBV-infected human B cells with endothelial cells.

    PubMed

    Delneste, Y; Lassalle, P; Jeannin, P; Mannessier, L; Dessaint, J P; Joseph, M; Tonnel, A B

    1993-08-01

    Vascular endothelial cells are suspected of being the target of autoimmune processes seen in many connective tissue diseases and in systemic vasculitis as evidenced by the detection of circulating autoantibodies against endothelial cell antigens. In order to select B cells recognizing endothelial cells antigens, Epstein-Barr virus (EBV)-infected B cells, obtained from one patient presenting a systemic vasculitis, were cocultured with human endothelial cells concurrently with a human endothelial cell line (EC-pSV1 cells). This coculture consisted of a first step of expansion of B cells specifically selected by adherence onto human umbilical vein endothelial cells (HUVEC). The adherence of selected B cells was specific to endothelial cells because no rosette formation around control cells (HeLa cells or COS cells) was observed. Adherent B cells were cloned by limiting dilution by coculture onto EC-pSV1 cells and screened for anti-HUVEC antibody production by endothelial cell ELISA. An increase in anti-HUVEC antibody production of IgM isotype was detected by endothelial cell ELISA, peaking at Day 9 and remaining constantly elevated, relative to B cell expansion. Among 21 B cell lines producing IgM, 6 presented high levels of anti-HUVEC antibodies, whereas 1 of 52 B cells cloned without EC-pSV1 cells showed such antibody production. Anti-HUVEC antibody production and B cell proliferation were dependent on the presence of endothelial cells. Two of these 6 B cell lines produced antibodies directed against an endothelial cell antigen with an apparent molecular weight of 192 kDa as determined by immunoblotting analysis. Our results demonstrate that adherence of EBV-infected B cells to endothelial cells and further cloning by adherence can efficiently select anti-HUVEC antibody-producing human B cells and might help to define antigens potentially involved in autoimmune diseases. PMID:7688268

  15. The role of anti-endothelial cell antibodies in Kawasaki disease – in vitro and in vivo studies

    PubMed Central

    Grunebaum, E; Blank, M; Cohen, S; Afek, A; Kopolovic, J; Meroni, P L; Youinou, P; Shoenfeld, Y

    2002-01-01

    Kawasaki disease (KD) is a systemic vasculitis with cardiac and noncardiac complications. Anti-endothelial cell antibodies (AECA) are found among many patients with KD. The aim of this study was to investigate the pathogenic role of AECA in KD using in vitro and in vivo experimental models. F(ab)2 fragments of IgG-AECA and IgM-AECA were affinity purified from a patient with active KD. Their endothelial binding and ability to induce a pro-adhesive and a pro-inflammatory phenotype were evaluated in vitro. Twenty Balb/C mice were immunized with KD-AECA or with control Ig (N-Ig) to induce AECA in a murine model by the idiotypic manipulation method. Both KD-AECA isotypes bind significantly to human umbilical vein endothelial cell (HUVEC) compared to N-Ig. The in vitro activity was demonstrated by the antibodies ability to activate endothelial cells resulting in increased IL-6 secretion, adhesion molecule expression and monocytic cell line (U937) adherence to HUVEC. Five of the mice that received KD-AECA developed murine AECA after 3 months. None of the mice that received N-Ig produced AECA. The murine AECA increased monocyte adhesion to EC in vitro, similarly to the AECA used for immunization. Furthermore, all the mice that developed AECA had proteinuria and IgG deposition in the renal mesangium. No histological or immunofluorescence evidence of cardiac vasculitis could be detected. AECA might play a role in the emergence of some of KD manifestations. PMID:12390310

  16. Antibodies to selected minor target antigens in patients with anti-neutrophil cytoplasmic antibodies (ANCA).

    PubMed

    Talor, M V; Stone, J H; Stebbing, J; Barin, J; Rose, N R; Burek, C L

    2007-10-01

    In patients with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis, indirect immunofluorescence (IF) distinguishes between cytoplasmic (C-ANCA) and perinuclear (P-ANCA) neutrophil staining patterns. In patients with primary systemic vasculitis such as Wegener's granulomatosis, microscopic polyangiitis and Churg-Strauss syndrome, these IF staining patterns correspond broadly with antibodies to the two major antigens: the C-ANCA pattern is associated generally with antibodies to serine protease 3 (PR3) and the P-ANCA pattern with antibodies to myeloperoxidase (MPO). However, some sera positive for ANCA by IF are negative for anti-PR3 and anti-MPO antibodies, suggesting the presence of antibodies to minor antigens of PMN granules. We tested sera from a previously well-defined clinical cohort of patients for antibodies to four possible minor antigens: bactericidal permeability increasing protein, elastase, cathepsin G and lactoferrin. IF-positive (+) sera had significantly higher antibody frequencies to the minor antigens than did the IF-negative (-) sera (P < 0.01). Patients with IF(+) PR3(-)MPO(-) sera showed the most varied reactivity to the minor antigens. Among the IF(+) groups, the IF(+) PR3(+)/MPO(-) sera showed the lowest reactivity to the minor antigens. Patients with well-defined ANCA specificities, e.g. the PR3-ANCA response associated with Wegener's granulomatosis, are less likely than are other patient subsets to have antibodies to minor antigen targets. Autoantibodies to these minor antigens contribute to the overall pattern of ANCA identified by IF and help to explain why the correlation between IF and enzyme immunoassays show discrepancies. While the pathophysiological significance of antibodies to minor target antigens needs further evaluation, they may be markers of inflammation associated with disease processes. PMID:17614969

  17. Localization of radiolabeled anti-DNA monoclonal antibodies in murine systemic lupus erythematosus (SLE)

    SciTech Connect

    Wahl, R.; Hahn, B.; Ebling, F.

    1984-01-01

    The diagnosis of SLE can be extremely difficult. This multi-system disease is characterized by the deposition of DNA-anti-DNA antibody (Ab) complexes in many tissues, producing glomerulonephritis and systemic vasculitis. This study evaluates an IGG monoclonal (Mo) Ab directe3d against DNA (MrSSl) for potential radioimmunodiagnosis of SLE. Six 15 wk. old F-1 female hybrids of NZB+NZW mice (an animal SLE model that develops vasculitis and nephritis) were injected with 50 ..mu..Cl of I-131 MrSSl and 15 ..mu..Cl of I-125 isotype-matched control mouse myeloma (LPC-1) (non-reactive with DNA). Imaging and tissue distribution were studied. Two animals were also imaged using I-131 LPC Ab. Images at 2 and 9 days showed no clear differences in scan patterns using MrSSl or LPC-1 Ab. Tissue distribution studies at six days, however, showed a significantly higher accumulation of MrSSl in the kidneys vs. control Ab (2.7% vs. 1.8% of injected dose) (p < .04). Similarly, higher levels of MrSS were also seen in the spleen, liver and lungs (p < .03). Blood levels tended to be higher with the specific antibody as well. These differences were not apparent at 3 days post injection. The increased concentration of MrSSl present at 9 days in several organs may be secondary to MrSSl binding to DNA containing immune complexes present in diseased tissues. Blocked clearance by immune complexes or DNA, or differences in electrical charges of the antibodies could be contributing to the higher MrSSl levels seen. Images did not suggest deiodination as responsible. Further studies are necessary to determine if the amount of MrSSl retained by diseased animals is indicative of SLE disease activity.

  18. CRDC: a Chinese rheumatology research platform.

    PubMed

    Li, Mengtao; Tian, Xinping; Zhang, Wen; Leng, Xiaomei; Zeng, Xiaofeng

    2015-08-01

    This review introduces the history of development, organizational structure, funding resources, data collection, and quality control of the Chinese Rheumatism Data Center (CRDC) and summarizes the collection of data. In 2009, Peking Union Medical College Hospital (PUMCH), together with several rheumatism centers, established the Chinese Systemic Lupus Erythematosus (SLE) Treatment and Research Group (CSTAR) to collect data on Chinese patients for the study of SLE disease characteristics. In 2011, CSTAR was extended with the formation of the CRDC at PUMCH with direction from the National Health and Family Planning Commission of the PRC. The CRDC currently includes 300 registration sites and 50 regional sites that have successively begun to collect data on 12 rheumatic diseases, including systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), ankylosing spondylitis (AS), systemic sclerosis, dermatomyositis, Takayasu arteritis, IgG4-related diseases, ANCA-associated vasculitis, gout, polyarteritis nodosa, unclassified systemic vasculitis, and Behcet disease. To date, 17,224 patients have been enrolled in the CRDC. Based on the SLE patients registered in the CRDC, papers investigating basic demographic characteristics and first symptom in Chinese SLE patients, risk factors of pulmonary hypertension, correlations between autoantibodies and clinical manifestations, and factors related to fetal loss have been published. The CRDC is a national registry that provides real-life data to improve clinical decision-making. At the same time, without additional work for the clinician, the CRDC is a powerful research database. The CRDC database provides sufficient information for Chinese clinical studies on rheumatology. Moreover, a mobile device application ensures convenient and efficient data collection without compromising data quality, thereby providing strong evidence-based data for the diagnosis and treatment of Chinese rheumatic patients. PMID:26160265

  19. Paradoxical Manifestation is Common in HIV-negative Tuberculous Meningitis.

    PubMed

    Tai, Mei-Ling Sharon; Nor, Hazman Mohd; Kadir, Khairul Azmi Abdul; Viswanathan, Shanthi; Rahmat, Kartini; Zain, Norzaini Rose Mohd; Ong, Kuo Ghee; Rafia, Mohd Hanip; Tan, Chong Tin

    2016-01-01

    Paradoxical manifestation is worsening of pre-existing tuberculous lesion or appearance of new lesions in patients whose condition initially improved with antituberculous treatment. Our hypothesis was that paradoxical manifestation in non-HIV tuberculous meningitis (TBM) patients was underestimated and this could contribute to patients' prognosis. This was the first systemic study of paradoxical manifestation in HIV-negative TBM patients.Between 2009 and 2014, TBM patients were studied prospectively in 2 hospitals. Clinical features, cerebrospinal fluid, and radiological findings were monitored. Paradoxical manifestation was divided into definite (4 weeks or more) and probable (between 14 and 27 d) after commencement of antituberculous treatment.Forty-one non-HIV TBM patients were recruited. Definite paradoxical manifestation occurred in 23/41 (56%) of the patients. Time to onset of paradoxical manifestation was between 28 days and 9 months, and majority was between 28 and 50 days.Neuroimaging manifestation in the brain (22/41 patients, 54%) and clinical manifestation (22/41 patients, 54%) were most commonly seen, followed by cerebrospinal fluid manifestation (7/41 patients, 17%). Neuroimaging changes most commonly seen were worsening of leptomeningeal enhancement, new infarcts, new tuberculomas, and enlargement of tuberculoma. Initial Computed Tomography Angiography/magnetic resonance angiography brain showed vasculitis in 14 patients, with 2 (12.5%) showing paradoxical vasculitis during follow-up.Recurrence of the paradoxical manifestation was seen in 7/23 (30%) of the patients. More than half (14/23, 61%) of the patients improved, 6 (26%) patients died, and 3 (13%) patients had persistent neurological deficit.Paradoxical manifestation was very common in non-HIV TBM patients. Neuroimaging paradoxical manifestation of 2-4 weeks may not be paradoxical manifestation but could be delayed treatment response. PMID:26735523

  20. [Selective immunoadsorption in neurologic complications of systemic lupus erythematosus].

    PubMed

    Harscher, S; Rummler, S; Oelzner, P; Mentzel, H-J; Brodhun, M; Witte, O W; Terborg, C; Isenmann, S

    2007-04-01

    Vasculitis of the nervous system is a rare cause of multifocal neurologic symptoms and may involve both the central and peripheral nervous systems. Typical symptoms include headache, encephalopathy with cognitive impairment and psychotic symptoms, epileptic seizures, and peripheral neuropathies. Here we report the case of a 71-year-old female presenting with Raynaud's syndrome and paresthesia of the feet. Several weeks later she was admitted to our hospital with a status epilepticus and complex partial seizures. On admission she had mild aphasia, distal paresis of the arms without sensory deficits, and disorientation with hallucinations. Cerebral MRI revealed small, multifocal infarctions in several arterial territories. Multiple cerebral artery stenoses were detected by ultrasound. Examination of the CSF was unremarkable. Serologic tests for autoimmune disorders detected Ro antibodies compatible with systemic lupus erythematosus or Sjögren's syndrome. A sural nerve biopsy revealed ischemic axonal neuropathy. During administration of i.v. methylprednisolone, the symptom progression stopped but dosages could not be tapered due to severe CNS symptoms (mental decline, disorientation, aphasia, hallucinations). Slow but sustained clinical improvement was achieved by immunoadsorption over 3 weeks followed by a combined high-dose immunosuppressive treatment with cyclophosphamide and prednisolone that paralleled a reduction in anti-Ro titers and normalization of cerebral blood flow velocities as detected by repeated transcranial Doppler sonography. Systemic vasculitis may present with multiple neurologic and psychiatric symptoms due to involvement of the central and peripheral nervous systems. After excluding systemic infection, immunosuppressive therapy should be started early. In our case a combination of high-dose methylprednisolone, immunoadsorption with elimination of Ro antibodies, and cyclophosphamide led to the patient's recovery. PMID:17342456