Sample records for hypocomplementemic urticarial vasculitis

  1. Hypocomplementemic Urticarial Vasculitis Syndrome

    PubMed Central

    Christensen, Jim; McCarty, Morgan

    2012-01-01

    Hypocomplementemic urticarial vasculitis syndrome, as opposed to urticarial vasculitis or urticarial vasculitis syndrome, is a rare disease process where the exact pathophysiology remains unknown. This article discusses the case of a 34-year-old Hispanic man with an ongoing history of chronic urticaria comprising episodes induced by low ambient temperatures, emotional stress, and spontaneous occurrences. This article serves as a consolidated reference for specialists to comprehensively review the plethora of systemic manifestations that may accompany urticarial vasculitis and highlights new systemic complications reported in association with this disease which are also observed in this case. PMID:22328958

  2. Urticarial vasculitis.

    PubMed

    Venzor, Joe; Lee, Wai L; Huston, David P

    2002-10-01

    Urticarial vasculitis is a clinico-pathologic entity typified by recurrent episodes of urticaria that have the histopathologic features of leukocytoclastic vasculitis. The cutaneous features may include painful, burning or pruritic skin lesions, the persistence of individual lesions greater than 24 hours, palpable purpura, pronounced central clearing of lesions, and residual hyperpigmentation following resolution. However, because clinical characteristics of urticarial vasculitis may overlap with those of allergic urticaria, confirmation of the diagnosis requires a lesional skin biopsy. This condition is idiopathic in many patients but can also occur in the context of autoimmune disorders, infections, drug reactions, or as a paraneoplastic syndrome. In idiopathic urticarial vasculitis common laboratory findings are an elevation of erythrocyte sedimentation rate and reduction of serum complement. An association between urticarial vasculitis and systemic lupus erythematosus has been hypothesized as some clinical manifestations of disease overlap and C1q autoantibodies may be present in both diseases. Normo-complementemic patients usually have minimal or no systemic involvement and often have a better prognosis. On-the-other-hand, hypocomplementemic patients have the propensity to have more severe multi-organ involvement. Response to treatment is variable and a wide variety of therapeutic agents may be efficacious. Initial recommendations for treatment of urticarial vasculitis manifest only as non-necrotizing skin lesions include antihistamines, dapsone, colchicine, hydroxychloroquine or indomethacin, but corticosteroids are often required. With necrotizing skin lesions or visceral involvement, corticosteroids are regularly indicated. Cases of severe corticosteroid resistant urticarial vasculitis or where corticosteroid morbidity is evident [table: see text] may require treatment with other immunosuppressive agents such as azathioprine, cyclophosphamide, or cyclosporine. PMID:12221865

  3. Urticarial Vasculitis

    MedlinePLUS

    ... vasculitis that affects the skin, causing wheals or hives and/or red patches due to swelling of ... the disease. Symptoms The most common symptoms are hives that cause itching, pain and a burning feeling. ...

  4. Urticarial vasculitis and urticarial autoinflammatory syndromes.

    PubMed

    Marzano, A V; Tavecchio, S; Venturini, M; Sala, R; Calzavara-Pinton, P; Gattorno, M

    2015-02-01

    Urticaria is a frequent disorder classified as acute and chronic forms, which presents with wheals that can be associated with angioedema. Several entities may manifest with urticarial skin lesions, encompassing a heterogeneous group of conditions that have to be differentiated from ordinary urticaria. This review is focused on two of these urticarial syndromes: urticarial vasculitis (UV), which represents the most important differential diagnosis with common urticaria, and autoinflammatory diseases such as cryopyrin-associated periodic syndromes (CAPS) and Schnitzler's Syndrome, both rare multisystem forms that may masquerade as common urticaria. UV is a small-vessel vasculitis with predominant skin involvement, characterized by wheals persisting for more than 24 hours, burning rather than itching and resolving with hyperpigmentation as well as by other cutaneous manifestations including purpura, papules, vesicles, bullae and necrotic-ulcerative lesions. Histology shows a classic pattern of leukocytoclastic vasculitis, with possible presence of upper dermal edema. CAPS are classified as three distinct entities: familial cold autoinflammatory syndrome, Muckle-Wells Syndrome and chronic infantile neurological cutaneous and articular syndrome, which represent a spectrum of disorders caused by different mutations in a single gene, NLRP3 (NOD-like receptor 3). This gene encodes for cryopyrin, an inflammasome protein that activates interleukin-1?, leading to an overproduction of this pivotal proinflammatory cytokine. Histologically, urticarial lesions are generally characterized by a perivascular neutrophilic infiltrate. Unlike urticaria, neither UV nor urticarial autoinflammatory syndromes do respond to antihistamines: thus, it is important not to misdiagnose such conditions in order to give the patients specific treatments, potentially preventing serious systemic complications. PMID:25586657

  5. Urticarial vasculitis in northern Spain: clinical study of 21 cases.

    PubMed

    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

    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

  6. Evaluation of D-dimer serum levels among patients with chronic urticaria, psoriasis and urticarial vasculitis*

    PubMed Central

    Criado, Paulo Ricardo; Antinori, Lidi Che Leon; Maruta, Celina Wakisaka; dos Reis, Vitor Manoel Silva

    2013-01-01

    BACKGROUND It has been demonstrated that neutrophils, eosinophils and monocytes, under appropriated stimulus, may express tissue factor and therefore, activate the extrinsic pathway of coagulation. We performed a transversal and case-control study of patients with chronic urticaria and patients with psoriasis, in our outpatient clinic to evaluate the production of D-dimer. OBJECTIVE To evaluate D-dimer serum levels in patients with chronic urticaria and its possible correlation with disease activity. PATIENTS AND METHODS The study was conducted from October 2010 until March 2011. We selected 37 consecutive patients from our Allergy Unit and Psoriasis Unit, and divided them into three groups for statistical analysis: (i) 12 patients with active chronic urticaria (CU); (ii) 10 patients with chronic urticaria under remission and (iii) 15 patients with psoriasis (a disease with skin inflammatory infiltrate constituted by neutrophils, lymphocytes and monocytes). Another five patients with urticarial vasculitis were allocated in our study, but not included in statistical analysis. The serum levels of D-dimer were measured by Enzyme Linked Fluorescent Assay (ELFA), and the result units were given in ng/ml FEU. RESULTS Patients with active chronic urticaria had the highest serum levels of D-dimer (p<0.01), when compared to patients with CU under remission and the control group (patients with psoriasis). CONCLUSIONS Patients with active chronic urticaria have higher serum levels of D-dimer, when compared to patients with chronic urticaria under remission and patients with psoriasis. We found elevated serum levels of D-dimer among patients with urticarial vasculitis. PMID:23793207

  7. Cutaneous adverse effects of hormonal adjuvant therapy for breast cancer: a case of localised urticarial vasculitis following anastrozole therapy and a review of the literature.

    PubMed

    Bock, Vanessa L; Friedlander, Michael; Waring, Dale; Kossard, Steven; Wood, Glenda K

    2014-11-01

    Hormonal therapy with either tamoxifen or aromatase inhibitors is commonly used to treat women with breast cancer in both the adjuvant and recurrent disease setting. Cutaneous adverse reactions to these drugs have been rarely reported in the literature. We report an unusual case of urticarial vasculitis following the aromatase inhibitor anastrozole that localised to the unilateral trunk and mastectomy scar, and review the literature on the cutaneous adverse effects of hormonal therapy for breast cancer. PMID:24575835

  8. Vasculitis

    MedlinePLUS

    ... in children than adults. On the other hand, giant cell arteritis occurs only in adults over 50 ... test that often finds large-vessel vasculitis. Detecting giant cell arteritis often involves a biopsy of an ...

  9. Primary cutaneous small vessel vasculitis

    Microsoft Academic Search

    James P. Russell; Roger H. Weenig

    2004-01-01

    Opinion statement  Disorders associated with cutaneous vasculitis include numerous well-described etiologies. Primary cutaneous vasculitis limits\\u000a discussion to primary leukocytoclastic vasculitis, essential mixed cryoglobulinemia, urticarial vasculitis, Henoch-Schönlein\\u000a purpura, and erythema elevatum diutinum. Although the therapeutics for these disorders are based on limited data, we attempt\\u000a to construct a consensus opinion on the management of primary cutaneous vasculitis. Therapy of primary cutaneous vasculitis

  10. Prevalence and Functional Role of Anti-IgE Autoantibodies in Urticarial Syndromes

    Microsoft Academic Search

    Barry L. Gruber; Maria L. Baeza; Mary J. Marchese; Vincent Agnello; Allen P. Kaplan

    1988-01-01

    The prevalence of autoantibodies of immunoglobulin G (IgG) and immunoglobulin M (IgM) classes directed against myeloma immunoglobulin E (IgE) were determined in distinct subsets of urticaria, using an enzyme immunoassay. IgG anti-IgE antibodies were found in five of nine patients (55%) with cold urticaria, four of eight patients (50%) with urticarial vasculitis, and tree of six patients (50%) with chronic

  11. Vasculitis Foundation

    MedlinePLUS

    ... to attend the 2015 International Vasculitis Symposium. The scholarship program is in honor of Mercy LaVilla, a ... the 2015 International Symposium 2015 International Vasculitis Symposium Scholarship Application Education Articles Feeling Alone – The Impact of ...

  12. Pulmonary Vasculitis

    PubMed Central

    Brown, Kevin K.

    2006-01-01

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

  13. PUPPP (Pruritic Urticarial Papules Plaques of Pregnancy)

    MedlinePLUS

    ... numerous red bumps of PUPPP sometimes look like hives and are intensely itchy. Overview Pruritic urticarial papules ... and form raised, red areas that look like hives. Blisters filled with liquid may also be seen. ...

  14. Pustular vasculitis

    PubMed Central

    Selvan, Shilpa; Shakir, Rebecca; Chan, Antoni

    2013-01-01

    We present a rare case of pustular vasculitis secondary to streptococcal infection. The majority of cases described in the literature predominantly involve the dorsal hands, and may subsequently spread to involve the lower limbs. However, our case demonstrates a rare case where primary involvement of the lower limbs was present with secondary involvement at the dorsum of the hands. The patient responded well to intravenous methylprednisolone reflected both clinically and by his acute phase response. PMID:23505282

  15. Pediatric Vasculitis Initiative

    ClinicalTrials.gov

    2014-12-02

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

  16. CNS vasculitis.

    PubMed

    John, Seby; Hajj-Ali, Rula A

    2014-09-01

    Central nervous system vasculitis (CNSV) is a complex disease that poses considerable diagnostic and therapeutic challenges. It is divided into primary angiitis of the CNS (PACNS), or secondary angiitis of the CNS when associated with systemic conditions. Clinical presentation can be extremely variable and there is no classic presentation. In addition, there is no single diagnostic test and the sensitivity/specificity of all currently available tests is suboptimal. As such, an exhaustive approach with thorough historical data, physical examination, and corroborating investigations should be performed to exclude or confirm a diagnosis of CNSV. Infectious, neoplastic, and autoimmune conditions should be carefully evaluated. Knowledge of CNSV mimics is crucial given the therapeutic and prognostic implications. Reversible cerebral vasoconstriction syndrome is now recognized as the most common mimicker of PACNS. Much progress has been made in the understanding of PACNS histopathology, and specific clinical subsets and their response to treatment. Contrary to historical reports, recent PACNS cohorts achieved favorable clinical outcomes when early diagnosis and prompt treatment was initiated. Successful treatment with newer drugs has also been reported in PACNS cases. Newer imaging modalities hold promise for distinguishing inflammatory from noninflammatory vascular lesions. In this review, the authors provide a broad overview with updates on the types of adult CNSV along with its clinical assessment, diagnosis, and treatment. PMID:25369436

  17. Types of Vasculitis

    MedlinePLUS

    ... to stop using tobacco of any kind. Central Nervous System Vasculitis Central nervous system (CNS) vasculitis usually occurs as a result of ... condition is called isolated vasculitis of the central nervous system or primary angiitis (an-jee-I-tis) of ...

  18. Managing mesenteric vasculitis.

    PubMed

    Angle, John Fritz; Nida, Berhanemeskel A; Matsumoto, Alan H

    2015-03-01

    Mesenteric vasculitis is a rare diagnosis, but it comprises a group of disorders that may have devastating manifestations. It is often difficult to diagnose using clinical symptoms and biomarkers. Vascular imaging often provides the best opportunity for the noninvasive diagnosis of vasculitis and obviates the need for performing a biopsy. The medical management of vasculitis involves controlling the inflammatory process with the use of steroids or other immunosuppressants, but medical therapy does not consistently provide regression of the vascular changes (ie, aneurysms or vascular occlusions) seen at the time of the initial diagnosis. Operative management remains the mainstay of therapy for focal occlusive or aneurysms, but the treatment options for multifocal disease remain challenging. Endovascular treatment is increasingly being used as a first line of treatment for symptomatic vasculitis. Interventionalists should be familiar with the indications and outcomes associated with the various therapeutic options for mesenteric vasculitis-associated occlusive disease and aneurysms. PMID:25814202

  19. Cryopyrin-associated periodic syndrome: an autoinflammatory disease manifested as neutrophilic urticarial dermatosis with additional perieccrine involvement.

    PubMed

    Kolivras, Athanassios; Theunis, Anne; Ferster, Aline; Lipsker, Dan; Sass, Ursula; Dussart, Anneliese; André, Josette

    2011-02-01

    A female newborn presented with a congenital urticarial rash that consisted of fluctuating well-demarcated pink or pale reddish macules or slightly raised papules and plaques. In addition, purulent cerebrospinal fluid was present in the absence of evidence of congenital infection. Skin biopsy revealed a sparse infiltrate throughout the entire dermis, including the eccrine adventitia. The infiltrate was composed mostly of neutrophils, but rarely lymphocytes and eosinophils could also be seen. No vasculitis was present. Because of the presenting attributes, a diagnosis of cryopyrin-associated periodic syndrome (CAPS) was considered and the neonatal-onset multisystem inflammatory disorder (NOMID) that represents the most severe expression of the CAPS clinical spectrum was favored. Diagnosis was confirmed by identification of a mutation in the cold-induced autoinflammatory syndrome-1 gene and by an observed response to treatment with the interleukin-1 receptor antagonist anakinra. Both the clinical and histopathological findings of the presented case may represent a distinct entity within the spectrum of aseptic neutrophilic dermatitis. We refer to this spectrum as neutrophilic urticarial dermatosis (NUD), which may serve as a cutaneous marker of autoinflammation. NUD with perieccrine involvement should prompt consideration of CAPS, especially NOMID, in the context of neonatal multisystem disease. PMID:21062341

  20. How Is Vasculitis Treated?

    MedlinePLUS

    ... into a vein. Certain types of vasculitis may require surgery to remove aneurysms that have formed as a ... Trials Clinical trials are research studies that explore whether a medical strategy, treatment, ...

  1. Central Nervous System Vasculitis

    MedlinePLUS

    ... CNS vasculitis is usually treated with steroids. High-dose steroids such as prednisone, in combination with cyclophosphamide (a medication that decreases the immune system’s response to autoimmune diseases), are generally used. In some ...

  2. [Unclear pruritic and urticarial skin inflammation. Bed bug attack].

    PubMed

    Arayesh, A; Wieczorek, D; Kapp, A; Raap, U

    2013-03-01

    A 27-year-old woman presented with severe pruritus for a few weeks and diffuse urticarial erythema with pinhead-sized papules over the entire body. The skin lesions occurred directly after a stay in her secondary residence in London. The medical history, clinical picture and histological feature of an arthropod reaction in the skin biopsy, coupled with patient offering a bed bug specimen as evidence, secured the diagnosis of a cimicosis. PMID:23223900

  3. CMR in inflammatory vasculitis

    PubMed Central

    2012-01-01

    Vasculitis, the inflammation of blood vessels, can produce devastating complications such as blindness, renal failure, aortic rupture and heart failure through a variety of end-organ effects. Noninvasive imaging with cardiovascular magnetic resonance (CMR) has contributed to improved and earlier diagnosis. CMR may also be used in serial evaluation of such patients as a marker of treatment response and as an indicator of subsequent complications. Unique strengths of CMR favoring its use in such conditions are its abilities to noninvasively visualize both lumen and vessel wall with high resolution. This case-based review focuses on the large- and medium-vessel vasculitides where MR angiography has the greatest utility. Because of increasing recognition of cardiac involvement in small-vessel vasculitides, this review also presents evidence supporting greater consideration of CMR to detect and quantify myocardial microvascular disease. CMR’s complementary role amidst traditional clinical, serological and other diagnostic techniques in personalized care for patients with vasculitis is emphasized. Specifically, the CMR laboratory can address questions related to extent and severity of vascular involvement. As ongoing basic and translational studies better elucidate poorly-defined underlying molecular mechanisms, this review concludes with a discussion of potential directions for the development of more targeted imaging approaches. PMID:23199343

  4. Cutaneous vasculitis complicating coeliac disease.

    PubMed Central

    Meyers, S; Dikman, S; Spiera, H; Schultz, N; Janowitz, H D

    1981-01-01

    A 38 year old female, with chronic uncontrolled coeliac disease, presented with the rare complication of cutaneous leucocytoclastic vasculitis. Detailed study failed to identify any cause for the vasculitis, other than the underlying coeliac disease. Haematuria and proteinuria with mesangial nephritis were also demonstrated on renal biopsy with electron microscopic study. It is speculated that exogenous or endogenous antigens permeated the abnormal small bowel mucosa leading to formation of circulating immune complexes. Subsequent tissue deposition of these complexes then resulted in vasculitis and nephritis. The skin lesions cleared completely after treatment with a strict gluten free diet. Images Fig. 1 Fig. 2 PMID:7461477

  5. Animal models of systemic vasculitis.

    PubMed

    Mathieson, P W; Qasim, F J; Esnault, V L; Oliveira, D B

    1993-04-01

    Necrotizing leucocytoclastic vasculitis is the histopathological hallmark of the small vessel systemic vasculitides (SV), a group of human diseases commonly associated with anti-neutrophil cytoplasm autoantibodies (ANCA). Necrotizing vasculitis is seen in a number of experimental systems, but none of these provide an ideal animal model for human SV. Vasculitis occurs in serum sickness reactions; in murine models of systemic lupus erythematosus; in association with infection, particularly chronic viral infections; and after treatment with certain drugs or inflammatory mediators. 'Spontaneous' vasculitis has been reported in specific mouse strains, especially with ageing, and in some larger species. The size of vessel involved and the type of inflammatory cells predominating are variable in these experimental situations, and none of these models feature antibodies analogous to ANCA. We have recently reported that Brown Norway rats treated with mercuric chloride (HgCl2) develop necrotizing leucocytoclastic vasculitis, especially in the gut, and also develop antibodies to myeloperoxidase (MPO) which recognize similar determinants on MPO to those bound by a subset of ANCA. Transfer of serum from HgCl2-treated rats to naive animals does not induce tissue injury. Preliminary experiments using pooled immunoglobulin or an anti-CD4 monoclonal antibody did not show useful therapeutic benefit from these treatments. HgCl2-induced vasculitis has weaknesses as an animal model of human SV, but is the only experimental model in which anti-MPO autoantibodies have so far been demonstrated, and therefore may be of particular relevance to ANCA-associated SV. PMID:8388693

  6. 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

  7. 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.

  8. The extent and nature of disability in different urticarial conditions.

    PubMed

    Poon, E; Seed, P T; Greaves, M W; Kobza-Black, A

    1999-04-01

    Chronic forms of urticaria are common, often adversely impacting on quality of life. No formal studies have assessed the extent and nature of disability in different types of urticaria. The Dermatology Life Quality Index (DLQI) is a simple and validated 10-item questionnaire designed to measure and compare disability in different skin conditions. In this study, we aimed to assess the disability in different urticarial groups using the DLQI, allowing comparison with previously published DLQI scores in common skin diseases. The DLQI was administered to 170 consecutive patients attending a specialist urticaria clinic over a 4-month period. Consistent with previous studies using the DLQI, mean scores were not influenced by gender or age. Patients with chronic idiopathic urticaria without a concurrent physical urticaria (n = 47) suffered moderate quality of life impairment (mean +/- SD DLQI 25 +/- 24%). In comparison, patients with chronic idiopathic urticaria with concurrent delayed pressure urticaria (DPU) (n = 26) suffered significantly higher quality of life impairment (mean +/- SD DLQI 43 +/- 23%, 95% confidence interval for difference 7-29%). Disability in this group was greatest in the dimensions of work/study, symptoms/feelings and leisure. Subjects with another form of physical urticaria, cholinergic urticaria, also endured high levels of disability (n = 9, mean +/- SD DLQI 50 +/- 34%). From our urticaria study group, we have shown that subjects with DPU and cholinergic urticaria endure the most quality of life impairment. The mean DLQI scores demonstrated in these groups are comparable with those previously seen in severe atopic dermatitis out-patients (60%) and higher than those seen in out-patients with psoriasis (29.7%), acne (24.3%) and vitiligo (16.1%). PMID:10233318

  9. DRESS syndrome with cerebral vasculitis.

    PubMed

    Sola, Daniele; Rossi, Luca; Sainaghi, Pier Paolo; Pirisi, Mario

    2013-01-01

    DRESS (drug rash with eosinophilia and systemic symptoms) syndrome is a severe reaction triggered by drugs that manifests as pyrexia and eosinophilia with involvement of the skin and internal organs. We herein describe the case of a patient who developed hyperuricemia after receiving treatment for tuberculosis, then took allpurinol and developed DRESS syndrome with a contextual coma and right hemisyndrome. This report describes for the first time the presence of vasculitic cerebral involvement in a patient with DRESS syndrome. The cerebral vasculitis responded to treatment, showing clinical and instrumental remission. In cases such as this, allergic cerebral vasculitis should be considered in the differential diagnosis because it can be treated if recognized early, thus leading to remission without the development of any sequelae. PMID:23774556

  10. Silica exposure and systemic vasculitis.

    PubMed

    Mulloy, Karen B

    2003-12-01

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

  11. Silica exposure and systemic vasculitis.

    PubMed Central

    Mulloy, Karen B

    2003-01-01

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

  12. New insights on biomarkers in systemic vasculitis.

    PubMed

    Perez-Alamino, Rodolfo; Maldonado-Ficco, Hernán

    2015-03-01

    The systemic vasculitis is a heterogeneous group of diseases characterized by the inflammation of blood vessels. The development of advanced diagnostic tests and genetic studies have resulted in greater improvement in our understanding of vasculitis pathogenesis and thus in the development of newer therapies. However, there is still an unmet need in the management of systemic vasculitis, focused on developing of new biomarkers that would enable distinction between active disease from damage or infection and predict treatment response and prognosis. PMID:25740705

  13. Pregnancy Outcomes Among Patients With Vasculitis

    PubMed Central

    Clowse, Megan E. B.; Richeson, Rachel L.; Pieper, Carl; Merkel, Peter A.

    2015-01-01

    Objective Pregnancy outcomes of patients with vasculitis are unknown, but are of great concern to patients and physicians. Through an online survey, this study assessed pregnancy outcomes among patients with vasculitis. Methods Participants in the Vasculitis Clinical Research Consortium Patient Contact Registry were invited to respond to an anonymous, internet-based survey that included questions about pregnancy outcomes, the timing of pregnancy relative to a diagnosis of vasculitis, and medication use. Results A total of 350 women and 113 men completed the survey. After a diagnosis of vasculitis, 74 pregnancies were reported by women and 18 conceptions were reported by men. The rate of pregnancy loss was higher among women who conceived after a diagnosis of vasculitis compared to those who conceived prior to diagnosis (33.8% versus 22.4%; P = 0.04). Among women, the rate of preterm births increased significantly for pregnancies conceived after a diagnosis of vasculitis relative to those conceived before diagnosis (23.3% versus 11.4%; P = 0.03). Only 18% of women reported worsening of vasculitis during pregnancy, but those who experienced increased vasculitis activity were more likely to deliver preterm. Exposure to cyclophosphamide or prednisone did not appear to impact pregnancy outcomes; however, the number of pregnancies among women taking these medications was small. Among the pregnancies conceived by men with vasculitis, the timing of diagnosis had no significant effect on the rate of pregnancy loss. Conclusion Women who conceived after a diagnosis of vasculitis had a higher rate of pregnancy loss than those who conceived prior to diagnosis. Vasculitis did not worsen during the majority of pregnancies conceived after diagnosis. PMID:23401494

  14. Vasculitis Syndromes of the Central and Peripheral Nervous Systems

    MedlinePLUS

    NINDS Vasculitis Syndromes of the Central and Peripheral Nervous Systems Information Page Synonym(s): Temporal Arteritis, Cranial Arteritis, Giant ... is Vasculitis Syndromes of the Central and Peripheral Nervous Systems ? Vasculitis is an inflammation of blood vessels, which ...

  15. 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

  16. Acute lymphoblastic leukemia presenting with urticarial plaques and hypereosinophilia in a child.

    PubMed

    Chien, Andy J; Argenyi, Zsolt B; Colven, Roy M; Kirby, Philip

    2004-11-01

    Our report describes a previously healthy 10-year-old female who was seen for urticarial plaques and mild loss of appetite. An initial laboratory workup revealed an elevated leukocyte count of 30,000/microL and a peripheral eosinophil count of 22,500/microL. A skin biopsy showed a marked hypersensitivity tissue response with abundant eosinophils. Further investigation of her peripheral eosinophilia uncovered Giardia lamblia in a stool sample. Despite treatment with the anti-parasitic agent furazolidone, the patient's urticarial plaques, leukocyte count, and peripheral eosinophil count remained unchanged. A bone marrow biopsy confirmed a diagnosis of acute lymphoblastic leukemia (ALL). ALL with hypereosinophilia (ALL/Eo) represents a rare and distinct subset of ALL, with more than 30 cases documented in the literature. Our discussion summarizes the clinical aspects of this disease and reviews the reported dermatological manifestations of ALL/Eo. PMID:15577757

  17. Intraoperative Localized Urticarial Reaction During Q-switched Nd:YAG Laser Tattoo Removal.

    PubMed

    Wilken, Reason; Ho, Derek; Petukhova, Tatyana; Jagdeo, Jared

    2015-03-01

    Q-switched lasers, such as the neodymium:yttrium-aluminum-garnet (Nd:YAG) laser, are the gold standard for tattoo removal. Allergy to tattoo pigment is well-documented, but adverse allergic reactions during or shortly after laser tattoo removal are rare with few reports in the medical literature. Here we describe an intraoperative, localized urticarial reaction that developed during treatment of a tattoo using a 1064-nm Nd:YAG laser. As laser tattoo removal becomes increasingly popular amongst our patients, it is important for dermatologists to be aware of urticarial allergic reactions as well as their management. We outline our recommendations for medical management of this condition and hope that these guidelines will facilitate patient care by dermatologists who encounter this immune skin reaction to laser tattoo removal.

    J Drugs Dermatol. 2015;14(3):303-304. PMID:25738853

  18. [Vasculitis and hereditary small vessel diseases].

    PubMed

    Opherk, C; Peters, N; Dichgans, M

    2009-11-01

    In younger patients with stroke, cerebral vasculitis and hereditary small vessel diseases should be considered as important differential diagnoses. Since the clinical course of cerebral vasculitis is highly variable, diagnostic workup, which includes laboratory tests, CSF analysis, cranial magnetic resonance imaging and biopsy, is often challenging. Therapy should be initiated on an interdisciplinary basis and includes immunosuppressive induction and maintenance regimes. Hereditary small vessel diseases, e.g. CADASIL or Fabry's disease, can mimic clinical features of cerebral vasculitis. Their diagnosis which is based on family history, typical clinical features and genetic analysis often has implications for treatment and genetic counselling. PMID:19838657

  19. 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

  20. Neutrophilic dermatosis of the dorsal hands: Pustular vasculitis revisited

    Microsoft Academic Search

    Noreen A. Galaria; Jacqueline M. Junkins-Hopkins; Douglas Kligman; William D. James

    2000-01-01

    An entity termed “pustular vasculitis of the hands” was recently described. Patients with this condition presented with low-grade fevers and erythematous plaques, pustules, and bullae limited to the dorsal hands and fingers, which were characterized histologically by a dense neutrophilic infiltrate and leukocytoclastic vasculitis. We describe patients with a similar clinical presentation, but who lacked vasculitis on biopsy findings. We

  1. Pathogenesis of ANCA-Associated Vasculitis

    PubMed Central

    Cartin-Ceba, Rodrigo; Peikert, Tobias; Specks, Ulrich

    2012-01-01

    Antineutrophil cytoplasmic autoantibodies (ANCA) associated vasculitis (AAV) are a group of systemic vasculitis characterized by inflammation and necrosis of blood vessel walls. Genetic, epigenetic and environmental factors contribute to the etiology and pathogenesis of AAV. Based on currently available clinical and experimental evidence, it is reasonable to conceptualize that in predisposed patients, different triggers can lead to the production of autoantibodies (ANCA) that in the context of an inflammatory environment can cause tissue inflammation and vascular injury. Several different pathways and mechanisms in the pathogenesis of AAV are described in this contemporary review. PMID:20688244

  2. Enhancement of mite antigen-induced histamine release by deuterium oxide from leucocytes of chronic urticarial patients

    SciTech Connect

    Numata, T.; Yamamoto, S.; Yamura, T.

    1981-09-01

    The mite antigen-induced histamine release from leucocytes of chronic urticarial patients was enhanced in the presence of deuterium oxide, which stabilizes microtubules. This enhancing effect of deuterium oxide on the histamine release from leucocytes may provide a useful means for the detection of allergens in vitro in chronic urticaria.

  3. Red blood cell distribution width as a useful indicator to predict systemic vasculitis in patients with cutaneous vasculitis.

    PubMed

    Kim, Dae Suk; Shin, Dongyun; Kim, Tae-Gyun; Kim, Sung Hee; Kim, Do Young; Kim, Soo Min; Lee, Min-Geol

    2015-04-01

    Cutaneous vasculitis can be limited to skin or a manifestation of primary systemic vasculitis. However, there are no definite markers to predict systemic involvements. Recent studies have shown that a higher red blood cell distribution width (RDW) is associated with disease activity in various disorders. We evaluated whether RDW can be used as an indicator for predicting systemic disease in patients with initial cutaneous involvements. We reviewed clinical and laboratory information of 143 patients with cutaneous vasculitis and 15 pigmented purpuric dermatosis patients seen at single academic hospital in Korea. Various parameters, including RDW, were evaluated in patients with primary cutaneous vasculitis and primary systemic vasculitis with initial cutaneous manifestations. The RDW value between cutaneous and systemic vasculitis patients was compared and RDW level was also investigated whether it can indicate systemic vasculitis in patients with cutaneous involvements. The mean age was 32.0 years, and 102 (64.6 %) patients were female. A total of 132 patients were patients with primary cutaneous vasculitis, and 11 were primary systemic vasculitis. Higher ratio of patients with high RDW was detected in systemic vasculitis group compared with cutaneous vasculitis group (36.4 vs. 7.6 %, P < 0.05). The mean RDW was significantly higher in systemic vasculitis patients (P < 0.05). RDW had the strongest association with systemic vasculitis (P < 0.05, OR 1.834). In conclusion, elevated level of RDW was significantly associated with systemic vasculitis. RDW can be used as one of the marker to predict systemic disease in patients with cutaneous vasculitis. PMID:25284376

  4. Cryoglobulinemia and cutaneous vasculitis in human brucellosis

    Microsoft Academic Search

    Juan L. Yrivarren; Luis R. Lopez

    1987-01-01

    We report the occurrence of cryoglobulinemia and cutaneous vasculitis in three patients with brucellosis caused byBrucella melitensis. The isolated cryoglobulins were characterized as mixed polyclonal or type III. Brucella agglutinin activity was not detected in any of the cryoglobulins analyzed. However, the same agglutinin titer and the presence of precipitin lines of identity in immuno-diffusion gels were observed in the

  5. Isolated vasculitis of the peripheral nervous system.

    PubMed

    Collins, M P; Periquet, M I

    2008-01-01

    Vasculitis restricted to the peripheral nervous system (PNS), referred to as nonsystemic vasculitic neuropathy (NSVN), has been described in many reports since 1985 but remains a poorly understood and perhaps under-recognized condition. There are no uniform diagnostic criteria. Classification is complicated by the occurrence of vasculitic neuropathies in many systemic vasculitides affecting small-to-medium-sized vessels and such clinical variants as nonsystemic skin/nerve vasculitis and diabetic/non-diabetic lumbosacral radiculoplexus neuropathy. Most patients present with painful, stepwise progressive, distal-predominant, asymmetric or multifocal, sensory-motor deficits evolving over months-to-years. NSVN is identical to but less severe than systemic vasculitis-associated neuropathies (SVNs). All vasculitic neuropathies are axonal by electrodiagnostic/pathologic criteria. Laboratory testing is unremarkable except for mildly elevated erythrocyte sedimentation rate (ESR) in 50%. Highly elevated ESRs, leukocytosis, rheumatoid factors, and anti-neutrophil cytoplasmic antibodies (ANCAs) raise concern for underlying systemic vasculitis. Without a specific clinical/laboratory marker, the condition depends on nerve biopsy for diagnosis. Biopsies showing necrotizing vasculitis are about 50% sensitive, mandating reliance on "suspicious" changes in many patients. Vasculitic lesions predominate in smaller epineurial vessels and are milder than those in SVNs. The disorder is often accompanied by subclinical involvement of adjacent muscles and skin. NSVN has the potential to spontaneously relapse and remit but neurologic deficits accumulate. No randomized controlled trials have been performed, but one retrospective cohort survey showed combination therapy to be more effective than prednisone alone. Although most patients have a good outcome, more than 30% relapse and 60% have residual pain. Many nosologic, pathogenic, diagnostic, and therapeutic questions remain unanswered. PMID:18799069

  6. Vasculitis in childhood – a dermatological approach.

    PubMed

    Sandrine, Benoit; Goebeler, Matthias

    2014-02-01

    Vasculitis, an inflammatory condition affecting the blood vessels, may be restricted to a single organ or involve several organ systems. The size of the involved vessels is an important criterion for categorization of vasculitides, which is a prerequisite for rapid diagnosis and initiation of treatment. In pediatric patients, this particularly applies to Kawasaki disease. However, making the diagnosis can be challenging for dermatologists as skin involvement may be variable and non-specific. In contrast, Henoch-Schönlein purpura (IgA vasculitis) presents with the classic picture of palpable purpura. It predominantly affects postcapillary venules frequently following upper respiratory tract infections. Severe organ involvement is relatively rare in children and the prognosis is good. As renal involvement may occur during the course of disease, continuous monitoring is required. Acute hemorrhagic edema of infancy is considered as a distinct type of immune complex vasculitis and is characterized by a triad of fever, edema and rosette-shaped purpura. The clinical course of this rare disease is usually benign and self-limited. Due to the variability of clinical symptoms and manifestations, management of childhood vasculitides represents a special challenge requiring interdisciplinary collaboration. Dermatologists should be aware of their important role especially for making an early diagnosis. PMID:24494640

  7. Vasculitis Syndromes of the Central and Peripheral Nervous Systems

    MedlinePLUS

    ... or PNS vasculitis will gather a comprehensive medical history of the individual, perform a physical examination, order laboratory tests ( ... Johns Hopkins Vasculitis Center, and Mayo Clinic College of Medicine. The Consortium’s internet site provides information about clinical research and clinical ...

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

    PubMed Central

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

    2013-01-01

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

  9. Limited Cutaneous Vasculitis Associated With Levamisole-Adulterated Cocaine

    PubMed Central

    Yachoui, Ralph; Kolasinski, Sharon L; Eid, Hala

    2012-01-01

    Levamisole is among the many contaminants that have been detected in seized cocaine throughout North America and Europe. Little is known about the association between levamisole-adulterated cocaine and vasculitis. Herein we describe a case of limited cutaneous vasculitis manifested as retiform purpura and skin necrosis in a user of cocaine contaminated with levamisole. PMID:23024742

  10. Limited cutaneous vasculitis associated with levamisole-adulterated cocaine.

    PubMed

    Yachoui, Ralph; Kolasinski, Sharon L; Eid, Hala

    2012-10-01

    Levamisole is among the many contaminants that have been detected in seized cocaine throughout North America and Europe. Little is known about the association between levamisole-adulterated cocaine and vasculitis. Herein we describe a case of limited cutaneous vasculitis manifested as retiform purpura and skin necrosis in a user of cocaine contaminated with levamisole. PMID:23024742

  11. Treatment of cystic fibrosis associated cutaneous vasculitis with chloroquine

    Microsoft Academic Search

    Ian D. Molyneux; Tanya Moon; A. Kevin Webb; Alyn H. Morice

    2010-01-01

    Vasculitis is a well recognised complication of Cystic Fibrosis. Corticosteroids are the mainstay of treatment but some cases can be resistant and may require additional disease modifying agents.We describe a case of steroid resistant cutaneous vasculitis which was successfully treated with chloroquine in addition to corticosteroids and a subsequent relapse with chloroquine alone.

  12. Renal paraneoplastic vasculitis complicating lung adenocarcinoma.

    PubMed

    Dhaou, Besma Ben; Boussema, Fatma; Aydi, Zohra; Ketari, Sonia; Baili, Lilia; Moussa, Fatma Ben; Rokbani, Lilia

    2014-09-01

    Renal paraneoplastic vasculitis (RNPV) is rare. It can be revealed by glomerulonephritis, microaneurysms or renal failure. RPNV may precede the onset of the primary tumor, and treatment and prognosis depend on the etiology (primary tumor). A 54-year-old man who had a primary lung adenocarcinoma was admitted for nephrotic syndrome. The investigations revealed RNPV. The patient was treated with corticosteroids at high dose and cyclophosphamide with improvement of the renal condition; however, the patient died from worsening of his pulmonary neoplasia. PMID:25193910

  13. Pathogenesis of ANCA-associated vasculitis.

    PubMed

    Cartin-Ceba, Rodrigo; Peikert, Tobias; Specks, Ulrich

    2012-12-01

    Antineutrophil cytoplasmic autoantibodies (ANCA)-associated vasculitides (AAV) are a group of systemic vasculitis syndromes characterized by inflammation and necrosis of blood vessel walls. Genetic, epigenetic, and environmental factors contribute to the etiology and pathogenesis of AAV. On the basis of currently available clinical and experimental evidence, it is reasonable to believe that, in predisposed patients, different triggers can lead to the production of autoantibodies (ANCA) that, in the context of an inflammatory environment, can cause tissue inflammation and vascular injury. Several different pathways and mechanisms in the pathogenesis of AAV are described in this contemporary review. PMID:22927039

  14. [Necrotizing cutaneous vasculitis with massive gastrointestinal bleeding following naproxen treatment].

    PubMed

    Horev, Amir; Etzion, Ohad; Halevy, Sima

    2010-03-01

    Cutaneous necrotizing vasculitis is usually induced by an acute infection or exposure to a drug. Cutaneous vasculitis may precede severe systemic involvement, and may end in death. Accordingly, diagnosis of cutaneous vasculitis, identification of etiological factors, follow-up for systemic involvement and treatment are important. The authors present a case study of a 58-year-old male with fever and extensive eruption involving the trunk and extremities which appeared two days after initiation of treatment with oral naproxen. The clinical and histological findings were consistent with cutaneous necrotizing leukocytoclastic vasculitis. Two events of massive upper gastrointestinal bleeding occurred during treatment with systemic corticosteroids. The possible etiological factors and the mechanisms involved in the induction of the vasculitis and the gastrointestinal bleeding are discussed. PMID:20684166

  15. Cerebral large vessel vasculitis in systemic lupus erythematosus.

    PubMed

    Böckle, B C; Jara, D; Aichhorn, K; Junker, D; Berger, T; Ratzinger, G; Sepp, N T

    2014-11-01

    Neuropsychiatric systemic lupus erythematosus (NPSLE) is defined by involvement of the central nervous system in systemic lupus erythematosus (SLE), with a wide range of both neurological and psychiatric manifestations. Although its aetiopathogenesis is not fully elucidated, NPSLE seems to be a consequence of cerebral vascular pathology including thromboembolism, small-vessel vasculopathy and, in rare cases, true vasculitis. Cerebral vasculitis is rare, and cerebral large-vessel vasculitis in SLE is even more unusual. We report the case of a female patient with the diagnosis of SLE. She presented with stroke-like symptoms, headache and vertigo, and palpable purpura on her legs. Further investigations revealed that she suffered from both vasculitis of the cerebral large vessels and coexisting cutaneous small-vessel vasculitis. PMID:24969082

  16. pANCA-vasculitis associated with rectal adenocarcinoma.

    PubMed

    Hommel, C; Rihova, Z; Mokaddem, F; Libotte, B

    2014-12-01

    We report the case of a 69-year-old male patient who was admitted for fever, dry cough, recurrent sinusitis with epistaxis, anorexia with weight loss of 20 kg over a 3-month period, myalgia, and mononeuritis multiplex. He was diagnosed with pANCA/anti-MPO associated vasculitis and rectal adenocarcinoma. The tumor was treated by surgical resection. Recurrence of vasculitis occurred during steroid tapering which prompted us to add Mycophenolate mofetyl. A complete remission was achieved. We conclude that in the present case the vasculitis was an independent disease, not a paraneoplastic phenomenon. We discuss the value of different ANCA serologies for diagnostics and follow-up, the epidemiology of vasculitis associated with malignancy, and the concept of vasculitis as a paraneoplastic syndrome. PMID:25113856

  17. Cryoglobulinemic vasculitis in a patient with CREST syndrome.

    PubMed

    Hurst, Rebecca L; Berianu, Florentina; Ginsburg, William W; Klein, Christopher J; Englestad, Janean K; Kennelly, Kathleen D

    2014-10-01

    Cryoglobulinemic vasculitis is a rare entity. Although it has been reported in diffuse systemic sclerosis, it has not been reported in calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia (CREST) syndrome. We report a patient with cryoglobulinemic vasculitis with CREST syndrome who did not have typical clinical features of vasculitis. This 58-year-old woman presented with mild generalized weakness and a diagnosis of CREST syndrome, which included Raynaud's syndrome, dysphagia and telangiectasias. She was positive for serum cryoglobulins, which led to a sural nerve biopsy. The biopsy results were consistent with cryoglobulinemic vasculitis. Cryoglobulinemic vasculitis has not been previously reported in CREST syndrome to our knowledge. Additionally, the patient also had limited clinical symptoms. Our patient displays the importance of checking for cryoglobulins and obtaining a nerve biopsy when the serum is positive. Both of these diagnostic tests were integral for directing appropriate treatment for this patient. PMID:24852904

  18. Diagnosis and treatment of cerebral vasculitis

    PubMed Central

    Berlit, Peter

    2010-01-01

    Vasculitides are characterized by inflammation and necrosis of the blood vessel wall. Large vessels including the aorta are affected in giant-cell arteritis, medium-size arteries in classic polyarteritis nodosa. The small-vessel vasculitides are separated in those with antineutrophil cytoplasm antibodies (ANCA) and those without. The primary angiitis of the central nervous system (PACNS) is a rare disorder affecting both medium- and small-sized vessels. Major symptoms of cerebral vasculitis are stroke, headache and encephalopathy. Diagnosis is based on laboratory and imaging findings. When cerebral affection occurs in systemic vasculitis an acute inflammatory response with raised erythrocyte sedimentation rate and increased values of C-reactive protein is present. In many cerebral vasculitides including PACNS, CSF studies reveal inflammatory findings. Magnetic resonance imaging, including ADC maps, diffusion and gradient echo sequences, is the investigation of choice to detect and monitor cerebral involvement. Certain MRI techniques and 18-fluorodeoxyglucose positron emission tomography allow the visualization of vessel wall inflammation when the lumen is still unaffected on angiography. The treatment recommendations for cerebral angitis are derived from protocols for systemic vasculitides. In general, a combination of steroids and pulse cyclophosphamide (CYC) is recommended for induction treatment. An alternative option is the use of the anti- CD20 antibody rituximab. Methotrexate, azathioprine and mycophenolate mofetil are recommended as alternatives to CYC once remission is achieved. PMID:21180634

  19. Vasculitis and stroke due to Lyme neuroborreliosis - a review.

    PubMed

    Zajkowska, Joanna; Garkowski, Adam; Moniuszko, Anna; Czupryna, Piotr; Ptaszy?ska-Sarosiek, Iwona; Tarasów, Eugeniusz; Ustymowicz, Andrzej; ?ebkowski, Wojciech; Pancewicz, S?awomir

    2015-01-01

    Abstract Lyme neuroborreliosis (LNB) is a rare cause of vasculitis and stroke. It may manifest as subarachnoid hemorrhage, intracerebral hemorrhage, and most often ischemic stroke due to cerebral vasculitis. The vast majority of reported cases have been described by European authors. A high index of suspicion is required in patients who live or have traveled to areas with high prevalence of tick-borne diseases, and in the case of stroke-like symptoms of unknown cause in patients without cardiovascular risk factors. In this review, we also present four illustrative cases of vasculitis and stroke-like manifestations of LNB. PMID:25342573

  20. What Are the Signs and Symptoms of Vasculitis?

    MedlinePLUS

    ... you have. Gastrointestinal Tract If vasculitis affects your gastrointestinal tract, you may get ulcers (sores) in your mouth or have stomach pain. In severe cases, blood flow to the intestines can be blocked. ...

  1. Historical perspective of vasculitis: Polyarteritis nodosa and microscopic polyangiitis

    Microsoft Academic Search

    Eric L. Matteson

    2002-01-01

    The original and early case reports of vasculitis provide a historical context and foundation for understanding current concepts\\u000a of these diseases. These early case reports are valuable as reference points for the current efforts in diagnosing, treating,\\u000a and classifying vasculitis. In addition, they emphasize the importance of careful clinical observation in these efforts and\\u000a the essential nature of medical science.

  2. Update on diffuse alveolar hemorrhage and pulmonary vasculitis.

    PubMed

    Krause, Megan L; Cartin-Ceba, Rodrigo; Specks, Ulrich; Peikert, Tobias

    2012-11-01

    Diffuse alveolar hemorrhage is a clinical syndrome that can be a manifestation of multiple different causes. Identification of the underlying etiology is of utmost importance and dictates treatment. Pulmonary vasculitis including antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a common cause of diffuse alveolar hemorrhage. For AAV, treatment includes induction followed by maintenance therapy. Rituximab has an increasing role in the treatment of AAV. PMID:23102067

  3. Testicular Vasculitis – Literature Review and Case Report in Queensland

    PubMed Central

    Lintern, Narelle; Johnson, Nigel R.; Mckenzie, Ian; Martin, Ben

    2013-01-01

    A 21-year-old male presented with acute onset, sharp right sided testicular pain. The testicle was removed with a histological diagnosis of testicular vasculitis. Anti-neutrophil cytoplasmic antibodies were negative. Although rare, males who present with acute onset pain should be screened for testicular vasculitis with a scrotal ultrasound and blood investigations including tumor markers and anti-neutrophil cytoplasmic antibodies. PMID:24917768

  4. Preliminary classification criteria for the cryoglobulinaemic vasculitis

    PubMed Central

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

    2011-01-01

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

  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. Isolated lower limb medium-vessel vasculitis: a new entity?

    PubMed Central

    Gore, David; Bukhari, Marwan

    2011-01-01

    A 47-year-old female was referred with bilateral ischaemic lesions affecting her toes with associated gangrene. Systemic examination was normal and her medical history was unremarkable. Close examination of the hands revealed splinter haemorrhages. Her feet were dusky in colour and the toes were dark and weeping, each becoming gangrenous, with erythema around the metatarsal phalangeal joint. Dorsalis pedis and posterior tibial pulses were intact with strong Doppler signals. Investigations for surgical pathology and malignancy were all negative as were routine blood tests and tests for autoimmune and viral markers. Vasculitis was diagnosed when her toes showed improvement with steroids, however, attempts to reduce the steroid dose were unsuccessful and azathioprine was introduced. Despite immunosuppression and immunomodulators, her toes deteriorated and were amputated. Histology revealed findings consistent with a medium-vessel vasculitis which antineutrophil cytoplasmic antibody was negative. A similar vasculitis has not been formally reported in the literature. PMID:22691585

  7. 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.

  8. Secondary Syphilis with Nodular Vasculitis Mimicking Behçet's Disease

    PubMed Central

    Jo, Jaemin; Kim, Jae Wang; Kim, Jinseok; Yu, Jung Re

    2013-01-01

    Although, erythema nodosum is a common skin manifestation associated with syphilis, nodular vasculitis is a rare feature. Here, we describe a case of a 22-year-old, human immunedeficiency virus negative, non-immunocompromised man who developed recurrent oral and scrotal ulcers with nodular lesions of the lower extremitie. Behçet's disease was initially suspected, however, his serologic test for syphilis was positive, and he was thus diagnosed with secondary syphilis, with a skin biopsy showing nodular vasculitis. The patient was treated with benzathine penicillin, and the skin lesions disappeared after treatment. PMID:24475361

  9. [Relation between vasculitis and hepatitis HBsAg].

    PubMed

    Parcero, S A; Orfus, S; Malamud, S W; Huberman, E D

    1978-01-01

    The authors present the development of a generalized necrotic vasculitis accompanied by positive Virus B Cronic Hepatitis. A 31 years old female, affected by a multisystemic disease with jaundice, dark urine, and light stools, with biologic values of necrosis of hepatic cells and positive dosages of HBsAg. The biopsy of muscle revealed the presence of an alergic vasculitis with leucocitoclasia and the biopsy of the liver showed and Active Cronic Hepatitis. References are made about the different pathogenical hypothesis of these unusual association. PMID:747085

  10. Leukocytoclastic Vasculitis Associated with Influenza A Virus Infection

    PubMed Central

    Lee, Hyo Jin; Choi, Jong Soo; Kim, Ki Hong

    2012-01-01

    Leukocytoclastic vasculitis (LCV) usually presents palpable purpura characterized by inflammation of vessel walls and fragmentation of nuclei. Various conditions can cause LCV, and it can be induced by influenza A virus infection. We report a 2-yr-old Korean girl who presented palpable purpuric and hemorrhagic lesions with fever. She was diagnosed as LCV by skin biopsy, and influenza A virus was isolated from nasopharyngeal swab. She was treated with oseltamivir (Tamiflu®) and prednisolone with dramatic effect of vasculitis and fever. PMID:23255867

  11. Leukocytoclastic vasculitis and acute allergic interstitial nephritis following ceftriaxone exposure.

    PubMed

    Agrawal, Sachin R; Rajput, Atul; Jain, A P

    2014-10-01

    Leukocytoclastic vasculitis (LCV), also known as hypersensitivity vasculitis is a small vessel inflammatory disease which mainly involves the postcapillary venules. A 17-year-old girl developed palpable purpura over lower limbs and acute allergic interstitial nephritis 5 days after exposure to ceftriaxone. Skin biopsy from the lesion over lower limbs confirmed the diagnosis of LCV. Discontinuation of drug and treatment with steroid resulted in resolution of skin lesions and normalization of kidney functions. Beta-lactams are commonly used antibiotics in various types of infection in day-to-day practice. LCV, a rare complication of ceftriaxone should be kept in mind while using this drug. PMID:25422573

  12. [Necrotizing glomerulonephritis in decursu vasculitis after vaccination against influenza].

    PubMed

    Hyla-Klekot, Lidia; Kucharska, Grazyna; Cieslak, Witold

    2005-07-01

    Vaccinations against influenza are common, acceptable way of prevention of influenza, which develops as epidemic on huge areas of Europe. We described 17 years old girl, who has developed vasculitis after vaccination against influenza vaccine Vaxigrip produced by Aventis Pasteur. Aggressive immunosuppressive treatments caused regression majority of clinical symptoms and normalization of renal function tests. PMID:16194032

  13. What’s new in the aetiopathogenesis of vasculitis?

    Microsoft Academic Search

    Paul A. Brogan

    2007-01-01

    The cause of the majority of childhood vasculitides is unknown although it is likely that a complex interaction between environmental\\u000a factors and inherited host responses trigger the disease and determine the vasculitis phenotype. Epidemiological clues continue\\u000a to implicate infectious triggers in Kawasaki syndrome (KS) and Henoch Sch?nlein purpura (HSP). Several genetic polymorphisms\\u000a have now been described in KS and HSP

  14. Central Nervous System Vasculitis: Still More Questions than Answers

    PubMed Central

    Alba, Marco A; Espígol-Frigolé, Georgina; Prieto-González, Sergio; Tavera-Bahillo, Itziar; García-Martínez, Ana; Butjosa, Montserrat; Hernández-Rodríguez, José; Cid, Maria C

    2011-01-01

    The central nervous system (CNS) may be involved by a variety of inflammatory diseases of blood vessels. These include primary angiitis of the central nervous system (PACNS), a rare disorder specifically targeting the CNS vasculature, and the systemic vasculitides which may affect the CNS among other organs and systems. Both situations are severe and convey a guarded prognosis. PACNS usually presents with headache and cognitive impairment. Focal symptoms are infrequent at disease onset but are common in more advanced stages. The diagnosis of PACNS is difficult because, although magnetic resonance imaging is almost invariably abnormal, findings are non specific. Angiography has limited sensitivity and specificity. Brain and leptomeningeal biopsy may provide a definitive diagnosis when disclosing blood vessel inflammation and are also useful to exclude other conditions presenting with similar findings. However, since lesions are segmental, a normal biopsy does not completely exclude PACNS. Secondary CNS involvement by systemic vasculitis occurs in less than one fifth of patients but may be devastating. A prompt recognition and aggressive treatment is crucial to avoid permanent damage and dysfunction. Glucocorticoids and cyclophosphamide are recommended for patients with PACNS and for patients with secondary CNS involvement by small-medium-sized systemic vasculitis. CNS involvement in large-vessel vasculitis is usually managed with high-dose glucocorticoids (giant-cell arteritis) or glucocorticoids and immunosuppressive agents (Takayasu’s disease). However, in large vessel vasculitis, where CNS symptoms are usually due to involvement of extracranial arteries (Takayasu’s disease) or proximal portions of intracranial arteries (giant-cell arteritis), revascularization procedures may also have an important role. PMID:22379458

  15. Mixed cryoglobulinemia as a model of systemic vasculitis

    Microsoft Academic Search

    Franco Dammacco; Domenico Sansonno

    1997-01-01

    Summary  Leukocytoclastic vasculitis is the dominant lesion of mixed cryoglobulinemia (MC). The high prevalence of antibodies to hepatitis\\u000a C virus (HCV) in association with the higher concentration of HCV RNA genomic sequences in the cryoglobulins suggests a close\\u000a relationship between MC and HCV infection and strongly supports the view that this virus plays a key role in causing vascular\\u000a damage. Analysis

  16. Cutaneous leukocytoclastic vasculitis associated with anti-tuberculosis drugs.

    PubMed

    Avcu, Gülhadiye; Sensoy, Gülnar; Çeliksoy, Mehmet Halil; Sogut, Ayhan; Kefeli, Mehmet; Karli, Arzu; Belet, Nursen

    2015-02-01

    Leukocytoclastic vasculitis (LCV), a disease characterized by inflammation of the small vessels, presents with palpable purpura, especially in the lower extremities. Its etiology is known to include drugs, infection, collagen tissue disease, and malignancy, but LCV caused by anti-tuberculosis drugs is very rarely seen. This report describes the case of a 12-year-old girl who developed LCV with rifampicin and ethambutol while undergoing anti-tuberculosis treatment due to extensive pulmonary involvement. PMID:25711253

  17. 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

  18. [Vasculitis : EULAR/ACR guidelines with respect to the clinical cardiological routine].

    PubMed

    Maisch, B

    2015-02-01

    In this article the diagnostics, differential diagnosis, laboratory findings, multimodal imaging and treatment of vasculitis of small, medium and large vessels as well as granulomatous and eosinophilic vascular diseases are described in the context of previous and current European League against Rheumatism (EULAR) and American College of Rheumatology (ACR) recommendations. Vasculitis is a syndrome which is part of various clinical disease entities, such as Wegener's granulomatosis, polyangiitis, Churg-Strauss syndrome, polyarteritis nodosa, cryoglobulinemia and other forms of vasculitis. PMID:25676009

  19. Rhinologic and sinonasal changes in PR3 ANCA pulmonary vasculitis

    PubMed Central

    2010-01-01

    Background Over 70-95% patients with PR3 ANCA pulmonary vasculitis present with upper respiratory tract symptoms or sings. Nasal cavity usually presents with obstruction and chronic refractory infections (rhinosinusitis) which commonly manifest as bloody discharge or crusting obstruction. Mucopurulent discharge may occur in the acute phase or remission, along with other symptoms suggesting sinusitis. Later on, saddle nose deformities can occur due to collapse of the nasal septum. Other common destruction areas are the maxillary ostia, erosion of the tubinates or damage of soft palate. Objective The aim of the study was to characterize pathologies of nasal and sinonasal CT scans in patients with PR3 pulmonary ANCA vasculitis and to establish the CT diagnostic criteria for WG. Between 2005-2009 sinonasal CT visualization was performed in 35 patients (19 female, 16 male) with PR3 ANCA positive WG. Results Bony destruction of the nasal cavity was revealed in 15 (42.8%), damage or distortion of the paranasal sinuses in 20 (57.1%), the mastoid cells in 7 (20%), and the orbits in 7 (20%) patients. Sclerosing osteitis of the nasal cavity and paranasal sinuses were observed in 11 (31.4%) and in 24 (68.5%), respectively. Bony thickening of the nasal cavity was shown in 5 (14.2%) patients and of the paranasal sinuses in 7 (20%) (unilateral in 2 and bilateral in 5 patients). Seven patients (20%) had orbital masses; all unilateral. Septal perforation was observed in 11 (31.4%) and saddle nose deformity in 7 (20%) patients. Conclusions Maxillary sinuses are regions which are most frequently affected during the course of PR3 ANCA pulmonary vasculitis. CT imagines may be a useful supplement to clinical and activity scoring of WG disease with pulmonary involvement. PMID:21147659

  20. Upregulated expression and function of integrin adhesive receptors in systemic lupus erythematosus patients with vasculitis.

    PubMed Central

    Takeuchi, T; Amano, K; Sekine, H; Koide, J; Abe, T

    1993-01-01

    Upregulation of integrin adhesive receptors has been implicated in various pathological conditions. We examined expression and function of integrin adhesive receptors on peripheral blood lymphocytes from patients with systemic lupus erythematosus (SLE), particularly those with the complication of vasculitis, and found that VLA-4 and LFA-1 expression was increased in SLE patients with vasculitis, while LFA-1 but not VLA-4 expression was increased in those without vasculitis. These results suggested a role of VLA-4 in the pathogenesis of vasculitis in SLE. Functional studies further demonstrated that adhesion to cytokine-activated human umbilical cord vein endothelial cells and to the CS-1 alternatively spliced domain of fibronectin was significantly increased in SLE patients with vasculitis. Analysis of the functional epitopes on the alpha 4 chain demonstrated that antigen densities of all the functional epitopes were increased in those with vasculitis, indicating that the increased expression of VLA-4 resulted from the increased number of VLA-4 molecules, and was not secondary to an increase in one particular functional epitope. Immunoprecipitation studies further support these results. Interestingly, high molecular weight bands associated with VLA-4 were observed in about half of the SLE patients with vasculitis. These results introduce a possibility that upregulation of integrin adhesive receptors has a potential role in the pathogenesis of vasculitis in SLE. Images PMID:8254055

  1. Disulfite-induced acute intermittent urticaria with vasculitis.

    PubMed

    Wüthrich, B; Kägi, M K; Hafner, J

    1993-01-01

    Recognition of sulfite sensitivity by the practicing dermatologist has become increasingly important. A wide spectrum of anaphylactoid reactions can occur after ingestion of sulfite additives in foods and medications. We report the case of a 47-year-old man with severe acute intermittent urticaria. A placebo-controlled oral challenge test with 50 mg sodium disulfite resulted in an acute urticaria attack. A biopsy taken 5 h after the appearance of the urticaria demonstrated a leukocytoclastic vasculitis with eosinophilia. Avoiding foods and drugs containing sulfites is often difficult due to its widespread use. Therefore, the patient should be equipped with a medical emergency kit. PMID:8274792

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

    PubMed Central

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

    2014-01-01

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

  3. Case of disseminated cutaneous Mycobacterium chelonae infection mimicking cutaneous vasculitis.

    PubMed

    Ichihara, Asako; Jinnin, Masatoshi; Fukushima, Satoshi; Inoue, Yuji; Ihn, Hironobu

    2014-05-01

    Mycobacterium chelonae is a non-tuberculous, rapidly growing mycobacteria and is widely distributed in the natural environment. In the immunocompetent status, localized cutaneous infections such as cellulitis and subcutaneous abscesses commonly occur after traumatic injury. However, disseminated cutaneous infections occur on a background of immunosuppression. Cutaneous M. chelonae infection presents with a variety of skin eruptions. We report a case of disseminated M. chelonae infection mimicking cutaneous vasculitis. The patient was treated with long-term oral corticosteroids and injected etanercept for the treatment of rheumatoid arthritis and asthma. Because the skin eruptions were preceded by asthma and rheumatoid arthritis and the pathological findings showed fibrinoid necrosis around the vascular of dermis, cutaneous vasculitis was first suspected. The culture from the pus revealed the bacterium which grew within 5 days on Ogawa's culture medium suggesting a rapidly growing mycobacteria. This bacterium was identified as M. chelonae by the DNA-DNA hybridization method. We chose 800 mg/day clarithromycin and 500 mg/day levofloxacin as a result of the drug-sensitivity test. After 6 months of the treatment, infection symptoms disappeared. Rapidly growing mycobacteria should be considered in the differential diagnosis of infections in patients under immunosuppression caused by diseases or drugs such as corticosteroids and biologic agents. Repeated bacterial examinations are important and required for the diagnosis of rapidly growing mycobacteria. PMID:24801916

  4. Role of ultrasound in the understanding and management of vasculitis

    PubMed Central

    2014-01-01

    Vasculitis is characterized by a circumferential vessel-wall thickening (‘halo’), which can be visualized by modern imaging techniques. In particular, the resolution of ultrasound has increased to 0.1 mm. Ultrasound detects abnormalities that are pathognomonic even in arteries with a diameter below 1 mm. It is particularly helpful in the diagnosis of large-vessel vasculitides, such as classic temporal arteritis, large-vessel giant-cell arteritis (GCA), Takayasu arteritis and idiopathic aortitis. Echocardiography is important for determining cardiac involvement in Takayasu arteritis and also for examining the coronary arteries of children with suspected Kawasaki disease, which is a medium-vessel vasculitis. In small vessel vasculitides ultrasound has only a role for determining the distribution or organ involvement. Fast-track clinics for the diagnosis of GCA help to initiate treatment before complications such as blindness occur; patients receive appointments within 24 h in these clinics. Clinical examination and ultrasound of temporal and axillary arteries are performed by an experienced rheumatologist. In most cases this is able to determine if GCA is present. Temporal artery biopsy can be still carried out in ambivalent cases. The wall swelling of temporal arteries disappears after 2–3 weeks of glucocorticoid treatment. After 3 days of treatment, diagnosis becomes more difficult with ultrasound in some cases. In larger arteries, such as the axillary arteries, wall thickening disappears within months. It tends to be darker (more hypoechoic) in acute disease because of oedema. PMID:24688604

  5. Role of ultrasound in the understanding and management of vasculitis.

    PubMed

    Schmidt, Wolfgang A

    2014-04-01

    Vasculitis is characterized by a circumferential vessel-wall thickening ('halo'), which can be visualized by modern imaging techniques. In particular, the resolution of ultrasound has increased to 0.1 mm. Ultrasound detects abnormalities that are pathognomonic even in arteries with a diameter below 1 mm. It is particularly helpful in the diagnosis of large-vessel vasculitides, such as classic temporal arteritis, large-vessel giant-cell arteritis (GCA), Takayasu arteritis and idiopathic aortitis. Echocardiography is important for determining cardiac involvement in Takayasu arteritis and also for examining the coronary arteries of children with suspected Kawasaki disease, which is a medium-vessel vasculitis. In small vessel vasculitides ultrasound has only a role for determining the distribution or organ involvement. Fast-track clinics for the diagnosis of GCA help to initiate treatment before complications such as blindness occur; patients receive appointments within 24 h in these clinics. Clinical examination and ultrasound of temporal and axillary arteries are performed by an experienced rheumatologist. In most cases this is able to determine if GCA is present. Temporal artery biopsy can be still carried out in ambivalent cases. The wall swelling of temporal arteries disappears after 2-3 weeks of glucocorticoid treatment. After 3 days of treatment, diagnosis becomes more difficult with ultrasound in some cases. In larger arteries, such as the axillary arteries, wall thickening disappears within months. It tends to be darker (more hypoechoic) in acute disease because of oedema. PMID:24688604

  6. Diffuse alveolar hemorrhage secondary to ANCA-associated vasculitis in a patient with Down syndrome.

    PubMed

    Hori, Ikumi; Nakaseko, Haruna; Kamei, Michi; Nomura, Takayasu; Iwata, Naomi; Ito, Yasuhiko; Saitoh, Shinji

    2015-04-01

    Diffuse alveolar hemorrhage (DAH) is a rare disease characterized by dyspnea, cough, hemoptysis, and new alveolar infiltrates. Among the various underlying disorders, vasculitis is believed to play a significant role in the pathogenesis of DAH. Here we report the first case of a patient with Down syndrome who developed DAH secondary to anti-neutrophil cytoplasmic antibody-associated vasculitis. This case highlights the significance of vasculitis as well as pulmonary hypoplasia and vulnerability associated with Down syndrome in the development of DAH. PMID:25868961

  7. Cutaneous and systemic vasculitis: cellular players and pathogenetic aspects.

    PubMed

    Cassisa, A

    2015-02-01

    Vascular wall damage, inflammatory cell recruitment and subsequent structural remodelling define a vasculitic process. Histopathological classification of vasculitis is based on the caliber of the vessel involved and on the prevalent type of inflammatory cells (neutrophils in acute forms, lymphocytic for chronic, histiocytic for granulomatous). A large amount of information is emerging from the literature on the complex pathophysiology of the cellular components of vessel wall. For instance, endothelial cells not only have the task to cover the inner surface of the vascular system but they also play an active role in tuning the immunological response in a very sophisticated way. Neutrophils are not only terminally differentiated cells sacrificed for a valuable cause. Cellular types of the perivascular microenvironment play roles one time not expected. The spread of the inflammatory process into the vascular wall is not necessarily inside-out. These and other selected concepts will be discussed in this paper. PMID:25502539

  8. A case report: Multaq-induced leukocytoclastic vasculitis.

    PubMed

    Smith, Suzanne M; Al-Bataineh, Mohammed; Iorfido, Stephen B; Macfarlane, Jarrod

    2014-01-01

    A 71-year-old white male presented to the emergency department complaining of a worsening lower extremity rash. Thirteen days before presentation, the patient was placed on Multaq (dronedarone) for his paroxysmal atrial fibrillation. Biopsy-proven leukocytoclastic vasculitis (LV) was diagnosed, and causes for the condition other than drug-induced were investigated and ruled out. Rash has been cited as a possible side effect of Multaq; however, a literature search has revealed this to be the first documented case of likely multaq-induced LV. Other patients on Multaq therapy should be monitored for signs and symptoms of LV and be referred to an acute care setting as indicated. PMID:23011162

  9. Cutaneous vasculitis and immune complexes in severe bronchiectasis.

    PubMed Central

    Hilton, A M; Hasleton, P S; Bradlow, A; Leahy, B C; Cooper, K M; Moore, M

    1984-01-01

    Four patients with severe bronchiectasis (chronic bronchial suppuration) are described who developed cutaneous lesions associated with exacerbations of their respiratory disease. The skin abnormalities consisted of purpuric lesions in three patients and an erythematous vasculitis in one. Circulating immune complexes were present in all patients and in three skin biopsy specimens showed deposition of C3, IgG, and IgA in dermal blood vessels. Haemophilus influenzae had been isolated from the sputum of all four patients and in two patients was present at the time the cutaneous lesions appeared. It is suggested that local immune complex deposition was responsible for the skin lesions which occurred during acute exacerbations of bronchiectasis. Images PMID:6710426

  10. Eosinophilic/T-cell Chorionic Vasculitis: Histological and Clinical Correlations.

    PubMed

    Cheek, Bradley; Heinrich, Stephen; Ward, Kenneth; Craver, Randall

    2014-10-22

    Eosinophilic T-cell chorionic vasculitis (E/TCV) is composed of eosinophils and T-lymphocytes originating within chorionic vessels, radiating toward the intervillous space and away from the amnion in a fashion different from the fetal vascular response seen in amnionitis. Clinical significance and risk factors are not well established. We report four pregnancies (five infants, one triplet was spared) with E/TCV, gestational ranging from 23 weeks to term. All had concurrent acute chorioamnionitis, three had the typical acute fetal inflammatory response. One had placental fetal obstructive vasculopathy and an upper extremity reduction defect (radio-ulnar synostosis), the mother had pre-eclampsia. A second case involved 2 of 3 23 week previable triplets. Our third case had a metatarsus varus resistant to casting, the mother had gestational diabetes. The last case was a normal infant. We review the literature, discuss the clinical findings and present the histologic characteristics of this infrequently recognized lesion. PMID:25338020

  11. 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

  12. A case of vasculitis, retinitis and macular neurosensory detachment presenting post typhoid fever

    PubMed Central

    2014-01-01

    Background Ocular and extraocular immune-mediated phenomena are known to occur following febrile illness. Vasculitis, retinitis and neurosensory detachment are not well-recognized sequelae of typhoid fever. Findings We report a case of vasculitis, retinitis and macular neurosensory detachment presenting post typhoid fever. A 27-year-old female presented with decreased vision in right eye with history of typhoid fever (treated adequately 6 weeks prior). Her best corrected visual acuity in right eye was 20/125, N36. Fundus showed a patch of vasculitis and retinitis superior to the disc associated with macular neurosensory detachment and disc pallor. With oral steroids, the inflammation resolved and visual acuity improved to 20/20 at 6 weeks. Conclusions Immune-mediated vasculitis and retinitis following typhoid fever may respond well to systemic steroids. PMID:25246983

  13. 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

  14. 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.

  15. Neutrophil priming and apoptosis in anti-neutrophil cytoplasmic autoantibody-associated vasculitis1

    Microsoft Academic Search

    Lorraine Harper; Paul Cockwell; Dwoma Adu; Caroline O. S. Savage

    2001-01-01

    Neutrophil priming and apoptosis in anti-neutrophil cytoplasmic autoantibody-associated vasculitis.BackgroundInteractions between anti-neutrophil cytoplasmic autoantibody (ANCA) and primed neutrophils (PMNs) may be central to the pathogenesis of primary small vessel vasculitis. PMNs from patients are primed, expressing proteinase 3 (PR3) on the cell surface, which permits interaction with ANCA. In vitro ANCA activates primed PMN to degranulate and generate a respiratory burst.

  16. Spontaneous massive bilateral peri-renal hemorrhage as a complication of ANCAnegative granulomatous vasculitis.

    PubMed

    Vo, Hieu; Showkat, Arif; Chikkalingaiah, K Mandya; Nguyen, Cuong; Wall, Barry M

    2015-04-01

    Spontaneous retroperitoneal hemorrhage (SRH) is a rare, life-threatening clinical entity most commonly associated with renal cell cancers. Systemic vasculitis has also been described as a rare cause of SRH. The current report describes a patient with acute kidney failure complicated by massive SRH, which occurred in the setting of anti-neutrophil cytoplasmic antibody (ANCA)-negative systemic necrotizing angiocentric granulomatous vasculitis involving multiple organs with minimal constitutional symptoms and no respiratory tract involvement. PMID:25707453

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

    PubMed Central

    Tervaert, J W Cohen

    2009-01-01

    Premature atherosclerosis has been observed during the course of different systemic inflammatory diseases such as rheumatoid arthritis and sytemic lupus erythematosus. Remarkably, relatively few studies have been published on the occurrence of accelerated atherosclerosis in patients with vasculitis. In giant cell arteritis (GCA), mortality because of ischaemic heart disease is not increased. In addition, intima media thickness (IMT) is lower in patients with GCA than in age-matched controls. In contrast, IMT is increased significantly in Takayasu arteritis, another form of large vessel vasculitis occurring in younger patients. In Takayasu arteritis and in Kawasaki disease, a form of medium-sized vessel vasculitis, accelerated atherosclerosis has been well documented. In small vessel vasculitis because of anti-neutrophil cytoplasmic autoantibodies-associated vasculitis, cardiovascular diseases are a major cause of mortality. IMT measurements reveal conflicting results. During active disease these patients experience acceleration of the atherosclerotic process. However, when inflammation is controlled, these patients have atherosclerotic development as in healthy subjects. Several risk factors, such as diabetes and hypertension, are present more often in patients with vasculitis compared with healthy controls. In addition, steroids may be pro-atherogenic. Most importantly, many patients have impaired renal function, persistent proteinuria and increased levels of C-reactive protein, well-known risk factors for acceleration of atherosclerosis. Enhanced oxidation processes, persistently activated T cells and reduced numbers of regulatory T cells are among the many pathophysiological factors that play a role during acceleration of atherogenesis. Finally, autoantibodies that may be relevant for acceleration of atherosclerosis are found frequently in elevated titres in patients with vasculitis. Because patients have an increased risk for cardiovascular events, vasculitis should be treated with as much care as possible. In addition, treatment should be considered with angiotensin-converting-enzyme inhibitors and/or angiotensin receptor-1 blockers, statins and acetylsalicyl acid. Finally, classical risk factors for cardiovascular disease should be monitored and treated as much as possible. PMID:19309350

  18. Chronic Urticaria: Recent Advances

    Microsoft Academic Search

    Malcolm W. Greaves; Kian Teo Tan

    2007-01-01

    Chronic urticaria is an umbrella term, which encompasses physical urticarias, chronic “idiopathic” urticaria and urticarial\\u000a vasculitis. It is important to recognize patients with physical urticarias as the investigation and treatment differs in important\\u000a ways from patients with idiopathic chronic urticaria or urticarial vasculitis. Although relatively uncommon, urticarial vasculitis\\u000a is an important diagnosis to make and requires histological confirmation by biopsy.

  19. Mast Cells Contribute to Peripheral Tolerance and Attenuate Autoimmune Vasculitis

    PubMed Central

    Gan, Poh-Yi; Summers, Shaun A.; Ooi, Joshua D.; O’Sullivan, Kim M.; Tan, Diana S.Y.; Muljadi, Ruth C.M.; Odobasic, Dragana; Kitching, A. Richard

    2012-01-01

    Mast cells contribute to the modulation of the immune response, but their role in autoimmune renal disease is not well understood. Here, we induced autoimmunity resulting in focal necrotizing GN by immunizing wild-type or mast cell-deficient (KitW-sh/W-sh) mice with myeloperoxidase. Mast cell-deficient mice exhibited more antimyeloperoxidase CD4+ T cells, enhanced dermal delayed-type hypersensitivity responses to myeloperoxidase, and more severe focal necrotizing GN. Furthermore, the lymph nodes draining the sites of immunization had fewer Tregs and reduced production of IL-10 in mice lacking mast cells. Reconstituting these mice with mast cells significantly increased the numbers of Tregs in the lymph nodes and attenuated both autoimmunity and severity of disease. After immunization with myeloperoxidase, mast cells migrated from the skin to the lymph nodes to contact Tregs. In an ex vivo assay, mast cells enhanced Treg suppression through IL-10. Reconstitution of mast cell-deficient mice with IL-10–deficient mast cells led to enhanced autoimmunity to myeloperoxidase and greater disease severity compared with reconstitution with IL-10–intact mast cells. Taken together, these studies establish a role for mast cells in mediating peripheral tolerance to myeloperoxidase, protecting them from the development of focal necrotizing GN in ANCA-associated vasculitis. PMID:23138486

  20. Immune Mechanisms in Medium and Large Vessel Vasculitis

    PubMed Central

    Weyand, Cornelia M.; Goronzy, Jörg J.

    2014-01-01

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

  1. [Takayasu arteritis: a chronic vasculitis that is rare in children].

    PubMed

    Greidanus, P M; Benninga, M A; Groothoff, J W; van Delden, O M; Davin, J C M A; Kuijpers, T W

    2006-11-18

    Takayasu arteritis was diagnosed in two children, a 15-year-old girl and a 10-year-old boy. The girl had suffered from fatigue, malaise, abdominal pain and weight loss for several months, but no clear cause could be found. A few weeks later, when a blood pressure of 222/155 mmHg was measured, the possibility of renal artery stenosis was considered and imaging studies revealed indications for Takayasu's disease. The patient was given methylprednisolone followed by a combination of prednisone and, initially, cyclophosphamide, later methotrexate. This resulted in a clinical remission of the inflammatory process. The boy presented with increasing fatigue and variable episodes of fever. After 3 years, sarcoidosis or Castleman's disease were considered. Imaging studies revealed aortic stenosis. He underwent stenting of the involved vessel segment. Takayasu arteritis is a chronic vasculitis of unknown origin, affecting mainly the aorta and its main branches. As a result of the inflammation, stenosis, occlusion or dilatation of the involved vessels may occur and cause a wide range of symptoms. Especially in the early phase, the symptoms often are non-specific. One should look for hypertension, blood pressure differences between the two arms, decreased peripheral pulsation or bruits over the aorta and its major branches. Radiological examination may consist ofangiography, magnetic resonance imaging or CT-scans. Treatment consists of corticosteroids and other immunosuppressants, such as cyclophosphamide, methotrexate, azathioprine, and antagonists of tumour-necrosis factor alpha. In addition, balloon dilatation or stenting is often necessary. PMID:17152333

  2. [Rare forms of hypertension : From pheochromocytoma to vasculitis].

    PubMed

    Haller, H; Limbourg, F; Schmidt, B M; Menne, J

    2015-03-01

    Secondary hypertension affects only 5-10?% of hypertensive patients. Screening is expensive and time-consuming and should be performed only in patients for whom there is a high clinical suspicion of secondary hypertension. Clinical signs of secondary forms of hypertension are new-onset hypertension in patients without other risk factors (i.e., family history, obesity, etc.), sudden increase of blood pressure (BP) in a previously stable patient, increased BP in prepubertal children, resistant hypertension, and severe hypertension or hypertensive emergencies. In adults, renal parenchymal and vascular diseases as well as obstructive sleep apnea are the most common causes of secondary hypertension. Medication-induced hypertension and non-adherence to medication have to be ruled out. Of the endocrine causes associated with hypertension, primary aldosteronism is the most common. Other endocrine causes of hypertension such as thyroid disease (hypo- or hyperthyroidism), hypercortisolism (Cushing's syndrome), hyperparathyroidism, and pheochromocytoma are rare. Monogenetic forms of hypertension are mostly of tubular origin and associated with alterations in mineralocorticoid handling or signaling. Rare causes of hypertension also include inflammatory vascular disease. Acute forms of vasculitis may present as "malignant" hypertension with associated thrombotic microangiopathy and organ damage/failure. It is important to diagnose these rare forms of hypertension in order to prevent acute organ damage in these patients or unnecessary invasive treatment strategies. PMID:25700646

  3. An Interferon-Induced Digital Vasculitis-Like Syndrome: A Case Report.

    PubMed

    Hamidi, Oksana; Reiser, Jochen; Hasler, Scott

    2014-04-11

    This report describes a patient with chronic hepatitis C undergoing therapy with interferon (IFN) alpha who developed bilateral ischemia of his fingers. We present a 43-year-old man with a failed renal transplant and chronic hepatitis C. He was treated with 6 months of IFN therapy with good reduction of his viral load. He presented with 2 days of pain and swelling in the second digits of both hands. Workup for extrahepatic manifestations of hepatitis C was initiated including assessment for vasculitis because of cryoglobulin- and noncryoglobulin-related causes. Extensive assessment with invasive and noninvasive vascular testing was performed. His workup for vasculitis did not reveal any specific reasons for the ischemic changes. Angiography of his fingers showed mild stenotic changes but no evidence of systemic vasculitis. IFN therapy was stopped and over several weeks his symptoms resolved. The ischemic changes were attributed to IFN therapy. The patient in this report is unique because although IFN has been historically reported to cause a variety of vascular syndromes, the reported experience in hepatitis C patients is small. In addition, the likelihood of encountering vasculitis and vasculitis-like syndromes in patients with hepatitis C is significant, and the increasing use of IFN in this population makes drug-induced vascular changes an essential consideration in this subset of patients. PMID:24732907

  4. Genome-wide association study of hepatitis C virus- and cryoglobulin-related vasculitis.

    PubMed

    Zignego, A L; Wojcik, G L; Cacoub, P; Visentini, M; Casato, M; Mangia, A; Latanich, R; Charles, E D; Gragnani, L; Terrier, B; Piazzola, V; Dustin, L B; Khakoo, S I; Busch, M P; Lauer, G M; Kim, A Y; Alric, L; Thomas, D L; Duggal, P

    2014-10-01

    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 hepatitis C virus (HCV) RNA-positive individuals with cryoglobulin-related vasculitis and 447 ethnically matched, HCV RNA-positive controls. All cases had both serum cryoglobulins and 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. 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 (odds ratio=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)). 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 mixed cryoglobulinemia vasculitis in this extended major histocompatibility complex region. PMID:25030430

  5. 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

  6. Decreased Neutrophil Apoptosis in Quiescent ANCA-Associated Systemic Vasculitis

    PubMed Central

    Abdgawad, Mohamed; Pettersson, Åsa; Gunnarsson, Lena; Bengtsson, Anders A.; Geborek, Pierre; Nilsson, Lars; Segelmark, Mårten; Hellmark, Thomas

    2012-01-01

    Background ANCA-Associated Systemic Vasculitis (AASV) is characterized by leukocytoclasis, accumulation of unscavenged apoptotic and necrotic neutrophils in perivascular tissues. Dysregulation of neutrophil cell death may contribute directly to the pathogenesis of AASV. Methods Neutrophils from Healthy Blood Donors (HBD), patients with AASV most in complete remission, Polycythemia Vera (PV), Systemic Lupus Erythematosus (SLE), Rheumatoid Arthritis (RA) and renal transplant recipients (TP) were incubated in vitro, and the rate of spontaneous apoptosis was measured by FACS. Plasma levels of cytokines and sFAS were measured with cytometric bead array and ELISA. Expression of pro/anti-apoptotic factors, transcription factors C/EBP-?, C/EBP-? and PU.1 and inhibitors of survival/JAK2-pathway were measured by real-time-PCR. Results AASV, PV and RA neutrophils had a significantly lower rate of apoptosis compared to HBD neutrophils (AASV 50±14% vs. HBD 64±11%, p<0.0001). In RA but not in AASV and PV, low apoptosis rate correlated with increased plasma levels of GM-CSF and high mRNA levels of anti-apoptotic factors Bcl-2A1 and Mcl-1. AASV patients had normal levels of G-CSF, GM-CSF and IL-3. Both C/EBP-?, C/EBP-? were significantly higher in neutrophils from AASV patients than HBD. Levels of sFAS were significantly higher in AASV compared to HBD. Conclusion Neutrophil apoptosis rates in vitro are decreased in AASV, RA and PV but mechanisms seem to differ. Increased mRNA levels of granulopoiesis-associated transcription factors and increased levels of sFAS in plasma were observed in AASV. Additional studies are required to define the mechanisms behind the decreased apoptosis rates, and possible connections with accumulation of dying neutrophils in regions of vascular lesions in AASV patients. PMID:22403660

  7. Effects of decomplementation with cobra venom factor on experimental vasculitis.

    PubMed Central

    Mathieson, P W; Qasim, F J; Thiru, S; Oldroyd, R G; Oliveira, D B

    1994-01-01

    Mercuric chloride (HgCl2) induces autoimmunity in susceptible rat strains, with hyper-IgE, appearance of a number of autoantibodies, and widespread tissue injury, including necrotizing vasculitis in the gut. In the early phase of tissue injury there is granulocyte infiltration; later there is immunoglobulin deposition along basement membranes in vessels. We have analysed the role of complement in this model using cobra venom factor (CVF), which causes decomplementation lasting around 5 days. The characteristic time course when HgCl2 is given over 10 days is that tissue injury and autoantibody levels reach a peak at around day 15 (start of HgCl2 = day 0). We therefore gave CVF either early (day 0), intermediate (day 5) or late (day 10); a fourth group (controls) received HgCl2 but no CVF. At each time point, CVF caused complete decomplementation which lasted for at least 5 days. Serum IgE and autoantibody levels were similar in all four experimental groups. Tissue injury in the 'early' CVF group and in the 'late' CVF group was not significantly different from controls, but in the intermediate group tissue injury was significantly more severe than in controls. These data indicate that the complement system does not play a major role in the induction of autoantibodies by HgCl2, nor in the effector phase of tissue injury. We speculate that the exacerbation of tissue injury by CVF in the group given this agent at an intermediate stage of the model is explained by the presence of products of C3 activation which have proinflammatory effects during the phase of active granulocyte-mediated tissue injury. PMID:8082302

  8. 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

  9. Failure of Recombinant Activated Factor VII in Treatment of Diffuse Alveolar Hemorrhage due to Cryoglobulinemic Vasculitis.

    PubMed

    Khoulani, Dania; Rao, Bharat; Khanshour, Ammar; Kuriakose, Philip; Yessayan, Lenar

    2014-01-01

    Diffuse alveolar hemorrhage (DAH) is a serious complication of the small vessel vasculitis syndromes and carries a high mortality. Recombinant activated factor VII (rFVIIa) is used to treat bleeding in patients with hemophilia and antibodies to factor VIII or IX. It is increasingly being used in life-threatening hemorrhage in a variety of other settings in which conventional therapy is unsuccessful. Randomized controlled trials of rFVIIa in DAH are lacking. However, several case reports have described a complete or sustained control of DAH using rFVIIa after patients failed to respond to medical treatment. There are no case reports in the literature describing the use or the failure of rFVIIa in DAH associated with cryoglobulinemic vasculitis. We here report the failure of rFVIIa to control DAH in a patient with CD5+ B-cell non-Hodgkin's lymphoma and cryoglobulinemic vasculitis. PMID:25114814

  10. Failure of Recombinant Activated Factor VII in Treatment of Diffuse Alveolar Hemorrhage due to Cryoglobulinemic Vasculitis

    PubMed Central

    Khoulani, Dania; Khanshour, Ammar; Kuriakose, Philip

    2014-01-01

    Diffuse alveolar hemorrhage (DAH) is a serious complication of the small vessel vasculitis syndromes and carries a high mortality. Recombinant activated factor VII (rFVIIa) is used to treat bleeding in patients with hemophilia and antibodies to factor VIII or IX. It is increasingly being used in life-threatening hemorrhage in a variety of other settings in which conventional therapy is unsuccessful. Randomized controlled trials of rFVIIa in DAH are lacking. However, several case reports have described a complete or sustained control of DAH using rFVIIa after patients failed to respond to medical treatment. There are no case reports in the literature describing the use or the failure of rFVIIa in DAH associated with cryoglobulinemic vasculitis. We here report the failure of rFVIIa to control DAH in a patient with CD5+ B-cell non-Hodgkin's lymphoma and cryoglobulinemic vasculitis. PMID:25114814

  11. 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

  12. Finger necrosis due to cryoglobulinemic vasculitis in association with membranous nephropathy.

    PubMed

    Porres-Aguilar, Mateo; Rodriguez-Castro, Carlos E; Osvaldo, Padilla; Saifuddin, Fátima; Siddiqui, Tariq; Fan, Jerry; Mukherjee, Debabrata; Pema, Kanchan; Abbas, Aamer

    2015-01-01

    Cryoglobulinemic vasculitis is a small vessel vasculitis that has been associated with chronic infections and autoimmune, lymphoproliferative, and neoplastic disorders. When no significant etiological factors are identified, it is called essential mixed cryoglobulinemia. A detailed and thorough laboratory investigation is required to exclude all possible causes of cryoglobulin formation. Although cryoglobulin testing is simple, careful temperature regulation is needed to avoid false-negative results. Consensus diagnosis should be developed and implemented for appropriate cryoglobulin detection and accurate clinical diagnosis for cryoglobulinemic vasculitis. Here we present an interesting, first-ever case report of a 54-year-old Hispanic-American woman with essential mixed cryoglobulinemia presenting with significant digital necrosis in association with membranous nephropathy. PMID:25552807

  13. Large Vessel Vasculitis Occurring in Rheumatoid Arthritis Patient under Anti-TNF Therapy

    PubMed Central

    Cestelli, Valentina; Spinella, Amelia; Campomori, Federica; Esposito, Carmela; Ciaffi, Sara; Sandri, Gilda; Ferri, Clodoveo

    2014-01-01

    Vasculitis is a heterogeneous group of disorders characterized by the presence of necrotic inflammatory phenomena and destruction of blood vessels. Vasculitis is classified as primary (idiopathic) or secondary to infections, connective tissue diseases and drugs but can also be considered as a paraneoplastic phenomenon. Evidence shows that the increasing use of biological agents results in a growing number of reports of autoimmune diseases induced by these therapies. An inflammatory articular chronic disease such as rheumatoid arthritis may be complicated by extra-articular manifestations, such as cutaneous or systemic vasculitis. Herewith, we describe the case of a great vessels arteritis in a patient affected by rheumatoid arthritis in therapy with an anti-TNF agent (etanercept). PMID:25544845

  14. Fiber optic LDF to monitor vascular dynamics of urticarial dermographism in pressure-tested patients before and after treatment with antihistamines

    NASA Astrophysics Data System (ADS)

    Eikje, Natalja Skrebova; Arase, Seiji

    2008-02-01

    The local microcirculatory dynamics underlying phenomenon of urticarial dermographism (UD) are not yet sufficiently elucidated in dermatological patients. A fiber optic laser Doppler flowmeter (LDF) was used to monitor skin blood flow (SBF) changes on the back of the patients with UD before and after application of the series of pressure stimuli (9.8×10 4, 14.7×10 4, 19.6×10 4 and 24.5×10 4 Pa). All patients acted as self-controls to assess their disease activity by means of SBF values based on response to pressure stimuli before and after treatment with antihistamines, when compared to baseline SBF. Throughout 30 minutes evaluation inter-subject SBF values at pressure-tested sites were noticeably distinguished as high, moderate and low. By LDF we could differentiate the highest development of vascular dynamics after 5 minutes, coming back to normal within about 30 minutes in one group of patients, and the vascular dynamics reaching its maximum in 15 minutes, but with no fade after 30 minutes, in another group of patients. All treatment regimens in both groups of patients by LDF produced a measurable reduction already during 1-2 days of therapy, accompanied by a reduction in SBF baseline values in patients with severe and moderate symptoms of UD.

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

    PubMed Central

    Berlit, P; Kraemer, M

    2014-01-01

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

  16. Vasculitis associated with all trans retinoic acid (ATRA) in a case with acute promyelocytic leukemia.

    PubMed

    Paydas, Semra; Yavuz, Sinan; Disel, Umut; Sahin, Berksoy; Canbolat, Tugba; Tuncer, Ilhan

    2003-03-01

    All trans retinoic acid is the drug of choice in the treatment of acute promyelocytic leukemia. But this drug has some side effects, some of which may be life-threatening. Retinoic acid syndrome is the most frequent and dangerous side effect of this differentiation inducing agent. Other side effects include Sweet's syndrome, vasculitis, hypercalcemia, bone marrow necrosis and fibrosis, thromboembolic events, erythema nodosum, granulomatous proliferation and some pulmonary complications. Here, we report vasculitis in a case with APL treated with ATRA and review the literature. PMID:12688331

  17. Recurrent erythema nodosum: a red flag sign of hidden systemic vasculitis

    PubMed Central

    Gupta, Monica; Singh, Kamal; Lehl, SS; Bhalla, Mala

    2013-01-01

    Takayasu's arteritis is a rare, systemic vasculitis with varied presentations across multiple medical specialities. Here, we present a young woman who had recurrent episodes of erythema nodosum on the background of a low-grade fever and no vascular manifestations. The presence of a high erythrocyte sedimentation rate generated a high index of suspicion for underlying vasculitis, and radioimaging confirmed the suspicion of Takayasu's arteritis. The patient was found to have type III diseases in the vasculitic stage and was managed with systemic corticosteroids. PMID:23576669

  18. Appearance of ANCA – associated vasculitis under Tumor necrosis factor-alpha inhibitors treatment

    PubMed Central

    Reitblat, Tatiana; Reitblat, Olga

    2013-01-01

    Summary Background: Tumor necrosis factor-alpha inhibitors treatment is accosiated with several side effects. The most common are injection side reactions, headache, nausea and infections. The more rare are development of systemic autoimmune diseases. Case Report: We describe two patients, who developed ANCA associated vasculitis during Tumor necrosis factor alpha inhibitors treatment. The diagnosis was confirmed by appropriate tissue picture, CT scan and laboratory findings. Conclusions: Our case series are unique, because vasculitis appeared after many years of the treatment and during complete patient’s remission of there main illness. PMID:23569570

  19. Anti-neutrophil cytoplasmic antibody-associated vasculitis associated with infectious mononucleosis due to primary Epstein–Barr virus infection: report of three cases

    PubMed Central

    Yamaguchi, Makoto; Yoshioka, Tomoki; Yamakawa, Taishi; Maeda, Matsuyoshi; Shimizu, Hideaki; Fujita, Yoshiro; Maruyama, Shoichi; Ito, Yasuhiko; Matsuo, Seiichi

    2014-01-01

    Although the aetiology of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis remains unclear, it is generally believed that environmental factors such as infections contribute to its development of ANCA-associated vasculitis. Prior Epstein–Barr virus (EBV) infection is reported to be a trigger of systemic vasculitis. We herein report three cases of ANCA-associated vasculitis presenting with infectious mononucleosis due to primary EBV infection. The causal link between the two pathologies could not be proved, but primary EBV infection may play a role in the initiation or exacerbation of ANCA-associated vasculitis. Future studies are necessary to determine the interaction between these diseases conditions.

  20. 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...

  1. IgG rheumatoid factors and anti-nuclear antibodies in rheumatoid vasculitis.

    PubMed Central

    Quismorio, F P; Beardmore, T; Kaufman, R L; Mongan, E S

    1983-01-01

    We studied the distribution and characteristics of circulating rheumatoid factors (RF) and anti-nuclear antibodies (ANA) in 30 rheumatoid arthritis (RA) patients who had polyarthritis alone (group I), 28 RA patients with polyarthritis and extra-articular disease (group II), 28 RA patients with systemic vasculitis (group III) and 60 healthy matched controls. IgG RF occurred more frequently and in higher serum titres in group III (100%) than RA patients in group I (40%), or in group II (18%) or in normal controls (5.8%). The serum titre of IgM RF was higher in vasculitis patients than in other RA patients. ANA were found in 74% of all RA patients and although the frequency did not differ in the three patient groups, the serum titre was significantly higher in the vasculitis group. Antibodies to extractable nuclear antigen were found only in group III (18.7%). Antibodies to histones were also more prevalent in group III than in the other RA groups. The serological abnormalities in rheumatoid vasculitis differed quantitatively as well as qualitatively from other RA patients. PMID:6602676

  2. Nuove acquisizioni in tema di terapia delle vasculiti ANCA-associate

    Microsoft Academic Search

    R. A. Sinico; E. Sabadini

    The prognosis of untreated ANCA-associated systemic vasculitis (AASV) is poor with up to 90% of patients dying within 2 years. The combination of prednisone and cyclophosphamide is now established as the treatment of choice and leads to con- trol of the disease in 80-90% of the patients. Such treatment has turned these acutely fatal diseases into chronic relapsing disorders with

  3. [Central nervous system vasculitis according to the 2012 revise international chapel hill consensus conference nomenclature of vasculitides].

    PubMed

    Koike, Haruki; Sobue, Gen

    2015-03-01

    Vasculitis can be primarily or secondarily to various underlying diseases. It frequently affects the nervous system, and neurological deficits may remain even after disease remission. Because the progression of vasculitides is usually acute to subacute, early initiation of treatment is important from the viewpoint of patients' functional status. However, early diagnosis may be difficult, particularly in patients with central nervous system vasculitis. Hence, it is important to understand the wide-ranging clinical manifestations of vasculitides. Here, we summarize the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides from the standpoint of central nervous system vasculitis. PMID:25846438

  4. Abnormal plasma fibrinolysis in patients with rheumatoid arthritis and impaired endothelial fibrinolytic response in those complicated by vasculitis.

    PubMed Central

    Lau, C S; McLaren, M; Hanslip, J; Kerr, M; Belch, J J

    1993-01-01

    OBJECTIVES--(a) To assess plasma fibrinolytic parameters in patients with rheumatoid arthritis (RA) and to determine whether there are differences between patients with RA alone and those with RA complicated by vasculitis. (b) To determine if patients with RA respond differently to venous occlusion compared with normal subjects and to assess whether such a response differs in patients with RA alone and those with rheumatoid vasculitis. (c) To determine the extent of vascular damage in patients with rheumatoid vasculitis and if this correlates with the levels of plasma fibrinolytic parameters. METHODS--Sixty three patients with RA (38 had RA only and 25 had evidence of rheumatoid vasculitis) were recruited. Plasma levels of tissue plasminogen activator antigen (t-PA Ag), plasminogen activator inhibitor (PAI) activity, and factor VIII von Willebrand factor (vWF) were measured before and 10 minutes after venous occlusion. RESULTS--Patients with RA, with or without rheumatoid vasculitis, had higher baseline PAI levels than control subjects. The difference was statistically significant for patients with RA alone but was not statistically significant for patients with rheumatoid vasculitis. After venous occlusion, t-PA Ag levels increased significantly in normal subjects and patients with RA alone, but not in patients with rheumatoid vasculitis. Plasma levels of vWF were significantly higher in patients with rheumatoid vasculitis than in normal subjects and those with RA alone. In patients with RA alone, baseline vWF correlated positively with t-PA Ag levels, whereas a negative correlation was found between these two parameters in patients with rheumatoid vasculitis. A negative correlation between vWF and t-PA Ag levels after venous occlusion was also found in patients with rheumatoid vasculitis. CONCLUSIONS--Patients with rheumatoid vasculitis showed evidence of vascular damage with increased levels of vWF and impaired t-PA Ag release after venous occlusion, a useful measurement of endothelial reserve to remove fibrin. This may be of pathophysiological importance in the development of vasculitis in these patients. PMID:8239758

  5. [Vasculitis 2013. What are the changes introduced in the 2012 Chapel Hill Consensus Conference?].

    PubMed

    Zeher, Margit

    2013-10-01

    Vasculitis is a heterogeneous group of rare disorders in which inflammation of blood vessels is the common feature. Due to the increasing number of diseases as well as overlaps and gaps in the definition and nomenclature, the classification criteria were constantly changing in the past decades. The classifications were based essentially on the size of affected blood vessels and pathologic characteristics of inflamed vessel walls. The standard procedures and validated diagnostic criteria are missing from the diagnostics of vasculitis, thus in clinical practice the classification criteria are applicable. The 2012 Chapel Hill Consensus Conference brought a change in the definition, nomenclature and classification of previously uncategorized diseases. The definitions of subgroups accurately determine the diagnosis of the specific disease, and they are suitable for establishing homogeneous disease groups. By better understanding of the etiopathogenetic factors, further diseases and subgroups may be defined in the near future. PMID:24077160

  6. [Acute renal failure and vasculitis - when to consider it and what to do about it?].

    PubMed

    Wilde, Benjamin; Bienholz, Anja; Witzke, Oliver; Kribben, Andreas

    2015-02-01

    Renal involvement in systemic autoimmune diseases such as ANCA-vasculitis is common. Acute kidney injury (AKI) limits the prognosis of the patient and needs to be detected early. However, AKI is in early phases clinically inapparent and at first contact with the patient, extra-renal symptoms may dominate the clinical presentation. Serum-Creatinine and urinary analysis provide useful information on the extent of renal involvement. A renal biopsy should confirm the diagnosis. Treatment needs to be adapted to the extent of ANCA-vasculitis and in cases with severe organ failure plasmapheresis should be performed. Maintenance immunosuppressive is necessary to prevent relapses and the patients should be monitored closely to detect renal flares early. PMID:25704521

  7. Reversal of rejection-induced coronary vasculitis detected early after heart transplantation with increased immunosuppression.

    PubMed

    Ballester, M; Obrador, D; Carrió, I; Bordes, R; Augé, J M; Crexells, C; Oriol, A; Padró, J M; Arís, A; Caralps, J M

    1989-01-01

    Four patients who underwent heart transplantation, in whom coronary obstruction was seen early after transplantation, are described. Repeated acute rejection episodes were detected within the first 2 months in each patient. Coronary obstruction or ischemia was shown through a combination of T1-201 isotopic study findings, evidence of vasculitis of a small coronary arteriole seen at endomyocardial biopsy, or coronary angiographic results. Vigorous treatment for rejection (antithymocyte globulin and bolus methylprednisolone) was given, and coronary artery lesions or myocardial ischemia resolved after treatment. Rejection-induced coronary obstruction should be considered in patients with repeated acute rejection episodes who are predisposed to the development of vascular rejection. Early after transplantation such obstruction is caused by diffuse vasculitis of small and medium-sized vessels and may be reversed with increased immunosuppression. PMID:2795284

  8. Acute respiratory failure as primary manifestation of antineutrophil cytoplasmic antibodies-associated vasculitis.

    PubMed

    Sourla, Evdokia; Bagalas, Vasilis; Tsioulis, Helias; Paspala, Asimina; Akritidou, Sofia; Pataka, Athanasia; Fekete, Katalin; Kioumis, Ioannis P; Stanopoulos, Ioannis; Pitsiou, Georgia

    2014-06-18

    The systemic vasculitides are multifocal diseases characterized by the presence of blood vessel inflammation in multiple organ systems. Their clinical presentation is variable extending from self-limited illness to critical complications including diffuse alveolar hemorrhage and glomerulonephritis. Alveolar hemorrhage is a life-threatening manifestation of pulmonary vasculitis that can rapidly progress into acute respiratory failure requiring ventilatory support. We present the case of a 74-year-old patient admitted to the Intensive Care Unit with severe hypoxic respiratory failure and diffuse alveolar infiltrates in chest imaging that was later diagnosed as antineutrophil cytoplasmic antibodies-associated vasculitis. The report highlights the importance of differentiate between alveolar hemorrhage and acute respiratory distress syndrome of other etiology because alveolar hemorrhage is reversible with prompt initiation of treatment. PMID:25332763

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

    PubMed

    Trask, R S; Bond, I P

    2010-06-01

    This paper presents the first conceptual study into creating a Plantae-inspired vascular network within a fibre-reinforced polymer composite laminate, which provides an ongoing self-healing functionality without incurring a mass penalty. Through the application of a 'lost-wax' technique, orthogonal hollow vascules, inspired by the 'ray cell' structures found in ring porous hardwoods, were successfully introduced within a carbon fibre-reinforced epoxy polymer composite laminate. The influence on fibre architecture and mechanical behaviour of single vascules (located on the laminate centreline) when aligned parallel and transverse to the local host ply was characterized experimentally using a compression-after-impact test methodology. Ultrasonic C-scanning and high-resolution micro-CT X-ray was undertaken to identify the influence of and interaction between the internal vasculature and impact damage. The results clearly show that damage morphology is influenced by vascule orientation and that a 10 J low-velocity impact damage event is sufficient to breach the vasculature; a prerequisite for any subsequent self-healing function. The residual compressive strength after a 10 J impact was found to be dependent upon vascule orientation. In general, residual compressive strength decreased to 70 per cent of undamaged strength when vasculature was aligned parallel to the local host ply and a value of 63 per cent when aligned transverse. This bioinspired engineering study has illustrated the potential that a vasculature concept has to offer in terms of providing a self-healing function with minimum mass penalty, without initiating premature failure within a composite structure. PMID:19955122

  10. Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis

    Microsoft Academic Search

    E Christiaan Hagen; Mohamed R Daha; Jo Hermans; Konrad Andrassy; Elena Csernok; Gillian Gaskin; Phillippe Lesavre; Jens Lüdemann; Niels Rasmussen; R Alberto Sinico; Allan Wiik; Fokko J van der Woude

    1998-01-01

    Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis. Anti-neutrophil cytoplasmic antibodies (ANCA) are widely used as diagnostic markers for Wegener’s granulomatosis (WG), microscopic polyangiitis (MPA), Churg-Strauss syndrome (CSS) and idiopathic rapidly progressive glomerulonephritis (iRPGN). The objective of this study was to evaluate the diagnostic value of ANCA measurement by the indirect immunofluorescence (IIF) test, and

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

    PubMed Central

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

    2010-01-01

    This paper presents the first conceptual study into creating a Plantae-inspired vascular network within a fibre-reinforced polymer composite laminate, which provides an ongoing self-healing functionality without incurring a mass penalty. Through the application of a ‘lost-wax’ technique, orthogonal hollow vascules, inspired by the ‘ray cell’ structures found in ring porous hardwoods, were successfully introduced within a carbon fibre-reinforced epoxy polymer composite laminate. The influence on fibre architecture and mechanical behaviour of single vascules (located on the laminate centreline) when aligned parallel and transverse to the local host ply was characterized experimentally using a compression-after-impact test methodology. Ultrasonic C-scanning and high-resolution micro-CT X-ray was undertaken to identify the influence of and interaction between the internal vasculature and impact damage. The results clearly show that damage morphology is influenced by vascule orientation and that a 10 J low-velocity impact damage event is sufficient to breach the vasculature; a prerequisite for any subsequent self-healing function. The residual compressive strength after a 10 J impact was found to be dependent upon vascule orientation. In general, residual compressive strength decreased to 70 per cent of undamaged strength when vasculature was aligned parallel to the local host ply and a value of 63 per cent when aligned transverse. This bioinspired engineering study has illustrated the potential that a vasculature concept has to offer in terms of providing a self-healing function with minimum mass penalty, without initiating premature failure within a composite structure. PMID:19955122

  12. A second case of Marburg's variant of multiple sclerosis with vasculitis and extensive demyelination.

    PubMed

    Elenein, Rania G A; Sharer, Leroy R; Cook, Stuart D; Pachner, Andrew R; Michaels, Jennifer; Hillen, Machteld E

    2011-12-01

    Marburg's variant of multiple sclerosis is a rapidly progressive and malignant form of multiple sclerosis (MS) that usually leads to severe disability or death within weeks to months without remission. Few cases have been described in the literature since the original description by Marburg. The classic pathological findings usually include highly destructive zones of extensive demyelination, necrosis with dense cellular infiltrate, and giant reactive astrocytes. We report a case of a 31-year-old woman with Marburg's variant of MS who, over a period of eight months, became totally disabled, blind, and quadriplegic, with vocal cord paralysis, requiring a tracheostomy. The patient underwent diagnostic stereotactic brain biopsy. Clinical findings, magnetic resonance imaging (MRI), serologic and cerebrospinal fluid (CSF) findings, and neuropathology are discussed. MRI showed extensive white matter involvement in the brain and spinal cord that continuously progressed over time. A diagnostic stereotactic brain biopsy revealed extensive active demyelination with unexpected finding of active vasculitis and fibrinoid necrosis with a vascular inflammatory cell infiltrate, including polymorphonuclear neutrophils and rare eosinophils. Serologic work-up for vasculitis and neuromyelitis optica was unremarkable and the CSF showed only one oligoclonal band (OCB) not present in serum. This is the second case of Marburg's variant of MS that demonstrated both demyelination and vasculitis. In our case these features were demonstrated simultaneously, even though the demyelination was the predominant pathological finding. Since vasculitis is not a feature of classic MS, these findings pose the question as to whether Marburg's variant of MS is a true variant or different entity altogether. PMID:21816761

  13. Radiation Retinopathy Caused by Low Dose Irradiation and Antithyroid Drug-Induced Systemic Vasculitis

    Microsoft Academic Search

    Koh-Hei Sonoda; Masahiro Yamamoto; Tatsuro Ishibashi

    2005-01-01

    Background: We report on a patient with Graves’ disease with radiation retinopathy caused by lowdose irradiation and antithyroid drug-induced antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis. Case: A 38-year-old woman with Graves’ disease presented with bilateral blurred vision, microaneurysms, telangiectasia, and macular edema. The patient was examined by ophthalmoscopy and fluorescein angiography, and radiation retinopathy was diagnosed. Observations: The patient had been

  14. Contrasting genetic association of IL2RA with SLE and ANCA – associated vasculitis

    Microsoft Academic Search

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

    2009-01-01

    BACKGROUND: Autoimmune diseases are complex and have genetic and environmental susceptibility factors. The objective was to test the genetic association of systemic lupus erythematosus (SLE) and anti-neutrophil cytoplasmic antibody (ANCA) – associated systemic vasculitis (AAV) with SNPs in the IL2RA region and to correlate genotype with serum levels of IL-2RA. METHODS: Using a cohort of over 700 AAV patients, two

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

    PubMed

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

    2014-11-01

    Vasculitis in systemic lupus erythematosus (SLE) has a broad spectrum of clinical manifestations from cutaneous to visceral involvement and its prognosis ranges from mild to life-threatening. We report the case of a previously healthy 17-year-old woman with eight months' history of arthralgias and myalgias. Subsequently, she developed facial and lower limbs edema, and hair loss. Two weeks before admission to a secondary level hospital, she developed fever up to 40°C followed by abdominal pain, rectal bleeding, hematemesis and blisters on both legs, reason for which she was hospitalized. With active bullous SLE with rapidly progressive glomerulonephritis suspected, she was treated with methylprednisolone pulses without response. After one week of treatment, she was transferred to a tertiary level hospital. On admission she presented acute arterial insufficiency of the lower extremities, respiratory failure with apnea, metabolic acidosis and shock; six hours later she died. Autopsy findings showed active diffuse lupus nephritis and diffuse systemic vasculitis that involved vessels from the skin, brain, myocardium, spleen, iliac and renal arteries. In addition, serositis of the small intestine and colon, acute and chronic pericarditis, pericardial effusion and myocarditis were found. Immunologic tests confirmed SLE diagnosis. In this case the fulminant course was the result of SLE high disease activity, visceral vasculitis of several organs and late diagnosis, referral and treatment. Early diagnosis, and opportune referral to the rheumatologist for intensive treatment can improve the outlook in these patients. PMID:25107938

  16. 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

  17. A role for mast cells in the development of adjuvant-induced vasculitis and arthritis.

    PubMed Central

    Johnston, B.; Burns, A. R.; Kubes, P.

    1998-01-01

    The objective of this study was to characterize the role of mast cells in the development of vasculitis and joint swelling in adjuvant-immunized rats. Leukocyte trafficking within mesenteric venules (rolling and adhesion) and mast cell activation (ruthenium red uptake) were examined in vivo. Elevated leukocyte trafficking was observed by 4 days after immunization, whereas joint swelling developed between days 10 and 12. Perivascular mast cells took up ruthenium red and appeared activated by electron microscopy at 4 but not 12 days after immunization. Treatment with the mast cell stabilizer cromolyn on days 1 to 4 after immunization blocked ruthenium red uptake at day 4 and reduced leukocyte rolling and adhesion by approximately 50%. This treatment also reduced rolling, adhesion, and joint swelling at day 12 by approximately 50%. Cromolyn treatment over days 9 to 12 reduced joint swelling but increased leukocyte emigration into the mesentery. Peritoneal mast cells isolated 4 days after immunization elicited significant neutrophil chemotaxis in vitro, whereas day 12 mast cells did not. Mast cell activation and vasculitis were absent in adjuvant-resistant Fisher/344 rats. These data suggest that mast cells play an early role in the initiation of vasculitis and may function by day 12 to limit infiltration of leukocytes from the vasculature. In the joint, however, mast cells appear to contribute to inflammation at early as well as later time points. Images Figure 2 PMID:9466582

  18. Systemic lupus erythematosus associated with ANCA-associated vasculitis: an overlapping syndrome?

    PubMed

    Hervier, B; Hamidou, M; Haroche, J; Durant, C; Mathian, A; Amoura, Z

    2012-10-01

    Systemic lupus erythematosus (SLE) and small-sized vessel vasculitis are usually two distinguishable autoimmune diseases. However, a vasculitis may be found in the course SLE but rarely corresponds to an ANCA-associated vasculitis (AAV). We report four cases of de novo SLE associated with AAV, our aim being to discuss the clinical significance of this association. We included four patients fulfilling the criteria for both SLE and AAV and followed in two different university hospitals between 1996 and 2009. In light of a 20-year literature review (25 described clinical cases), we discussed the etiopathogeny of such an association. All patients presented a severe renal involvement (creatininemia ranging from 120 to 370 ?mol/l) and thrombopenia (ranging from 45,000 to 137,000 platelets/mm(3)). The other main clinical symptoms were arthritis (n = 3), serositis (n = 2) and intra-alveolar hemorrhage (n = 2). An inflammatory syndrome was noticed at diagnosis in all cases. ANCAs were MPO-ANCAs in all cases. Two out of these four patients were also diagnosed with antiphospholipid syndrome. The frequency of this association seems not fortuitous. Although the etiopathogenic mechanisms of such an association remain to be more precisely described, several clinical, histological and immunological features support the hypothesis of the existence of a SLE-AAV overlapping syndrome. Moreover, clinicians must be aware of such an overlapping syndrome, notably because its initial presentation can be very severe. PMID:21805174

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

    SciTech Connect

    Both, M.; Jahnke, T. [Department of Radiology, Christian-Albrechts-University of Kiel, Kiel(Germany); Reinhold-Keller, E. [Department of Rheumatology, University of Luebeck, Rheumaklinik Bad Bramstedt (Germany); Reuter, M.; Grimm, J.; Biederer, J.; Brossmann, J. [Department of Radiology, Christian-Albrechts-University of Kiel, Kiel (Germany); Gross, W.L. [Department of Rheumatology, University of Luebeck, Rheumaklinik Bad Bramstedt (Germany); Heller, M.; Mueller-Huelsbeck, S. [Department of Radiology, Christian-Albrechts-University of Kiel, Kiel (Germany)

    2003-02-15

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

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

    PubMed

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

    2014-10-01

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

  1. Goodpasture's Syndrome and p-ANCA Associated Vasculitis in a Patient of Silicosiderosis: An Unusual Association

    PubMed Central

    Bal, Amanjit; Das, Ashim; Gupta, Dheeraj; Garg, Mandeep

    2014-01-01

    Introduction. Goodpasture's syndrome is a rare clinical entity and is characterized by circulating autoantibodies which are principally directed against the glomerular/alveolar basement membrane. The etiology of Goodpasture's syndrome is still unknown. Lung involvement occurs as a result of lung injury and the exposure of new epitopes to the immune system. Recently, several studies have suggested the role of silica as one of etiological factors in ANCA associated vasculitis and glomerulonephritis. Materials and Methods. We present a case of a 40-year-old welder with silicosiderosis, who developed anti-GBM disease with p-ANCA positivity. Case Report. Patient presented to an emergency with gradually increasing breathlessness along with renal failure and died after short hospital stay. Autopsy pathology findings revealed crescentic glomerulonephritis with linear glomerular basement membrane antibody deposition, splenic vasculitis, pulmonary haemorrhage, and pulmonary silicosiderosis. Conclusion. This case reinforces the role of environmental triggers like exposure to silica, metal dust, and tobacco in pathogenesis of Goodpasture's syndrome and p-ANCA associated vasculitis. PMID:25349763

  2. Septic vasculitis and vasculopathy in some infectious emergencies: the perspective of the histopathologist.

    PubMed

    Tomasini, C

    2015-02-01

    Sepsis is a potentially life-threatening complication of an infection where cutaneous lesions often represent one of the early signs. A myriad of microorganisms including bacteria, fungi, yeasts, viruses, protozoas, helminths and algae can be implicated. A broad spectrum of clinical and histopathologic findings can be observed in the skin and the common denominator is a thrombotic vasculopathy. The pathogenesis of cutaneous septic vasculitis (SV)/vasculopathy is complex and includes five main mechanisms: disseminated intravascular coagulation, direct invasion and occlusion of blood vessel walls by microorganisms, hypersensitivity reaction with immune complex deposition into blood vessel walls, embolism from a distant infectious site and vascular effects of toxins. Herein we describe the clinicopathologic findings of some selected cases of SV recently observed in our hospital, including purpura fulminans, necrotizing fasciitis, cutaneous meningococcemia, malignant syphilis and disseminated alternaria infection. Histopathologically, a wide spectrum of histopathologic changes was observed in skin specimens from the various entities, involving the intensity and composition of the inflammatory infiltrate, the degree of vascular changes and the presence of microorganisms, that ranged from a predominant not inflammatory, thrombotic-occlusive vasculopathy in purpura fulminans to leukocytoclastic vasculitis like changes in cutaneous meningococcemia to a dermal angiomatosis-like pattern in disseminated Alternaria infection. The different pathologic presentations may be related to the microorganism involved, the main pathogenetic mechanism that induced the vascular injury and the individual immunologic burden. Early skin biopsy for histopathologic examination and microbiologic culture is a cornerstone in the diagnosis of life-threatening diseases that present with cutaneous septic vasculitis. Ancillary techniques, such as immunohistochemistry and polymerase chain reaction are additional novel and helpful tools to identify pathogens, leading to definite diagnosis in cases with challenging or ambiguous clinical and/or pathologic findings. PMID:25592669

  3. 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

  4. Transformation from tubulointerstitial nephritis to crescentic glomerulonephritis: an unusual presentation of ANCA-associated renal vasculitis.

    PubMed

    Wen, Yao-Ko; Chen, Mei-Ling

    2006-01-01

    A 44-year-old man with acute renal failure and antineutrophil cytoplasmic antibodies (ANCA) positivity was described. The first renal biopsy specimen showed tubulointerstitial nephritis (TIN) with normal glomeruli. However, delayed recovery of renal function with low-dose steroid treatment for TIN prompted a second renal biopsy 1 month later; and the specimen demonstrated a dramatically different morphology, with necrotizing and crescentic glomerulonephritis. Improvement in renal function occurred, together with reduction of ANCA titers, following intensive immunosuppressive therapy. This case illustrates an unusual presentation of TIN in ANCA-associated renal vasculitis. The possible pathogenetic mechanism are discussed. PMID:16538980

  5. Generalized alopecia with vasculitis-like changes in a dog with babesiosis.

    PubMed

    Tasaki, Yumi; Miura, Naoki; Iyori, Keita; Nishifuji, Koji; Endo, Yasuyuki; Momoi, Yasuyuki

    2013-10-01

    A locally bred, 12-year-old, intact female Satsuma dog presented with generalized alopecia. Erythema, crusts and desquamation were observed primarily on the truck. Papules and erosions were present in the pinnae, and there were multiple areas of skin necrosis on the right forelimb. The cutaneous lesions had not responded to treatment with systemic antibiotics and prednisolone. The dog also had progressive anemia. Babesia gibsoni was detected in the blood, and the dog was treated with antiprotozoal agents. The skin lesions and anemia improved, but relapsed after the treatment was discontinued. Histopathological examination of skin biopsies revealed findings suggestive of early leukocytoclastic vasculitis or ischemic vasculopathy. PMID:23676299

  6. Therapy insight: The recognition and treatment of retinal manifestations of systemic vasculitis.

    PubMed

    Aristodemou, Petros; Stanford, Miles

    2006-08-01

    A variety of retinal signs can occur in patients who have systemic vasculitides, or who experience complications of these diseases or their treatment. Although treatment of these retinal manifestations is usually the treatment of the systemic disease, specific treatment is occasionally indicated to preserve vision. The more prevalent of the systemic vasculitides are giant cell arteritis, polyarteritis nodosa, Wegener's granulomatosis, Churg-Strauss syndrome, relapsing polychondritis and systemic lupus erythematosus. Less frequently occurring vasculitides include Takayasu's arteritis, Goodpasture's disease, microscopic polyangiitis and Henoch-Schönlein purpura, as well as vasculitis secondary to scleroderma and rheumatoid arthritis. This article describes the pathogenesis, clinical features and treatment of retinal manifestations of systemic vasculitides. PMID:16932736

  7. Generalized Alopecia with Vasculitis-Like Changes in a Dog with Babesiosis

    PubMed Central

    TASAKI, Yumi; MIURA, Naoki; IYORI, Keita; NISHIFUJI, Koji; ENDO, Yasuyuki; MOMOI, Yasuyuki

    2013-01-01

    ABSTRACT A locally bred, 12-year-old, intact female Satsuma dog presented with generalized alopecia. Erythema, crusts and desquamation were observed primarily on the truck. Papules and erosions were present in the pinnae, and there were multiple areas of skin necrosis on the right forelimb. The cutaneous lesions had not responded to treatment with systemic antibiotics and prednisolone. The dog also had progressive anemia. Babesia gibsoni was detected in the blood, and the dog was treated with antiprotozoal agents. The skin lesions and anemia improved, but relapsed after the treatment was discontinued. Histopathological examination of skin biopsies revealed findings suggestive of early leukocytoclastic vasculitis or ischemic vasculopathy. PMID:23676299

  8. Vasculitis and long standing ankylosing spondylitis in a patient with familial Mediterranean fever

    PubMed Central

    Taylan, Ali; Yildiz, Yasar; Sari, Ismail; Ozkok, Guliz

    2014-01-01

    Coexistence of familial Mediterranean fever (FMF) and other inflammatory disorders has been frequently reported, but no specific underlying factor has been identified. We report a patient with FMF who is presented with long-standing ankylosing spondylitis (AS) and cutaneous leukocytoklastic vasculitis (LV) of the lower limbs. It is the first report on combination of FMF with AS and LV. The Mediterranean Fever (MEFV) gene mutation of heterozygote (R202Q/R726A) and HLA-B27 are detected in this case, and are believed to form genetic susceptibility to LV. PMID:25538787

  9. ANCA-positive vasculitis associated with simvastatin/ezetimibe: expanding the spectrum of statin-induced autoimmunity?

    PubMed

    Sen, Deepali; Rosenstein, Elliot D; Kramer, Neil

    2010-08-01

    Although autoimmune syndromes such as systemic lupus erythematosus and dermatomyositis have been previously reported in association with statin use, vasculitis has not been well described. We present a patient with an antineutrophil cytoplasmic antibody-positive, predominantly cutaneous vasculitis, the temporal course of which was associated with simvastatin/ezetimibe use. The patient's serologic findings were consistent with drug-induced disease, with high titer antimyeloperoxidase, in addition to antinuclear and anti-Ro (SSA) antibodies. The patient demonstrated complete resolution of symptoms simply by withdrawing the drug. PMID:20704607

  10. Vasculitis secondary to presumptive leptospirosis treated with long-term corticosteroids in a captive lesser kudu (Tragelaphus imberbis australis).

    PubMed

    Fogelberg, Katherine; Ferrell, Shannon T

    2010-09-01

    A 2-yr-old female lesser kudu (Tragelaphus imberbis australis) was presented for lethargy. Empirical antibiotic treatment appeared to improve its clinical signs, although no etiology for the symptoms was determined. The kudu again presented with lethargy, diffusely swollen limbs, and subcutaneous ecchymoses of 1 day's duration after completion of the initial therapy. Vasculitis secondary to presumptive leptospirosis infection was diagnosed based on a skin biopsy and decreasing paired serologic titers for Leptospira grippotyphosa. The vasculitis was responsive to intramuscular antibiotic therapy and dexamethasone treatment. This case provides evidence that corticosteroids can be used in ruminants at moderate doses for chronic treatment without clinically relevant detrimental effects. PMID:20945659

  11. Varicella zoster-associated retinal and central nervous system vasculitis in a patient with multiple sclerosis treated with natalizumab

    PubMed Central

    2014-01-01

    We report the first case of combined retinal and CNS varicella zoster-associated vasculitis in a 49-year-old patient with multiple sclerosis who had been treated with natalizumab. He presented with a progressive bilateral visual loss. The diagnosis of a vasculitis was based on the fundoscopic examination and MRI findings. We confirmed the varicella zoster virus (VZV) infection of the CNS by PCR and increased intrathecal antibody indices in the cerebrospinal fluid. The patient was stabilized with antiviral treatment, methylprednisolone, plasmapheresis and cycophosphamide. Natalizumab was discontinued. This case illustrates the neuroimmunological and neuroinfectiological consequences of treatments with biologicals that influence the immune system. PMID:24479415

  12. Vasculitis-like hemorrhagic retinal angiopathy in Wegener’s granulomatosis

    PubMed Central

    2013-01-01

    Background Granulomatosis with polyangiitis, also known as Wegener’s granulomatosis, is a chronic systemic inflammatory disease that can also involve the eyes. We report a case of massive retinal and preretinal hemorrhages with perivascular changes as the initial signs in granulomatosis with polyangiitis (Wegener’s granulomatosis). Case presentation A 39-year-old Caucasian male presented with blurred vision in his right eye, myalgia and arthralgia, recurrent nose bleeds and anosmia. Fundus image of his right eye showed massive retinal hemorrhages and vasculitis-like angiopathy, although no fluorescein extravasation was present in fluorescein angiography. Laboratory investigations revealed an inflammation with increased C-reactive protein, elevated erythrocyte sedimentation rate and neutrophil count. Tests for antineutrophil cytoplasmic antibodies (ANCA) were positive for c-ANCA (cytoplasmatic ANCA) and PR3-ANCA (proteinase 3-ANCA). Renal biopsy demonstrated a focal segmental necrotizing glomerulonephritis. Granulomatosis with polyangiitis (Wegener’s granulomatosis) was diagnosed and a combined systemic therapy of cyclophosphamide and corticosteroids was initiated. During 3 months of follow-up, complete resorption of retinal hemorrhages was seen and general complaints as well as visual acuity improved during therapy. Conclusion Vasculitis-like retinal changes can occur in Wegener’s granulomatosis. Despite massive retinal and preretinal hemorrhages that cause visual impairment, immunosuppressive therapy can improve ocular symptoms. PMID:24021028

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

    PubMed

    Yoshida, Naohiro; Iino, Yukiko

    2014-12-01

    Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is histologically characterized by systemic necrotizing vasculitis and is clinically classified into two phases, systemic or localized. Recently, otological symptoms such as otitis media and hearing loss, not previously often associated with AAV, have been reported in AAV cases. In these cases we propose a diagnosis of otitis media with AAV (OMAAV). The ANCA titer is important for the diagnosis of OMAAV, and in most cases rapid progressive hearing loss is observed as localized AAV. Peripheral facial nerve palsy or hypertrophic pachymeningitis are coupled with 25% of cases and 18% of cases respectively. Proteinase 3-ANCA (PR3-ANCA) positive otitis media causes granulomatous formation or middle ear effusion in the middle ear, on the other hand myeloperoxidase-ANCA (MPO-ANCA) positive otitis media predominantly presents as otitis media with effusion. The early diagnosed case and the sensorineural hearing loss not progressed deaf could be recovered by the immunosuppressive therapy. Delayed diagnosis of AAV occasionally leads to progression to the irreversible phase; therefore, diagnosis at the early-localized stage is important for treating AAV. In this review, we discuss the current understanding of this newly proposed concept of OMAAV. PMID:25339433

  14. Immune complex glomerulonephritis and dermal vasculitis following intestinal bypass for morbid obesity.

    PubMed

    Gamble, C N; Kimchi, A; Depner, T A; Christensen, D

    1982-03-01

    A kidney and skin biopsy were performed on a patient who developed cryoglobulinemia, polyarthritis, a purpuric skin rash, and acute renal failure four years following jejunoileal bypass for morbid obesity. Morphologic studies revealed a diffuse glomerulonephritis characterized by the presence of numerous subendothelial deposits containing IgG, IgA, C3, Clq, C4, and properdin, and an acute dermal vasculitis associated with similar immune complex deposits. Identical immunoglobulin and complement components were present in the cryoglobulin. In addition, both the cryoglobulin and a renal biopsy eluate containing anti-IgG antibody and antibody against Klebsiella pneumoniae which were present in the patient's stool in large numbers. Combined therapy with steroids and chloramphenicol resulted in marked improvement in the patient's arthritis, skin rash, and renal function. The findings indicate that glomerulonephritis and dermal vasculitis due to the deposition of bacterial antigen-antibacterial antibody complexes may occur as part of a systemic immune complex disease complicating small intestinal bypass. PMID:7041613

  15. The role of IgM rheumatoid factor in experimental immune vasculitis.

    PubMed Central

    Floyd, M; Tesar, J T

    1979-01-01

    The effect of IgM rhematoid factor (RF) on reversepassive cutaneous Arthus reaction in rats was studied. The RF was obtained from the serum cryoglobulin of a patient with symptoms of purpura, arthralgia and digital gangrene. The cryoglobulins was of IgG-IgM type and when given i.v it induced a prompt hypocomplementaemia in experimental animals. The purified RF also induced low serum complement levels when injected i.v. along with complexes of non-complement-fixing, aggregated IgG. A reverse passive Arthus reaction was induced by intradermal injection of IgG anti-bovine serum albumin (BSA), followed by an i.v. dose of antigen (Ag). The cutaneous inflammatory reaction was aggravated by simultaneous administration of IgM RF intradermally, but not by IgM without antibody (Ab) properties. Intradermal injection of low concentrations of non-complement-fixing IgG anti-BSA, along with normal human IgM, followed by i.v. injection of BSA, resulted in a complete lack of cutaneous inflammation. At higher Ab concentrations there was only a mild inflammation. However, when IgM RF was substituted for normal IgM and injected with non-complement-fixing anti-BSA, an effective reverse passive cutaneous Arthus reaction and vasculitis was induced. The inflammatory response was greatly suppressed by decomplementation of animals by cobra venom factor. This study provides evidence favouring an inflammatory, complement-dependent role for RF in vasculitis. PMID:157238

  16. Hypersensitivity Vasculitis

    MedlinePLUS

    ... with ongoing infections, there may be ongoing or ‘chronic’ symptoms of HV. Drugs used to manage the skin rash and joint pains associated with HV might include corticosteroids and/or nonsteroidal anti-inflammatory drugs. In patients with more severe or ongoing ...

  17. Simultaneous Automated Screening and Confirmatory Testing for Vasculitis-Specific ANCA

    PubMed Central

    Sowa, Mandy; Grossmann, Kai; Knütter, Ilka; Hiemann, Rico; Röber, Nadja; Anderer, Ursula; Csernok, Elena; Bogdanos, Dimitrios P.; Borghi, Maria Orietta; Meroni, Pier Luigi; Schierack, Peter; Reinhold, Dirk; Conrad, Karsten; Roggenbuck, Dirk

    2014-01-01

    Anti-neutrophil cytoplasmic antibodies (ANCA) are the serological hallmark of small vessel vasculitis, so called ANCA-associated vasculitis. The international consensus requires testing by indirect immunofluorescence (IIF) on human ethanol-fixed neutrophils (ethN) as screening followed by confirmation with enzyme-linked immunosorbent assays (ELISAs). This study evaluates the combination of cell- and microbead-based digital IIF analysis of ANCA in one reaction environment by the novel multiplexing CytoBead technology for simultaneous screening and confirmatory ANCA testing. Sera of 592 individuals including 118 patients with ANCA-associated vasculitis, 133 with rheumatoid arthritis, 49 with infectious diseases, 77 with inflammatory bowel syndrome, 20 with autoimmune liver diseases, 70 with primary sclerosing cholangitis and 125 blood donors were tested for cytoplasmic ANCA (C-ANCA) and perinuclear ANCA (P-ANCA) by classical IIF and ANCA to proteinase 3 (PR3) and myeloperoxidase (MPO) by ELISA. These findings were compared to respective ANCA results determined by automated multiplex CytoBead technology using ethN and antigen-coated microbeads for microbead immunoassays. There was a good agreement for PR3- and MPO-ANCA and a very good one for P-ANCA and C-ANCA by classical and multiplex analysis (Cohen's kappa [?]?=?0.775, 0.720, 0.876, 0.820, respectively). The differences between classical testing and CytoBead analysis were not significant for PR3-ANCA, P-ANCA, and C-ANCA (p<0.05, respectively). The prevalence of confirmed positive ANCA findings by classical testing (IIF and ELISA) compared with multiplex CytoBead analysis (IIF and microbead immunoassay positive) resulted in a very good agreement (??=?0.831) with no significant difference of both methods (p?=?0.735). Automated endpoint-ANCA titer detection in one dilution demonstrated a very good agreement with classical analysis requiring dilution of samples (??=?0.985). Multiplexing by CytoBead technology can be employed for simultaneous screening and quantitative confirmation of ANCA. This novel technique provides fast and cost-effective ANCA analysis by automated digital IIF for the first time. PMID:25225805

  18. Lack of association of alpha-1 antichymotrypsin gene polymorphism with PR3-ANCA and MPO-ANCA associated vasculitis.

    PubMed

    Borgmann, Stefan; Haubitz, Marion; Schwab, Sibylle G

    2002-11-01

    In patients with PR3-ANCA associated vasculitides the carrier frequency of alpha1-antitrypsin (AAT) deficiency allele PI*Z is increased and linkage disequilibrium between polymorphic markers within a cluster of serine protease inhibitor (serpin) genes, including AAT gene, at chromosome 14q32.1 has been described. A1-antichymotrypsin (AACT), part of an extended serpin gene cluster, was discussed to contribute to PR3-ANCA associated vasculitis formation. To analyse, if an AACT gene polymorphism within the signal peptide sequence is associatedwith antineutrophil cytoplasm autoantibodies (ANCA) vasculitis allelic frequencies of AACT polymorphism were analysed in 128 control persons, 79 PR3-ANCA, and 30 MPO-ANCA patients. In MPO-ANCA patients also phenotyping of AAT was performed as well as frequency and linkage analysis of simple tandem repeat polymorphisms in the genes of cortisol-binding globulin, AAT, protein C inhibitor, and three extragenic markers (S48, S55, S51) of the gene cluster. Allelic frequencies of the AACT polymorphism did not differ between controls and vasculitis patients. In the MPO-ANCA group no patient expressed the Pi*Z defective allele of AAT and the allelic frequencies of polymorphic markers within the serpin gene cluster did not differ from those of the controls. Strong linkage disequilibrium was detected in MPO-ANCA patients neither. Therefore, we can not support the hypothesis that AACT polymorphism contributes to the pathogenesis of PR3-ANCA vasculitis. Nor is it probable that any factor, coded by the serpin gene cluster, contributes to MPO-ANCA vasculitis. PMID:12685871

  19. 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

  20. Successful rechallenge with reduced dose of erlotinib in a patient with lung adenocarcinoma who developed erlotinib-associated leukocytoclastic vasculitis: A case report

    PubMed Central

    SU, BO-AN; SHEN, WAN-LIN; CHANG, SHENG-TSUNG; FENG, LI-YIA; WU, CHIA-JUNG; FENG, YIN-HSUN

    2012-01-01

    The oral tyrosine kinase inhibitors of epidermal growth factor, erlotinib and gefitinib, are active in the treatment of non-small cell lung cancer (NSCLC). However, a number of skin manifestations have been found in patients receiving erlotinib therapy. Leukocytoclastic vasculitis is a rare side-effect of erlotinib therapy. However, whether or not erlotinib treatment should be continued when disseminated ulceration of leukocytoclastic vasculitis is encountered remains to be determined. In this study, we report a patient with NSCLC who remains responsive to erlotinib treatment following successful rechallenge with a reduced dose of erlonitib after presenting with severe degree of leukoclastic vasculitis. PMID:22783433

  1. Non-infectious cryoglobulinemia vasculitis (CryoVas): update on clinical and therapeutic approach.

    PubMed

    Perez-Alamino, Rodolfo; Espinoza, Luis R

    2014-05-01

    CryoVas is a small vessel vasculitis associated with the presence of circulating cryoglobulins. In the absence of HCV infection, several disorders have been identified in association of CryoVas. Although evidence is limited, a few studies have recently described the clinical presentation, prognosis, and therapeutic management of non-infectious CryoVas. Patients with type I CryoVas, especially associated with hematologic malignancies, have shown a worse clinical presentation. Recent studies have also identified prognostic factors in mixed CryoVas. Therapeutic management in non-infectious CryoVas remains to be defined. Overall, treatment options should be individualized based on severity of involvement. In this setting, new data have emerged regarding the role of biologic therapy in non-infectious CryoVas. Off-label use of rituximab should be highlighted, based on the assessment of benefits and risks, especially infections. PMID:24647999

  2. [Goodpasture syndrome associated with p-ANCA microscopic vasculitis: a rare entity to recognize].

    PubMed

    Pralong, G; Fournier, C; Dayer, E; Meier, P

    2009-06-10

    Some autoimmune diseases may be found presenting simultaneously antibodies (Abs) against basal membrane and anti-neutrophil cytoplasm (ANCA). The clinical picture is that of the Goodpasture syndrome with anti-basement membrane Abs associated with an ANCA-associated small-vessel vasculitis (micro-PAN). The arisen of these two pathological entities is nevertheless too frequent to be the fruit of the only fate. The pathophysiological hypothesis remains that of the micro-PAN initially creates lesions of the basement membrane facilitating the formation of Abs against some constituents of this latter. The prognosis remains controversial, leaving open this issue. This article aims to present a recent literature revue dealing with the simultaneous presence of these two auto-immune diseases. PMID:19626934

  3. Primary and secondary central nervous system vasculitis: clinical manifestations, laboratory findings, neuroimaging, and treatment analysis.

    PubMed

    Vera-Lastra, Olga; Sepúlveda-Delgado, Jesús; Cruz-Domínguez, María Del Pilar; Medina, Gabriela; Casarrubias-Ramírez, Moisés; Molina-Carrión, Luis E; Pineda-Galindo, Luis F; Olvera-Acevedo, Arturo; Hernández-Gonzalez, Claudia; Jara, Luis J

    2015-04-01

    The objectives of this study are to compare the initial clinical, laboratory, and imaging features in primary central nervous system vasculitis (PCNSV) vs secondary central nervous system vasculitis (SCNSV) and follow up after treatment with intravenous cyclophosphamide (IV-CYC) plus glucocorticosteroids (GCS): methylprednisolone (MP). Neurological, laboratory, and neuroimaging findings were analyzed in PCNSV and SCNSV patients. Cerebral biopsy (CB) was performed in nine patients. Both groups received at onset MP plus IV-CYC for 6 months, followed by bimonthly IV-CYC plus prednisone (PND) for 12 months. All patients were followed during 36 months. Thirty patients were included (12 PCNSV and 18 SCNSV). Focal and non-focal neurological manifestations were similar in both groups, headache being the most frequent manifestation in both groups. Fatigue, myalgias, arthralgias, neuropathy, low leukocytes and platelets, elevated erythrocyte sedimentation rate, positive antinuclear antibodies (ANA), anti-double-stranded DNA (dsDNA), antineutrophil cytoplasmic antibodies (ANCA), low complement, and rheumatoid factor were more frequent in SCNSV (p?

  4. Infliximab leads to a rapid but transient improvement in endothelial function in patients with primary systemic vasculitis

    PubMed Central

    Raza, K; Carruthers, D M; Stevens, R; Filer, A D; Townend, J N; Bacon, P A

    2006-01-01

    Objective To assess the immediate effects of tumour necrosis factor ? (TNF?) blockade on endothelial function in systemic vasculitis. Methods Endothelial function was assessed by laser Doppler flowmetry in patients with active vasculitis after 10 infusions of infliximab. For comparison endothelial responses were assessed after five infusions of cyclophosphamide plus methylprednisolone. Results Endothelial dependent vasodilatation (EDV) improved significantly within 24?hours of infliximab infusion. The median change in red blood cell flux (interquartile range) was 5.7 (4.3–8.2) before infusion v 8.4 (7.5–10.9) at 24?hours; p?=?0.027. This was not maintained at day 14. No improvement was seen in EDV after cyclophosphamide plus methylprednisolone infusion. Conclusion The rapid but transient improvement in EDV after TNF? inhibition suggests that TNF? may have a direct role in the impairment of endothelial function. PMID:16361277

  5. PR3ANCA Related Cerebral Vasculitis in Ulcerative Colitis Presenting with Orbital Involvement: A Case Report with Review of Literature

    PubMed Central

    Uchiyama, Robert C.

    2014-01-01

    PR3 ANCA is a classic marker of granulomatosis with polyangiitis (GPA). There have been several recent reports of increased prevalence of PR3ANCA in ulcerative colitis (UC) patients, the clinical implication of which is not well defined. We are reporting a case of 27-year-old Caucasian male with 14-year history of UC presenting with unilateral proptosis, conjunctival congestion, and chemosis who developed acute hemiparesis within three days of hospital admission, followed by rapid neurological deterioration correlating with brain imaging findings. Serologically he had atypical PANCA with high PR3 antibody titer with a negative infectious workup. His cerebral angiogram was normal but the brain biopsy showed necrotizing vasculitis. He was diagnosed with PR3 ANCA mediated cerebral and orbital vasculitis associated with UC. Treatment was initiated with high dose steroids, plasmapheresis, and cyclophosphamide. He improved significantly with residual left hemiparesis. PMID:25097791

  6. A case of necrotizing vasculitis with panniculitis, during sorafenib treatment for hepatocellular carcinoma, appeared in disease progression

    PubMed Central

    Ragazzi, Moira; Asensio, Nuria Maria; Pagano, Maria; Gnoni, Roberta; Boni, Corrado

    2014-01-01

    Hepatocellular carcinoma (HCC) is the sixth most common cancer worldwide and is the third most common cause of death from cancer. Sorafenib is the only drug which improves survival in first line advanced HCC. Sorafenib has been associated with several dermatologic toxicities and toxic effects have been related to a better treatment response. We report the case of a well-circumscribed panniculitis and necrotizing vasculitis due to sorafenib, appeared in disease progression in a man affected by advanced HCC. PMID:25436135

  7. Long-Term Maintenance Therapy Using Rituximab-Induced Continuous B-Cell Depletion in Patients with ANCA Vasculitis

    PubMed Central

    Pendergraft, William F.; Cortazar, Frank B.; Wenger, Julia; Murphy, Andrew P.; Rhee, Eugene P.; Laliberte, Karen A.; Niles, John L.

    2014-01-01

    Background and objectives Remission in the majority of ANCA vasculitis patients is not sustained after a single course of rituximab, and risk of relapse warrants development of a successful strategy to ensure durable remission. Design, setting, participants, & measurements A retrospective analysis of ANCA vasculitis patients who underwent maintenance therapy using rituximab-induced continuous B-cell depletion for up to 7 years was performed. Maintenance therapy with rituximab was initiated after achieving remission or converting from other prior maintenance therapy. Continuous B-cell depletion was achieved in all patients by scheduled rituximab administration every 4 months. Disease activity, serologic parameters, adverse events, and survival were examined. Results In the study, 172 patients (mean age=60 years, 55% women, 57% myeloperoxidase–ANCA) treated from April of 2006 to March of 2013 underwent continuous B-cell depletion with rituximab. Median remission maintenance follow-up time was 2.1 years. Complete remission (Birmingham Vasculitis Activity Score [BVAS]=0) was achieved in all patients. Major relapse (BVAS?3) occurred in 5% of patients and was associated with weaning of other immunosuppression drugs. Remission was reinduced in all patients. Survival mirrored survival of a general age-, sex-, and ethnicity-matched United States population. Conclusion This analysis provides evidence for long-term disease control using continuous B-cell depletion. This treatment strategy in ANCA vasculitis patients also seems to result in survival rates comparable with rates in a matched reference population. These findings suggest that prospective remission maintenance treatment trials using continuous B-cell depletion are warranted. PMID:24626432

  8. Renal cell carcinoma presenting as Henoch–Schönlein purpura with leukocytoclastic vasculitis, hematuria, proteinuria and abdominal pain

    Microsoft Academic Search

    Young Hoon Hong

    2010-01-01

    Some infectious, rheumatic, allergic diseases, and malignancies have been associated with leucocytoclastic vasculitis (LCV).\\u000a LCV and cancer occur most frequently in patients with hematological malignancies such as lymphomas and leukemias. There have\\u000a been a few prior cases reported of LCV associated with renal cell carcinoma (RCC). A 25-year-old male patient was referred\\u000a from the department of dermatology and nephrology because

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

    PubMed

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

    2014-01-01

    We report the case of a 45-year-old patient who presented with acute dilated cardiomyopathy. During admission the patient was consecutively diagnosed with cryoglobulinaemic vasculitis and beriberi. In both diseases, cardiac involvement may occur as dilated cardiomyopathy. Thiamin deficiency was the final cause for the severe cardiac manifestations (cardiac acute beriberi or Shoshin syndrome), which returned to normal after thiamin supplementation. PMID:24429381

  10. Cutaneous leukocytoclastic vasculitis due to Salmonella enteritidis in a child with interleukin-12 receptor beta-1 deficiency.

    PubMed

    Filiz, Serkan; Kocacik Uygun, Dilara F; Verhard, Els M; van Dissel, Jaap T; Uygun, Vedat; Bassorgun, Cumhur; Bingol, Aysen; Yegin, Olcay; van de Vosse, Esther

    2014-01-01

    Defects in the interleukin 12 (IL-12)/interferon gamma (IFN-?) pathway result in Mendelian susceptibility to mycobacterial disease (MSMD). IL-12 receptor beta 1 (IL-12R?1) deficiency, the most common form of MSMD, is associated with weakly virulent mycobacteria and salmonella. Infections in patients with this deficiency are extraintestinal, or septicemic, recurrent infections with nontyphoid salmonellae. Here we report a case of an IL-12R?1 deficiency with cutaneous leukocytoclastic vasculitis due to Salmonella enteritidis. PMID:23004925

  11. Unusual cause of acute neurologic deficit in childhood: primary central nervous system vasculitis presenting with basilar arterial occlusion

    Microsoft Academic Search

    Eren Kale Çekinmez; Nurcan Cengiz; ?lknur Erol; Osman K?z?lk?l?ç; Yasemin Uslu

    2009-01-01

    Introduction  Primary central nervous system (CNS) vasculitis of childhood is a rare disorder. The most common signs and symptoms are acute\\u000a severe headache and focal neurologic deficit. It should be suspected in children who have an acquired neurologic deficit that\\u000a remains unexplained after an initial basic evaluation. Diagnosis usually depends on brain magnetic resonance imaging and conventional\\u000a angiography of cerebral vasculature.

  12. 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.

  13. Mycobacterium avium-intracellulare pulmonary infection complicated by cutaneous leukocytoclastic vasculitis in a woman with anorexia nervosa.

    PubMed

    Walsh, T L; Baca, V; Stalling, S S; Natalie, A A; Veldkamp, P J

    2014-06-01

    A 53-year-old Caucasian woman with a history of anorexia nervosa developed a bilateral lower extremity rash comprised of palpable red to violaceous, sub-centimeter papular lesions that increased in quantity rapidly. She also noted a 2-month history of non-productive cough. Imaging modalities revealed a thin-walled cavitary lesion in the right lung apex and scattered nodular opacities. Acid fast bacilli (AFB) were found in sputum and subsequently identified by culture as Mycobacterium avium-intracellulare (MAI). Punch biopsies of her skin lesions yielded a histological diagnosis of small-to-medium vessel vasculitis. Stains and cultures for organisms were negative. Her skin lesions resolved quickly after the initiation of antimicrobial therapy for MAI. Hypersensitivity vasculitis associated with an atypical mycobacterial infection is unusual. The postulated underlying mechanism is the deposit of immune complexes and not the bacillus itself. While cutaneous leukocytoclastic vasculitis (CLV) due to MAI is certainly a rare entity, it should be entertained in patients with vasculitic skin lesions and a concomitant pulmonary disease. PMID:24363210

  14. Relapsing polychondritis with p-ANCA associated vasculitis: Which triggers the other?

    PubMed

    File, Ibolya; Trinn, Csilla; Mátyus, Zsolt; Ujhelyi, László; Balla, József; Mátyus, János

    2014-12-16

    Relapsing polychondritis (RP) is a rare autoimmune disease with chronic inflammatory/destructive lesions of the cartilaginous tissues. In one third of the cases it is associated with other autoimmune disorders, mostly with anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). We report three cases of RP with p-ANCA positive AAV. In the first patient RP developed 1.5 years after the onset of AAV. In the others the signs of RP were present before the onset of severe crescent glomerulonephritis. Patients responded well on steroid and cyclophosphamide. In dialysis dependent cases plasmapheresis was also used successfully. During the 2 and 1.5 years of follow up, they were symptom-free, and had stable glomerular filtration rate. The first patient died after four years of follow-up due to the complications of sudden unset pancytopenia, which raises the possibility of associated hemophagocytic syndrome. In the setting of RP or AAV physicians should always be aware of the possibility of sudden or insidious appearance of the other disease. PMID:25516870

  15. Presumed reactive polyarthritis and granulomatous vasculitis in a Mississippi sandhill crane (Grus canadensis pulla).

    PubMed

    MacLean, Robert; Beaufrère, Hugues; Heggem-Perry, Brittany; Field, Cara; Garner, Michael

    2013-12-01

    A 1.5-year-old female Mississippi sandhill crane (Grus canadensis pulla) was presented and managed for a polyarthritis of the intertarsal and tarsophalangeal articulations. Results of aerobic bacterial cultures, Mycoplasma species culture, and polymerase chain reaction testing of articular fluid did not identify any causative organisms. Results of radiographs and cytologic examination of articular fluid were consistent with an inflammatory, nonerosive polyarthritis. The arthritis did not improve with systemic anti-inflammatory and antibiotic treatment and with joint lavage. A large necrotic granulomatous mass was detected on the right shoulder area from which Staphylococcus aureus and Enterococcus species were isolated as opportunistic pathogens. Two days after surgical resection of the mass, the distal polyarthritis resolved. Histopathologic examination of the mass was consistent with granulomatous vasculitis with abscess formation of unknown origin. In this crane, the unresponsiveness to standard therapy, the presence of an infected and inflammatory mass, and the resolution of the polyarthritis after the resection of the mass strongly supported a diagnosis of reactive immune-mediated nonerosive polyarthritis. Analysis of this case suggests that immune-mediated idiopathic arthritis should be a differential diagnosis of distal polyarthritis in cranes and that an inciting source remote from the joints should be investigated in case of lack of response to standard therapy. PMID:24640933

  16. Relapsing polychondritis with p-ANCA associated vasculitis: Which triggers the other?

    PubMed Central

    File, Ibolya; Trinn, Csilla; Mátyus, Zsolt; Ujhelyi, László; Balla, József; Mátyus, János

    2014-01-01

    Relapsing polychondritis (RP) is a rare autoimmune disease with chronic inflammatory/destructive lesions of the cartilaginous tissues. In one third of the cases it is associated with other autoimmune disorders, mostly with anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). We report three cases of RP with p-ANCA positive AAV. In the first patient RP developed 1.5 years after the onset of AAV. In the others the signs of RP were present before the onset of severe crescent glomerulonephritis. Patients responded well on steroid and cyclophosphamide. In dialysis dependent cases plasmapheresis was also used successfully. During the 2 and 1.5 years of follow up, they were symptom-free, and had stable glomerular filtration rate. The first patient died after four years of follow-up due to the complications of sudden unset pancytopenia, which raises the possibility of associated hemophagocytic syndrome. In the setting of RP or AAV physicians should always be aware of the possibility of sudden or insidious appearance of the other disease. PMID:25516870

  17. 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

  18. [A case with juvenile rheumatoid arthritis who developed cerebral vasculitis and venovascular hypertension].

    PubMed

    Yamamoto, T; Abe, T; Mastuya, M; Kobayashi, K; Akaike, J; Ikeda, Y; Hosokawa, A; Shimoji, H; Sakai, H; Yonezawa, K; Tosaka, M; Imai, K; Matsumoto, H

    2000-10-01

    On November, 1997, a 15-year-old boy visited our hospital because of headache, fever and arthralgia. He was treated with 5 mg/day of prednisolone thereafter. On October 21, 1998, he was admitted because of remittent fever and multiple arthralgia and diagnosis of juvenile rheumatoid arthritis (JRA) was made. He was also found to have hypertension of 210/110 mmHg, and soon developed ptosis of the eye, facial paresis and perceptive deafness of the right side. Cerebrospinal fluid showed protein of 98 mg/dl and mildly elevated IgG, IgA and IgM levels with normal cell count. Brain MRI examination revealed multiple cerebral lesions in the frontal, parietal and cerebellar areas on the right, whose cause was thought to be vasculitis. Renal angiography demonstrated a right renal artery stenosis, compatible with renovascular hypertension. He was treated with 60 mg of prednisolone per day, which brought about a satisfactory improvement of the above rheumatic and neurologic signs. On November 17, 1998, he received a follow-up study of MRI, which failed to show any cerebral lesions, supporting the effectiveness of prednisolone. An angiotensin converting enzyme inhibitor successfully normalized hypertension and renin activity in serum, although renal blood flow did not increase. PMID:11215160

  19. Leukocytoclastic vasculitis masking chronic vascular changes in previously undiagnosed erythropoietic protoporphyria.

    PubMed

    Thom, Graham; Lam, Minh; Lucas, Michaela; Rossi, Enrico

    2013-11-01

    A 31-year old man presented with swelling and purpura of the dorsum of the hands following sun exposure. There was a preceding lifelong history of photosensitivity, but this episode, which occurred after the recent commencement of oral iron therapy, and after recent alcohol ingestion, was much more severe than any preceding episode. Skin biopsy performed 48 h after the onset of symptoms showed features consistent with the early stages of leukocytoclastic vasculitis. Direct immunofluorescence showed homogeneous thick staining of the vessel walls with IgG, IgM and IgA, together with abundant perivascular fibrinogen. A subsequent periodic acid-Schiff (PAS) stain on the skin biopsy revealed thickening of the walls of dermal vessels, which was not discernible in routinely stained (hematoxylin/eosin) sections. The diagnosis of erythropoietic protoporphyria (EPP) was confirmed by significantly elevated erythrocyte protoporphyrin levels and positive plasma fluorimetry. The diagnosis of porphyria may have been missed by routine skin microscopy if not for the additional information provided by clinical history, direct immunofluorescence and PAS stain. The pathogenesis and histopathology of acute and chronic vascular changes in EPP are reviewed. PMID:24102632

  20. Clinical diagnosis and management of large vessel vasculitis: giant cell arteritis.

    PubMed

    Chatterjee, Soumya; Flamm, Scott D; Tan, Carmela D; Rodriguez, E Rene

    2014-07-01

    Large vessel vasculitis (LVV) covers a spectrum of primary vasculitides predominantly affecting the aorta and its major branches. The two main subtypes are giant cell arteritis (GCA) and Takayasu arteritis (TA). Less commonly LVV occurs in various other diseases. Clinical manifestations result from vascular stenosis, occlusion, and dilation, sometimes complicated by aneurysm rupture or dissection. Occasionally LVV is discovered unexpectedly on pathological examination of a resected aortic aneurysm. Clinical evaluation is often unreliable in determining disease activity. Moreover, the diagnostic tools are imperfect. Acute phase reactants can be normal at presentation and available imaging modalities are more reliable in delineating vascular anatomy than in providing reliable information on degree of vascular inflammation. Glucocorticoids are the mainstay of therapy of LVV. Patients may develop predictable adverse effects from long-term glucocorticoid use. Several steroid-sparing agents have also shown some promise and are currently in use. Endovascular revascularization procedures and open surgical treatment for aneurysms and dissections are sometimes necessary, but results are not always favorable and relapses are common. This article, the first in a series of two, will be devoted to GCA and isolated (idiopathic) aortitis, while TA will be covered in detail in the next article. PMID:24893935

  1. Acute abdominal pain in systemic lupus erythematosus: focus on lupus enteritis (gastrointestinal vasculitis)

    PubMed Central

    Lee, C; Ahn, M; Lee, E; Shin, J; Cho, Y; Ha, H; Yoo, B; Moon, H

    2002-01-01

    Methods: A retrospective study was carried out for all patients admitted with SLE from 1993 to March 2001. The SLEDAI and laboratory data were collected at the time of diagnosis of SLE and at the time of acute abdominal pain. Lupus enteritis (gastrointestinal vasculitis) was diagnosed by clinical investigation and abdominal computed tomographic findings. Results: Chart review identified 175 patients (20 male, 155 female) who had been admitted with SLE. Of these patients, 38 (22%) presented with acute abdominal pain. Lupus enteritis was the most common cause of acute abdominal pain. Patients were divided into three groups: group 1: lupus enteritis (n=17), group 2: acute abdominal pain without lupus enteritis (n=21), and group 3: SLE without acute abdominal pain (n=137). There was no difference in age and sex among the three groups. Antiphospholipid, anti-RNP, anti-Sm, anti-Ro, and anti-La antibodies did not differ among the three groups. There was no difference in the SLEDAI at the time of diagnosis and at the time of acute abdominal pain between groups 1 and 2. Complement, erythrocyte sedimentation rate, C reactive protein, and anti-dsDNA measured at the time of acute abdominal pain did not differ between groups 1 and 2. A drop in the white blood cell count at the time of abdominal pain was more prominent in group 1 than group 2. In lupus enteritis, the jejunum and ileum were the sites most commonly affected. Rectal involvement was rare. Even though four patients relapsed, all the patients with lupus enteritis, including those who relapsed, responded well to corticosteroid. Conclusion: Lupus enteritis is the most common cause of acute abdominal pain in SLE. All patients with lupus enteritis responded well to a high dose of a corticosteroid without surgical intervention. The SLEDAI and laboratory data, except leucopenia, do not correlate with the occurrence of lupus enteritis. PMID:12006332

  2. ANCA Associated Vasculitis and Renal Failure Related to Propylthiouracil and Hyperthyroidism Induced Cholestasis in the Same Case

    PubMed Central

    Tuncay, Mehmet; Kivrakoglu, Emine; Yegenaga, Itir; Dervisoglu, Erkan

    2014-01-01

    Introduction. Liver involvement due to hyperthyroidism and also ANCA positive vasculitis related renal failure cases were reported separately several times before. However, to our knowledge, these two complications together in the same case had never been observed before. Case Presentation. The case of an ANCA positive 71-year-old Caucasian male with renal failure and lung involvement, subclinical hyperthyroidism, and intrahepatic cholestatic jaundice was presented in this paper. After exclusion of all of the other possibilities, cholestatic hepatitis was explained by subclinical hyperthyroidism; renal failure and lung involvement were interpreted as ANCA related vasculitis which might be a side effect of propylthiouracil use. Conclusion. The coexistence of these rare conditions in the same patient deserves emphasis and it is worth reporting. This case demonstrates that following the clinical course of the patient is essential after prescribing any medications to see whether any complication occurs or not. If the complications of this case were noticed earlier, it would be possible to treat and to prevent the permanent damages. PMID:25506446

  3. Successful outcome of pregnancy in patients with anti-neutrophil cytoplasm antibody-associated small vessel vasculitis.

    PubMed

    Croft, Adam P; Smith, Stuart W; Carr, Sue; Youssouf, Sajeda; Salama, Alan D; Burns, Aine; Pusey, Charles D; Hamilton, Patrick; Brown, Nina; Venning, Michael; Harper, Lorraine; Morgan, Matthew D

    2015-04-01

    Pregnancy in patients with anti-neutrophil cytoplasm antibody-associated vasculitis is reportedly associated with a high risk of fetal and maternal complications. Here we describe the outcome of pregnancies in patients with granulomatosis with polyangiitis and microscopic polyangiitis at five centers in the United Kingdom using a retrospective case review of all women who became pregnant following diagnosis. We report 15 pregnancies in 13 women resulting in 15 live births including one twin pregnancy and 13 singleton pregnancies. One patient had an unplanned pregnancy and a first trimester miscarriage while taking methotrexate. All other pregnancies were planned following a minimum of 6 months clinical remission. Eleven successful pregnancies were delivered vaginally at full term, whereas three were delivered by cesarean section. All infants were healthy with no neonatal complications on their initial health check within the first 24?h of delivery and no evidence of neonatal vasculitis. One relapse occurred during pregnancy and was successfully treated with an increased dose of azathioprine and corticosteroids, intravenous immunoglobulin, and plasma exchange therapy. One patient developed tracheal crusting and subglottic stenosis of infective etiology in the third trimester requiring tracheal debridement post delivery. No patient had a relapse in the first 12 months postpartum. Thus, successful pregnancy outcomes can occur following planned pregnancy in women in sustained remission on non-teratogenic therapies. PMID:25272233

  4. Classification and characteristics of Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study

    PubMed Central

    2014-01-01

    Introduction We investigated the clinical and serological features of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in Japan using data from a nationwide, prospective, inception cohort study. Methods In total, 156 Japanese patients with newly diagnosed AAV were classified according to the European Medicines Agency (EMEA) algorithm with exploratory surrogate markers for AAV-related non-granulomatous pulmonary lesions, predefined as alveolar haemorrhage and interstitial lung disease (ILD), and their clinical and serological features were evaluated. Results Using the EMEA algorithm, we identified 14 patients (9.0%) with eosinophilic granulomatosis with polyangiitis (EGPA), 33 (21.2%) with granulomatosis with polyangiitis (GPA), 78 (50.0%) with microscopic polyangiitis and renal-limited vasculitis (MPA/RLV), and 31 (19.9%) with unclassifiable vasculitis. The average ages of patients with EGPA (male/female, 5/9), GPA (12/21), and MPA/RLV (35/43) and unclassifiable (9/22) were 58.0, 63.6, 71.1, and 70.6 years, respectively. Myeloperoxidase (MPO)-ANCA and proteinase-3 ANCA positivity was 50.0% and 0% for EGPA, 54.6% and 45.5% for GPA, 97.4% and 2.6% for MPA/RLV, and 93.5% and 3.2% for unclassifiable, respectively. According to the Birmingham Vasculitis Activity Score (BVAS), cutaneous (71.4%) and nervous system (92.9%) manifestations were prominent in EGPA and ear, nose, and throat manifestations (84.9%) and chest manifestations (66.7%) in GPA. Renal manifestations developed frequently in MPA/RLV (91.0%) and GPA (63.6%). The average serum creatinine levels were 0.71 mg/dL for EGPA, 1.51 mg/dL for GPA, 2.46 mg/dL for MPA/RLV, and 0.69 mg/dL for unclassifiable. The percentages of patients with ILD were 14.3% for EGPA, 9.0% for GPA, 47.4% for MPA/RLV, and 61.3% for unclassifiable. Patients with ILD (n?=?61) had significantly lower BVAS (P?=?0.019) with fewer ear, nose, and throat and cardiovascular manifestations than patients without ILD (n?=?95). Conclusions MPO-ANCA-positive MPA/RLV is the most common form of AAV in Japanese patients, and one-half of patients with GPA were positive for MPO-ANCA. ILD is an important clinical manifestation in Japanese patients with AAV. Unclassifiable vasculitis with MPO-ANCA positivity and ILD may represent a novel variant of MPA. Trial Registration The University Hospital Medical Information Network Clinical Trials Registry: UMIN000001648. Registered 28 February 2009. PMID:24758294

  5. 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...

  6. Urinary Levels of High Mobility Group Box-1 Are Associated with Disease Activity in Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis

    PubMed Central

    Wang, Chen; Chang, Dong-Yuan; Zhao, Ming-Hui; Chen, Min

    2015-01-01

    Background High mobility group box-1 (HMGB1), a kind of pro-inflammatory mediator, is associated with inflammatory conditions and tissue damage. Our previous study demonstrated that the circulating levels of HMGB1 correlated with disease activity of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). In the current study, we aimed to measure urinary levels of HMGB1 in AAV patients, correlated them to clinical activity index and analysed the immunohistochemical HMGB1 staining in kidney specimens. Methods 50 patients with AAV in active stage and 56 patients with AAV in remission were recruited. The urinary levels of HMGB1 were determined by enzyme-linked immunosorbent assay. Moreover, renal biopsy specimens from 27 patients with active AAV were randomly collected to evaluate the deposition of HMGB1. Results Urinary HMGB1 levels in AAV patients in active stage were significantly higher than those in AAV patients in remission and healthy controls (1.46 [0.56-3.43] versus 0.38 [0.10-1.35] mg/?molCr, P=0.001; 1.46 [0.56-3.43] versus 0.48 [0.40-0.60] mg/?molCr, P=0.000, respectively). Further analysis found that urinary levels of HMGB1 correlated with erythrocyte sedimentation rate (r=0.354, p=0.012), C-reactive protein (r=0.289, p=0.042), and Birmingham Vasculitis Activity Score (r=0.350, p=0.013). Renal tissue of active AAV patients showed HMGB1 was mainly expressed in the cytoplasm and the extracellular space. The percentage of HMGB1-negative nuclei in renal tissue of patients with active AAV was significantly higher than that in normal controls (60.6±20.2 % versus 2.7±0.6 %, p<0.01). Conclusion Urinary levels of HMGB1 may be associated with the disease activity in AAV patients. PMID:25884225

  7. Systemic vasculitis with prolonged pyrexia, recurrent facial urticaria, skin nodules, pleural effusions and venous thrombosis: an unusual presentation of an uncommon disease

    PubMed Central

    Hassan, Imad Salah; Dar, Javeed

    2011-01-01

    Classically presenting with multiple or single peripheral cytopenias of variable severity, the myelodysplastic syndromes may occasionally present with bizarre manifestations that confuse the clinical picture and result in significant delays in making the correct diagnosis. We describe the case of an elderly male patient whose presentation with prolonged unexplained fever coupled with cutaneous, pulmonary and other systemic features of inflammation was finally diagnosed as having a primary myelodysplastic syndrome with associated vasculitis after a delay of 4 years. PMID:22031795

  8. Tissue microscopic changes and artifacts in multi-phase post-mortem computed tomography angiography in a hospital setting: a fatal case of systemic vasculitis.

    PubMed

    Capuani, Caroline; Guilbeau-Frugier, Céline; Mokrane, Fatima-Zohra; Delisle, Marie-Bernadette; Marcheix, Bertrand; Rousseau, Hervé; Telmon, Norbert; Rougé, Daniel; Dedouit, Fabrice

    2014-09-01

    A 27-year-old man suddenly died in hospital of acute respiratory distress syndrome secondary to severe systemic vasculitis. Multi-phase post-mortem computed tomography angiography followed by scientific autopsy of the thoracic and abdominal cavity and histology was performed, illustrating the advantages and drawbacks of such techniques. Imaging enabled us to examine the cranium, as the family refused cerebral dissection. MPMCTA revealed absence of opacification of the left middle cerebral artery. But parenchymal findings of thoracic and abdominal organs were still difficult to interpret after both imaging and macroscopic examination during the autopsy. Microscopic examination provided the definitive diagnosis of cause of death. Analysis revealed systemic vasculitis of the lung complicated by diffuse alveolar, mediastinal, splenic and retroperitoneal lesions. We were unable to determine the type of vasculitis, whether polyarteritis nodosa or microscopic polyangiitis, because of artifactual glomerular collapse. We observed some structural changes in tissue secondary to contrast agent injection, affecting the vascular system and renal parenchyma in particular. Such artifacts must be known in order to avoid misinterpreting them as pathological findings. MPMCTA and conventional autopsy are two complementary techniques showing both their specific advantages and limits which have to be known in order to choose the appropriate technique. One limit of both techniques is the detection of microscopic findings which can only be obtained by additional histological examination. This case report underlines this fact and demonstrates that caution is required in some cases if microscopic analyses are carried out after contrast agent injection. PMID:25085763

  9. Levamisole-induced leukocytoclastic vasculitis and neutropenia in a patient with cocaine use: An extensive case with necrosis of skin, soft tissue, and cartilage

    PubMed Central

    2012-01-01

    Levamisole-induced vasculitis is a relatively new entity in people who use cocaine. We describe a 44-year-old woman with a history of cocaine use who presented with a complaint of a painful rash of 2-3 month’s duration on her extremities, cheeks, nose, and earlobes. She had not experienced fever, weight loss, alopecia, dry eyes, oral ulcers, photosensitivity, or arthralgia. Examination revealed tender purpuric eruptions with central necrosis on her nose, cheeks, earlobes, and extremities. Laboratory investigations revealed neutropenia, an elevated erythrocyte sedimentation rate (ESR), presence of lupus anticoagulant, low complement component 3 (C3), and presence of perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA). A urine toxicology screen was positive for cocaine, and gas chromatography–mass spectrometry was positive for levamisole. Skin biopsy showed leukocytoclastic vasculitis and small vessel thrombosis. Necrotic lesions of the nose led to its self-amputation. Large bullae on the lower extremities ruptured, leading to wound infection and extensive necrosis that required multiple surgical debridements. When necrosis progressed despite debridement, bilateral above-knee amputation of the legs was performed. Once new lesions stopped appearing, the patient was discharged home. Two months later, she had a recurrence related to cocaine use. To the best of our knowledge, this is only the second reported case of levamisole-induced vasculitis that required above-knee amputation. PMID:23186390

  10. 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

  11. Anti-idiotypes against anti-neutrophil cytoplasmic antigen autoantibodies in normal human polyspecific IgG for therapeutic use and in the remission sera of patients with systemic vasculitis.

    PubMed Central

    Rossi, F; Jayne, D R; Lockwood, C M; Kazatchkine, M D

    1991-01-01

    Anti-neutrophil cytoplasmic antigen (ANCA) activity was inhibited in 15 out of 21 sera from patients with acute systemic vasculitis following incubation with normal polyspecific IgG for therapeutic use (IVIg). ANCA antibodies reacted with IVIg through idiotypic-anti-idiotypic interactions, as shown in competitive binding assays using F(ab')2 fragments from IVIg and affinity chromatography of ANCA IgG on Sepharose-bound F(ab')2 fragments from IVIg. Co-incubation of sera from patients with acute systemic vasculitis with paired autologous remission stage sera also resulted in inhibition of ANCA activity in acute sera. Remission sera contain IgM and IgG capable of interacting with beta and or alpha idiotypes of ANCA IgG from acute sera. Anti-idiotypic IgM may account for the lack of expression of ANCA activity in whole serum from patients in remission from systemic vasculitis, which were found to contain high titres of ANCA IgG. These observations suggest that remission of systemic vasculitis is associated with the generation of anti-idiotypes against autoantibodies rather than the suppression of production of ANCA autoantibodies. IVIg may modulate the activity of systemic vasculitis in vivo. PMID:1993362

  12. Establishment of a vascular endothelial cell-reactive type II NKT cell clone from a rat model of autoimmune vasculitis.

    PubMed

    Iinuma, Chihiro; Waki, Masashi; Kawakami, Ai; Yamaguchi, Madoka; Tomaru, Utano; Sasaki, Naomi; Masuda, Sakiko; Matsui, Yuki; Iwasaki, Sari; Baba, Tomohisa; Kasahara, Masanori; Yoshiki, Takashi; Paletta, Daniel; Herrmann, Thomas; Ishizu, Akihiro

    2015-02-01

    We previously generated a rat model that spontaneously developed small vessel vasculitis (SVV). In this study, a T cell clone reactive with rat vascular endothelial cells (REC) was established and named VASC-1. Intravenous injection of VASC-1 induced SVV in normal recipients. VASC-1 was a TCR??/CD3-positive CD4/CD8 double-negative T cell clone with expression of NKG2D. The cytokine mRNA profile under unstimulated condition was positive for IL-4 and IFN-? but negative for IL-2 and IL-10. After interaction with REC, the mRNA expression of IL-2, IL-5 and IL-6 was induced in VASC-1, which was inhibited by blocking of CD1d on the REC surface. Although the protein levels of these cytokines seemed to be lower than the detection limit in the culture medium, IFN-? was detectable. The production of IFN-? from the VASC-1 stimulated with LPS-pre-treated REC was inhibited by the CD1d blockade on the REC. These findings indicated VASC-1 as an NKT cell clone. The NKT cell pool includes two major subsets, namely types I and II. Type I NKT cells are characterized by expression of semi-invariant TCRs and the potential to bind to marine sponge-derived ?-galactosylceramide (?-GalCer) loaded on CD1d; whereas, type II NKT cells do not manifest these characteristics. VASC-1 exhibited a usage of TCR other than the type I invariant TCR ? chain and did not bind to ?-GalCer-loaded CD1d; therefore, it was determined as a type II NKT cell clone. The collective evidence suggested that REC-reactive type II NKT cells could be involved in the pathogenesis of SVV in rats. PMID:25239132

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

    PubMed

    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

  14. 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

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

    PubMed

    Nelson, Danielle D; Taus, Naomi S; Schneider, David A; Cunha, Cristina W; Davis, William C; Brown, Wendy C; Li, Hong; O'Toole, Donal; Oaks, J Lindsay

    2013-10-25

    American bison (Bison bison) are particularly susceptible to developing fatal sheep-associated malignant catarrhal fever (SA-MCF) caused by ovine herpesvirus-2 (OvHV-2), a ?-herpesvirus in the Macavirus genus. This generally fatal disease is characterized by lymphoproliferation, vasculitis, and mucosal ulceration in American bison, domestic cattle (Bos taurus), and other clinically susceptible species which are considered non-adapted, dead-end hosts. The pathogenesis and cellular tropism of OvHV-2 infection have not been fully defined. An earlier study detected OvHV-2 open reading frame 25 (ORF25) transcripts encoding the viral major capsid protein in tissues of bison with SA-MCF, and levels of viral transcript expression positively correlated with lesion severity. To further define the cellular tropism and replication of OvHV-2 infection in vascular lesions of bison, immunofluorescence studies were performed to identify cell type(s) expressing ORF25 protein within tissues. Cytoplasmic and not nuclear ORF25 protein was demonstrated in predominantly perivascular fibroblasts in six bison with experimentally-induced SA-MCF, and there was no evidence of immunoreactivity in vascular endothelium, smooth muscle, or infiltrating leukocytes. The cytoplasmic distribution of viral major capsid protein suggests that viral replication in perivascular fibroblasts may be abortive in this dead-end host. These findings provide a novel foundation for defining the pathogenesis of vasculitis in non-adapted hosts with SA-MCF. PMID:23953727

  16. 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

  17. Large vessel vasculitis

    Microsoft Academic Search

    Ashima Gulati; Arvind Bagga

    2010-01-01

    Takayasu arteritis is a chronic granulomatous disease of the aorta and its major branches that usually affects women during\\u000a the second and third decades of life, but it has been reported in young children. This review details the clinical, pathological\\u000a and radiological features, differential diagnoses and management of the condition, focusing chiefly on the disease in children.\\u000a The recent definition

  18. How Is Vasculitis Diagnosed?

    MedlinePLUS

    ... blood vessel with a color image of the blood flow through that vein or artery. 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) identifies areas that show higher glucose metabolism leading to problems in the blood vessels. Angiography ...

  19. Unusual urticarias.

    PubMed

    Black, A K

    2001-11-01

    Typical urticarial lesions are transient cutaneous swellings of sudden onset, often itchy, persisting for less than 24 hours and resolving to leave normal appearing skin. Angioedema lesions are similar subcutaneous lesions. Atypical urticarias persist for longer than 24 hours, may be painful and bruised in appearance and accompanied with severe systemic symptoms. Conditions where prolonged weals are present include delayed pressure urticaria and urticarial vasculitis. These conditions do not respond well to antihistamine therapy. In delayed pressure urticaria, weals appear after a delay of hours at sites of sustained pressure on the skin and occur in association with ordinary chronic 'idiopathic' urticaria. Weals of urticarial vasculitis show histological features of venulitis, and can be accompanied by arthralgia and abdominal pain. Rarely, the condition is due to infective or autoimmune disease. Urticarial diseases, sometimes with features of urticarial vasculitis, and with associated systemic features include Schnitzler's Syndrome, Still's disease and Muckle-Wells syndrome. The latter syndrome is linked with chromosome 1q44, as is autosomal dominant cold urticaria, an unusual physical urticaria. Persistent cholinergic erythema, a variant of cholinergic urticaria, has been mistaken for a drug eruption or cutaneous mastocytosis. Rarely, food and exercise induced urticaria and anaphylaxis occur when exercise follows a specific food or any meal within a few hours. The early stages of inflammatory disease may be mistaken for urticaria and angioedema, but lesions usually persist for longer than 48 hours and are accompanied by epidermal changes. PMID:11770721

  20. Local and systemic activation of the whole complement cascade in human leukocytoclastic cutaneous vasculitis; C3d,g and terminal complement complex as sensitive markers.

    PubMed Central

    Dauchel, H; Joly, P; Delpech, A; Thomine, E; Sauger, F; Le Loet, X; Lauret, P; Tron, F; Fontaine, M; Ripoche, J

    1993-01-01

    We have studied complement activation both in plasma samples and in lesional skin from patients with leukocytoclastic cutaneous vasculitis (LCV). Enzyme immunoassay (EIA) quantification of the complement activation markers, C3d,g and the terminal complement complex (TCC) in plasma, showed that their levels were significantly increased in 66% and 55% of the patients, respectively (n = 29) compared with healthy controls, whereas the standard measurements of C3, factor B, C1q, C4 and C2 were generally within normal range. Elevations of C3d,g and TCC levels in plasma were significantly correlated. Importantly, a significant correlation was found between the severity of the vasculitis and both C3d,g and TCC plasma levels. Immunofluorescence studies of skin biopsy specimens demonstrated simultaneous presence of perivascular dermal deposits of C3d,g and TCC in lesional skin from 96% and 80% respectively of the patients (n = 25). There was a significant correlation between the intensity of the deposits of both markers. Clusterin, a TCC inhibitory protein, was always found at the same sites of perivascular TCC deposits. Immunofluorescence studies at the epidermal basement membrane zone (BMZ) revealed in each case deposits of C3d,g which were accompanied by TCC deposits in 52% of the biopsy specimens. These data demonstrate that there is a local and systemic activation of the whole complement cascade in human LCV. The presence of both C3d,g and clusterin-associated TCC perivascular deposits suggests an intervention of a regulatory mechanism of local complement activation in LCV. Finally, measurement of plasma C3d,g and TCC appears to be a sensitive indicator of systemic complement activation and disease severity in LCV. Images Fig. 1 Fig. 2 PMID:8485913

  1. BULLOUS PRESENTATION OF HENOCH-SCHÖNLEIN PURPURA IN AN ADULT

    Microsoft Academic Search

    Fouad EL SAYED; Rola DHAYBI; Alfred AMMOURY

    Henoch-Schönlein purpura is a small vessel vasculitis occurring mainly in childhood and rarely in adulthood. Typical cutaneous eruption may begin as macular or urticarial erythematous lesions progressing to a palpable purpura. In adults, the dis - ease has a propensity to be more severe and chronic and affects mainly the ankles and feet. Bullae, vesi - cles and ulcers are

  2. C-antineutrophil cytoplasmic antibody positivity in vasculitis patients is associated with the Z allele of alpha-1-antitrypsin, and P-antineutrophil cytoplasmic antibody positivity with the S allele

    Microsoft Academic Search

    M. E. Griffith; J. U. Lovegrove; G. Gaskin; D. B. Whitehouse; C. D. Pusey

    1996-01-01

    Background. Antineutrophil cytoplasmic antibodies (ANCA) in vasculitis have either cANCA or pANCA patterns as defined by immunofluorescence. The target autoantigen of cANCA is usually proteinase 3 (PR3), whereas that of pANCA is usually myeloperoxidase (MPO). Alpha-1-antitrypsin (alAT) is the major physiological inhibitor of PR3, while MPO is an inhib- itor of a 1 AT. Methods. To determine whether there was

  3. Emergency testing for antineutrophil cytoplasmic antibodies combined with a dialog-based policy between clinician and biologist: effectiveness for the diagnosis of ANCA-associated vasculitis.

    PubMed

    Sayegh, Johnny; Poli, Caroline; Chevailler, Alain; Subra, Jean-François; Beloncle, François; Deguigne, Pierre Antoine; Beauvillain, Céline; Augusto, Jean-François

    2015-04-01

    A prompt immunosuppressive treatment initiation is crucial in ANCA-associated vasculitis (AAV) to minimize organ injury. The aim of the present work was to analyze the accuracy of emergency ANCA screening to identify rapidly patients with AAV. In our Institution, emergency ANCA screening is based on a telephone call between a Clinician and a Biologist. Indirect immunofluorescence (IIF) for ANCA detection was performed using a commercial kit (Euroimmun(®) Granulocyte Mosaic 12). Positive serums for c- or p-ANCA at IIF are subsequently screened for antigenic specificity (MPO or PR3) by an immunodot technique (immunodot, D-Tek(®).) Positive samples with atypical c- or p-ANCA pattern at IIF are subsequently screened for antigenic specificity by ELISA. Data were retrieved from patients' medical records and confronted to emergency ANCA screening results. Between 2005 and 2012, 114 patients were screened. IIF was positive in 27.2 % of patients, but c-/p-ANCA anti-MPO/-PR3 was detected in 13.2 % of patients. The sensibility and specificity of IIF combined with immunodot for newly diagnosed AAV were 83.3 and 100 %, respectively. Ten patients were newly diagnosed with AAV. In these patients, a specific AAV treatment was initiated less than 24 h following ANCA screening. Emergency ANCA screening based on a clinical gating policy was relevant to identify patients with AAV diagnosis, and was associated with a rapid treatment initiation. PMID:25343851

  4. Giant Cell Arteritis: A Systematic Review of the Qualitative and Semiquantitative Methods to Assess Vasculitis with 18F-Fluorodeoxyglucose Positron Emission Tomography

    PubMed Central

    Puppo, Cristina; Massollo, Michela; Paparo, Francesco; Camellino, Dario; Piccardo, Arnoldo; Shoushtari Zadeh Naseri, Mehrdad; Villavecchia, Giampiero; Rollandi, Gian Andrea; Cimmino, Marco Amedeo

    2014-01-01

    Giant cell arteritis (GCA) is the most common vasculitis affecting medium and large vessels. It shows a close clinical association with polymyalgia rheumatica (PMR), a musculoskeletal inflammatory disorder, which is clinically characterized by girdles pain and stiffness. 18F-Fluorodeoxyglucose (18F-FDG) positron emission tomography (PET) is an effective tool for the diagnosis, grading, and follow-up of patients affected by GCA involving the aorta and its proximal branches, but the lack of a standardized method for the assessment of vascular inflammation remains a critical issue, potentially leading to misclassification. In our systematic review, including 19 original articles for a total of 442 GCA patients (with or without PMR symptoms) and 535 healthy controls, we described the different qualitative, semiquantitative and combined methods that have been proposed throughout the literature for assessing the presence and grading the severity of GCA-related vascular inflammation on 18F-FDG PET scans, focusing on the diagnostic performance and examining their respective advantages and limitations. The majority of the included studies adopted qualitative methods of PET image analysis, which are less sensitive but more specific than semiquantitative ones. Among the semiquantitative approaches, the aortic-to-blood pool uptake ratio of the aortic arch seems to be the most accurate method. PMID:25254211

  5. Myocarditis, pancreatitis, polyarthritis, mononeuritis multiplex and vasculitis with symmetrical peripheral gangrene of the lower extremities as a rare presentation of leptospirosis: a case report and review of the literature

    PubMed Central

    2014-01-01

    Introduction Leptospirosis is a zoonosis caused by the spirochete, Leptospira interrogans. While most cases of leptospirosis are mild to moderate, the course may be complicated by multiorgan dysfunction. We present a rare case of leptospirosis with acute myocarditis, pancreatitis, polyarthritis, mononeuritis multiplex and severe vasculitis with necrosis of the extremities. Case presentation A 32-year-old man from Congo presented with high-grade fever, confusion and headache. He developed tachycardia and hypotension followed by electrocardiogram changes and elevation of troponin I levels suggesting myocarditis. A physical examination revealed conjunctival suffusion, polyarthritis of his lower extremities and cutaneous necrosis of his feet due to vasculitis. Laboratory findings included amylase levels 10-fold the upper normal serum levels and thrombocytopenia. The diagnosis was confirmed by a positive leptospira immunoglobulin M, negative immunoglobulin G and a positive rapid agglutination test. Our patient recovered progressively with antimicrobials and supportive care. Conclusions Because the clinical features and diagnostic findings of leptospirosis are not specific, a high index of suspicion must be maintained for the diagnosis. Serology is the most important tool for accurate and quick diagnosis in order to administer the appropriate therapy. PMID:24885926

  6. Urticaria mimickers in children.

    PubMed

    Mathur, Anubhav N; Mathes, Erin F

    2013-01-01

    Acute urticaria is a self-limited cutaneous condition marked by transient, erythematous, and pruritic wheals. It is a hypersensitivity response that is often secondary to infection, medications, or food allergies in children. In contrast, the urticarial "mimickers" described in this review article are often seen in the context of fever and extracutaneous manifestations in pediatric patients. The differential diagnosis ranges from benign and self-limited hypersensitivity responses to multisystem inflammatory diseases. Establishing the correct diagnosis of an urticarial rash in a pediatric patient is necessary to both prevent an unnecessary work up for self-limited conditions and to appropriately recognize and evaluate multisystem inflammatory disorders. Herein, we describe two cases to illustrate the clinical manifestations, laboratory findings, histopathology and differential diagnoses for several mimickers of acute urticaria including: urticaria multiforme, serum sickness like reaction, Henoch-Schönlein purpura, acute hemorrhagic edema of infancy, systemic onset juvenile idiopathic arthritis, cryopyrin associated periodic syndromes, and urticarial vasculitis. PMID:24552410

  7. Autologous stem cell therapy for systemic vasculitis

    Microsoft Academic Search

    David M. Carruthers; Paul A. Bacon

    \\u000a Stem cell transplantation (SCT) has been proposed as a potentially highly effective therapy for a variety of autoimmune diseases.\\u000a In this group of diseases a disturbed immune response results in an attack by the immune system on host antigens with B- and\\u000a T-cell components involved in the process. The hypothesis behind SCT in autoimmune disease is that a vigorous immuno-ablative

  8. Small vessel vasculitis of the lung

    Microsoft Academic Search

    S K Frankel; G P Cosgrove; K K Brown

    2005-01-01

    Interstitial Lung Disease Program, National Jewish Medical and Research Center, Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Health Sciences Center, Denver, Colorado, USA The diagnosis and management of SVV remains one of the most challenging clinical scenarios encountered by a clinician. Careful attention to detail and a thorough knowledge of the specific disorders, their therapies, and

  9. Assessment of disease activity in systemic vasculitis.

    PubMed Central

    Tse, W. Y.; Cockwell, P.; Savage, C. O.

    1998-01-01

    The systemic vasculitides are a group of inflammatory disorders characterised by relapses and remission. Before the introduction of immunosuppressive drugs, mortality was unacceptably high. Immunosuppressive therapy has had a therapeutic impact, but at the cost of increased risk of infection and other adverse effects. Differentiating infection from active disease can be difficult, and the inappropriate prescription of immunosuppressive drugs can be fatal. Hence disease indices which can aid physicians to identify the active phase of disease and enable early treatment, will be valuable in the management of this group of disorders. PMID:9538478

  10. [Muckle-Wells syndrome or association of joint pain attacks, urticarial outbreaks and sensory deafness?].

    PubMed

    Serratrice, G; Pouget, J

    1987-02-01

    The Muckle and Well's syndrome corresponding to a transmission of the autosomic dominant type, combines bouts of urticaria, episodes of arthralgias to a shrinking of the ear and a sensory deafness. Sometimes, it evolves into a renal amylosis. Sometimes, as the case presented here, it combines multiple malformations. Its place in nosology is imprecise. It is, at the same time, close to systemic urticaria, sensory deafness, amylosis and specially amylosis of the periodic disease. But the common link between the various elements of the syndrome remains undetermined, for the time being. PMID:3563375

  11. The intestinal bypass: arthritis-dermatitis syndrome.

    PubMed

    Stein, H B; Schlappner, O L; Boyko, W; Gourlay, R H; Reeve, C E

    1981-05-01

    Of the 31 patients who developed polyarthritis following jejunoileal bypass for obesity, 24 had cutaneous vasculitis (urticarial, pustular, and nodular), 11 paresthesias, 10 Raynaud's phenomenon, and 1 pericarditis. Blind loop symptoms (14 of 26 patients), cryoglobulinemia (10 of 28), and immune deposits in biopsied skin lesions (5 of 7) support the theory of a relationship between bowel bacteria and immune complexes. Treating the blind loop with antibiotics and sphincteroplasty to prevent bacterial reflux into the blind loop helped 5 of 10 and 6 of 9 patients, respectively. A comparison is made to other bowel associated arthritides. PMID:7236324

  12. Necrolytic acral erythema: an expanding spectrum.

    PubMed

    Halpern, Analisa V; Peikin, Steven R; Ferzli, Pascal; Heymann, Warren R

    2009-12-01

    Hepatitis C virus (HCV) infection is the most common chronic blood-borne viral infection in the United States. Well-described cutaneous manifestations of HCV infection include polyarteritis nodosa, porphyria cutanea tarda, type II cryoglobulinemia-associated vasculitis, pruritus, erythema nodosum, urticaria and urticarial vasculitis, lichen planus, and erythema multiforme. First described in 1996, necrolytic acral erythema (NAE) is now recognized as a cutaneous acral eruption uniquely associated with HCV infection. Most patients present with chronic, acral, erythematous, and psoriasiform lesions. Acute presentations of NAE are rare and patients may present with atypical clinical features; in these cases, suspicion for HCV infection may be delayed for weeks to months until more classic chronic lesions develop. In many cases, NAE presents before the patient has been diagnosed with HCV infection, which allows dermatologists the unique opportunity to suspect and diagnose HCV infection based on skin findings alone. PMID:20166571

  13. 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...

  14. Forty years’ experience treating septic arteritis and vasculitis

    PubMed Central

    Dieter, Raymond A; Kuzycz, George B; Dieter, Raymond A; Dieter, Robert S

    2009-01-01

    By their natures, septic arteritis and infected grafts present a high morbidity and mortality situation for both the patient and the treating physician. In the experience of the authors, therapy frequently includes removing the previous infected graft, use of long-term antibiotics, repeat grafting and omental wraps. When possible, aortic endografting followed by future elective primary repair may be considered an option. Leaving wounds open to granulate from the ‘bottom up’ has been successful in the extremities and the groin. Again, avoidance of infection is primary. Antibiotics are frequently used at the time of vascular grafting, both intravenously and as irrigation. Examples of the authors’ techniques and results demonstrate the variety of procedures available. PMID:22477474

  15. Forty years' experience treating septic arteritis and vasculitis.

    PubMed

    Dieter, Raymond A; Kuzycz, George B; Dieter, Raymond A; Dieter, Robert S

    2009-01-01

    By their natures, septic arteritis and infected grafts present a high morbidity and mortality situation for both the patient and the treating physician. In the experience of the authors, therapy frequently includes removing the previous infected graft, use of long-term antibiotics, repeat grafting and omental wraps. When possible, aortic endografting followed by future elective primary repair may be considered an option. Leaving wounds open to granulate from the 'bottom up' has been successful in the extremities and the groin. Again, avoidance of infection is primary. Antibiotics are frequently used at the time of vascular grafting, both intravenously and as irrigation. Examples of the authors' techniques and results demonstrate the variety of procedures available. PMID:22477474

  16. Diffuse alveolar haemorrhage secondary to propylthiouracil-induced vasculitis.

    PubMed

    Ferreira, Catarina; Costa, Teresa; Marques, Ana Vieira

    2015-01-01

    Propylthiouracil is a drug used to treat hyperthyroidism. It can cause several side effects including pulmonary disorders that, although rare, can be severe. The authors describe the case of a woman treated with propylthiouracil who developed diffuse alveolar haemorrhage with severe respiratory failure and anaemia, which improved with discontinuation of the antithyroid drug and on starting systemic corticosteroid therapy. PMID:25661751

  17. Reversible posterior leucoencephalopathy syndrome in systemic lupus and vasculitis

    Microsoft Academic Search

    A Primavera; D Audenino; N Mavilio; L Cocito

    2001-01-01

    OBJECTIVESReversible posterior leucoencephalopathy syndrome (RPLS) may develop in patients with renal insufficiency, hypertension, and immunosuppression, and is managed by prompt antihypertensive and anticonvulsant treatment. Four patients with renal insufficiency and fluid overload associated with Wegener's granulomatosis (one patient) and systemic lupus erythematosus (SLE) (three patients) are described, whose clinical picture and neuroimaging indicated RPLS.CASE REPORTSAll patients had headache, seizures, visual

  18. 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

  19. 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

  20. Treatment of autoimmune diseases and systemic vasculitis with pooled human intravenous immune globulin.

    PubMed Central

    Jordan, S C; Toyoda, M

    1994-01-01

    Treatment of autoimmune and vasculitic disorders with intravenous immune globulin (IVIG) has shown great promise. IVIG appears to provide large amounts of immunoregulatory substances that have the capacity to regulate the immune system in various ways. The ability of IVIG to regulate deleterious autoimmune responses and disease is largely directly related to its ability to stimulate the production of anti-idiotypic autoantibodies after infusion. In this respect, IVIG represents a novel immunoregulatory agent with the ability to control autoimmune and vasculitic disorders without subsequent predisposition to infectious complications. Future work and controlled clinical trials will be necessary to prove the efficacy of this therapy for specific autoimmune and vasculitic disorders. Images Fig. 1 PMID:8033432

  1. occlusion or retinal ischemia commonly occurs in blood disorders, hypertension, retinal vasculitis, reti-

    E-print Network

    Palczewski, Krzysztof

    , reti- nopathy of prematurity, and diabetic retinopathy.7 The retinal lesions in our patient could and Reconstructive Surgery. Baltimore: Williams & Wilkins, 1990:849­858. ALBIPUNCTATE RETINOPATHY WITH CONE

  2. Increased Levels of Soluble ST2 in Patients with Active Newly Diagnosed ANCA-Associated Vasculitis

    PubMed Central

    Hladinova, Z.; Hruskova, Z.; Svobodova, B.; Malickova, K.; Lanska, V.; Konopásek, P.; Jancova, E.; Rysava, R.; Edelstein, C. L.; Tesar, V.

    2015-01-01

    Objective. ST2, a member of the interleukin-1 receptor family, is selectively expressed on Th2 cells and mediates important Th2 functions. IL-33 is a specific ligand of ST2. The aim of the study was to determine whether serum levels of soluble ST2 (sST2) or IL-33 predict activity of the disease in patients with ANCA-associated vasculitides (AAV). Methods. 139 AAV patients and 62 controls were studied. IL-33 and sST2 in the blood were measured with a commercially available ELISA. Results. Newly diagnosed AAV patients had higher sST2 levels than controls (P < 0.01). Levels of sST2 were significantly higher in active newly diagnosed AAV patients than in patients with remission (P < 0.001). IL-33 levels were higher in AAV patients than in the control groups (P = 0.002). However, serum IL-33 levels were not increased in patients with active AAV compared to patients in remission. IL-33 levels were higher in patients with granulomatosis with polyangiitis than in patients with microscopic polyangiitis (P = 0.012). Conclusions. Serum sST2, but not serum IL-33, may be a marker of activity in AAV patients.

  3. Intraoperative recognition of retinal vasculitis in a patient with early lens-induced uveitis.

    PubMed

    Besen, G; Freeman, W R

    1997-01-01

    The purpose of this article is to describe a clinical finding not previously reported in lens-induced uveitis. An 86-year-old woman was seen by the authors 2 weeks after she had undergone phacoemulsification surgery complicated by retained lens material. A pars plana vitrectomy was indicated. Intraoperatively, retinal arteritis and phlebitis were documented in the retina immediately adjacent to the lens material. This resolved after removal of the material by vitrectomy. This previously unreported finding demonstrates the focal inflammatory effect of the retained lens material on the retinal vasculature. PMID:9031309

  4. 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

    allele of rs3087243 might thus contribute to T cell activation and autoimmune disease susceptibility. Taken together these data suggest that gen- otype at rs3087243 could impact upon both the expres- PTPN22 encodes a second negative regulator of T cell... the clinical syndromes Wegener's granulomato- sis (WG), microscopic polyangiitis (MPA) and Churg- Strauss Syndrome (CSS). AAV is a complex disease with both genetic and environmental factors involved in pathogenesis [1]. The magnitude of the increased familial...

  5. Large-Vessel Vasculitis: Interobserver Agreement and Diagnostic Accuracy of 18F-FDG-PET/CT

    PubMed Central

    Lensen, K. D. F.; Comans, E. F. I.; Voskuyl, A. E.; van der Laken, C. J.; Brouwer, E.; Zwijnenburg, A. T.; Pereira Arias-Bouda, L. M.; Glaudemans, A. W. J. M.; Slart, R. H. J. A.; Smulders, Y. M.

    2015-01-01

    Introduction. 18F-FDG-PET visualises inflammation. Both atherosclerosis and giant cell arteritis cause vascular inflammation, but distinguishing the two may be difficult. The goal of this study was to assess interobserver agreement and diagnostic accuracy of 18F-FDG-PET for the detection of large artery involvement in giant cell arteritis (GCA). Methods. 31 18F-FDG-PET/CT scans were selected from 2 databases. Four observers assessed vascular wall 18F-FDG uptake, initially without and subsequently with predefined observer criteria (i.e., vascular wall 18F-FDG uptake compared to liver or femoral artery 18F-FDG uptake). External validation was performed by two additional observers. Sensitivity and specificity of 18F-FDG-PET were determined by comparing scan results to a consensus diagnosis. Results. The highest interobserver agreement (kappa: 0.96 in initial study and 0.79 in external validation) was observed when vascular wall 18F-FDG uptake higher than liver uptake was used as a diagnostic criterion, although agreement was also good without predefined criteria (kappa: 0.68 and 0.85). Sensitivity and specificity were comparable for these methods. The criterion of vascular wall 18F-FDG uptake equal to liver 18F-FDG uptake had low specificity. Conclusion. Standardization of image assessment for vascular wall 18F-FDG uptake promotes observer agreement, enables comparative studies, and does not appear to result in loss of diagnostic accuracy compared to nonstandardized assessment. PMID:25695092

  6. Vasculitis reminds us that ‘Ockham's razor’ may still apply in the elderly

    PubMed Central

    Mount, Hillary R

    2013-01-01

    A 76-year-old woman with a history of stage IV chronic kidney disease and hypertension was admitted to the hospital for progressive weakness and acute on chronic kidney injury. She was found to have anaemia requiring transfusion. On the evening of admission, she had worsening respiratory status with subsequent diagnosis of a non-ST-elevation myocardial infarction, pulmonary oedema on chest x-ray presumed to be a result of heart failure, and a possible transfusion reaction. A kidney biopsy performed as part of her ongoing nephrology evaluation revealed granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis). Subsequent bronchoalveolar lavage confirmed diffuse alveolar haemorrhage as the explanation for her anaemia and respiratory decline. She underwent immunosuppressive therapies and initiated dialysis, but subsequently chose hospice care and died 2?months after her diagnosis. PMID:23505087

  7. Recent Understanding on Diagnosis and Management of Central Nervous System Vasculitis in Children

    PubMed Central

    Iannetti, Ludovico; Zito, Roberta; Bruschi, Simone; Papetti, Laura; Ulgiati, Fiorenza; Nicita, Francesco; Del Balzo, Francesca; Spalice, Alberto

    2012-01-01

    Central nervous system vasculitides in children may develop as a primary condition or secondary to an underlying systemic disease. Many vasculitides affect both adults and children, while some others occur almost exclusively in childhood. Patients usually present with systemic symptoms with single or multiorgan dysfunction. The involvement of central nervous system in childhood is not frequent and it occurs more often as a feature of subtypes like childhood polyarteritis nodosa, Kawasaki disease, Henoch Schönlein purpura, and Bechet disease. Primary angiitis of the central nervous system of childhood is a reversible cause of severe neurological impairment, including acute ischemic stroke, intractable seizures, and cognitive decline. The first line therapy of CNS vasculitides is mainly based on corticosteroids and immunosuppressor drugs. Other strategies include plasmapheresis, immunoglobulins, and biologic drugs. This paper discusses on current understanding of most frequent primary and secondary central nervous system vasculitides in children including a tailored-diagnostic approach and new evidence regarding treatment. PMID:23008735

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

    PubMed

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

    2014-05-16

    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

  9. Disseminated Herpes Zoster Mimicking Rheumatoid Vasculitis in a Rheumatoid Arthritis Patient on Etanercept

    Microsoft Academic Search

    S. Tresch; R. M. Trüeb; J. Kamarachev; L. E. French; G. F. L. Hofbauer

    2009-01-01

    Tumor necrosis factor-? (TNF?)-blocking agents are immunomodulating agents introduced for treatment of a variety of chronic inflammatory disease conditions. Adverse effects include an increased incidence of infections. Clinically, these infections often have atypical presentations that may hamper prompt diagnosis. In our report of a patient on etanercept therapy for rheumatoid arthritis, the correct diagnosis was delayed because disseminated herpes zoster

  10. 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

  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

    of Rheumatol- ogy revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1997, 40:1725. 13. Graham DS, Graham RR, Manku H, Wong AK, Whittaker JC, Gaffney PM, Moser KL, Rioux JD, Altshuler D, Behrens TW, Vyse TJ: Poly- 14... , Timothy J Vyse4, Lavanya Kamesh5, Richard A Watts6, Paul A Lyons1,2 and Kenneth GC Smith*1,2 Address: 1Cambridge Institute for Medical Research, University of Cambridge School of Clinical Medicine, Addenbrooke's Hospital, Hills Road, Cambridge, CB2 0XY...

  12. Extra- and intracranial cerebral vasculitis in giant cell arteritis: an observational study.

    PubMed

    Larivière, Delphine; Sacre, Karim; Klein, Isabelle; Hyafil, Fabien; Choudat, Laurence; Chauveheid, Marie-Paule; Papo, Thomas

    2014-12-01

    Recognizing giant cell arteritis (GCA) in patients with stroke may be challenging. We aimed to highlight the clinical spectrum and long-term follow-up of GCA-specific cerebrovascular accidents. Medical charts of all patients followed in a French Department of Internal Medicine for GCA between January 2008 and January 2014 were retrospectively reviewed. Patients with cerebrovascular accidents at GCA diagnosis were included. Diagnosis of GCA was based on American College of Rheumatology criteria. Transient ischemic attacks and stroke resulting from an atherosclerotic or cardioembolic mechanism were excluded. Clinical features, GCA-diagnosis workup, brain imaging, cerebrospinal fluid (CSF) study, treatment, and follow-up data were analyzed. From January 2008 to January 2014, 97 patients have been followed for GCA. Among them, 8 biopsy-proven GCA patients (mean age 70±7.8 years, M/F sex ratio 3/1) had stroke at GCA diagnosis. Six patients reported headache and visual impairment. Brain MR angiography showed involvement of vertebral and/or basilar arteries in all cases with multiple or unique ischemic lesions in the infratentorial region of the brain in all but one case. Intracranial cerebral arteries involvement was observed in 4 cases including 2 cases with cerebral angiitis. Long lasting lesions on diffusion-weight brain MRI sequences were observed in 1 case. All patients received steroids for a mean of 28.1±12.8 months. Side effects associated with long-term steroid therapy occurred in 6 patients. Relapses occurred in 4 patients and required immunosuppressive drugs in 3 cases. After a mean follow-up duration of 36.4±16.4 months, all but 1 patient achieved complete remission without major sequelae. The conjunction of headache with vertebral and basilar arteries involvement in elderly is highly suggestive of stroke associated with GCA. Intracranial cerebral arteries involvement with cerebral angiitis associated with long lasting brain lesions on diffusion-weight brain MRI sequences may occur in GCA. Both frequent relapses and steroid-induced side effects argue for the use of immunosuppressive agents combined with steroids as first-line therapy. PMID:25526454

  13. Revisiting cutaneous adverse reactions to pemetrexed

    PubMed Central

    Piérard-Franchimont, Claudine; Quatresooz, Pascale; Reginster, Marie?Annick; Piérard, Gérald E.

    2011-01-01

    Pemetrexed (Alimta®) is a multitargeted antifolate drug approved as a single agent or in combination with cisplatin for the treatment of a small number of malignancies including advanced and metastatic non-squamous non-small cell lung cancer (NSCLC), and malignant pleural mesothelioma. This review reports the recent peer-reviewed publications and original findings regarding cutaneous adverse reactions (CARs) to pemetrexed. Pemetrexed-related CARs are frequently reported under the unspecific term ‘skin rash’. However, more specific diseases were tentatively identified as alopecias, urticarial vasculitis, acute generalized exanthematous pustulosis, toxic epidermal necrolysis, radiation recall dermatitis and pityriasis lichenoides. Most of the skin reactions occur shortly after pemetrexed administration. As with methotrexate-related CARs, the cell cycle arrest in the S phase may be regarded as a direct and major cause of the cytotoxic pathobiology. An adverse immune reaction is unlikely. In conclusion, pemetrexed is responsible for CARs exhibiting a variety of clinical presentations. Their origin is likely attributed to direct cytotoxicity following the cell cycle arrest in the S phase and cell necrosis. PMID:22866124

  14. The Role of Biological Agents in the Management of Large Vessel Vasculitis (LVV): A Systematic Review and Meta-Analysis

    PubMed Central

    Osman, Mohammed; Pagnoux, Christian; Dryden, Donna M.; Storie, Dale; Yacyshyn, Elaine

    2014-01-01

    Background Giant cell arteritis (GCA) and Takayasu's arteritis (TAA) are large vessel vasculitides (LVV) for which corticosteroids (CS) are the mainstay for treatment. In patients with LVV unable to tolerate CS, biological agents have been used with variable effectiveness. Objective To systematically review the effectiveness and safety of biological agents in patients with LVV. Methods We searched 5 electronic databases (inception to October 2012) and conference abstracts with no language restrictions. Two reviewers independently selected studies, extracted data and assessed methodological quality. Our protocol was registered in PROSPERO. Results We included 25 studies (3 RCTs and 22 case series with ?2 cases). 95 GCA and 98 TAA patients received biological agents. The RCTs using anti-TNF agents (infliximab, etanercept and adalimumab) did not suggest a benefit in GCA. GCA patients receiving tocilizumab, in case series, achieved remission (19 patients) and reduction of corticosteroid dose (mean difference, –16.55 mg/day (95% CI: –26.24, –6.86)). In case series, 75 patients with refractory TAA treated with infliximab discontinued CS 32% of the time. Remission was variably defined and the studies were clinically heterogeneous which precluded further analysis. Conclusion This systematic review demonstrated a weak evidence base on which to assess the effectiveness of biological treatment in LVV. Evidence from RCTs suggests that anti-TNF agents are not effective for remission or reduction of CS use. Tocilizumab and infliximab may be effective in the management of LVV and refractory TAA, respectively, although the evidence comes from case series. Future analytical studies are needed to confirm these findings. PMID:25517966

  15. Limited Cutaneous Systemic Sclerosis Associated With MPO-ANCA Positive Renal Small Vessel Vasculitis of the Microscopic Polyangiitis Type

    Microsoft Academic Search

    Bart Maes; An Van Mieghem; Thierry Messiaen; Dirk Kuypers; Boudewijn Van Damme; Yves Vanrenterghem

    2000-01-01

    Renal disease in systemic sclerosis may present in various patterns. A 66-year-old woman with a history of longstanding limited cutaneous systemic sclerosis of the CREST syndrome variant presented with a sudden left foot drop and rapidly progressive renal insufficiency associated with mild proteinuria, a nephritic urine sediment, and a urinary output of 900 mL\\/d. There was no history of intake

  16. ANCA-associated renal vasculitis at the end of the twentieth century--a disease of older patients

    Microsoft Academic Search

    L. Harper; C. O. Savage

    2005-01-01

    Objective. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides are increasingly recognized in older patients. However, it is unknown whether disease presentation and response to treatment differs from younger patients. We aimed to examine the presentation, response to treatment and outcome of patients over 65 yr of age compared with a younger cohort. Methods. This retrospective, single centre, sequential cohort study reports presenting

  17. Acute blast crisis with EBV-infected blasts, in a patient with chronic myeloid leukemia, and vasculitis.

    PubMed

    Ariad, S; Argov, S; Manor, E; Yermiahu, T; Kedar, I

    2000-04-01

    Unless they undergo transplantation, all patients with chronic myeloid leukemia (CML) will eventually develop a late phase of acute blast crisis (ABC). Although additional chromosomal abnormalities to the Philadelphia (Ph) chromosome may herald ABC in many CML cases, the mechanisms leading to this fatal event are obscure. Viral etiology, including the Epstein-Barr virus (EBV) has never been implicated in the pathogenesis of ABC in CML. Iloprost is an analogue of epoprostenol (prostacyclin; PGI2) commonly used for the treatment of peripheral vascular diseases and acts via inhibition of platelet activation, and by vasodilation. A case of ABC with blasts of undetermined lineage showing EBV infection in a male patient with Ph positive CML is described here. This unusual event developed during a course of treatment with the prostacyclin analogue, iloprost administered for vasculopathic leg ulcers. The proliferating blasts stained positively by immunohistochemistry only for the leukocyte common antigen (LCA/CD-45), and the EBV-latent membrane protein 1 (LMP-1). The only chromosomal abnormality detected by cytogenetic analysis was the conventional Ph-chromosome. It is suggested that ABC in this case of CML, was associated with EBV-activated blasts of undetermined lineage. PMID:10752995

  18. Cryoglobulinemia Induced by a Murine IgG3 Rheumatoid Factor: Skin Vasculitis and Glomerulonephritis Arise from Distinct Pathogenic Mechanisms

    Microsoft Academic Search

    Luc Reininger; Thierry Berney; Takanori Shibata; Francois Spertini; Ramon Merino; Shozo Izui

    1990-01-01

    MRL-lpr\\/lpr mice spontaneously develop a lupus-like syndrome characterized by immunopathological manifestations such as necrotizing vascular lesions of ear tips and severe glomerulonephritis. Similar skin vascular and glomerular lesions associated with cryoglobulinemia can be induced in normal mice by injection of a monoclonal antibody (mAb)-6.19 (gamma 3 heavy chain and kappa light chain), exhibiting both cryoglobulin and anti-IgG2a rheumatoid factor (RF)

  19. CD4CD25 regulatory T-cell deficiency in patients with hepatitis C-mixed cryoglobulinemia vasculitis

    Microsoft Academic Search

    Olivier Boyer; David Saadoun; Julien Abriol; Melanie Dodille; Jean-Charles Piette; Patrice Cacoub; David Klatzmann

    2004-01-01

    Patients who are chronically infected with hepatitis C virus (HCV) often develop mixed cryoglobulinemia (MC), a B-cell proliferative disorder with polyclonal acti- vation and autoantibody production. We investigated if MC is associated with a deficit of CD4CD25 immunoregulatory T (Treg) cells, which have been shown to control autoimmunity. Because Treg cells express higher amounts of CD25 than activated CD4 T

  20. 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; on behalf of the PEXIVAS Investigators

    2013-03-14

    100,000 [1]. The most common severe AAV manifestations are glomerulonephritis, leading to renal failure and alveolar capillaritis causing lung hemorrhage. The current standards of care for initial treatment are either combination cyclophosphamide (CYC... ) and glu- cocorticoid (GC) therapy or rituximab and GC therapy. Although these treatments have significantly improved survival compared to untreated AAV, overall survival is still poor and many patients suffer from chronic morbi- dity including end...

  1. Occupational urticaria from welding polyurethane

    SciTech Connect

    Kanerva, L.; Estlander, T.; Jolanki, R.; Laehteenmaeki, M.T.Ke.; Keskinen, H. (Institute of Occupational Health, Helsinki (Finland))

    1991-05-01

    An urticarial reaction associated with high fever developed in a welder on four occasions while he was welding steel profiles filled with polyurethane. The fumes emitted during pyrolysis of polyurethane and inhaled by the patient probably caused the urticarial reaction. Provocation tests with two pyrolysis products, 4,4-diphenylmethane diisocyanate and 4,4-diaminophenylmethane, were negative. This case demonstrates the difficulty in detecting the cause of urticaria induced by airborne chemicals.

  2. Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema

    Microsoft Academic Search

    Franco Rongioletti; Alfredo Rebora

    2001-01-01

    Lichen myxedematosus (LM) is an idiopathic cutaneous mucinosis; its classification dates back to 1953, when Montgomery and Underwood distinguished 4 types of LM: a generalized lichenoid eruption, later called scleromyxedema, a discrete papular form, a localized or generalized lichenoid plaque form, and an urticarial plaque form. In the literature, the terms LM, papular mucinosis, and scleromyxedema have been often used

  3. Anti-endothelial cell auto-antibodies in hepatitis C virus mixed cryoglobulinemia

    Microsoft Academic Search

    Patrice Cacoub; Pascale Ghillani; Ronan Revelen; Vincent Thibault; Vincent Cálvez; Frédéric Charlotte; Lucile Musset; Pierre Youinou; Jean-Charles Piette

    1999-01-01

    Background\\/Aim: Hepatitis C virus (HCV) infection is often associated with mixed cryoglobulins (MC) and may manifest as small-vessel vasculitis. It has been suggested that antibody (Ab) or sensitized T cells to HCV-containing endothelial cells may initiate the vasculitis process. Anti-endothelial cell antibodies (AECA) have been found in various connective tissue disorders, with a high prevalence in systemic vasculitis. The aim

  4. Vasculitides associated with HIV infection

    PubMed Central

    Chetty, R

    2001-01-01

    The manifestations of human immunodeficiency virus (HIV) infection are protean and vasculitides are one of the less common but nonetheless important consequences. A wide range of vasculitides can be encountered, ranging from vasculitis resulting from specific infective agents to a non-specific vasculitis. Among the infective causes, cytomegalovirus and tuberculosis are probably the most common. A polyarteritis nodosa-like vasculitis with important differences to classic polyarteritis nodosa is also described. Hypersensitivity vasculitis resulting in several patterns of vasculitis and angiocentric immunoproliferative vasculitis are well recognised. As part of the immunocompromise caused by HIV, a granulomatous inflammation involving small arteries and veins of the brain surface and leptomeninges, termed a primary angiitis of the central nervous system, is a rare vasculitis associated with high mortality. A recently described large vessel (aorta, femorals, carotids) vasculopathy resulting in either multiple aneurysm formation or occlusive disease is seen in young adults. An infective agent is not found but aetiologically some of these lesions might be the result of a leucocytoclastic vasculitis of vasa vasora or periadventitial vessels. A final group of non-specific vasculitides not fitting into any of the characteristic patterns described accounts for the residue of vasculitides associated with HIV. Key Words: human immunodeficiency virus • vasculitis • immunocompromise PMID:11304843

  5. Immunologic aspects of cardiovascular disease.

    PubMed

    Ledford, D K

    1992-11-25

    The spectrum of vasculitis is diverse, and numerous entities do not fit the aforementioned broad categories. Examples of these include Buerger's disease; vaso-occlusive vasculitis of the lower extremities associated with cigarette smoking; Behçet's disease, which is prevalent in the Orient and Middle East and is characterized by recurrent aphthous stomatitis, genital ulcerations, uveitis, meningoencephalitis, and phlebitis; and isolated central nervous system vasculitis, a rare disease with a poor prognosis that primarily affects intracranial arteries without a systemic acute-phase response. Improvement in the classification and definitive therapy of vasculitis awaits identification of etiologic agents and definition of host factors and the immune response responsible for the pathology. PMID:1359164

  6. Compassionate Use Protocol for the Treatment of Autoinflammatory Syndromes

    ClinicalTrials.gov

    2015-02-17

    Chronic Atypical Neutrophilic Dermatosis With Lipodystrophy and Elevated Temperature (CANDLE); Juvenile Dermatomyositis (JDM); Stimulator of Interferon Genes (STING)-Associated Vasculitis With Onset in Infancy (SAVI)

  7. 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

  8. Endothelin 1 levels in relation to clinical presentation and outcome of Henoch Schonlein purpura

    Microsoft Academic Search

    S Fessatou; P Nicolaidou; D Gourgiotis; H Georgouli; K Douros; M Moustaki; A Fretzayas

    2008-01-01

    BACKGROUND: Henoch Schonlein purpura (HSP) is a common vasculitis of small vessels whereas endothelin-1 (ET-1) is usually reported elevated in vasculities and systematic inflammation. The aim of the present study was to investigate whether ET-1 levels are correlated with the clinical presentation and the outcome of HSP. METHODS: The study sample consisted of thirty consecutive patients with HSP. An equal

  9. Microscopic polyangiitis (microscopic polyarteritis) with late emergence of generalised Wegener’s granulomatosis

    Microsoft Academic Search

    Xavier Bosch

    1999-01-01

    OBJECTIVESRecent proposals for the nomenclature of systemic vasculitis have focused on a distinction between (classic) polyarteritis nodosa (PAN) and microscopic polyangiitis or polyarteritis (MPA). Thus, MPA may cause necrotising vasculitis of medium sized or small arteries but, unlike PAN, involvement of “microscopic” vessels must always be present in the former. This study aimed to show that the term “MPA” may

  10. Intravenous Immunoglobulin (IVIg) Therapy in MPO-ANCA Related Polyangiitis with Rapidly Progressive Glomerulonephritis in Japan

    Microsoft Academic Search

    Eri Muso; Toshiko Ito-Ihara; Takahiko Ono; Enyu Imai; Kunihiro Yamagata; Akira Akamatsu; Kazuo Suzuki

    Jpn. J. Infect. Dis., 57, 2004 scarring is more evident in MPO-ANCA vasculitis than PR3-ANCA disease and indicates chronicity and a poorer outcome. A similar pathogenesis is responsible for other organ manifestations, for example, alveolar capillaritis in the lung is the cause of the lung hemorrhage. Serology: The association of ANCA with vasculitis has provided both a diagnostic tool and

  11. Mixed cryoglobulinemia: demographic, clinical, and serologic features and survival in 231 patients

    Microsoft Academic Search

    Clodoveo Ferri; Marco Sebastiani; Dilia Giuggioli; Massimiliano Cazzato; Giovanni Longombardo; Alessandro Antonelli; Rodolfo Puccini; Claudio Michelassi; Anna Linda Zignego

    2004-01-01

    BackgroundMixed cryoglobulinemia (MC) is a systemic vasculitis secondary to circulating immune complex deposition in the small vessels. In the overwhelming majority of patients, hepatitis C virus (HCV) infection represents the triggering factor of the disease. MC is characterized by multiple organ involvement, mainly skin, liver, renal, peripheral nerves, and less frequently by widespread vasculitis and cancer.

  12. Urticaria due to polyethylene glycol-3350 and electrolytes for oral solution in a patient with jejunal nodular lymphoid hyperplasia

    PubMed Central

    Zhang, Hongfeng; Henry, Winoah A.; Chen, Lea Ann; Khashab, Mouen A.

    2015-01-01

    Both jejunal nodular lymphoid hyperplasia (NLH) and polyethylene glycol (PEG)-3350 hypersensitivity are extremely rare. We describe a 30-year-old female who had previously taken a PEG-3350 bowel preparation without adverse effects, and presented for evaluation of chronic diarrhea. An upper and lower gastrointestinal endoscopy, and small bowel series were scheduled. PEG-3350 and electrolytes for oral solution was prescribed for bowel cleansing. During consumption of the bowel preparation she developed urticarial hypersensitivity. An alternative bowel preparation was used. Colonoscopy and upper endoscopy were normal, but small bowel series revealed innumerable sand-like lucencies in the jejunum. NLH was confirmed on biopsy from antegrade enteroscopy. This is the first case report on the pathological jejunal NLH in association with the PEG-3350 urticarial hypersensitivity. The potential pathophysiological etiology of this association is discussed. PMID:25608714

  13. Muckle-Wells syndrome/neonatal-onset multisystem inflammatory disease overlap associated with myelodysplasia and cerebrovascular accident.

    PubMed

    Madan, V; Kingston, H; Jamieson, L A; Goyal, N; Ead, R

    2010-10-01

    We describe a patient with overlapping clinical features of Muckle-Wells syndrome and neonatal-onset multisystem inflammatory disease with an absence of mutation in exon 3 of the CIAS1 ? PYPAF1 ? NALP3 gene. Myelodysplasia and cerebrovascular accident were additional features in this patient, which to our knowledge have not been previously described in association with these disorders. The urticarial rash, myelodysplasia and raised inflammatory markers responded to treatment with the interleukin-1 receptor antagonist, anakinra. PMID:20456407

  14. UVB-sensitive solar urticaria possibly associated with terbinafine.

    PubMed

    Kuo, Sandy; Sivamani, Raja K

    2014-03-01

    Solar urticaria is an uncommon condition characterized by erythema and whealing shortly after exposure to ultraviolet (UV) and/or visible light. We report a 25-year-old woman with an erythematous, edematous, pruritic reaction minutes after sun exposure while she was taking terbinafine for onychomycosis. Phototesting revealed a UVB-sensitive urticarial reaction, confirming the diagnosis of solar urticaria. This report describes the first patient with possible terbinafine-associated solar urticaria. PMID:24656267

  15. Autoantibody Reactivity in a Case of Schnitzler’s Syndrome: Evidence for a Th1Like Response and Detection of IgG2 Anti-FcεRI? Antibodies

    Microsoft Academic Search

    Wolfgang R. Sperr; Susanne Natter; Mehrdad Baghestanian; Josef Smolen; Klaus Wolff; Bernd R. Binder; Michael R. Müller; Klaus Lechner; Rudolf Valenta; Peter Valent

    2000-01-01

    Schnitzler’s syndrome is a rare disease characterized by chronic urticaria, monoclonal IgM, and clinical and laboratory signs of inflammation. In a subset of patients, the urticarial lesions cause pruritus. However, the pathophysiology of the disease and the biochemical basis of urticaria are not known. We describe a female patient with Schnitzler’s syndrome suffering from chronic urticaria associated with pruritus. The

  16. Mast cells respond to urticating extract from lepidoptera larva Morpheis ehrenbergii in the rat.

    PubMed

    Galicia-Curiel, María Fernanda; Quintanar, J Luis; Jiménez, Mariela; Salinas, Eva

    2014-01-01

    Mast cells and histamine participate in toxic effects of hairs from some caterpillars. This study reports that a crude extract of Morpheis ehrenbergii caterpillar hairs induces in vitro mast cells activation, triggers the release of histamine and causes a rapid urticarial reaction in the rat skin. Heating of the extract abolishes the inflammatory reaction. These results suggest that the use of antihistamines may improve the adverse skin reactions caused by the Mexican caterpillar M. ehrenbergii. PMID:24269786

  17. [Association of chronic urticaria with Helicobacter pylori-induced antrum gastritis].

    PubMed

    Bohmeyer, J; Heller, A; Hartig, C; Wetenberger-Treumann, M; Huchzermeyer, H; Otte, H G; Stadler, R

    1996-02-01

    In spite of its high incidence, not much is known about the etiology of chronic urticaria. We performed gastroscopic evaluation of 10 patients in whom no cause for chronic urticaria had been found. In 8 of these 10 patients, Helicobacter pylori was identified in the gastric mucosa. The chronic urticarial lesions disappeared within a few days after starting therapy with amoxicillin and omeprazol. PMID:8868453

  18. 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

  19. Cryoglobulinemia and Glomerular Rhomboid Inclusions in a Child With Acute Kidney Injury

    PubMed Central

    Nester, Carla M.; Benner, Eric J.; Latour, Mathieu; Nickeleit, Volker; Hamrick, Harvey; Primack, William

    2011-01-01

    Cryoglobulinemia is rarely reported in children, and kidney failure secondary to cryoglobulinemia is even more uncommon. We report the case of a 7-year-old boy with cryoglobulins and a systemic illness, including persistent fever, arthralgias, rash, hypocomplementemia, and acute kidney injury associated with nephritic urine sediment. An extensive workup showed no infectious, neoplastic, or rheumatological cause of his kidney injury. The kidney biopsy specimen showed membranoproliferative glomerulonephritis type 1 with electron microscopic evidence of rhomboid crystalloid inclusions. These inclusions have rarely been reported in adult patients with cryoglobulinemia. The patient underwent spontaneous remission, including full recovery of kidney function, and required no immune suppression. The patient’s course is consistent with cryoglobulinemia-associated kidney injury, which supports the inclusion of essential cryoglobulinemia in the differential diagnosis of pediatric patients with hypocomplementemic glomerulonephritis. PMID:19303686

  20. Pulmonary and central nervous system involvement in Sweet's syndrome: a very rare case report.

    PubMed

    Aydemir, Hande; Oztoprak, Nefise; Celebi, Güven; Altinyazar, Cevdet; Barut, Figen; Piskin, Nihal; Akduman, Deniz

    2008-01-01

    Sweet's syndrome is a multisystem inflammatory disorder characterized by painful, erythematous plaques and aseptic neutrophilic infiltration of various organs. The absence of vasculitis is a histological criterion for diagnosis, but recent reports suggest that vasculitis can occur in Sweet's syndrome. Involvement of the central nervous system and the pulmonary system is very rare. In this case study we describe a chronic alcoholic man with Sweet's syndrome associated with acute-onset encephalitis and severe pulmonary involvement. The patient's symptoms responded dramatically to steroid treatment, and notably, a skin biopsy of his lesions showed vasculitis. PMID:18703859

  1. Cold hard facts of cryoglobulinemia: updates on clinical features and treatment advances.

    PubMed

    Ghetie, Daniela; Mehraban, Navid; Sibley, Cailin H

    2015-01-01

    Cryoglobulins are immunoglobulins that precipitate at temperatures less than 37°C. They occur secondary to infectious, autoimmune, and malignant processes. In the Brouet classification, type I cryoglobulinemia is caused by hyperviscosity, whereas type II and III manifestations are caused by vasculitis in target organs (primarily skin, peripheral nerves, and kidney). New classification criteria were recently proposed that may help with study and treatment of cryoglobulinemic vasculitis (CryoVas). Hepatitis C virus is the most common cause of CryoVas and treatment with antivirals can be curative in mild cases, whereas rituximab is highly effective in treating active vasculitis in more severe cases. PMID:25399942

  2. Giant Cell Arteritis

    MedlinePLUS

    ... polymyalgia rheumatica (also called PMR). What is giant cell arteritis? GCA is a type of vasculitis or ... right away to their doctors. What causes giant cell arteritis? As with PMR, the cause of GCA ...

  3. 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

  4. 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

  5. Chemical Sensitivities

    MedlinePLUS

    ... inflammation of the blood vessels (vasculitis-related disorder). Ozone and Nitrogen Dioxide Temporary or perhaps permanent bronchial hypersensitivity has been connected to inhaled ozone and nitrogen dioxide. Long-term exposure to nitrogen ...

  6. Buerger's Disease

    MedlinePLUS

    ... below ). This decreased blood flow (“ischemia”) led to ulcers of the fingers and severe pain. An abnormal ... unlike many other forms of vasculitis. Even as ulcers and gangrene develop in the digits, organs such ...

  7. 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.

  8. The CARRA Registry

    ClinicalTrials.gov

    2014-03-17

    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

  9. Isolated Aortitis

    MedlinePLUS

    ... are forms of large vessel vasculitis, most commonly giant cell arteritis and Takayasu arteritis. Other autoimmune diseases ... cases are due to a rheumatic cause like giant cell arteritis and Takayasu arteritis. Who gets Isolated ...

  10. Kawasaki disease: Current therapeutic perspectives

    Microsoft Academic Search

    Erik C. Michelfelder; David Shim

    2002-01-01

    Opinion statement  Kawasaki disease is an acute systemic vasculitis of unknown etiology that has become the most common form of acquired heart\\u000a disease in young children in developing countries. The most serious threat from Kawasaki disease is the development of coronary\\u000a vasculitis, with subsequent development of aneurysms, thrombosis, and coronary compromise. Standard treatment during the acute\\u000a phase of Kawasaki disease now

  11. Popliteal artery thrombosis in a patient with Cogan syndrome: Treatment with thrombolysis and percutaneous transluminal angioplasty

    SciTech Connect

    Bastug, Demir E.; Dominic, Anthony; Ortiz, Orlando [Robert C. Byrd Health Sciences Center, Department of Radiology, West Virginia University (United States); DiBartolomeo, Anthony G. [Robert C. Byrd Health Sciences Center, Department of Medicine, Rheumatology Section, West Virginia University (United States); Kotzan, Jeffrey M. [Robert C. Byrd Health Sciences Center, Department of Radiology, West Virginia University (United States); Abraham, F. Matthew [Robert C. Byrd Health Sciences Center, Department of Medicine, West Virginia University (United States)

    1997-01-15

    A 31-year-old woman with Cogan syndrome (a rare form of systemic vasculitis) was evaluated for a cold, painful left foot with diminished pulses. Arteriography demonstrated thrombosis of the left popliteal artery with evidence of vasculitis. Thrombolytic therapy was begun with initial success but eventual rethrombosis. After reinitiating thrombolytic therapy combined with intraarterial vasodilator therapy, successful angioplasty was performed with sustained results, at 6-month follow-up.

  12. Fatal cardiac involvement complicating antisynthetase syndrome.

    PubMed

    Brady, Stefen; Melath, Sunil; Scalco, Renata S; Penn, Henry

    2014-01-01

    A 35-year-old Afro-Caribbean woman presented with dyspnoea, urticarial rash and myalgia 1?month after treatment for a community-acquired respiratory tract infection. Investigations revealed raised antisynthetase antibodies, lung fibrosis and an inflammatory myopathy. The patient was diagnosed with antisynthetase syndrome (ASS) and started on immunosuppressive medication. Despite treatment she died 4?weeks after presentation from a fulminant cardiomyopathy. ASS is a rare condition and is not typically associated with a cardiomyopathy. This case report intends to raise awareness that cardiomyopathy is a potentially fatal complication of ASS. PMID:25155488

  13. Acebrophylline-induced angioedema.

    PubMed

    Kuriachan, Sanitha; Amberkar, Mohan Babu V; Mohan, Manu K; Shahul, Hameed Aboobackar; Kishore, Meenakumari Kamal

    2015-01-01

    A 53-year-old woman visited her physician complaining of acute breathlessness and productive cough. Her medications included budesonide and formoterol for asthma, fixed-dose combination aspirin 150 mg + clopidogrel 75 mg + atorvastatin 20 mg for ischemic heart disease. History revealed that she had allergic rhinitis and was hypersensitive to penicillins. The patient was prescribed acebrophylline (ABP). Six hours after ABP therapy she presented with generalized urticarial lesions, swelling of hands, feet, lips and face, suggestive of angioedema. ABP was stopped immediately, and the patient was treated symptomatically. This case was categorized as probable as per standard causality assessment scale. PMID:25878387

  14. Acebrophylline-induced angioedema

    PubMed Central

    Kuriachan, Sanitha; Amberkar, Mohan Babu V; Mohan, Manu K.; Shahul, Hameed Aboobackar; Kishore, Meenakumari Kamal

    2015-01-01

    A 53-year-old woman visited her physician complaining of acute breathlessness and productive cough. Her medications included budesonide and formoterol for asthma, fixed-dose combination aspirin 150 mg + clopidogrel 75 mg + atorvastatin 20 mg for ischemic heart disease. History revealed that she had allergic rhinitis and was hypersensitive to penicillins. The patient was prescribed acebrophylline (ABP). Six hours after ABP therapy she presented with generalized urticarial lesions, swelling of hands, feet, lips and face, suggestive of angioedema. ABP was stopped immediately, and the patient was treated symptomatically. This case was categorized as probable as per standard causality assessment scale. PMID:25878387

  15. Follicular mucinosis and mycosis-fungoides-like drug eruption due to leuprolide acetate: a case report and review.

    PubMed

    Shalin, Sara C; Brantley, Julie; Diwan, A Hafeez

    2012-11-01

    Leuprolide acetate represents a gonadotropin-releasing hormone agonist, used as part of the treatment of prostate cancer. We report an unusual case of disseminated urticarial rash following leuprolide injection in a 67-year-old man that histopathologically and immunohistochemically resembled mycosis-fungoides, including the presence of follicular mucinosis and eosinophils in the follicles. This histopathologic pattern has not been previously described as a drug reaction pattern due to leuprolide, and it underscores the importance of correlation with the clinical impression to arrive at a correct diagnosis. PMID:22882386

  16. Specific dermatoses of pregnancy and their treatment.

    PubMed

    Lehrhoff, Stephanie; Pomeranz, Miriam Keltz

    2013-01-01

    The specific dermatoses of pregnancy represent a diverse group of intensely pruritic dermatoses, occurring only in the puerperal state. The relative rarity of these conditions, the often variable clinical appearance, and the lack of definitive diagnostic tests have led to confusion regarding the appropriate diagnosis and management of the specific dermatoses of pregnancy. Herein we review the clinical characteristics, diagnosis and treatment of five dermatoses occurring during pregnancy: pruritic urticarial papules and plaques of pregnancy, atopic eruption of pregnancy, pemphigoid gestationis, intrahepatic cholestasis of pregnancy, and pustular psoriasis of pregnancy. PMID:23914884

  17. Pruritus in pregnancy

    PubMed Central

    Bergman, Hagit; Melamed, Nir; Koren, Gideon

    2013-01-01

    Abstract Question Some of my pregnant patients complain about pruritus. Are there conditions in pregnancy that present with pruritus that might put the mother or fetus at risk? Answer Although most cases of pruritus can be attributed to itchy dry skin, there are conditions unique to pregnancy that involve pruritus as a leading symptom. These include pemphigoid gestationis, pruritic urticarial papules and plaques of pregnancy, intrahepatic cholestasis of pregnancy, and atopic eruption of pregnancy. These conditions are associated with severe pruritus and some might be associated with adverse fetal outcomes. Clinical history and physical examination are the most important diagnostic clues when evaluating pruritus in pregnancy. PMID:24336540

  18. Asystole after the first dose of lansoprazole.

    PubMed

    Candar, Melik; Gunes, Harun; Boz, Behic Volkan; Kandis, Hayati; Kutlucan, Leyla; Saritas, Ayhan

    2014-10-01

    Allergic reactions due to drug intake are responsible from an important amount of emergency admissions. Patients mostly complain of urticarial lesions. But clinical picture may sometimes include a large scale of signs and symptoms ranging from a simple confusion to serious conditions like coma and even cardiopulmonary arrest. In this article, a case of anaphylactic shock and respiratory arrest after lansoprazole intake is presented. Delays in reaching basic and advanced life support decrease chance of positive results of life support in anaphylactic shock victims. It is important to remember that any medication we usually prescribe in our daily practice for treatment of any disease has a potential to kill the patient. PMID:24768227

  19. Adrenergic urticaria: a new form of stress-induced hives.

    PubMed

    Shelley, W B; Shelley, E D

    1985-11-01

    A distinctive new type of autonomic-system-dependent urticaria was seen in two patients. This "adrenergic" urticaria is to be contrasted with cholinergic urticaria. Widespread pruritic, urticarial papules developed at times of stress, each papule being surrounded by a striking white halo. Associated with these lesions was an increase in plasma noradrenaline and adrenaline concentrations. In severe attacks large plaques of urticaria appeared as well. The halo hives could be replicated with an intradermal injection of noradrenaline, but not with acetylcholine. Treatment with a beta-adrenoreceptor-blocker, propranolol, prevented attacks. PMID:2865515

  20. Are circulating neutrophils intravascularly activated in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides?

    PubMed Central

    MULLER KOBOLD, A C; MESANDER, G; STEGEMAN, C A; KALLENBERG, C G M; COHEN TERVAERT, J W

    1998-01-01

    Vascular injury in vasculitis may be due to activation of circulating neutrophils resulting in their increased adhesiveness to locally activated endothelium (Shwartzman phenomenon). Previously, we demonstrated up-regulation of endothelial intercellular adhesion molecule-1 (ICAM-1) and vascular cell adhesion molecule-1 (VCAM-1) in biopsies from patients with ANCA-associated vasculitis. In the present study, we investigated the expression of adhesion molecules (CD11b, ICAM-1, VLA-4, l-selectin) and activation markers (CD66b, CD64, CD63) on circulating neutrophils from patients with ANCA-associated vasculitis in comparison with their expression on cells from healthy volunteers and patients with sepsis. We related these findings to parameters of disease activity. Surface marker expression was determined by using a non-activating whole blood flow cytometric assay. The expression of activation markers, but not the expression of adhesion molecules, was increased on neutrophils from patients with active vasculitis. The expression of CD63 and CD66b on neutrophils correlated with disease activity as determined by the Birmingham Vasculitis Activity Score (BVAS). In contrast to patients with active vasculitis, patients with sepsis showed up-regulation of all markers, including adhesion molecules, suggesting that circulating neutrophils are fully activated in sepsis. We conclude that in ANCA-associated vasculitis, circulating neutrophils are not fully activated, since they do not express increased levels of adhesion molecules as sepsis or in the Shwartzman reaction. These findings are compatible with the concept that in vivo vascular damage in ANCA-associated vasculitides does not occur due to a Shwarzman-like reaction but only after ANCA-induced neutrophil activation at the endothelial cell surface. PMID:9844062

  1. [Cerebral vasculitides].

    PubMed

    de Boysson, Hubert; Faivre, Anthony; Pagnoux, Christian

    2012-11-01

    Cerebral vasculitides can be secondary to a broad variety of conditions such as systemic disease, infections, malignancies and toxic exposures, or can be isolated to the central nervous system. When cerebral vasculitis is suspected, the diagnostic work up must be exhaustive to rule out all these potential causes of secondary vasculitis. Most often, the diagnosis of cerebral vasculitis relies on the combination of clinical, biological and radiological findings. It is definite only when based on a positive leptomeningeal biopsy. This latter should be done when the diagnostic uncertainty remains high, especially in patients with underlying systemic disease and/or infrequent or rapidly-progressive symptoms. One can probably rule out the diagnostic of cerebral vasculitis in patients in whom both MRI and cerebrospinal fluid examination are normal. For secondary cerebral vasculitis, treatment depends on the identified underlying etiology. However, corticosteroids combined with immunosuppressive drugs are usually used. Actually, there is no consensual treatment for primary angiitis of the central nervous system. The prognosis is better when diagnosis is made early and treatment started promptly. PMID:22326667

  2. Measurement of anti-IgA antibodies by a two-site immunoradiometric assay

    SciTech Connect

    Homburger, H.A.; Smith, J.R.; Jacob, G.L.; Laschinger, C.; Naylor, D.H.; Pineda, A.A.

    1981-01-01

    To enable the detection of IgG class, anti-IgA antibodies and to investigate the possible occurrence of IgE class, anti-IgA antibodies, we developed a solid phase immunoradiometric assay, which uses purified IgA coupled covalently to microcrystalline cellulose as an immunosorbent. Radiolabeled, Fc specific anti-IgG and anti-IgE antibodies were used to detect specific aIgA after incubation of test sera or controls with the immunosorbent. IgG-aIgA were detected by the IRA in 100 and 67% of control sera with class specific and limited specificity aIgA. The IRA was sensitive to approximately two ng of class specific IgG-aIgA. IgG-aIgA also were detected by IRA in 7.9% of sera from patients with urticarial transfusion reactions and 73% of sera from patients with ataxia telangiectasia and IgA deficiency. Sera from 50 normal blood donors did not have detectable IgG-aIgA. Tests for IgE-aIgA were negative in all cases, including control sera with class specific IgG-aIgA. We conclude that the IRA is a sensitive and reproducible method for detection of class specific and limited specificity IgG-aIgA, and that IgE-aIgA do not mediate urticarial transfusion reactions.

  3. Mast cells mediate neutrophil recruitment and vascular leakage through the NLRP3 inflammasome in histamine-independent urticaria

    PubMed Central

    Nakamura, Yuumi; Saito, Megumu; Nishikomori, Ryuta; Kim, Yun-Gi; Murakami, Makoto; Núñez, Gabriel; Matsue, Hiroyuki

    2009-01-01

    Urticarial rash observed in cryopyrin-associated periodic syndrome (CAPS) caused by nucleotide-binding oligomerization domain–leucine-rich repeats containing pyrin domain 3 (NLRP3) mutations is effectively suppressed by anti–interleukin (IL)-1 treatment, suggesting a pathophysiological role of IL-1? in the skin. However, the cellular mechanisms regulating IL-1? production in the skin of CAPS patients remain unclear. We identified mast cells (MCs) as the main cell population responsible for IL-1? production in the skin of CAPS patients. Unlike normal MCs that required stimulation with proinflammatory stimuli for IL-1? production, resident MCs from CAPS patients constitutively produced IL-1?. Primary MCs expressed inflammasome components and secreted IL-1? via NLRP3 and apoptosis-associated speck-like protein containing a caspase recruitment domain when stimulated with microbial stimuli known to activate caspase-1. Furthermore, MCs expressing disease-associated but not wild-type NLRP3 secreted IL-1? and induced neutrophil migration and vascular leakage, the histological hallmarks of urticarial rash, when transplanted into mouse skin. Our findings implicate MCs as IL-1? producers in the skin and mediators of histamine-independent urticaria through the NLRP3 inflammasome. PMID:19364881

  4. Acute antibody-mediated rejection of skin grafts without involvement of granulocytes or complement

    SciTech Connect

    Bogman, M.J.; Cornelissen, I.M.; Koene, R.A.

    1984-05-01

    In immunosuppressed mice that carry rat skin xeno-grafts, acute antibody-mediated graft rejection (AAR) can be induced by intravenous administration of mouse anti-rat globulin. Dependent on the amount of antibody injected and on the complement status of the recipient, an Arthus-like or a Shwartzman-like pattern of vasculitis occurs. The role of polymorphonuclear granulocytes (PMNs) in either type of vasculitis was tested by inducing AAR in recipients depleted of PMNs by total body irradiation. Despite the absence of PMNs in the graft vessels, AAR occurred both in the Arthus-like and in the Shwartzman-like type. Moreover, AAR could be elicited in PMN-depleted recipients that were complement-depleted by cobra venom factor treatment or were congenitally C5-deficient. We conclude that neither the PMN nor complement is an essential mediator the PMN nor complement is an essential mediator in this form of antibody-mediated vasculitis.

  5. Idiopathic Nonviral Cryoglobulinemia Treated Successfully With Rituximab.

    PubMed

    Kamel, Mahmoud; Thajudeen, Bijin; Bracamonte, Erika; Madhrira, Machaiah

    2014-06-01

    Cryoglobulinemia is a systemic inflammatory syndrome that generally involves small-to-medium vessel vasculitis due to cryoglobulin-containing immune complexes. The therapeutic management of idiopathic cryoglobulinemic vasculitis has yet to be defined because no study has evaluated the best strategies. However, treatment of severe vasculitis is traditionally based on a combination of corticosteroids and immunosuppressants or plasmapheresis, and more recently rituximab. We report a case of 77-year-old female patient diagnosed with idiopathic cryoglobulinemia, treated successfully with 6 months prednisone tapering and 2 doses of rituximab (1 g each dose). After receiving the above-mentioned treatment, her creatinine went back to normal with resolution of proteinuria and hematuria, normalization of serum complements, and significant improvement in her clinical picture. We conclude that rituximab could be an effective treatment for idiopathic cryoglobulnemia. PMID:24914502

  6. [Thrombotic vasculopathy probably associated with cocaine contaminated with levamisole: report of 2 cases].

    PubMed

    Martínez-Velasco, María Abril; Flores-Suárez, Luis Felipe; Toussaint-Caire, Sonia; Rodríguez-Carreón, Angélica; Díaz-Lozano, Marisol; Sánchez-Armendáriz, Karen

    2015-01-01

    The vasculities are complex diseases. Their cutaneous manifestations are very important and often mirror several pathologies. Cocaine use has been related to both, vasculitis and thrombotic vasculopathy and pseudovasculitis. A new syndrome has been described in association with its adulteration with levamisole. It can be very serious, leading patients to death. This is relevant as levamisole-adultered cocaine seems to be increasingly offered to consumers. Our goal is to report the first two cases in Mexico, which faces an important raise in cocaine use, emphasizing that a high suspicion based on certain characteristics allows for early recognition and adequate treatment. PMID:25680649

  7. Takayasu arteritis in a young woman: a 4-year case history.

    PubMed

    Saab, Fadi; Giugliano, Robert P; Giugliano, Gregory R

    2009-01-01

    Takayasu arteritis is a chronic, progressive, autoimmune, idiopathic, large-vessel vasculitis that usually affects young adults. The disease has been reported to occur in all races and ethnicities. The diffuse nature of this vasculitis can affect multiple-organ systems to varying degrees. Herein, we report the case of a young woman whose exertional angina and claudication were the initial presentation of active Takayasu arteritis. During more than 4 years of ongoing treatment, therapy, and follow-up, she has displayed differing disease symptoms of varying intensity. We discuss the challenges of managing Takayasu arteritis in our patient and describe different treatments for this rare vasculitic disorder. PMID:19876432

  8. Kawasaki disease.

    PubMed

    Sundel, Robert P

    2015-01-01

    Kawasaki disease (KD) is the archetypal pediatric vasculitis, exemplifying the unique aspects and challenges of vascular inflammation in children. The condition is almost unheard of in adults, is closely associated with infections, and is self-limited, with fever resolving after an average of 12 days even without treatment. Yet KD is also a potentially fatal disease and the most common cause of acquired heart disease in the developed world. Unraveling of the developmental, immunologic, and genetic secrets of Kawasaki disease promises to improve our understanding of vasculitis in particular, and perhaps also to provide a window on the fundamental mysteries of inflammatory diseases in general. PMID:25399940

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

    PubMed

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

    2015-05-01

    Behçet's disease is an autoimmune systemic vasculitis that can occur after exposure to infectious agents. Behçet's disease also has been associated with HIV infection, including de novo development of this condition during chronic HIV infection and resolution of Behçet's disease symptoms following initiation of antiretroviral therapy. We describe a patient who presented with systemic vasculitis with skin and mucous membrane ulcerations in the setting of acute HIV infection, who was eventually diagnosed with Behçet's disease, demonstrating a possible link between acute HIV infection, immune activation and development of autoimmunity. PMID:24912539

  10. 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

  11. Marked Retroperitoneal Lymphadenopathy in Hairy Cell Leukemia: A Case Report

    PubMed Central

    Shackelford, R.E.; Heldmann, M.; Eskandari, F.; Joshi, N.; Browning, J.; Maxwell, N.; Coteligam, J.

    2013-01-01

    Hairy cell leukemia (HCL) is uncommonly associated with lymphadenopathy, while retroperitoneal lymphadenopathy is extremely uncommon. We report on a patient with a 12-year history of HCL who developed painless jaundice and ascites, accompanied by positional discomfort with persistent nausea. Computed tomography examination revealed 2 large retroperitoneal masses, which at autopsy consisted of HCL with focally intermixed pancreatic and peripancreatic tissue. Lymphadenopathy was not identified above the diaphragm or below the aortic bifurcation. No vasculitis or an unusual HCL histology was identified. As previous reports, our findings suggest that HCL with massive lymphadenopathy has a specific site predilection, but it is not necessarily accompanied by vasculitis or an unusual histology. PMID:24163667

  12. Fibrin(ogen) mediates acute inflammatory responses to biomaterials

    PubMed Central

    1993-01-01

    Although "biocompatible" polymeric elastomers are generally nontoxic, nonimmunogenic, and chemically inert, implants made of these materials may trigger acute and chronic inflammatory responses. Early interactions between implants and inflammatory cells are probably mediated by a layer of host proteins on the material surface. To evaluate the importance of this protein layer, we studied acute inflammatory responses of mice to samples of polyester terephthalate film (PET) that were implanted intraperitoneally for short periods. Material preincubated with albumin is "passivated," accumulating very few adherent neutrophils or macrophages, whereas uncoated or plasma- coated PET attracts large numbers of phagocytes. Neither IgG adsorption nor surface complement activation is necessary for this acute inflammation; phagocyte accumulation on uncoated implants is normal in hypogammaglobulinemic mice and in severely hypocomplementemic mice. Rather, spontaneous adsorption of fibrinogen appears to be critical: (a) PET coated with serum or hypofibrinogenemic plasma attracts as few phagocytes as does albumin-coated material; (b) in contrast, PET preincubated with serum or hypofibrinogenemic plasma containing physiologic amounts of fibrinogen elicits "normal" phagocyte recruitment; (c) most importantly, hypofibrinogenemic mice do not mount an inflammatory response to implanted PET unless the material is coated with fibrinogen or the animals are injected with fibrinogen before implantation. Thus, spontaneous adsorption of fibrinogen appears to initiate the acute inflammatory response to an implanted polymer, suggesting an interesting nexus between two major iatrogenic effects of biomaterials: clotting and inflammation. PMID:8245787

  13. Bench-to-bedside review: Pulmonary–renal syndromes – an update for the intensivist

    Microsoft Academic Search

    Spyros A Papiris; Effrosyni D Manali; Ioannis Kalomenidis; Giorgios E Kapotsis; Anna Karakatsani; Charis Roussos

    2007-01-01

    The term Pulmonary–renal syndrome refers to the combination of diffuse alveolar haemorrhage and rapidly progressive glomerulonephritis. A variety of mechanisms such as those involving antiglomerular basement membrane antibodies, antineutrophil cytoplasm antibodies or immunocomplexes and thrombotic microangiopathy are implicated in the pathogenesis of this syndrome. The underlying pulmonary pathology is small-vessel vasculitis involving arterioles, venules and, frequently, alveolar capillaries. The underlying

  14. 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

  15. Coeliac disease in patients with Kawasaki disease. Is there a link?

    Microsoft Academic Search

    S. Stagi; G. Simonini; L. Ricci; M. de Martino; F. Falcini

    2006-01-01

    Objective. Kawasaki disease (KD) is an acute febrile systemic vasculitis, mainly affecting infants and young children. Immunological abnormalities during the acute phase of KD have been described extensively. However, the occurrence of a second immunological disorder in a patient with a history of KD is rarely reported. We evaluated the presence of autoimmune thyroiditis and coeliac disease (CD) in patients

  16. Increased nitric oxide production in patients with Behçet's disease: Is it a new activity marker?

    Microsoft Academic Search

    Cem Evereklioglu; Yusuf Turkoz; Hamdi Er; H. Serhat Inaloz; Emin Ozbek; Mustafa Cekmen

    2002-01-01

    Background: The origin of Behçet's disease (BD) is unclear. One of the prominent features of BD is vasculitis and thrombosis as a result of endothelial dysfunction. Because nitric oxide (NO) is synthesized by endothelium, we considered it as an interesting target of investigation in BD. Objective: Our purpose was to define the level of NO in the serum of patients

  17. REVIEW Open Access Lupus enteritis: from clinical findings to

    E-print Network

    Paris-Sud XI, Université de

    REVIEW Open Access Lupus enteritis: from clinical findings to therapeutic management Peter Janssens steroid responsiveness, timely diagnosis becomes primordial for the adequate management of this rare [8]. In the BILAG 2004, lupus enteritis is de- fined as either vasculitis or inflammation

  18. Microscopic Polyangiitis in a Pediatric Patient

    Microsoft Academic Search

    Sujatha Ramesh; Yongxin Chen; Cazibe Celik; Edwin Jenis; Julian L. Ambrus

    icroscopic polyangiitis (MPA), previously called hypersensitivity angiitis, is a sys- temic necrotizing vasculitis that involves many organ systems including the skin, joints, kidneys, and lungs. Microscopic polyangiitis most commonly affects adults in the fourth and fifth decades of life, with only a few cases reported in children. We describe a pediatric patient with microscopic polyangiitis. Arch Fam Med. 2000;9:1189-1192 REPORT

  19. Oral lesions as the first clinical sign of microscopic polyangiitis: A case report

    Microsoft Academic Search

    Caroline H. Shiboski; Joseph A. Regezi; Henry C. Sanchez; Sol Silverman

    2002-01-01

    Microscopic polyangiitis (MPA) is defined as a systemic necrotizing vasculitis that clinically and histologically affects small-sized vessels without granulomata. The main clinical feature of MPA is renal involvement characterized by rapidly progressing glomerulonephritis. We report a case of oral lesions, initially thought to be associated with Wegener's granulomatosis, as the first sign of MPA in an otherwise healthy man. To

  20. Atrial myxoma masquerading as Takayasu’s arteritis

    PubMed Central

    Fung, Kenneth; Edmondson, Stephen

    2014-01-01

    Lesson We describe the case of a 48-year-old woman whose atrial myxoma was mistaken for vasculitis. The case report highlights the reasons why these two disorders may become confused, the dangers of initiating the wrong treatment and a simple means of avoiding misdiagnosis. PMID:25408917

  1. Advances in the Etiology and Management of Immune-mediated Glomerulonephritides

    Microsoft Academic Search

    JEREMY S. DUFFIELD; AMIR QAMAR; Joseph V. Bonventre; Barry M. Brenner; Jessamyn Bagley; D. Sangeeta Bhattia; Steven Brunelli; Anil K. Chandraker; David M. Charytan; Mary Choi; Kenneth B. Christopher; Gary C. Curhan; Bradley M. Denker; John P. Forman; Markus H. Frank; Monica Grafals; Indira Guleria; Dirk M. Hentschel; Andreas Herrlich; Li-Li Hsiao; Benjamin D. Humphreys; John J. Iacomini; D. Vicki Rubin Kelley; Julie Lin; M. P. H. Edgar; L. Milford; David B. Mount; Nader Najafian; Shona Pendse; Martin R. Pollak; Mohamed H. Sayegh; Julian L. Seifter; Jagesh V. Shah; Alice M. Sheridan; Ajay K. Singh; Theodore I. Steinman; Eric N. Taylor; John K. Tucker; Takuya Ueno; Sushrut S. Waikar; Wolfgang C. Winkelmayer; D. Xueli Yuan

    Immune-mediated inflammatory disease of the glomerulus remains a particular chal - lenge for the nephrologist, whether it is limited to the kidney or a part of a multisystem disease. Studies on animal models and the development of new viral detection assays have led to notable advances in our understanding of disease mechanisms in antineutrophil cyto - plasmic antibody (ANCA)-associated vasculitis

  2. Anthrax vaccine associated deaths in miniature horses.

    PubMed

    Wobeser, Bruce K

    2015-04-01

    During a widespread anthrax outbreak in Canada, miniature horses were vaccinated using a live spore anthrax vaccine. Several of these horses died from an apparent immune-mediated vasculitis temporally associated with this vaccination. During the course of the outbreak, other miniature horses from different regions with a similar vaccination history, clinical signs, and necropsy findings were found. PMID:25829553

  3. [Wegener's granulomatosis diagnosed by orbital-meningeal presentation: a case report].

    PubMed

    Louati, H; Chebbi, A; Ben Hassen, I; Douira, W; Ben Hassine, L; Lahmar, L; Ayed, S; Bellagha, I

    2012-10-01

    Wegener's granulomatosis is a necrotizing granulomatous vasculitis with a strong affinity for the upper respiratory tract, lung and kidney. The ophthalmologic manifestation most often presents as inflammatory orbital pseudotumor or scleritis. We report a case of a 27-year-old woman with an orbital-meningeal presentation leading to a diagnosis of Wegener's granulomatosis. PMID:22975270

  4. Henoch–Schönlein purpura: a clinical case with dramatic presentation

    PubMed Central

    Bento, João; Magalhães, Adriana; Moura, Conceição Souto; Hespanhol, Venceslau

    2010-01-01

    A case study involving a 55-year-old Caucasian male with end-stage glomerulosclerosis is presented here. Kidney biopsies showed no deposits on imunofluorescent microscopy. Relapsing massive haemoptysis and suspected bronchovascular malformation required lung lobectomy which revealed malformative and tortuous small blood vessels, with no vasculitis. Blood antinuclear antibodies, antineutrophil cytoplasmic antibodies and antiglomerular basement membrane antibodies were undetectable. Seric immunoglobulins and complement levels were normal. Three months later, arthralgia and joint oedema first appeared. Skin biopsy revealed vasculitis immune-reactive to immunoglobulin A. Systemic corticotherapy was then started. Two weeks later, the patient presented with abdominal pain melena and rectal bleeding (haematoquesia). Endoscopic study showed diffuse gastrointestinal haemorrhage. Angiographic study revealed diffuse lesions compatible with vasculitis and haemorrhage from multiple spots. Cyclophosphamide and then intravenous immunoglobulin were associated to treatment without response. Increasing blood loss occurred, with massive gastrointestinal haemorrhage and haemorrhagic ascitis. Death occurred due to uncontrolled diffuse bleeding. Necropsy findings showed generalised small vessels vasculitis compatible with Henoch–Schönlein purpura. PMID:22778211

  5. 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...

  6. zentrum chirurgie ABTEIlUNG THORAX-, HERZ-UND GEFSSCHIRURGIE centre for Surgery DEPARTMENT OF THORACIC AND CARDIOVASCUlAR SURGERy

    E-print Network

    Gollisch, Tim

    OF THORACIC AND CARDIOVASCUlAR SURGERy Abteilungsdirektor/in | Head of Department Prof. Dr. med. DiplHirurGie Centre for Surgery depArtment of tHorAcic And cArdioVAsculAr surGery Einleitung Die Abteilung vertrittzentrum chirurgie ABTEIlUNG THORAX-, HERZ- UND GEFäSSCHIRURGIE centre for Surgery DEPARTMENT

  7. Endothelial progenitor cell dysfunction in rheumatic disease

    Microsoft Academic Search

    Marianne C. Verhaar; Peter E. Westerweel

    2009-01-01

    Rheumatic disease is characterized by inflammation and endothelial dysfunction, which contribute to accelerated atherosclerosis. Circulating endothelial progenitor cells (EPCs) can restore dysfunctional endothelium and thereby protect against atherosclerotic vascular disease. The number and function of EPCs are, however, affected in rheumatic diseases such as psoriatic arthritis, rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, and antineutrophil cytoplasmic autoantibody-associated vasculitis. Rheumatic disease

  8. Detection and Multigenic Characterization of a Herpesvirus Associated with Malignant Catarrhal Fever in White-Tailed Deer (Odocoileus virginianus) from Missouri

    Microsoft Academic Search

    Steven B. Kleiboeker; Margaret A. Miller; Susan K. Schommer; Jose A. Ramos-Vara; Magalie Boucher; Susan E. Turnquist

    2002-01-01

    Between 1998 and 2001, tissues from four captive white-tailed deer were observed to have histologic lesions of systemic lymphocytic vasculitis. These lesions suggested malignant catarrhal fever, although epizootic hemorrhagic disease and bluetongue were included in the differential diagnosis. Initial diagnostic efforts, including virus isolation and reverse transcription-PCR for epizootic hemorrhagic disease virus and blue- tongue virus, failed to identify an

  9. Experimental aerosol infection of cattle ( Bos taurus) with ovine herpesvirus 2 using nasal secretions from infected sheep

    Microsoft Academic Search

    Naomi S. Taus; J. Lindsay Oaks; Katherine Gailbreath; Donald L. Traul; Donal O’Toole; Hong Li

    2006-01-01

    Infection of clinically susceptible ruminants, including domesticated cattle and American bison, with ovine herpesvirus 2 (OvHV-2) can result in the fatal lymphoproliferative and vasculitis syndrome known as malignant catarrhal fever (MCF). A reliable experimental infection model is needed to study the pathogenesis of MCF and to develop effective vaccination strategies to control the disease. An experimental aerosol infection model using

  10. [What's new in internal medicine?].

    PubMed

    Doutre, M-S

    2013-11-01

    In this paper based on a review of medical articles from September 2012 to September 2013, new data were selected about IgG4-related disease, connections between vitamin D and systemic lupus erythematosus, revised nomenclature of vasculitis, effects of salt on autoimmunity, new autoinflammatory syndromes and some diseases as systemic sclerosis and thrombangiitis obliterans. PMID:24365498

  11. Granulomatosis with Polyangiitis Presenting as a Parotid Gland Abscess

    PubMed Central

    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.

  12. The relevance of large-vessel vascular disease and restricted ankle movement to the aetiology of leg ulceration in rheumatoid arthritis

    Microsoft Academic Search

    E. R. MCRORIE; C. V. RUCKLEY; G. NUKI

    1998-01-01

    SUMMARY Leg ulceration in rheumatoid arthritis (RA) without systemic vasculitis is a diYcult clinical problem and a common cause of morbidity. We have assessed venous function, arterial pressures and range of ankle movement in 23 RA patients with a leg ulcer and compared the results with those in the non-ulcerated contralateral limb and in 25 RA patients matched for age

  13. Renal Abnormalities in Psoriatic Patients: A Review

    Microsoft Academic Search

    J. Heuvels; A. Maximus; J. L. Bosmans; J. Lambert; M. E. De Broe

    1999-01-01

    Numerous systemic diseases affect both skin and kidneys: autoimmune diseases (e.g. lupus, vasculitis), hematologic abnormalities, genetic disorders and others have been described (table 1). Although psoriasis is considered an immunomediated disease, it is assumed to affect the skin and joints exclusively. Psoriasis [1] is a common chronic inflammatory disorder of the skin, which affects more than 2% of people with

  14. Myeloperoxidase-antineutrophil cytoplasmic antibody-positive crescentic glomerulonephritis complicating the course of Graves' disease: Report of three adult cases

    Microsoft Academic Search

    Masayuki Tanemoto; Hiroshi Miyakawa; Junichi Hanai; Masako Yago; Masafumi Kitaoka; Shunya Uchida

    1995-01-01

    Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis has been recently recognized in Graves' disease patients treated with propylthiouracil. We have experienced three adult cases of Graves' disease with main features being renal derangements. All three patients, who were between the ages of 22 and 82 years, had been treated with propylthiouracil for 2 to 5 years after a diagnosis of Graves' disease.

  15. Textbook of rheumatology

    SciTech Connect

    Turner, R.A.; Wise, C.M.

    1986-01-01

    This book contains 23 papers. Some of the titles are: Diagnostic Radiology in the Rheumatic Diseases; Laboratory Testing in Rheumatology; Arthritis Nursing and the Team Approach in the Management of Rheumatic Disease; The Surgical Management of Arthritis; Vasculities; Neoplasms of Bone and Joints; and Rheumatic Disease of Childhood.

  16. Oligoclonal IgA Response in the Vascular Wall in Acute Kawasaki Disease

    Microsoft Academic Search

    Anne H. Rowley; Stanford T. Shulman; Benjamin T. Spike; Carrie A. Mask; Susan C. Baker

    2001-01-01

    Kawasaki Disease (KD) is a potentially fatal acute vasculitis of childhood. Although KD is the leading cause of acquired heart disease in children in developed nations, its pathogenesis remains unknown. We previously reported the novel observation that IgA plasma cells infiltrate the vascular wall in acute KD. We have now examined the clonality of this IgA response in vascular tissue

  17. Use and Perceived Credibility of Medication Information Sources for Patients with a Rare Illness: Differences by Gender

    Microsoft Academic Search

    Delesha M. Carpenter; Robert F. DeVellis; Susan L. Hogan; Edwin B. Fisher; Brenda M. DeVellis; Joanne M. Jordan

    2011-01-01

    Patients with rare illnesses may use medication information sources that are appreciably different from those used by patients with more common illnesses. This article's purpose is to describe vasculitis patients' most frequently used medication information sources, determine which sources patients perceive as credible, and explore gender differences in source use and perceived credibility. Using an online questionnaire, patients (n = 232) indicated

  18. [Vasculitic peripheral neuropathy].

    PubMed

    Oya, Yasushi

    2013-11-01

    The typical clinical manifestation of vasculitic peripheral neuropathy is sensory-dominant multiple mononeuropathy, although it can progress to distal-dominant sensorimotor polyneuropathy. It is painful in most cases. Peripheral nerves may be the most prone to produce symptoms of the vasculitis. Nerve conduction studies show reduced amplitude of M wave or sensory nerve action potential, which depends on the degree of injury of a nerve examined. Wallerian degeneration can cause pseudo-conduction block in the acute stage and temporal dispersion in the chronic stage. However, a definite diagnosis requires histological confirmation. Combined biopsy of the sural nerve and the peroneus brevis muscle can be performed by a single incision. Skin biopsy can also be performed. To increase the diagnostic yield, biopsy specimens are prepared in different manners to observe as many cross sections as possible: frozen unfixed, formalin-fixed paraffin-embedded, and glutaraldehyde-fixed epon embedded specimens, as well as teased fiber preparation of a nerve. Vasculitic peripheral neuropathy usually results from small-vessel vasculitis. There are still controversies regarding the classification of vasculitides. Differential diagnosis of vasculitis includes infection and lymphoma. Delayed diagnosis and treatment of neuropathy result in the impairment of ADL and QOL. Recovery from axonal degeneration usually takes time and is not always possible. Treatment includes corticosteroid, cyclophosphamide, and intravenous immunoglobulin administration; however, the intensity of treatment depends on the disease activity of vasculitis. PMID:24200608

  19. Coil embolization of an inferior pancreaticoduodenal artery aneurysm associated with celiac artery occlusion

    Microsoft Academic Search

    Brian G Peterson; Scott A Resnick; Mark K Eskandari

    2003-01-01

    Pancreaticoduodenal arcade aneurysms are rare. Untreated, these lesions enlarge progressively and have the potential for spontaneous rupture. Aneurysmal degeneration of pancreaticoduodenal arcade vessels is known to be associated with celiac artery occlusion, vasculitis, and certain connective tissue disorders. Given their precarious location, surgical expiration is a challenging endeavor. Innovations in endovascular techniques offer a possible alternative.We report a case of

  20. 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

  1. 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

  2. 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

  3. Suspected Sarcocystis infections of the bovine placenta and foetus

    Microsoft Academic Search

    B. L. Munday; H. Black

    1976-01-01

    Summary Protozoan parasites resembling Sarcocystis schizonts were found in the brains of 2 bovine foetuses and the placentas of another 4. As typical of Sarcocystis, the organisms were located mainly within vascular endothelial cells, and frequently the zoites within the schizonts were arranged in a rosette or palisade fashion. Pathological changes noted were placentitis, myocarditis, pulmonary vasculitis and encephalitis. Attempts

  4. 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 ...

  5. A novel bocavirus in canine liver

    E-print Network

    Li, Linlin; Pesavento, Patricia A.; Leutenegger, Christian M.; Estrada, Marko; Coffey, Lark L.; Naccache, Samia N.; Samayoa, Erik; Chiu, Charles; Qiu, Jianming; Wang, Chunlin; Deng, Xutao; Delwart, Eric

    2013-02-13

    gastroenteritis, necrotizing vasculitis, granulomatous lymphadenitis and anuric renal failure, we identified and characterized a novel bocavirus we named Canine bocavirus 3 (CnBoV3). The three major ORFs of CnBoV3 (NS1, NP1 and VP1) shared less than 60% aa...

  6. Rituximab Induced Left Bundle Branch Block

    PubMed Central

    Sheikh, Mujeeb; Moza, Ankush; Grubb, Blair P.

    2015-01-01

    Rituximab (a monoclonal antibody directed against CD 20) therapy can be acutely complicated by infusion reactions and cardiac arrhythmia on rare occasions. We report the first case of a new onset left bundle branch block (LBBB) after rituximab therapy for Wegener's vasculitis.

  7. A 27-year-old woman presenting with refractory hypoxaemic respiratory failure, haemoptysis and thyrotoxicosis: a rare manifestation of propylthiouracil therapy.

    PubMed

    Ortiz-Diaz, Enrique O

    2014-01-01

    Pulmonary manifestations of hyperthyroidism not only include pulmonary hypertension and hydrostatic pulmonary oedema, but also treatment/drug-associated pulmonary diseases have to be considered as an exclusion diagnosis. A 27-year-old woman with hypoxaemic respiratory failure under an arterial-venous extra-corporeal membrane oxygenator (AV-ECMO) was admitted to the intensive care unit (ICU). The patient had progressive dyspnoea with haemoptysis, palpitations and failure to thrive. The patient had Graves' disease treated previously with propylthiouracil (PTU). Diffuse alveolar haemorrhage is a non-specific syndrome characterised by evidence of diffuse alveolar damage, exclusion of infectious aetiology and progressively bloodier bronchoalveolar lavage (and/or 20% hemosiderin laden macrophages on cytological examination). PTU associated perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) vasculitis appears to be more common in younger female patients presenting with leukocytoclastic vasculitis, myalgias and arthralgias. The latter compared to non-drug associated ANCA vasculitis which are more common in older males with visceral involvement. PTU-induced ANCA vasculitis prognosis appears to be better compared to primary ANCA syndromes. PMID:25150234

  8. 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...

  9. Cryoglobulinemia presenting after liver transplantation

    Microsoft Academic Search

    J Gournay; LD Ferrell; JP Roberts; NL Ascher; TL Wright; JR Lake

    1996-01-01

    Essential mixed cryoglobulinemia is frequently associated with chronic hepatitis C. Three patients undergoing transplantation for end-stage chronic hepatitis C in whom cryoglobulinemia with vasculitis developed after transplantation are described. Hepatitis C virus (HCV) infection was confirmed in the 3 patients by the presence of HCV RNA detected by polymerase chain reaction. The time interval between transplantation and the first expression

  10. Infection of Peripheral Blood Mononuclear Cells by Hepatitis C Virus in Mixed Cryoglobulinemia

    Microsoft Academic Search

    Clodoveo Ferri; Monica Monti; Luca La Civita; Giovanni Longombardo; Francesco Greco; Giampiero Pasero

    1993-01-01

    IXED CRYOGLOBULINEMIA (MC) is a lympho- M proliferative disorder characterized clinically by purpura, arthralgias, weakness, and various organ involve- ments such as peripheral neuropathy, chronic hepatitis, glo- merulonephritis, and generalized vasculitis.' The typical pathologic lesions involving small- and medium-sized ves- sels are due to deposition of circulating immune-complexes (CIC) and complement.',' In MC patients, a monoclonal rheumatoid factor in the

  11. Efficacy and safety of rituximab in type II mixed cryoglobulinemia

    Microsoft Academic Search

    Francesco Zaja; Salvatore De Vita; Cesare Mazzaro; Stefania Sacco; Daniela Damiani; Ginevra De Marchi; Angela Michelutti; Michele Baccarani; Renato Fanin; Gianfranco Ferraccioli

    ready administered at the time of recruit- ment. All patients had active disease, poorly controlled or difficult to manage with previous treatments, including corti- costeroids in all. Efficacy and safety of rituximab therapy were evaluated in the following 6 months. The overall follow-up after rituximab treatment ranged from 9 to 31 months. Rituximab proved effective on skin vasculitis manifestations (ulcers,

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

    PubMed

    Nobles, Irma J; Khan, Safdar

    2015-04-01

    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

  13. Churg-Strauss syndrome in childhood: a case report.

    PubMed

    Albahri, Ziad; Minxová, Lenka; Lukeš, Antonín; Mawiri, Abdul Al; Štefá?ková, Šárka

    2014-10-01

    Churg-Strauss syndrome is a rare form of small-vessel vasculitis. In the current report, we describe the case of a 17-year-old Czech girl predominantly characterized by peripheral neuropathy, the presence of cardiac and pulmonary involvement, hypereosinophilia, asthma, and sinusitis that led to the diagnosis of Churg-Strauss syndrome. PMID:24170259

  14. Factors influencing immune complex localisation

    Microsoft Academic Search

    L. Schrieber; R. Penny

    1984-01-01

    In systemic immune complex (IC) diseases such as SLE and rheumatoid vasculitis, IC accumulate in a number of tissues, either after deposition from the circulation or from in situ formation. The tissue localisation of IC depends on a delicate balance between the production of IC and the ability of the mononuclear phagocytic system (MPS) to remove them from blood. At

  15. 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

  16. Polyarteritis nodosa in patients with Familial Mediterranean Fever (FMF): A concomitant disease or a feature of FMF?

    Microsoft Academic Search

    Seza Ozen; Eldad Ben-Chetrit; Aysin Bakkaloglu; Hanan Gur; Keriman Tinaztepe; Meral Calguneri; Cetin Turgan; Aydin Turkmen; Ilkser Akpolat; Murat Danaci; Nesrin Besbas; Tekin Akpolat

    2001-01-01

    Background: Familial Mediterranean Fever (FMF) is caused by mutations in the gene encoding pyrin and is characterized by self-limited, recurrent attacks of fever and serositis. Vasculitis has been increasingly reported in FMF. A study evaluating the prognosis in FMF and polyarteritis nodosa (PAN) patients has not been reported previously. Objectives: To determine the special characteristics and the prognosis of PAN

  17. Anticardiolipin Antibodies in Myelodysplastic Syndromes

    Microsoft Academic Search

    Izidore S. Lossos; Yaacov Matzner

    1999-01-01

    The myelodysplastic syndromes (MDS) are a heterogeneous group of clonal hematopoietic stem cell disorders characterized by bone marrow dysplasia, chronic cytopenias and a high propensy for evolution into acute leukemia. A spectrum of immunological abnormalities has been reported in MDS, including cutaneous vasculitis, peripheral neuropathy and lupus-like syndromes [1]. Laboratory studies may reveal raised or reduced levels of serum immunoglobulins,

  18. Therapy for vasculitic neuropathies.

    PubMed

    Gorson, Kenneth C

    2006-03-01

    The term vasculitis refers to a pathologic condition defined by inflammatory cell infiltration and destruction of blood vessels. Systemic vasculitis is classified as primary (eg, polyarteritis nodosa, Churg-Strauss syndrome) or secondary, the latter associated with connective tissue disorders, infections, medications, and rarely, as a paraneoplastic phenomenon. Neuropathy is a common complication of systemic vasculitis and is related to ischemic nerve fiber damage with axon loss. Peripheral neuropathy may be the sole manifestation of vasculitis, a condition termed nonsystemic vasculitic neuropathy (NSVN). Treatment of vasculitic neuropathy requires long-term immunosuppressive therapies with potential side effects. The diagnosis of vasculitis should be established by tissue (preferably nerve) biopsy. High-dose prednisone is the standard platform therapy for patients with systemic and NSVN; for those with systemic vasculitis, at least 3 to 12 months of treatment with cyclophosphamide (monthly intravenous pulse or daily oral therapy) is also necessary to sustain remission and allow successful prednisone tapering. The use of cyclophosphamide in patients with NSVN is controversial, but recent retrospective data suggest that those treated with prednisone and cyclophosphamide from the outset fare better than those initially treated only with prednisone. If prednisone is administered as monotherapy, cyclophosphamide should be added after several months if there is no improvement or relapse occurs with tapering of prednisone. Intravenous pulse and daily oral cyclophosphamide probably offer similar efficacy, although the risk of complications is greater with oral therapy. Azathioprine can be safely substituted for cyclophosphamide after 3 months without an increased relapse rate. Azathioprine, methotrexate, intravenous immune globulin, mycophenolate mofetil, plasma exchange, and rituximab can be offered to patients who are intolerant or have a contraindication to cyclophosphamide. However, efficacy is unproven for any of these therapies. Interferon-alpha, sometimes combined with plasma exchange, is used to treat vasculitis associated with hepatitis B infection. Some patients also may improve with corticosteroids. The classification of diabetic lumbosacral radiculoplexus neuropathy as a vasculitic disorder remains controversial. However, there is compelling pathological evidence that this condition represents a T-cell-mediated microvasculitis. Some patients treated with intravenous corticosteroids may have greater recovery and improved pain control. PMID:16464407

  19. Epidemiology: Most common in children with over half less than 5 yrs old. Disease is more severe in older children and adults, especially with regards to renal involvement. Often follows an upper respiratory infection but a recent study suggests that an occult malignancy may be the cause. Pathophysiology: Correlation with recent illness suggests an antigenic precipitant to the ensuing vasculitis. Very similar to IgA nephropathy and will see IgA deposits in skin and kidneys on biopsy. Interestingly, pts with renal involvement also have IgG antibodies against mesangial cells

    Microsoft Academic Search

    Henoch-Schonlein Purpura

    Treatment: Most recover on their own without treatment. Dapsone may help articular, GI, and skin disease. In those with renal disease, particularly if it is severe, steroids (methylprednisolone followed by 3 months of oral prednisone at 1mg\\/kg\\/d) are often given. Other therapies (usually in conjuction with steroids) that have been tried are cyclophosphamide, azathioprine, plamapheresis, and IVIG; none have been

  20. 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

  1. Host modifier genes affect mouse autoimmunity induced by the lpr gene.

    PubMed Central

    Wang, Y.; Nose, M.; Kamoto, T.; Nishimura, M.; Hiai, H.

    1997-01-01

    A defect in apoptotic signal transmission through CD95 is an essential genetic mechanism for lymphoproliferation and autoimmunities in lpr or gld mice. However, disease manifestations are largely affected by the host genetic background. To identify and map such host genes modifying lpr gene effect, ie, the lpr modifier (Lprm) genes, 82 MRL/lpr x (MRL/lpr x C3H/lpr) F1 mice were subjected to immunopathological and genetical analyses. High-grade vasculitis and glomerulonephritis among backcross mice were observed in separate groups of mice. Microsatellite analysis revealed that there were two host genes affecting the occurrence of vasculitis, Lprm1 (chromosome 4) and Lprm2 (chromosome 3). A recessive MRL allele at Lprm1 enhanced vasculitis to occur in both sexes, whereas that of Lprm2 inhibited its development selectively in females. Genotype combinations of these two genes explained the severity of vasculitis in crosses of MRL/lpr and C3H/lpr mice and also the vasculitis-prone recombinant inbred strain McH5/lpr. A recessive MRL allele at Lprm3 (chromosome 14) suppressed glomerulonephritis. The weight of the spleen was increased by a recessive MRL allele at Lprm4 (chromosome 5) yielding a logarithm of odds score of 2.02 in a quantitative trait locus analysis. In contrast, the weight of axillary lymph nodes was increased by a recessive MRL allele at a locus on chromosome 2, but its presence was not supported by the quantitative trait locus analysis. The titer of anti-dsDNA autoantibody was controlled by the locus Lprm5 on chromosome 16, which had an logarithm of odds score of 3.41. Possible candidate genes for Lprm genes deduced from their map locations are discussed and compared with the autoimmunity genes reported thus far. In conclusion, autoimmune disease manifestations by the lpr mutation are affected by multiple host genes separately. PMID:9403730

  2. [Intermittent rheumatism revealing a familial syndrome. Arthritis--urticarian eruptions--deafness: Muckle-Wells syndrome without kidney amylosis].

    PubMed

    Prost, A; Barrière, H; Legent, F; Cottin, S; Wallez, B

    1976-03-01

    At a consultation with a 31-year-old man, motivated by painful episodes of joint pain that had started considerably earlier, a familial disease entity was discovered that included the three clinical signs of the Muckle and Wells syndrome : urticarial eruption, intermittent pain in the limbs originating in the joints, and bilateral deafness of perception. In spite of the absnece of renal amylosis, the similarity of the characteristics of these three elements, as well as the nature of the biochemical disturbances, and the mode of transmission, led the authors to consider these observations in the contest of this syndrome. The joint manifestations being indicative, the signs of this intermittent rheumatism are described, as well as the characteristics of the other disorders. PMID:1083554

  3. NOMID: the radiographic and MRI features and review of literature.

    PubMed

    Zaki, Faizah Mohd; Sridharan, Radhika; Pei, Tan Sook; Ibrahim, Sharaf; Ping, Tang Swee

    2012-03-01

    Neonatal onset multisystem inflammatory disease (NOMID) is a rare autoinflammatory disorder, which manifests early in infancy. We describe a case of a 10-year-old boy who has been unwell since infancy. He presented with urticarial rash, intermittent fever and hepatosplenomegaly followed by progressive arthropathy. His joint symptoms started at two years of age, which progressively involved multiple joints, resulting in bone and joint deformities. A series of joint radiographs demonstrated bizarre enlarging physeal mass with heterogenous calcification. Magnetic resonance imaging (MRI) of the involved right ankle and knee showed characteristic thickened and calcified physeal lesions, which enhanced post-gadolinium. This debilitating disease is also known to involve the central nervous system and eyes. This case report aims to highlight the conventional radiographic and magnetic resonance imaging (MRI) findings of this physeal abnormality in NOMID syndrome. PMID:22690285

  4. Purpuric bullous pemphigoid.

    PubMed

    Marovt, Maruska; El Shabrawi-Caelen, Laila

    2015-02-01

    : Rare clinical variants of bullous pemphigoid (BP) include vesicular BP, dyshidrosiform BP, pemphigoid nodularis, seborrheic BP, pemphigoid vegetans, localized BP, erythrodermic BP, and juvenile BP. To our knowledge, this is the first report of an unusual case of purpuric BP. We present a case of 85-year-old white man who presented with a 2-week history of blisters and pruritic urticarial lesions all over his body. The diagnosis of purpuric BP was made on the basis of history, clinical presentation, histopathology report, direct and indirect immunofluorescence studies the diagnosis of purpuric BP was made. The reason for the development of palmoplantar purpuric lesions concomitant to ordinary patches and plaques of BP is unknown. PMID:25607328

  5. Bedbugs and Infectious Diseases

    PubMed Central

    Blanc, Véronique; Del Giudice, Pascal; Levy-Bencheton, Anna; Chosidow, Olivier; Marty, Pierre; Brouqui, Philippe

    2011-01-01

    Bedbugs are brown and flat hematophagous insects. The 2 cosmopolite species, Cimex lectularius and Cimex hemipterus, feed on humans and/or domestic animals, and recent outbreaks have been reported in occidental countries. Site assessment for bedbug eradication is complex but can be assured, despite emerging insecticide resistance, by hiring a pest-control manager. The common dermatological presentation of bites is an itchy maculopapular wheal. Urticarial reactions and anaphylaxis can also occur. Bedbugs are suspected of transmitting infectious agents, but no report has yet demonstrated that they are infectious disease vectors. We describe 45 candidate pathogens potentially transmitted by bedbugs, according to their vectorial capacity, in the wild, and vectorial competence, in the laboratory. Because of increasing demands for information about effective control tactics and public health risks of bedbugs, continued research is needed to identify new pathogens in wild Cimex species (spp) and insecticide resistance. PMID:21288844

  6. Metabisulfite challenge in patients with idiopathic anaphylaxis.

    PubMed

    Sonin, L; Patterson, R

    1985-01-01

    Bronchospasm, urticaria, angioedema, and anaphylactoid reactions have been attributed to sulfiting agents. These compounds are commonly used as preservatives in some drugs and foods, often in restaurants. Oral challenges with sodium metabisulfite were performed in 12 patients with idiopathic anaphylaxis (IA). Nine of these had episodes possibly caused by sodium metabisulfite because of clear temporal relation of episodes of IA to restaurant meals. One patient who had severe urticaria with exacerbations with restaurant meals and 10 control subjects were studied. No urticarial, asthmatic, or anaphylactoid reactions occurred in our patients. Sulfiting agents did not trigger episodes of IA in these 12 patients or urticaria in the patient with severe chronic idiopathic urticaria. PMID:3968330

  7. Myocardial infarction associated with eosinophilia and plasma extravasation at multiple sites. A variant of Kounis syndrome.

    PubMed

    Gunawardena, Mudalige Don Vajira Malin; Weerasinghe, Anura; Herath, Jagath; Amarasena, Naomali

    2015-01-01

    Myocardial infarction occurring during the course of type I hypersensitivity constitutes Kounis syndrome. We report a case of a 38-year-old man who presented with anterior ST elevation myocardial infarction and peripheral blood eosinophilia. He had rhinitis and malaise for several days prior to presentation. There was no urticarial rash or pruritus to suggest hypersensitivity. Coronary angiogram revealed only mild plaque disease. Blood investigations revealed moderate eosinophilia and elevated IgE levels. CT of the thorax revealed fluid extravasation at multiple sites. Screening for a possible secondary cause for eosinophilia revealed hypersensitivity to multiple antigens. A diagnosis of Kounis syndrome was made. Within days of starting steroids and antihistamines, the patient's eosinophil count returned to normal with improvement of clinical picture. This case differs from classical Kounis syndrome as there was no acute allergic reaction (except atopic rhinitis). Fluid extravasation at multiple sites has not been described in previous cases. PMID:25608982

  8. Identification of Helicobacter pylori in skin biopsy of prurigo pigmentosa.

    PubMed

    Missall, Tricia A; Pruden, Samuel; Nelson, Christine; Fohn, Laurel; Vidal, Claudia I; Hurley, M Yadira

    2012-06-01

    A 23-year-old Chinese man presented with a 3-year history of a pruritic eruption. On examination, pink urticarial papules associated with hyperpigmented reticulated patches were noted on his neck, back, and upper chest. Histopathology revealed vacuolar interface dermatitis and numerous gram-negative rods within a dilated hair follicle. The organisms were reactive with anti-Helicobacter pylori immunohistochemisty. The histologic findings and clinical presentation support the diagnosis of prurigo pigmentosa. Additional testing demonstrated a positive urease breath test and serum H. pylori IgG antibodies. The patient was referred to gastroenterology and treated with appropriate antibiotics. After treatment, esophagogastroduodenoscopy revealed chronic gastritis without evidence of H. pylori infection and his skin showed reticulated hyperpigmented patches without evidence of active inflammatory papules. Although previous reports have associated prurigo pigmentosa to H. Pylori gastritis, this is the first report of H. pylori organisms identified in a skin biopsy of prurigo pigmentosa. PMID:22197863

  9. A 41-year-old woman with shortness of breath and history of rash and recurrent laryngeal edema.

    PubMed

    Ataya, Ali; Faruqi, Ibrahim; Salgado, Juan C

    2015-02-01

    A 41-year-old Hispanic woman with a 20 pack-year smoking history presented with worsening shortness of breath on exertion that gradually started 2 years ago, then significantly deteriorated over the last 4 months. She was diagnosed with COPD 2 months prior to her presentation and started on treatment with fluticasone propionate and albuterol. Her medical history was relevant for undifferentiated connective tissue disorder diagnosed 5 years prior due to a positive antinuclear antibody test, arthralgia, recurrent urticarial skin rash, peripheral neuropathy, abdominal pain, and diffuse body swelling. She was started on treatment with prednisone and azathioprine at the time and had substantial improvement in the occurrence of her urticaria. She also had a history of recurrent laryngeal edema of unclear etiology. She had no history of IV drug abuse, no exposure to animals, was not sexually active, and had no recent travel outside of Florida. There was no significant family history of lung diseases. PMID:25644915

  10. Cryopyrin-associated periodic syndrome.

    PubMed

    Giat, Eitan; Lidar, Merav

    2014-10-01

    CAPS is a rare autoinflammatory disease associated with mutations in the NLRP3 gene that result in overactivation of the inflammasome, increased secretion of IL-1beta and IL-18, and systemic inflammation. Genetic testing has allowed for grouping of the three, previously distinct clinical syndromes of FCAS, MWS and NOMID, into a single syndrome termed CAPS. The clinical features include urticarial rash and fever, CNS and musculoskeletal involvement, ocular disorders and progressive deafness. Onset, severity and complications (mainly retardation, seizures, destructive arthropathy and amyloidosis) depend on the specific mutation. Diagnosis is determined by genetic tests but is often delayed due to lack of awareness. In Israel, the relative abundance of other autoinflammatory disorders (FMF, Behçet's disease) may result in misdiagnosis. Treatment is based on IL-1 antagonism, which usually results in prompt clinical response and may prevent amyloidosis. PMID:25438464

  11. Cryopyrin-associated periodic syndrome.

    PubMed

    Giat, Eitan; Lidar, Merav

    2014-10-01

    CAPS is a rare autoinflammatory disease associated with mutations in the NLRP3 gene that result in overactivation of the inflammasome, increased secretion of IL-1beta and IL-18, and systemic inflammation. Genetic testing has allowed for grouping of the three, previously distinct clinical syndromes of FCAS, MWS and NOMID, into a single syndrome termed CAPS. The clinical features include urticarial rash and fever, CNS and musculoskeletal involvement, ocular disorders and progressive deafness. Onset, severity and complications (mainly retardation, seizures, destructive arthropathy and amyloidosis) depend on the specific mutation. Diagnosis is determined by genetic tests but is often delayed due to lack of awareness. In Israel, the relative abundance of other autoinflammatory disorders (FMF, Behçet's disease) may result in misdiagnosis. Treatment is based on IL-1 antagonism, which usually results in prompt clinical response and may prevent amyloidosis. PMID:25507981

  12. Management of heparin allergy during pregnancy with danaparoid.

    PubMed

    Harrison, S J; Rafferty, I; McColl, M D

    2001-03-01

    We report a patient who presented with a left proximal deep vein thrombosis at 25 + 5 weeks gestation. She developed a severe urticarial rash 3 weeks following initiation of therapy with Enoxaparin. The patient was heterozygous for the factor V Leiden mutation. She was treated with subcutaneous twice-daily danaparoid (Orgaran) for the remainder of the pregnancy, achieving anti-Xa levels in the therapeutic range 0.5-1.0 IU/ml. Delivery was at term by caesarean section 2 days after spontaneous rupture of membranes and failure to progress in labour. Danaparoid was withheld during this time. Danaparoid was restarted 3 h post delivery and the patient anticoagulated with warfarin in the post-partum period. There was no recurrence of thrombosis or bleeding events during therapy with danaparoid. No anti-Xa activity was demonstrated in breast milk. PMID:11302479

  13. Physical urticarias.

    PubMed

    Mahmood, T

    1994-05-01

    Chronic urticaria can be produced by a number of stimuli that cause mast cell mediator release. Patients with urticaria caused by physical agents account for roughly one-fifth of all cases of chronic urticaria. There are about 20 different types of physical urticaria. Two forms, dermographism and cholinergic urticaria, are quite common and represent more than two-thirds of all cases of physically caused urticaria. More than one agent may precipitate urticaria in a given individual. Urticarial response can be easily reproduced in the sensitive patient and, generally, lasts less than one hour. Systemic features such as flushing, dizziness, headaches, and even hypotension, may occur during severe episodes. Identification of the causative physical agent is necessary for effective therapy. PMID:8172038

  14. Increased risk of major depression subsequent to a first-attack and non-infection caused urticaria in adolescence: a nationwide population-based study

    PubMed Central

    2014-01-01

    Background Non-infection caused urticaria is a common ailment in adolescents. Its symptoms (e.g., unusual rash appearance, limitation of daily activities, and recurrent itching) may contribute to the development of depressive stress in adolescents; the potential link has not been well studied. This study aimed to investigate the risk of major depression after a first-attack and non-infection caused urticaria. Methods This study used the Taiwan Longitudinal Health Insurance Database. A total of 5,755 adolescents hospitalized for a first-attack and non-infection caused urticaria from 2005 to 2009 were recruited as the study group, together with 17,265 matched non-urticarial enrollees who comprised the control group. Patients who had any history of urticaria or depression prior to the evaluation period were excluded. Each patient was followed for one year to identify the occurrence of depression. Cox proportional hazards models were generated to compute the risk of major depression, adjusting for the subjects’ sociodemographic characteristics. Depression-free survival curves were also analyzed. Results Thirty-four (0.6%) adolescents with non-infection caused urticaria and 59 (0.3%) non-urticarial control subjects suffered a new-onset episode of major depression during the study period. The stratified Cox proportional analysis showed that the crude hazard ratio (HR) of depression among adolescents with urticaria was 1.73 times (95% CI, 1.13-2.64) than that of the control subjects without urticaria. Moreover, the HR were higher in physical (HR: 3.39, 95% CI 2.77-11.52) and allergy chronic urticaria (HR: 2.43, 95% CI 3.18-9.78). Conclusion Individuals who have a non-infection caused urticaria during adolescence are at a higher risk of developing major depression. PMID:25012668

  15. Deficient Serum Bactericidal Activity Against Escherichia Coli in Patients with Cirrhosis of the Liver

    PubMed Central

    Fierer, Joshua; Finley, Fred

    1979-01-01

    The serum bactericidal activity (SBA) of cirrhotic patients was compared with that of normal individuals using the release of 51Cr from radiolabeled Escherichia coli as the assay method. 80% (22/27) of patients were found to have deficient SBA against at least one of three smooth, serum-sensitive test strains of E. coli. Cirrhotic patients were found to have normal levels of serum lysozyme. Although some patients were mildly hypocomplementemic, this abnormality did not correlate with the presence of a bactericidal defect. Bactericidal antibody in normal and cirrhotics' sera was limited to the immunoglobulin (Ig)M class. Purified IgM from patients with deficient SBA against E. coli 0111 had lower concentrations of bactericidal antibody for that E. coli than did IgM from normal sera; the calculated bactericidal activity of total serum IgM was also lower. The bactericidal defect in cirrhotic serum could be completely corrected by either human antiserum to the homologous strain of E. coli or by purified, normal human IgM. However, because higher concentrations of IgM were required to restore normal SBA to a cirrhotic's serum than to agammaglobulinemic serum, there may be an inhibitor of bactericidal antibody in addition to a deficiency of bactericidal IgM antibody to E. coli in the serum of patients with cirrhosis. The bactericidal activity of the alternative complement pathway was also assessed. Sera from cirrhotic patients had no deficit in SBA attributable to the alternative complement pathway. In fact, in some, the activity of the alternative complement pathway was supernormal, compensating in part for the deficit in IgM-mediated SBA. PMID:376551

  16. Treatment of severe henoch-schonlein purpura nephritis with mycophenolate mofetil.

    PubMed

    Nikibakhsh, Ahmad Ali; Mahmoodzadeh, Hashem; Karamyyar, Mohamad; Hejazi, Sasan; Noroozi, Mehran; Macooie, Ali Agayar

    2014-07-01

    Henoch-Schonlein purpura (HSP) is the most common childhood vasculitis. Renal involvement in HSP is one of the major causes of chronic renal failure in children. It is important to start effective and relatively safe medication to prevent end-stage renal disease (ESRD). Mycophenolate mofetil (MMF) appears to be a promising therapeutic agent in many autoimmune diseases such as lupus nephritis and vasculitis. Herein, we describe the treatment with MMF of three patients with HSP nephritis. In two cases with rapidly progressive glomerulonephritis without response to steroid, after treatment with MMF, significant improvement in kidney function and proteinuria were observed. In another patient with HSP nephritic-nephrotic syndrome who showed resistance to steroid, MMF offered a favorable effect. MMF seems to be a promising therapeutic agent in the treatment of the severe HSP nephritis. PMID:24969202

  17. [Treatment of Behçet's disease].

    PubMed

    Comarmond, C; Wechsler, B; Cacoub, P; Saadoun, D

    2014-02-01

    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, and gastrointestinal manifestations. Therapeutic management of BD depends on the clinical presentation and organ involved. Although colchicine, non-steroidal anti-inflammatory agents and topical treatments with corticosteroids are often sufficient for mucocutaneous and joint involvement, a more aggressive approach with immunosuppressive agents is warranted for severe manifestations such as posterior uveitis, retinal vasculitis, vascular, neurological and gastrointestinal involvement. However, some patients still have refractory disease, relapses, 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 therapies for BD. In contrast to current non-specific immunosuppressive agents, the emergence of immunomodulatory drugs provides the possibility of interfering with specific pathogenic pathways. Novel targeted immunosuppressive therapies might be used in the future for BD. PMID:24438860

  18. Churg-Strauss Syndrome Presented With Hearing Impairment and Facial Palsy

    PubMed Central

    Byun, Jeong-Hyun; Lee, Jong-Hoo

    2014-01-01

    Churg-Strauss syndrome (CSS) is a rare systemic necrotizing vasculitis. Cranial nerve involvement is very rare in CSS. A 59-year-old woman had complained of both hearing impairments for eight months and left facial palsy for three months. Left facial and cochlear neuropathies were detected in electrodiagnostic studies. Paranasal sinus computed tomography (CT) showed chronic pansinusitis. Chest CT revealed eosinophilic infiltration in the right upper lobe. Tissue biopsy of the right inferior turbinate displayed necrotizing vasculitis with eosinophilic infiltration. She was diagnosed as CSS, based on the presence of eosinophilia, pulmonary infiltration, paranasal sinusitis, and biopsy containing blood vessels with extravascular eosinophils. She was treated with intravenous and oral steroids and azathioprine, showing relatively good prognosis on facial palsy and hearing impairment. We report a very rare case of CSS presented with hearing impairment and facial palsy. PMID:25566487

  19. Experimental posterior uveitis. I: A clinical, angiographic, and pathological study.

    PubMed Central

    Stanford, M R; Brown, E C; Kasp, E; Graham, E M; Sanders, M D; Dumonde, D C

    1987-01-01

    The clinical, angiographic, and histopathological features of experimental posterior uveitis in the black hooded Lister rat are described. This mild form of experimental allergic uveoretinitis (EAU) is induced by sensitisation with retinal S antigen in Freund's complete adjuvant, and the inflammation produced is confined to the posterior segment of the eye. This allows for the first time precise photographic and angiographic documentation of the evolution of clinical signs, because there is minimal clouding of the vitreous by inflammatory cells. Clinically the disease is characterised by the appearance of disc oedema and periphlebitis, followed by focal infiltrates in the deep retinal layers, with eventual atrophy of the pigment epithelium. Histologically, retinal vasculitis is associated with focal mononuclear cell infiltration and necrosis of the photoreceptor layers. This model closely resembles the clinical features of idiopathic retinal vasculitis seen in man. Images PMID:3651374

  20. Neurorickettsioses: A Rare Presentation with Stroke in a Young Adult

    PubMed Central

    K, Pramod

    2014-01-01

    Acute stroke-like presentations due to rickettsial infections have been sparsely reported in literature. We report a young patient who presented with high grade fever and acute encephalopathy with right hemiplegia. CT head showed left cerebral, bilateral thalamic and midbrain infarcts. The stroke in young work-up for conventional and non-conventional risk factors including few infections known to cause cerebral vasculitis was negative. He did not respond to empirical antimalarials and antibiotics. With a high index of suspicion of his ‘febrile cerebrovasculitis’ like presentation, serological test for rickettsia was done and found to be positive. He responded to doxycycline. This case expands the spectrum of presentation of neurorickettsioses and highlights the importance of considering rickettsial vasculitis as one of the potentially treatable causes of infections causing stroke. PMID:25478386

  1. [Systemic vasculitides: novel nomenclature and novel therapeutic approaches].

    PubMed

    Allali, D; Chizzolini, C

    2014-04-16

    A novel nomenclature of systemic vasculitides is proposed by the 2012 Chapel Hill Consensus Conference. It aims at substituting established eponyms and introducing new terms and groups closer to our current understanding of vasculitis pathophysiology. In parallel, a therapeutic revolution is taking place partially based on the concept of re-induction of immune tolerance for ANCA-associated vasculitis (AAV). Two major multicentric randomized studies have shown that rituximab (RTX), monoclonal antibody capable of selectively killing B lymphocytes, is not inferior when compared to cyclophosphamide (CYC) to induce remission in AAV, and superior in the case of disease relapse. Thus, a hot debate is taking place whether or not to maintain CYC or use RTX in AAV. An individual-based choice may be wise for the moment being. PMID:24834643

  2. Cerebral aneurysms associated with Behçet's disease: a case report

    PubMed Central

    Nakasu, S; Kaneko, M; Matsuda, M

    2001-01-01

    Cerebral aneurysms in Behçet's disease are very rare. The role of vasculitis in the aetiology of these aneurysms has not been clarified. A 57 year old man with Behçet's disease is described, who had a subarachnoid hemorrhage due to rupture of a peripheral middle cerebral artery aneurysm. He underwent a successful aneurysmal clipping. Three years later he had seizures and was found to have a new aneurysm on the contralateral peripheral middle cerebral artery as well as some radiological features of vasculitis. After 3 months of steroid therapy, the aneurysm disappeared. Although surgical treatment is the first choice for ruptured aneurysms, steroid therapy may be effective for unruptured small aneurysms in patients with Behçet's disease.?? PMID:11309468

  3. A presentation of massive hemoptysis in a patient with Churg-Strauss syndrome.

    PubMed

    Hikmat, Fadi; Pearse, David B; Damarla, Mahendra

    Churg-Strauss syndrome (CSS) is a systemic small-vessel vasculitis. When involving the lungs, small-vessel vasculitides typically cause capillaritis, leading to diffuse alveolar hemorrhage and submassive hemoptysis. In contrast, massive hemoptysis primarily originates from the bronchial arteries; therefore, small-vessel vasculitis is not considered when a patient presents with massive hemoptysis. The authors describe a patient with CSS who presented with the novel finding of massive hemoptysis. Computed tomography scans lacked alveolar infiltrates and bronchoalveolar lavage lacked hemosiderin-laden macrophages. Bronchoscopy demonstrated a raised mucosal lesion in the right mainstem bronchus and computed tomography angiogram revealed aberrant dilated bronchial arteries underlying the same region, suggesting this as the source of the hemoptysis. To the authors' knowledge, the present report describes the first reported case of CSS to present with massive hemoptysis with likely involvement of the bronchial arterial circulation. CSS should be considered in patients with unexplained massive hemoptysis. PMID:25255461

  4. Coronary involvement in Churg-Strauss syndrome: a case report with CT findings.

    PubMed

    Doo, Kyung Won; Yong, Hwan Seok; Kang, Eun-Young

    2013-12-01

    We report a case of Churg-Strauss syndrome (CSS) associated with coronary artery involvement, as demonstrated on coronary CT angiography (CCTA), without specific cardiac symptoms. A 69-year-old male had an 8-year history of bronchial asthma and chronic sinusitis with hypereosinophilia (35 %), polyneuropathy, and a positive antineutrophil cytoplasmic antibody titer, so he was diagnosed with CSS. The patient had no specific cardiac symptoms, but CCTA showed vasculitis and a saccular aneurysm involving the proximal coronary arteries. The 3-year follow-up CCTA demonstrated an increase in the extent of soft-tissue wall thickening and infiltration involving the coronary arteries. Although vasculitis of the major coronary arteries is not a prominent feature of CSS, our case suggests that the coronary arteries may also be targeted in this syndrome. PMID:24155026

  5. Bilateral Facial Palsy in Rapidly Progressive Course of Wegener's Granulomatosis: A Case Report

    PubMed Central

    Roszkowska, Anna; Morawska-Kochman, Monika; Temporale, Hanna; Sikorska-?uk, Ma?gorzata; Kr?cicki, Tomasz

    2013-01-01

    Introduction. Wegener's granulomatosis belongs to a group of systemic vasculitis diseases, which is characterized by necrotizing vasculitis and presence of granulomas. In a lot of cases, the first symptoms of the disease are observed in the head and neck region, but the bilateral facial nerve palsy occurs very rarely. Objective. The objective of our report was to describe the unusual course of Wegener's granulomatosis with the bilateral facial nerve paralysis, which subsided after application of steroids and immunosuppressive therapy in combination with surgical treatment. Results and Conclusions. Hearing loss may precede other symptoms in Wegener's granulomatosis. Ear pain and otorrhea may suggest the diagnosis of bacterial purulent otitis media and delay the proper diagnosis. In the presented case, considering the clinical course, it was necessary to apply both pharmacological and surgical treatments. PMID:24187641

  6. 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

  7. Extrahepatic manifestations of HCV infection: A brief review and update

    Microsoft Academic Search

    Nidhi Singh; Nancy Reau

    2006-01-01

    Hepatitis C affects approximately 3% of the population worldwide and extrahepatic manifestations may occur in nearly 40% of\\u000a all patients. Recent efforts have focused on incidence, prevalence, clinical significance, mechanism of disease, and the role\\u000a of antiviral therapy in treatment. Cryoglobulinemia, cryoglobulinemic vasculitis, membranoproliferative glomerulonephritis,\\u000a and lymphoproliferative disorders have a well-established link to hepatitis C, and many other endocrine, autoimmune,

  8. Massive proteinuria and acute glomerulonephritis picture in a patient with Familial Mediterranean fever and E148Q mutation.

    PubMed

    Ardalan, Mohammadreza; Nasri, Hamid

    2014-11-01

    Familial Mediterranean fever (FMF) is an inherited auto-inflammatory disorder. Secondary AA amyloidosis is the most devastating complication of FMF. Nonamyloid renal involvements have also been reported in association with FMF, including vasculitis, focal and diffuse glomerulonephritis, and IgA nephropathy. We describe a patient with FMF and E148Q mutation who presented with massive proteinuria, elevated serum creatinine level, and acute glomerulonephritis picture. Disease remission was achieved after treatment with corticosteroids and colchicine. PMID:25362225

  9. Serum homocysteine level is increased and correlated with endothelin-1 and nitric oxide in Behc?et's disease

    Microsoft Academic Search

    H Er; C Evereklioglu; T Cumurcu; Y Tu?rko?z; E O?zerol; K S?ahin; S Doganay

    2002-01-01

    Background\\/aims: Behc?et's disease (BD) is a systemic inflammatory vasculitis of young adults with unknown aetiology, characterised by endothelial dysfunction and occlusion in both deep venous and retinal circulation. Ocular involvement occurs in 70% of cases and is characterised by periphlebitis, periarteritis, vascular occlusion, and thrombosis leading to blindness despite vigorous treatment. Endothelin-1 (ET-1) is a vasoconstricting peptide while nitric oxide

  10. Proteinase3 as the major autoantigen of c-ANCA is strongly expressed in lung tissue of patients with Wegener's granulomatosis

    Microsoft Academic Search

    Holger Brockmann; Andreas Schwarting; Jörg Kriegsmann; Peter Petrow; Andreas Gaumann; Klaus-Michael Müller; Peter Robert Galle; Werner Mayet

    2002-01-01

    Proteinase-3 (PR-3) is a neutral serine proteinase present in azurophil granules of human polymorphonuclear leukocytes and serves as the major target antigen of antineutrophil cytoplasmic antibodies with a cytoplasmic staining pattern (c-ANCA) in Wegener's granulomatosis (WG). The WG disease appears as severe vasculitis in different organs (e.g. kidney, nose and lung). Little is known about the expression and distribution of

  11. 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

  12. [Rare complication in a patient with giant cell arteritis].

    PubMed

    Olivera, S; Gonzalez, S Olivera; Amores, B; Arriaga, B Amores; Torralba, M A; Cabeza, M A Torralba; Pérez Calvo, J I; Calvo, J I Pérez

    2010-01-01

    Giant cell arteritis (temporal arteritis) is a chronic vasculitis that usually affects older people. Although this is a systemic disease, it most often affects the cranial arteries. The most frequent complication of this disorder is visual loss. We report the case of a patient who suffered several rare complications, including tongue necrosis, as a result of being diagnosed with giant cell arteritis following the start of treatment. PMID:21233870

  13. A new antibody in rheumatoid arthritis targeting glycated IgG: IgM anti- IgG-AGE

    Microsoft Academic Search

    S. LIGIER; P. R. FORTIN; M. M. NEWKIRK

    1998-01-01

    SUMMARY Hyperglycaemia and\\/or oxidative stress can cause IgG to be modified by advanced glycation end products (AGE). Three patients with aggressive rheumatoid arthritis (RA) and vasculitis are described who have high titres of IgM antibodies against AGE-modified IgG (IgM anti-IgG-AGE ). Diabetics and randomly selected patients with rheumatic diseases, including 50 additional RA patients, were tested for IgM and IgA

  14. Palpable purpura with foot drop: Common presentations in an uncommon disease.

    PubMed

    Mathanda, Teena Ramesh; Bhat, Ramesh; Alva, Jayaprakash

    2015-01-01

    Churg-Strauss syndrome is a rare disease manifested by hypereosinophilia, vasculitis and tissue infiltration. This report describes the case of a 45-year-old man who presented with a history of fever, difficulty in breathing, reddish lesions over the extremities and inability to walk since two weeks. The cutaneous features prompted us to conduct serial lab investigations which led to an early, potentially life saving diagnosis. PMID:25821734

  15. Anti-MPO-ANCA-Positive Microscopic Polyangiitis following Subacute Bacterial Endocarditis

    Microsoft Academic Search

    B. Hellmich; M. Ehren; M. Lindstaedt; M. Meyer; M. Pfohl; H. Schatz

    2001-01-01

    :   Although infectious agents such as Staphylococcus aureus have been implicated in the pathogenesis of Wegener’s granulomatosis, the role of bacterial infections in the pathogenesis\\u000a of other types of small-vessel vasculitides associated with antineutrophil cytoplasmic antibodies (ANCA) is less clear. We\\u000a describe a patient who developed a non-granulomatous necrotising small vessel vasculitis and perinuclear ANCA (p-ANCA) directed\\u000a against myeloperoxidase (MPO)

  16. Association of HLA-DRB1*0901-DQB1*0303 haplotype with microscopic polyangiitis in Japanese

    Microsoft Academic Search

    N Tsuchiya; S Kobayashi; H Hashimoto; S Ozaki; K Tokunaga

    2006-01-01

    Microscopic polyangiitis (MPA) is a rare and severe form of systemic necrotizing vasculitis associated with myeloperoxidase (MPO)-specific antineutrophil cytoplasmic antibody (ANCA). We previously reported significant association of HLA-DRB1*0901 with MPA. To define the susceptibility loci within the HLA region, we determined the genotypes of HLA-DQB1, DPB1, B and C in 50 patients with MPA and 77 unrelated Japanese controls. In

  17. Fatal hemoperitoneum due to rupture of the left gastric artery in a patient with microscopic polyangiitis

    Microsoft Academic Search

    Kensei Yahata; Chinatsu Okamoto; Hirotaka Imamaki; Koichi Seta; Tsuyoshi Terashima; Sachiko Minamiguchi; Akira Sugawara

    2009-01-01

    Microscopic polyangiitis is a vasculitis which primarily affects capillaries, venules or arterioles. Involvement of small\\u000a and medium-sized arteries may also occur. A 70-year-old Japanese female with a fever and cough was diagnosed with pneumonia\\u000a and antibiotics were administered. Her symptoms initially improved, but her fever recurred and she experienced malaise and\\u000a loss of appetite. Her renal function gradually worsened and

  18. Polyarteritis nodosa and microscopic polyangiitis: Etiologic and diagnostic considerations

    Microsoft Academic Search

    Laura B. Hughes; S. Louis Bridges

    2002-01-01

    Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis of medium-sized vessels with clinical manifestations resulting\\u000a from ischemia and infarction of affected tissues and organs. Although the cause of most cases of PAN and the related disorder\\u000a microscopic polyangiitis (MPA) remains largely unknown, there has been significant progress in understanding the pathogenesis\\u000a of vascular inflammation. The diagnostic approach to PAN and

  19. Spontaneous Duodenal Perforation as a Complication of Kawasaki Disease

    PubMed Central

    Forouzan, Arash; Saidi, Hossein; Javaherizadeh, Hazhir; Khavanin, Ali; Bahadoram, Mohammad

    2015-01-01

    Kawasaki disease is generally known as a systemic vasculitis that often concerns doctors due to its serious cardiac complications; however, other visceral organs may get involved as well. Surgical manifestations of the intestinal tract in Kawasaki disease are rare. In this report, we describe the case of a 2.5-year-old boy with typical Kawasaki disease who presented with GI bleeding and surgical abdomen. The diagnosis of duodenal perforation was confirmed.

  20. The current status of neutrophil cytoplasmic antibodies.

    PubMed Central

    van der Woude, F J; Daha, M R; van Es, L A

    1989-01-01

    Several studies in the past 10 years have demonstrated the occurrence of autoantibodies against cytoplasmic constituents in patients with vasculitis and glomerulonephritis. In this review the nomenclature of these antibodies is discussed and assays and clinical associations are summarized. Although the antigens involved are not completely identified, antibodies and T cells reactive with myeloid lysozomal enzymes may both play a significant role in pathogenesis. PMID:12412739

  1. Indium-111 leukocyte scintigraphy in Wegener's granulomatosis involving the spleen

    SciTech Connect

    Morayati, S.J.; Fink-Bennett, D.

    1986-12-01

    Indium-111-labeled leukocyte scintigraphy was performed on a 44-yr-old man to exclude an occult abscess. Four- and twenty-four-hour images of the abdomen revealed splenic photopenia except for a rim of activity medially. A subsequent computed tomography (CT) study demonstrated necrosis or hemorrhage of the spleen except for a medial rim. Exploratory laparotomy demonstrated necrotizing vasculitis with granuloma formation consistent with Wegener's granulomatosis and a rim of viable splenic tissue corresponding to the radionuclide and CT studies.

  2. Evaluation of current therapeutic strategies in Behçet’s disease

    Microsoft Academic Search

    Iliana Alexoudi; Violetta Kapsimali; Aristides Vaiopoulos; Meletios Kanakis; George Vaiopoulos

    2011-01-01

    Behçet’s disease (BD) is a chronic relapsing vasculitis with multifunctional pathogenesis. The mucocutaneous and ocular lesions\\u000a are the commonest manifestations, but BD also affects the musculoskeletal, intestinal, cardiac, and central nervous system.\\u000a BD therapy is based on the suppression of the inflammatory process, using immunomodulating and immunosuppressive agents. In\\u000a selected cases, invasive procedures may be required.

  3. Henoch–Schönlein purpura: a clinical case with dramatic presentation

    Microsoft Academic Search

    João Bento; Adriana Magalhães; Conceição Souto Moura; Venceslau Hespanhol

    2010-01-01

    A case study involving a 55-year-old Caucasian male with end-stage glomerulosclerosis is presented here. Kidney biopsies showed no deposits on imunofluorescent microscopy. Relapsing massive haemoptysis and suspected bronchovascular malformation required lung lobectomy which revealed malformative and tortuous small blood vessels, with no vasculitis. Blood antinuclear antibodies, antineutrophil cytoplasmic antibodies and antiglomerular basement membrane antibodies were undetectable. Seric immunoglobulins and complement

  4. Bullous Fixed Drug Eruption to Ciprofloxacin: A Case Report

    PubMed Central

    Jain, Sonia Pramod; Jain, Pramod Ajit

    2013-01-01

    Adverse reactions to medications are extremely common and display a characteristic clinical morphology such as fixed drug eruption (FDE), Stevens-Johnson syndrome, urticaria, morbilliform exanthem, hypersensitivity syndrome, pigmentary changes, lichenoid, dermatitis, acute generalized exanthematous pustulosis, photosensitivity, vasculitis etc. Here we report a case of a 60 year old male who presented to us with multiple bullous eruptions over both the hands and feet after oral ingestion of ciprofloxacin. PMID:23730666

  5. Role of antineutrophil cytoplasmic antibodies and glomerular basement membrane antibodies in the diagnosis and monitoring of systemic vasculitides.

    PubMed

    Sinclair, David; Stevens, Judith M

    2007-09-01

    Systemic vasculitis, although rare, is often diagnosed late and long after the onset of symptoms. The small vessel vasculitides are recognized clinically by their multisystem presentation, markers of inflammation and evidence for an acute glomerulonephritis (GN), with the most apparent organ involved directing referral to secondary care. Routine laboratory tests are usually non-specific in systemic vasculitis but the use of anti-neutrophil cytoplasmic antibodies (ANCAs) and glomerular basement membrane (GBM) antibodies can aid diagnosis, treatment and monitoring decisions. These antibodies are detected and quantified by indirect immunofluorescence (IIF) and antigen-specific enzyme-linked immunosorbent assay (ELISA), usually in combination for ANCA, and ELISA systems (or direct IIF on kidney biopsy) for GBM antibodies. The presence or absence of ANCA does not confirm or exclude the diagnosis of systemic vasculitis but negative and positive predictive values will be strongly influenced by clinical presentation. Various large studies have been unable to conclude that following serial ANCA titres has great clinical utility in each case but each patient must be considered on its own merits; for example the reappearance of ANCA in a patient who was rendered ANCA negative following treatment is more likely to indicate relapse. The adoption of consensus guidelines that direct testing towards patients with rapidly progressive GN, pulmonary haemorrhage, persistent and destructive ear, nose and upper airways problems, such as subglottic tracheal stenosis, a retro-orbital mass and cutaneous vasculitis with systemic features or peripheral neuropathy, will greatly increase the clinical utility and positive predictive value of these tests. PMID:17761028

  6. Takayasu’s arteritis associated with Crohn’s disease

    Microsoft Academic Search

    You-shi Liu; You-hong Fang; Ling-xiang Ruan; You-ming Li; Lin Li; Ling-ling Jiang

    2009-01-01

    Takayasu’s arteritis (TA), also known as the “pulseless disease,” is a chronic vasculitis of the aorta and aortic branches.\\u000a TA with Crohn’s disease is rare and has not been documented in China before. In this paper we report on a case of Takayasu’s\\u000a arteritis associated with concurrent Crohn’s disease. A 17-year-old Chinese male developed upper limb sourness and a sensation

  7. Role of Advanced MRI Brain Sequences in Diagnosing Neurological Complications of Scrub Typhus

    PubMed Central

    Sood, Shikha; Sharma, Sanjeev; Khanna, Shweta

    2015-01-01

    Scrub typhus is a rare disease affecting many organs and causing vasculitis by affecting the endothelium of blood vessels. Review of literature shows that there are only a few case reports describing the neuroradiological manifestations of scrub typhus. This case report describes how newer and advanced MRI sequences are able to diagnose neurological complications of scrub typhus, such as hemorrhages, meningoencephalitis, infarctions, cranial nerve involvement, thrombosis, and hypoperfusion, that are not picked up on routine magnetic resonance imaging (MRI) sequences.

  8. Henoch - Schönlein Purpura, a Rare Disease in Pregnancy

    Microsoft Academic Search

    TABASSUM PARVEEN; FIROZA BEGUM

    2008-01-01

    Introduction: Henoch-Schönlein purpura is an IgA-mediated small vessel vasculitis involving mainly skin, gastrointestinal system and kidneys. It is predominantly a disease of young children. Most common symptoms are rash (95-100%), sometimes necrotizing involving specially the legs, subcutaneous oedema (20-50%), abdominal pain and vomiting(85%), bloody stool and joint pain (60-80%) involving mainly the knees and ankles. Diagnosis is clinical and not

  9. Vascularite restreinte au système nerveux périphérique : présentation clinique atypique

    Microsoft Academic Search

    V Marcaud; C Gauthier; T Maisonobe; J Hogenhuis; R Morizot-Koutlidis; C. F Degos

    2002-01-01

    Introduction. – Peripheral neuropathy is a common feature of many vasculitic syndromes. In some patients, the neuropathy may be the sole manifestation of vasculitis.Exegesis. – A 74-year-old lady complained of pain and weakness of the lower limbs. In her history, we noted right optic neuritis, monoclonal gammopathy and dyslipidemia treated by fenofibrate. Clinical examination showed proximal muscle strength deficit of lower limbs,

  10. Detection of inflammatory processes during various diseases by the method of flow cytofluorometry.

    PubMed

    Varfolomeeva, E Yu; Ivanov, E I; Drobchenko, E A; Semenova, E V; Filatov, M V

    2010-10-01

    Oxidative (respiratory) burst is an important manifestation of inflammation. Precise quantitative assessment of this reaction by flow cytometry made it possible to record and evaluate the severity of the inflammatory processes in a wide spectrum of diseases including diphtheria, hepatitis, pneumonia, bronchial asthma, arthritis, vasculitis, postoperative complications, tuberculosis, psoriasis, rheumatoid arthritis, systemic lupus erythematosus, and myocardial infarction. This approach can be employed as a highly sensitive method of detection of inflammatory reactions and monitoring of their course in various pathological processes. PMID:21234449

  11. Detection of Inflammatory Processes during Various Diseases by the Method of Flow Cytofluorometry

    Microsoft Academic Search

    E. Yu. Varfolomeeva; E. I. Ivanov; E. A. Drobchenko; E. V. Semenova; M. V. Filatov

    2010-01-01

    Oxidative (respiratory) burst is an important manifestation of inflammation. Precise quantitative assessment of this reaction\\u000a by flow cytometry made it possible to record and evaluate the severity of the inflammatory processes in a wide spectrum of\\u000a diseases including diphtheria, hepatitis, pneumonia, bronchial asthma, arthritis, vasculitis, postoperative complications,\\u000a tuberculosis, psoriasis, rheumatoid arthritis, systemic lupus erythematosus, and myocardial infarction. This approach can

  12. Use of drug therapy to manage acute cutaneous necrosis of the skin.

    PubMed

    Wallace, Jill S; Hall, John C

    2010-04-01

    Acute cutaneous necrosis is defined as a sudden onset of gangrenous skin changes in the skin, associated with significant morbidity and mortality. The following diseases are included in this discussion: coumadin necrosis, heparin necrosis, brown recluse spider bite, necrotizing fasciitis, vasculitis, pyoderma gangrenosum, calciphylaxis, clotting abnormalities and embolic phenomena. The importance of early diagnosis, early distinction and early drug therapy or drug withdrawal must match the diagnosis for maximal preservation of the skin and underlying tissue. PMID:20514791

  13. [Arthritis, erythema nodosum and genital ulcerations. Behçet disease].

    PubMed

    Arnold, C; Revaz, S; Dudler, J

    2011-04-13

    Behcet's disease is a disease of unknown etiology resting in between vasculitis, spondyloarthropathy and autoinflammatory diseases. If his predilection for the population originating from the Silk Road is well known, as are its cutaneous, ocular and vascular manifestations, this case illustrates the non-specificity of those manifestations, the diagnostic difficulties and the importance of routinely assessing for eyes and bowel diseases in this type of patient. PMID:21484713

  14. Palpable purpura with foot drop: Common presentations in an uncommon disease

    PubMed Central

    Mathanda, Teena Ramesh; Bhat, Ramesh; Alva, Jayaprakash

    2015-01-01

    Churg–Strauss syndrome is a rare disease manifested by hypereosinophilia, vasculitis and tissue infiltration. This report describes the case of a 45-year-old man who presented with a history of fever, difficulty in breathing, reddish lesions over the extremities and inability to walk since two weeks. The cutaneous features prompted us to conduct serial lab investigations which led to an early, potentially life saving diagnosis.

  15. Assessment of alveolar epithelial permeability in Behçet’s disease with 99m Tc-DTPA aerosol scintigraphy

    Microsoft Academic Search

    Fikriye G. Gumuser; Timur Pirildar; Dilek Batok; Ays?n Sakar; Ebru Ruksen; Elvan Sayit

    2008-01-01

    Objective  Behçet’s disease (BD) is a multisystem disorder characterized by vasculitis, and consists of a triad of recurrent ulcers of\\u000a the oral and genital mucosa with relapsing uveitis. The prevalance of pulmonary involvement varies in the range of 1–10% in\\u000a various studies and its complications are severe and life threatening. In this study, we investigated the changes of pulmonary\\u000a epithelial permeability

  16. Microarray Studies of Gene Expression in Circulating Leukocytes in Kidney Diseases

    Microsoft Academic Search

    David Alcorta; Gloria Preston; William Munger; Pamela Sullivan; Jia Jin Yang; Iwao Waga; J. Charles Jennette; Ronald Falk

    2002-01-01

    The molecular characterization of changes in mRNA expression in renal tissue during disease is hampered by the acquisition of sufficient mRNA to do genomewide expression profiling. In many renal diseases, such as systemic lupus erythematosus, IgA nephropathy, antineutrophil cytoplasmic autoantibody (ANCA) associated glomerulonephritis, and small-vessel vasculitis (ANCA disease), circulating leukocytes play a role in onset, progression, and severity of the

  17. Cutaneous manifestations associated with rheumatoid arthritis

    Microsoft Academic Search

    T. Yamamoto

    2009-01-01

    Rheumatoid arthritis presents various cutaneous manifestations, either specific or nonspecific skin features, which are induced\\u000a by the activation of inflammatory cells (neutrophils, lymphocytes, macrophages), vasculopathy, vasculitis, acral deformity,\\u000a drugs, and so on. These include (1) specific findings, (2) findings due to vascular impairment, (3) findings due to immune\\u000a dysfunction, (4) characteristic neutrophilic conditions, and (5) miscellaneous conditions. On the other

  18. Computerized information-gathering in specialist rheumatology clinics: an initial evaluation of an electronic version of the Short Form 36

    Microsoft Academic Search

    A. S. Wilson; G. D. Kitas; D. M. Carruthers; C. Reay; J. Skan; S. Harris; G. J. Treharne; S. P. Young; P. A. Bacon

    2002-01-01

    Objectives. Longitudinal outcome data are important for research and are becoming part of routine clinical practice. We assessed an initial version of an electronic Short Form 36 (SF-36), a well-established health assessment questionnaire, in comparison with standard paper forms, in two specialist rheumatology clinics. Methods. Out-patients (20 with systemic lupus erythematosus and 31 with vasculitis) were randomly selected to complete

  19. Churg-Strauss syndrome associated with montelukast therapy

    Microsoft Academic Search

    J M Tuggey; H S R Hosker

    2000-01-01

    Churg-Strauss syndrome is a rare form of eosinophilic vasculitis associated with asthma. There have been several recent case reports of the condition in association with leukotriene antagonists and it has been speculated that the Churg-Strauss syndrome was unmasked when oral corticosteroids were withdrawn. We report a case of Churg-Strauss syndrome associated with montelukast therapy in an asthmatic patient in whom

  20. Evaluation of the ameliorative effects of immunosuppressants on crescentic glomerulonephritis in SCG\\/Kj mice

    Microsoft Academic Search

    Kan Saiga; Minako Yoshida; Iwao Nakamura; Eriko Toyoda; Kazuhiro Tokunaka; Hirohisa Morohashi; Fuminori Abe; Kyuichi Nemoto; Masato Nose

    2008-01-01

    The therapeutic efficacy of immunosuppressants for treating rapidly progressive glomerulonephritis (RPGN) with crescent formation remains controversial. SCG\\/Kj mice spontaneously develop RPGN-like symptoms, characteristic of crescentic glomerulonephritis and systemic small vessel vasculitis, associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCA). We evaluated the “ameliorative”, not prophylactic, effects of immunosuppressive agents, deoxyspergualin (DSG), cyclophosphamide (CYC) and prednisolone (PDN), on RPGN in

  1. Clinical and surgical therapeutic approach in erithema elevatum diutinum--case report.

    PubMed

    Zacaron, Luciana Helena; Gonçalves, Júlia Costa de Faria; Curty, Vanessa Maria Amoreira; D'Acri, Antonio Macedo; Lima, Ricardo Barbosa; Martins, Carlos José

    2013-01-01

    Erythema elevatum diutinum is a rare chronic cutaneous vasculitis which usually affects adults. It is characterized by symmetrical and persistent papules, plaques, and nodules. These lesions are usually located on the extensor surfaces of the extremities. We report a case of erythema elevatum diutinum in which the association of dapsone and surgical excision promoted complete remission of skin lesions, providing a new approach to the treatments described in the literature. PMID:24346869

  2. Giant cell arteritis

    PubMed Central

    Calvo-Romero, J

    2003-01-01

    Giant cell arteritis (GCA), temporal arteritis or Horton's arteritis, is a systemic vasculitis which involves large and medium sized vessels, especially the extracranial branches of the carotid arteries, in persons usually older than 50 years. Permanent visual loss, ischaemic strokes, and thoracic and abdominal aortic aneurysms are feared complications of GCA. The treatment consists of high dose steroids. Mortality, with a correct treatment, in patients with GCA seems to be similar that of controls. PMID:13679546

  3. Interstitial Lung Disease Induced by Drugs and Radiation

    Microsoft Academic Search

    Philippe Camus; Annlyse Fanton; Philippe Bonniaud; Clio Camus; Pascal Foucher

    2004-01-01

    An ever-increasing number of drugs can reproduce variegated patterns of naturally occurring interstitial lung disease (ILD), including most forms of interstitial pneumonias, alveolar involvement and, rarely, vasculitis. Drugs in one therapeutic class may collectively produce the same pattern of involvement. A few drugs can produce more than one pattern of ILD. The diagnosis of drug-induced ILD (DI-ILD) essentially rests on

  4. Clinical utility of peripheral nerve biopsy

    Microsoft Academic Search

    David Lacomis

    2005-01-01

    Histopathologic evaluation of nerve biopsy specimens provides important diagnostic information in some patients with peripheral\\u000a neuropathy. The role of nerve biopsy is more restricted than that of muscle biopsy. Nerve biopsy is utilized mainly for diagnosis\\u000a of vasculitis and infiltrative neuropathies. It is also utilized in diagnosis of atypical inflammatory demyelinating neuropathies\\u000a in which the clinical, electrodiagnostic, and laboratory features

  5. Influence of inherited and acquired thrombophilic defects on the clinical manifestations of mixed cryoglobulinaemia

    Microsoft Academic Search

    M. Casato; M. Carlesimo; A. Francia; C. Timarco; A. Antenucci; M. Bove; H. Martini; M. Visentini; M. Fiorilli; L. Conti

    2008-01-01

    Objective. To investigate the contribution of inherited and acquired thrombophilic defects to the clinical manifestations of mixed cryoglobulinaemia vasculitis. Methods. The following thrombophilic defects were investigated in 64 consecutive patients with HCV-associated mixed cryoglobulinaemia: aPLs, lupus anti-coagulant, homocysteinaemia, protein C and protein S concentrations, activated protein C resistance, plasminogen activator inhibitor-1 4G4G and 5G5G genotypes, and the presence of mutations

  6. Induction of the Gene Encoding Macrophage Chemoattractant Protein 1 by Orientia tsutsugamushi in Human Endothelial Cells Involves Activation of Transcription Factor Activator Protein 1

    Microsoft Academic Search

    Nam-Hyuk Cho; Seung-Yong Seong; Myung-Sook Huh; Na-Hyun Kim; Myung-sik Choi; Ik-sang Kim

    2002-01-01

    Human macrophage chemoattractant protein 1 (MCP-1) is a potent mediator of macrophage migration and therefore plays an essential role in early events of inflammation. In endothelial cells, at least three independent pathways regulate MCP-1 expression by NF-B and AP-1. Orientia tsutsugamushi causes vasculitis in humans by replicating inside macrophages and endothelial cells. In the present study, we investigated the cis-acting

  7. Peripheral neuropathy with essential mixed cryoglobulinemia: biopsies from 5 cases

    Microsoft Academic Search

    C. Vital; C. Deminière; A. Lagueny; F. X. Bergouignan; J. L. Pellegrin; M. S. Doutre; A. Clement; J. Beylot

    1988-01-01

    Essential mixed cryoglobulinemia, which can cause hypersensitivity vasculitis, was observed in five patients with peripheral neuropathy. Three cases presented with multifocal neuropathies and two cases with symmetrical polyneuropathy. One had cryoglobulinemia with IgM monoclonal gammopathy and IgG polyclonal gammopathy, and the other four had cryoglobulinemia with polyclonal gammopathy. Biopsies showed perivascular infiltration by mononuclaer cells around medium, and mainly small-sized

  8. Localization of hepatitis c virus antigens in liver and skin tissues of chronic hepatitis c virus—Infected patients with mixed cryoglobulinemia

    Microsoft Academic Search

    Domenico Sansonno; Vito Cornacchiulo; Anna Rina Jacobelli; Rita Di Stefano; Mario Lospalluti; Franco Dammacco

    1995-01-01

    Skin and\\/or liver biopsy specimens were obtained from the following patients: 15 anti-hepatitis C virus (HCV), HCV RNA-positive patients and 3 anti-HCV, HCV RNA-negative patients with type II mixed cryoglobulinemia (MC); 7 anti-HCV, HCV RNA-positive patients with chronic active liver disease (CALD); 5 anti-HCV, HCV RNA-negative patients with noncryoglobulinemic vasculitis; and 7 anti-HCV, HCV RNA-negative patients with lichen ruber planus.

  9. Rupture of Abdominal Aortic Aneurysms in Behçet’s Disease

    Microsoft Academic Search

    Vedat Erentug; Nilgün Bozbuga; Suat Nail Ömeroglu; Hasan Ardal; Ercan Eren; Murat Güçlü Elevli; Füsun Güzelmeriç; Kaan Kirali; Esat Akinci; Cevat Yakut

    2003-01-01

    Behçet’s disease is a systemic disease of unknown etiology with a chronic relapsing course, characterized by oral aphtous, genital ulcers, ocular lesions, and occasionally vasculitis. Major asymptomatic vascular complications should always be considered in patients with Behçet’s disease. We present the surgical treatment of two male Behçet’s patients of 41 and 30 years of age with ruptured infrarenal abdominal aortic

  10. Pulmonary-Renal vasculitic disorders: Differential diagnosis and management

    Microsoft Academic Search

    Luis J. Jara; Olga Vera-Lastra; Maria C. Calleja

    2003-01-01

    Pulmonary-renal syndrome (PRS) is a combination of diffuse pulmonary hemorrhage and glomerulonephritis. Pulmonary-renal syndrome\\u000a is not a single entity and is caused by a variety of conditions, including Goodpasture’s syndrome associated with autoantibodies\\u000a to the glomerular and alveolar basement membranes, various forms of primary systemic vasculitis associated with serum positivity\\u000a for antineutrophil cytoplasmic antibodies (ANCA), cryoglobulinemia, systemic lupus erythematosus, systemic

  11. Aortic root dilation secondary to giant cell aortitis in a human immunodeficiency virus-positive patient

    Microsoft Academic Search

    Muhammad A. Javed; Mary N. Sheppard; John Pepper

    2006-01-01

    HIV-associated vasculitis rarely involves the aorta. There is no well-established association of HIV and giant cell arteritis. We present the case of a 31-year-old HIV positive Indian woman who was referred to us with complaints of dyspnea and chest pain. Physical examination revealed a diastolic murmur in the aortic area and echocardiography showed a dilated aortic root causing severe aortic

  12. Aortic root dilation secondary to giant cell aortitis in a human immunodeficiency virus-positive patient

    Microsoft Academic Search

    Muhammad A. Javed; Mary N. Sheppard; John Pepper

    2010-01-01

    HIV-associated vasculitis rarely involvestheaorta. Thereis no well-establishedassociationof HIVand giantcell arteritis.We present thecaseof a 31-year-old HIV positive Indian woman who was referred to us with complaints of dyspnea and chest pain. Physical examination revealed a diastolic murmur in the aortic area and echocardiography showed a dilated aortic root causing severe aortic regurgitation. She was being adequately treated with anti-HIV therapy. She

  13. Schönlein-Henoch purpura during pregnancy with successful outcome for mother and newborn

    Microsoft Academic Search

    Robert Feldmann; Walter Rieger; Paul G Sator; Friedrich Gschnait; Friedrich Breier

    2002-01-01

    ABSTRAC: BACKGROUND: Schönlein-Henoch purpura is a systemic vasculitis that affects vessels of a small caliber and rarely reported in the literature. CASE PRESENTATION: We report on a 35-year-old woman who developed palpable purpura with necrotizing cutaneous lesions on the lower limbs at 27 weeks of gestation. She also complained of epigastric pain and arthralgias. Histologic examination of a skin biopsy

  14. Perineurial cells filled with collagen in ‘atypical’ Cogan’s syndrome

    Microsoft Academic Search

    Kay W. Nolte; Volkmar J. Hans; Christoph Schattenfroh; Joachim Weis; J. Michael Schröder

    2008-01-01

    Cogan’s syndrome is a rare clinical entity characterized by non-infectious interstitial keratitis with vestibuloauditory dysfunction.\\u000a The clinical course is extremely variable. In the majority of patients, there appears to be an underlying systemic process,\\u000a often a “vasculitis”. We were able to study for the first time a sural nerve biopsy of a 38-year-old female with clinically\\u000a suggested Cogan’s syndrome associated

  15. Ataxia and peripheral neuropathy: rare manifestations in Henoch-Schönlein purpura

    Microsoft Academic Search

    A. Bulun; R. Topaloglu; A. Duzova; I. Saatci; N. Besbas; A. Bakkaloglu

    2001-01-01

    .   Henoch-Schönlein purpura (HSP) is a multisystemic vasculitis. Nervous system involvement is usually underestimated. Headaches,\\u000a mental status changes and seizures are the most frequent neurologic symptoms. Ataxia and mononeuropathy are both very rare.\\u000a We present an 11-year-old boy with HSP who suffered from ataxia during the initial presentation and peripheral neuropathy\\u000a at the time of a relapse. Brainstem vasculitic involvement

  16. A Challenging Twist in Pulmonary Renal Syndrome

    PubMed Central

    Namas, Rajaie; Rubin, Bernard

    2014-01-01

    Case. We report a rare case of hydralazine-induced anti-neutrophil cytoplasmic antibody-associated vasculitis. A 75-year-old African American woman with history of high blood pressure on hydralazine for 3 years presented with acute onset of shortness of breath and hemoptysis. Lab workup revealed a severe normocytic anemia and a serum creatinine of 5.09?mg/dL (baseline 0.9). Bronchoscopy demonstrated active pulmonary hemorrhage. A urine sample revealed red cell casts and a renal biopsy demonstrated pauci-immune, focally necrotizing glomerulonephritis with small crescents consistent with possible anti-neutrophil cytoplasmic antibody-positive renal vasculitis. Serologies showed high-titer MPO-ANCA and high-titer anti-histone antibodies. She was treated with intravenous steroids and subsequently with immunosuppression after cessation of hydralazine. The patient was subsequently discharged from hospital after a rapid clinical improvement. Conclusion. Hydralazine-induced anti-neutrophil cytoplasmic antibody-positive renal vasculitis is a rare adverse effect and can present as a severe vasculitic syndrome with multiple organ involvement. Features of this association include the presence of high titer of anti-myeloperoxidase anti-neutrophil cytoplasmic antibody with multiantigenicity, positive anti-histone antibodies, and the lack of immunoglobulin and complement deposition. Prompt cessation of hydralazine may be sufficient to reverse disease activity but immunosuppression may be needed. PMID:25525550

  17. Granulomatosis with polyangiitis (Wegener's granulomatosis) in children: report of three cases with cutaneous manifestations and literature review.

    PubMed

    Gajic-Veljic, Mirjana; Nikolic, Milos; Peco-Antic, Amira; Bogdanovic, Radovan; Andrejevic, Sladjana; Bonaci-Nikolic, Branka

    2013-01-01

    Granulomatosis with polyangiitis (GPA), also known as Wegener's granulomatosis, is a rare disease in childhood. Of 39 GPA patients that we diagnosed during a 20-year period, only 3 (7.7%) were younger than 18 years. We report the course of GPA in three girls whose disease started at the ages of 16, 11, and 6 years. All had cutaneous manifestations: the first had necrotizing vasculitis, the second had palpable purpura, and the third had right upper-eyelid edema and infiltration and proptosis caused by extraocular pseudotumor, initially histologically misdiagnosed as orbital immunoglobulin G4 (IgG4)-related disease. Unlike with skin vasculitis and glomerulonephritis, upper-airway and orbital inflammation were resistant to immunosuppressive therapy. Our report emphasizes that children presenting with cutaneous vasculitis, chronic eyelid swelling, sinusitis, or hoarseness should be tested for antineutrophil cytoplasmic antibodies. We emphasize that the upper-eyelid edema and infiltration, with histologic characteristics of orbital IgG4-related disease, may be the initial presentation of localized GPA in children, a feature that, until now, has been described only in adults. PMID:23145989

  18. 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.

  19. [An autopsy case of subarachnoid and intracerebral hemorrhage and necrotizing angitis associated with methamphetamine abuse].

    PubMed

    Shibata, S; Mori, K; Sekine, I; Suyama, H

    1988-11-01

    We report an autopsy case of methamphetamine-related intracranial hemorrhage and vasculitis. The possible relationship between drug usage and the occurrence of intracranial bleeding and cerebral vasculitis in such patients is discussed. A 22-year-old woman died after an intravenous injection of unknown dose of methamphetamine. A computed tomography head scan demonstrated massive subarachnoid hemorrhage and hematoma in corpus callosum. Cerebral angiography revealed nonfilling of bilateral intracranial carotid arteries and extravasation of contrast medium from right pericallosal artery which was visualized retrogradely via vertebral artery. Postmortem studies showed cerebral edema, subarachnoid and intracerebral hemorrhage, and intracranial vasculitis in the absence of aneurysm, arteriovenous malformation or chronic hypertension. Histological findings of necrosis of blood vessel walls with destruction of the elastica and smooth muscle layer, and without leukocytotic infiltration of the blood vessel walls were observed in order of anterior cerebral, middle cerebral, vertebral, posterior cerebral and basilar arteries. These angiographic and histological evidence suggests that such hemorrhage results from the development of fibrinonecrosis in the large intracerebral vessels, in addition to a sudden rise in blood pressure. PMID:3219243

  20. Schönlein-Henoch purpura during pregnancy with successful outcome for mother and newborn

    PubMed Central

    Feldmann, Robert; Rieger, Walter; Sator, Paul G; Gschnait, Friedrich; Breier, Friedrich

    2002-01-01

    Background Schönlein-Henoch purpura is a systemic vasculitis that affects vessels of a small caliber and rarely reported in the literature. Case presentation We report on a 35-year-old woman who developed palpable purpura with necrotizing cutaneous lesions on the lower limbs at 27 weeks of gestation. She also complained of epigastric pain and arthralgias. Histologic examination of a skin biopsy showed leukocytoclastic vasculitis with intravascular fibrin thrombi. The direct immunofluorescence analysis evidenced vascular deposits of IgA and C3 in the upper and mid-dermis. These findings were consistent with Schönlein-Henoch purpura. There was no evidence of renal involvement or placental dysfunction. The patient was treated with low-dose oral corticosteroids and a healthy infant was delivered by cesarean section. Examination of the placenta and the navel string disclosed no signs of vasculitis or infarction. Conclusions Schönlein-Henoch purpura is rarely reported in pregnancy. Treatment with orally administred corticosteroids may lead to a beneficial outcome for mother and newborn. PMID:11866865

  1. Acute onset distal symmetrical vasculitic polyneuropathy associated with acute hepatitis B.

    PubMed

    Mehndiratta, Manmohan; Pandey, Sanjay; Nayak, Rajeev; Saran, Ravindra K

    2013-02-01

    Hepatitis B can have varied extrahepatic manifestations involving the skin, renal, haematological and nervous systems. Neurological manifestations in hepatitis B may take the form of Guillain-Barré syndrome and secondary systemic vasculitis-related mononeuritis multiplex. The clinical course of hepatitis B-related, vasculitis-related neuropathy is usually subacute to chronic and clinical evolution is relatively benign. To our knowledge, acute hepatitis B-associated vasculitis manifesting as acute distal symmetric polyneuropathy has not been reported. We report a 60-year-old man who presented with fever, mild hepatomegaly, skin lesions in the form of non-palpable purpura and acute onset distal symmetric sensorimotor polyneuropathy. Serum transaminase levels were raised and viral serological markers revealed acute hepatitis B. The patient remained anicteric throughout his clinical course. Nerve conduction studies showed severe axonal sensorimotor polyneuropathy and histopathological examination of sural nerve biopsy was suggestive of vasculitic neuropathy. The patient was first given a course of intravenous immunoglobulin with the antiviral drug entecavir. The fever subsided after 1 week of treatment. The patient was started on prednisolone in addition to the entecavir, and showed significant improvement in motor power and marked resolution in paresthesia after 2 weeks of treatment. Thus, acute onset distal symmetric sensorimotor polyneuropathy of vasculitic etiology can be a manifestation of acute hepatitis B. PMID:23164826

  2. The Usefulness of Interferon-gamma Release Assay for Diagnosis of Tuberculosis-related Uveitis in Korea

    PubMed Central

    Ahn, Seong Joon; Kim, Ko Eun; Park, Kyu Hyung

    2014-01-01

    Purpose To evaluate the usefulness of the interferon-gamma release assay (IGRA) for diagnosing tuberculosis (TB)-related uveitis (TRU). Methods Records from 181 patients with ocular signs and symptoms suggestive of TRU and intraocular inflammation of unknown etiology were reviewed. All subjects underwent clinical and laboratory testing, including IGRA, to rule out presence of underlying disease. A diagnosis of presumed TRU was made based on an internist's TB diagnosis and a patient's response to anti-TB therapy. Sensitivity, specificity, and positive predictive values of IGRA for TRU diagnosis were calculated. Clinical characteristics were compared between patients with positive and negative IGRA results. Results The sensitivity and specificity of IGRA for TRU were 100% and 72.0%, respectively. Mean age, percentage of patients with retinal vasculitis, and the anatomic type of uveitis were significantly different between patients with positive and negative IGRA results (all p ? 0.001). Positive IGRA rates and false-positive rates were significantly different between age and anatomic type groups (both p = 0.001). The positive predictive value of the IGRA among patients with intraocular inflammation was high (70%) when all of younger age (?40 years), posterior uveitis, and retinal vasculitis were present. Conclusions The IGRA is useful for diagnosing TRU in the Korean population, especially when it is used as a screening test. Clinical characteristics, including younger age (?40 years), posterior uveitis, and retinal vasculitis in IGRA-positive patients, increase the likelihood of the patient having TRU. PMID:24882956

  3. Effectiveness of Heparin during Long-Term Tocolysis

    PubMed Central

    Inagaki, Tetsunori; Makino, Shintaro; Yorifuji, Takashi; Sugimura, Motoi; Takeda, Satoru

    2013-01-01

    Objective. Drip infusion during long-term tocolysis causes mechanical and infectious vasculitis and increases the frequency of peripheral venous catheter exchange (PVC), thereby placing a burden on patients. Our study aim is to confirm whether heparin ameliorates pain due to vasculitis during long-term tocolysis and reduces the frequency of peripheral venous catheter exchange. Design. Prospective study. Setting and Sample. All the patients requiring admission because of the presence of uterine contraction or progressive cervical dilatation from August 2009 to June 2011 at Juntendo University in Japan. Methods. Heparin was used for patients at the time the total number of peripheral venous catheter exchanges exceeded 5 in two weeks, and we evaluated whether heparin reduced the frequency of peripheral venous catheter exchange and improved the visual analog scale (VAS) for patients. The main outcome measures frequency of PVC exchange and VAS. Results. This study demonstrated that heparin reduced the frequency of peripheral venous catheter exchange (P = 0.0069) and VAS (P = 0.042). No side effects were noted. Conclusion. Heparin could satisfy patients during long-term tocolysis in terms of ameliorating pain due to vasculitis and reducing the PVC exchange frequency. PMID:23589778

  4. Spontaneous and antiviral-induced cutaneous lesions in chronic hepatitis B virus infection

    PubMed Central

    Grigorescu, Ioana; Dumitrascu, Dan Lucian

    2014-01-01

    AIM: To describe spontaneous, or interferon (IFN)- or immunization-induced skin lesions in hepatitis B virus (HBV) infection. METHODS: A comprehensive literature search of all the papers presenting case reports of dermatological lesions in patients with chronic HBV infection was carried out. We included only patients with histologically proven skin lesions that appeared in the normal course of hepatitis B infection, or after immunization for hepatitis B or antiviral treatment. RESULTS: We found 44 papers on this topic, reporting 151 cases. About 2% of patients with hepatitis B infection, mainly men, presented with skin lesions. Among patients with chronic hepatitis B, vasculitis and essential mixed cryoglobulinemia seemed to be the most frequent skin lesion (53.3%), followed by papular changes, rashes and Gianotti-Crosti syndrome, skin carcinoma and Henoch-Schönlein purpura were rare. IFN treatment seemed to be effective against HBV-associated and immunoglobulin-complex-mediated disease (vasculitis). Two cutaneous lesions (lichen planus and granuloma annulare) were described after hepatitis B vaccination. Systemic lupus and lupus-like lesions were the most frequently encountered lesions after antiviral treatment. Immunosuppressive and steroid therapy ameliorates lichen planus lesions in 50% of cases. CONCLUSION: Vasculitis was the most frequent spontaneous skin lesion found in chronic hepatitis B. Lichen planus was most frequent after immunization and lupus/lupus-like lesions after IFN. PMID:25400473

  5. Clinical Course, Prognosis, and Cause of Death in Primary Sjögren's Syndrome

    PubMed Central

    Horvath, Ildiko Fanny; Szanto, Antonia; Papp, Gabor; Zeher, Margit

    2014-01-01

    The aim of this retrospective, single-centre study was to investigate the clinical and laboratory features and disease outcomes of 547 patients diagnosed with primary Sjögren's syndrome (pSS) between 1975 and 2010. The patients were followed up for 11.4 ± 6.2 years. We evaluated the clinical and laboratory features, and assessed their influence on the time of diagnosis, survival, and mortality ratios, and compared them within subgroups defined by gender, glandular and extraglandular manifestations (EGMs), associated diseases, and immunoserological abnormalities. The most frequent EGMs were polyarthritis, Raynaud's phenomenon, and vasculitis among our patients; the most common associated disease was thyroiditis. During the follow-up period, 51 patients died; the median survival time was 33.71 years. Our results revealed a negative effect of cryoglobulinemia on survival ratios; additionally, the presence of vasculitis and lymphoproliferative diseases at the time of diagnosis increased the risk of mortality. The development of vasculitis was the most powerful predictor of mortality. Mortality in the group of patients with extraglandular symptoms was two- to threefold higher than in the glandular group. Attention is drawn to the importance of close monitoring and targeted diagnostic approaches in those pSS subgroups with obviously increased mortality risk. PMID:24963499

  6. Monocyte activation in patients with Wegener's granulomatosis

    PubMed Central

    Kobold, A.; Kallenberg, C.; Tervaert, J. W.

    1999-01-01

    OBJECTIVE—Wegener's granulomatosis (WG) is an inflammatory disorder characterised by granulomatous inflammation, vasculitis, and necrotising vasculitis and is strongly associated with anti-neutrophil cytoplasmic antibodies (ANCA). Activated monocytes/macrophages are present in renal biopsy specimens and participate in granuloma formation by synthesising and secreting a variety of chemoattractants, growth factors, and cytokines. In view of these findings, in vivo monocyte activation was evaluated in patients with WG and the findings related to parameters of clinical disease activity.?METHODS—Monocyte activation was analysed by measuring plasma concentrations of soluble products of monocyte activation, that is neopterin and interleukin 6 (IL6), by ELISA, and by quantitating the surface expression of activation markers on circulating monocytes by flow cytometry.?RESULTS—Twenty four patients with active WG were included in this study. Ten of these patients were also analysed at the time of remission. Twelve patients with sepsis served as positive controls, and 10 healthy volunteers as negative controls for monocyte activation. Patients with active disease had increased monocyte activation compared with healthy controls as shown by increased concentrations of neopterin (p <0.0001) and increased surface expression of CD11b (p < 0.05) and CD64 (p < 0.05). In those patients with increased concentrations of IL6 during active disease plasma concentrations of IL6 decreased during follow up when patients went into remission (p < 0.0001). In addition, neopterin (r = 0.37, r = 0.44), IL6 (r = 0.37, r = 0.60) and CD63 expression (r = 0.39, r = 0.45) correlated significantly with disease activity as measured by the Birmingham Vasculitis Activity Score and C reactive protein values, respectively. Compared with patients with sepsis, all markers of monocyte activation in patients with vasculitis were lower.?CONCLUSION—It is concluded that disease activity in WG correlates with the extent of activation of monocytes, compatible with their role in the pathophysiology of this disease.?? Keywords: anti-neutrophil cytoplasmic antibody; monocyte activation; vasculitis; flow cytometry PMID:10364903

  7. Recurrent urticaria: clinical investigation of 330 patients.

    PubMed

    Juhlin, L

    1981-04-01

    A questionnaire was used from 1972-1978 for 330 consecutive patients with recurrent urticaria of 3 months to 40 years duration. Fifty men and fifty women had only urticaria. The rest had both urticaria and angio-oedema and most of them were women between 24 and 38 years of age. Urticarial attacks were less frequent during the daytime. A personal history of rhinitis, asthma or atopic dermatitis was recorded in more than one-third. Nasal polyps, migraine and arthralgia were found in 6-7% of the patients. Severe psychiatric problems were mentioned by 16%. Abdominal problems, mainly gastritis, were described by 44%. A history of side effects from drugs was found in 32% of the patients. Food was mentioned as a factor worsening the weals by 30% and drinks by 18%. Fruits, vegetables and nuts were the most common. Despite all cases with physical urticaria having been excluded, physical factors such as exercise were considered by 20% to make the urticaria worse. Provocation tests with various food additives such as azo dyes, benzoates, butylated hydroxytoluene (BHT), butylated hydroxyanisole (BHA) sorbic acid, quinoline yellow, carotene, canthaxanthine, annatto and nitrite revealed one or more positive reactions in one-third of the patients, one-third showed negative provocation tests and, in the rest, one or several tests were questionable. Routine history of other diseases. The fibrin microclot generation test which test which indicates the presence of circulating endotoxins was positive in 24%. PMID:7236502

  8. Extensive protein hydrolysate formula effectively reduces regurgitation in infants with positive and negative challenge tests for cow’s milk allergy

    PubMed Central

    Vandenplas, Y; De Greef, E

    2014-01-01

    Aim Cow’s milk protein allergy (CMPA) is treated using an elimination diet with an extensive protein hydrolysate. We explored whether a thickened or nonthickened version was best for infants with suspected CMPA, which commonly causes regurgitation/vomiting. Methods Diagnosis of CMPA was based on a positive challenge test. We compared the efficacy of two casein extensive hydrolysates (eCH), a nonthickened version (NT-eCH) and a thickened version (T-eCH), using a symptom-based score covering regurgitation, crying, stool consistency, eczema, urticarial and respiratory symptoms. Results A challenge was performed in 52/72 infants with suspected CMPA and was positive in 65.4%. All confirmed CMPA cases tolerated eCH. The symptom-based score decreased significantly in all infants within a month, and the highest reduction was in those with confirmed CMPA. Regurgitation was reduced in all infants (6.4 ± 3.2–2.8 ± 2.9, p < 0.001), but fell more with the T-eCH (?4.2 ± 3.2 regurgitations/day vs. ?3.0 ± 4.5, ns), especially in infants with a negative challenge (?3.9 ± 4.0 vs. ?1.9 ± 3.4, ns). Conclusion eCH fulfilled the criteria for a hypoallergenic formula, and the NT-eCH and T-eCH formulas both reduced CMPA symptoms. The symptom-based score is useful for evaluating how effective dietary treatments are for CMPA. PMID:24575806

  9. Phase Ib clinical trial of starch-conjugated deferoxamine (40SD02): a novel long-acting iron chelator.

    PubMed

    Harmatz, Paul; Grady, Robert W; Dragsten, Paul; Vichinsky, Elliott; Giardina, Patricia; Madden, Jacqueline; Jeng, Michael; Miller, Becky; Hanson, Gregory; Hedlund, Bo

    2007-08-01

    The most widely used drug for iron chelation is deferoxamine (DFO) mesylate. While effective in promoting iron excretion, it requires prolonged daily infusions, often resulting in poor compliance. A clinical trial was conducted using starch-conjugated DFO (S-DFO; 40SD02), a high-molecular-weight iron chelator possessing prolonged vascular retention. Single doses of S-DFO were infused intravenously into groups of four transfusion-dependent patients with beta-thalassaemia at doses of 150, 300, 600 and 900 mg/kg. Urinary iron excretion and various pharmacologic parameters were evaluated for 1 week and safety for 3 weeks. No drug-related effects were observed on clinical chemistries, haematological and coagulation parameters, urinalyses, vital signs or electrocardiograms. Drug-related adverse events were limited to four urticarial reactions, none requiring termination of the infusion. The drug stimulated clinically significant urinary iron excretion, with the highest dose (900 mg/kg) inducing excretion of 1.31 mg of iron/kg (range 0.79-1.90 mg/kg) over 1 week, with residual iron-binding capacity present in the plasma for over 6 d. In summary, treatment with S-DFO, administered weekly, has the potential to achieve iron balance in the poorly compliant patient. PMID:17614825

  10. Review: dermatitis herpetiformis*

    PubMed Central

    Mendes, Fernanda Berti Rocha; Hissa-Elian, Adaucto; de Abreu, Marilda Aparecida Milanez Morgado; Gonçalves, Virgínica Scaff

    2013-01-01

    Dermatitis herpetiformis (DH) or Duhring-Brocq disease is a chronic bullous disease characterized by intense itching and burning sensation in the erythematous papules and urticarial plaques, grouped vesicles with centrifuge growth, and tense blisters. There is an association with the genotypes HLA DR3, HLA DQw2, found in 80-90% of cases. It is an IgA-mediated cutaneous disease, with immunoglobulin A deposits appearing in a granular pattern at the top of the dermal papilla in the sublamina densa area of the basement membrane, which is present both in affected skin and healthy skin. The same protein IgA1 with J chain is found in the small intestinal mucosa in patients with adult celiac disease, suggesting a strong association with DH. Specific antibodies such as antiendomysium, antireticulina, antigliadin and, recently identified, the epidermal and tissue transglutaminase subtypes, as well as increased zonulin production, are common to both conditions, along with gluten-sensitive enteropathy and DH. Autoimmune diseases present higher levels of prevalence, such as thyroid (5-11%), pernicious anemia (1-3%), type 1 diabetes (1-2%) and collagen tissue disease. The chosen treatment is dapsone and a gluten-free diet. PMID:24068131

  11. Phenotype-genotype analysis of cryopyrin-associated periodic syndromes (CAPS): description of a rare non-exon 3 and a novel CIAS1 missense mutation.

    PubMed

    Jesus, Adriana A; Silva, Clovis A; Segundo, Gesmar R; Aksentijevich, Ivona; Fujihira, Erika; Watanabe, Mônica; Carneiro-Sampaio, Magda; Duarte, Alberto J S; Oliveira, João B

    2008-03-01

    We describe in this paper the phenotype-genotype analysis of a Brazilian cohort of patients with cryopyrin-associated periodic syndromes (CAPS). Patient 1 presented with an urticarial rash and recurrent fever exacerbated by cold weather, arthritis, and anterior uveitis, thus, receiving a clinical diagnosis of familial cold autoinflammatory syndrome. CIAS1 sequencing identified the T436I mutation, previously associated to a clinical phenotype of chronic infantile neurological cutaneous and articular/neonatal onset multisystem inflammatory disease. Patient 2 developed a papular exanthema with daily fever shortly after birth, frontal bossing, patellae enlargement, and cognitive and motor impairments. Sequencing identified the exceedingly rare G755R CIAS1 mutation in exon 4. Patient 3 developed skin rash and articular symptoms 6 h after birth, followed by aseptic meningitis. He was found to have the novel C148Y missense mutation in CIAS1. This report expands the spectrum of CIAS1 mutations associated to clinical disease, suggests that the same mutation can be associated with different clinical syndromes, and supports the evidence that CAPS patients should always be screened for mutations outside exon 3. PMID:18080732

  12. [An approach to the patients with cryopyrin-associated periodic syndrome (CAPS) : a new biologic response modifier, canakinumab].

    PubMed

    Yokota, Shumpei; Kikuchi, Masako; Nozawa, Tomo; Kizawa, Toshiatsu; Kanetaka, Taichi; Miyamae, Takako; Mori, Masa-aki; Nishikomori, Ryohta; Takata, Hidetoshi; Heike, Toshio; Hara, Toshiro; Imagawa, Tomoyuki

    2012-01-01

    Cryopyrin-associated periodic syndrome (CAPS) comprises a group of rare, but severe, autoinflammatory syndrome, and includes 3 distinct conditions, familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease (MONID). These syndromes are characterized by urticarial-like rash, periodic fever, central nervous system inflammation, an arthropathy, and the risk of amyloidosis. About 20% die by age 20 years in the most severe cases. The disease is associated with mutations in the NLRP3 gene that encodes for the protein cryopyrin, a component of the inflammasome complex that regulates the production and secretion of IL-1?. Canakinumab is a human IgG monoclonal antibody targeting IL-1?. The clinical trials of canakinumab for patients with CAPS in both western countries and Japan were well-tolerated in most patients, and provided significant advantages over existing competitive therapies. Although no serious adverse effects have been reported, the frequencies of common infectious diseases including nasopharyngitis, upper respiratory tract infections, and gastroenteritis were reported presumably due to the blockade of proinflammatory cytokine, IL-1?. For us pediatrician, it will be important to be more careful for infectious diseases to provide the maximum safety of canakinumab for these patients. PMID:22374439

  13. Association between Helicobacter pylori Infection and Chronic Urticaria: A Meta-Analysis

    PubMed Central

    Gu, Huiyuan; Li, Lin; Zhang, Guoxin

    2015-01-01

    Background. Some studies have shown the possible involvement of Helicobacter pylori (H. pylori) infection in chronic urticaria, but the relationship remains controversial. The aim of this meta-analysis was to quantitatively assess the association between H. pylori infection and chronic urticaria. Methods. Observational studies comparing the prevalence of H. pylori infection in patients with chronic urticaria and control subjects were identified through a systematic search in MEDLINE and EMBASE up to July 2014. H. pylori infection was confirmed by serological or nonserological tests. For subgroup analyses, studies were separated by region, publication year, and H. pylori detection method to screen the potential factors resulting in heterogeneity. Results. 16 studies involving 965 CU cases and 1235 controls were included. Overall, the prevalence of H. pylori infection was higher in urticarial patients than in controls (OR = 1.66; 95% CI: 1.12–2.45; P = 0.01). This result persisted in subanalysis of nine high-quality studies (OR = 1.36; 95% CI: 1.03–1.80; P = 0.03). Subgroup analysis showed that detection method of H. pylori is also a potential influential factor for the overall results. Conclusions. Our present meta-analysis suggests that H. pylori infection is significantly, though weakly, associated with an increased risk of chronic urticaria. PMID:25861258

  14. [Allergic contact urticaria caused by a chameleon. Expression of sensitization to Ficus benjamina].

    PubMed

    Sesztak-Greinecker, G; Hemmer, W; Götz, M; Jarisch, R

    2005-12-01

    A 31-year-old man presented with a long history of rhinoconjunctivitis and sneezing that lasted from March to May. The man kept a chameleon (Chamaeleo calyptratus) as a pet and reported about recurrent urticarial lesions, erythema, and itch after skin contact with the animal. The reactions started within a few minutes and were restricted specifically to the contact sites with the animal's claws. No comparable reactions occurred in other subjects. Allergy testing confirmed allergy to hazel, alder, birch, and ash pollen, and additionally revealed sensitization to house dust mite, cat, and Ficus benjamina. Apparently the contact dermatitis originated from passive transfer of Ficus benjamina allergens to the patient's skin by the reptile which habitually climbed on a big Ficus benjamina tree in the patient's home, thereby contaminating its claws with the plant's allergenic milky sap. Careful examination revealed strong perforation of many leaves by the pointed claws. The patient denied respiratory symptoms from Ficus benjamina and intolerance of Ficus-associated fruits. PMID:15657732

  15. Recognizing Presentations of Pemphigoid Gestationis: A Case Study

    PubMed Central

    2014-01-01

    Introduction. Pemphigoid gestationis (PG) is an autoimmune blistering disease that occurs in approximately 1 in 50,000 pregnancies. Failing to recognize PG may lead to inadequate maternal treatment and possible neonatal complications. Case Report. At 18 weeks of gestation, a 36-year-old otherwise healthy Caucasian G4P1 presented with pruritic papules on her anterior thighs, initially treated with topical steroids. At 31 weeks of gestation, she was switched to oral steroids after her rash and pruritus worsened. The patient had an uncomplicated SVD of a healthy female infant at 37 weeks of gestation and was immediately tapered off steroid treatment, resulting in a severe postpartum flare of her disease. Discussion. This case was similar to reported cases of pruritic urticarial papules followed by blisters; however, this patient had palm, sole, and mucous membrane involvement, which is rare. Biopsy for direct immunofluorescence or ELISA is the preferred test for diagnosis. Previous case reports describe severe postdelivery flares that require higher steroid doses. Obstetrical providers need to be familiar with this disease although it is rare, as this condition can be easily confused with other dermatoses of pregnancy. Adequate treatment is imperative for the physical and psychological well-being of the mother and infant. PMID:25506008

  16. Churg-Strauss syndrome: A case report

    PubMed Central

    2010-01-01

    A fifty-year-old female presented with a one month history of progressive dyspnea, productive cough, pain of elbows and knees, and 40°C fever despite antibiotic treatment. She has been diagnosed of bronchial asthma over 25 years before admission and oral and depot glucocorticosteroids as a long-term therapy was applied. Recently, an attempt of inhaled corticosteroids and LABA treatment was introduced with no success. Four years before admission she also developed peripheral neuropathy. Physical examination revealed tachypnea, wheezes, rhonchi and wet cracles on auscultation, tachycardia, skin nodules, urticarial rash and necrotic bullae all over the body. Chest X-ray showed transient, patchy, nonsegmental areas of consolidation with predilection for lower zones with the area of consolidation in lower left zone. Obstruction was found on spirometry. Tachycardia on ECG and myocardial fluid on ECHO were also detected. Lab exams revealed elevated CRP, WBC, eosinophils, and IgE levels. ANA and ANCA antibodies were not found. Patient was diagnosed of Churg Strauss Syndrome and initial treatment of prednisone was introduced. After four days of treatment, temperature normalized, and dyspnea diminished. After one month of therapy skin lesions regressed. After 18 months of the treatment patient reports no signs, nor symptoms of the disease. Patient continues oral corticosteroid therapy. PMID:21147630

  17. Managing urticaria in primary care.

    PubMed

    Tidman, Michael J

    2015-02-01

    Urticaria is characterised by transient wheals that consist of a swollen palpable centre often surrounded by an erythematous flare, associated with itching or, less commonly, a burning sensation. Individual wheals usually disappear within 1 to 24 hours leaving normal skin. Wheals may be accompanied by angioedema, a more deep-seated flesh-coloured or erythematous swelling of skin or mucous membrane, which may last longer than 24 hours. Urticaria is classified as acute when it resolves within six weeks and chronic when its duration exceeds six weeks. Chronic urticaria is now sub-classified into chronic spontaneous urticaria (CSU) and chronic inducible urticaria. The prognosis for eventual recovery from spontaneous and inducible urticaria is excellent. However, the time course is unpredictable and may extend to years, often following a relapsing and remitting course. Urticaria results from the release of inflammatory mediators from dermal mast cells, resulting in vasodilatation, plasma extravasation, recruitment of immunologically active cells and sensory nerve stimulation. The cause of urticaria cannot usually be precisely identified for most affected individuals. IgE-mediated food allergy is rarely the cause of CSU in patients with the daily appearance of urticarial lesions, although it should be considered in CSU patients with intermittent symptoms. For patients with CSU a differential full blood count and inflammatory markers are all that are routinely recommended. It is also reasonable to test thyroid function and check for circulating thyroid autoantibodies as there is an association between CSU and thyroid autoimmunity. PMID:25816502

  18. Cutaneous manifestations of adult-onset Still's disease: a case report and review of literature.

    PubMed

    Cozzi, Alessandra; Papagrigoraki, Anastasia; Biasi, Domenico; Colato, Chiara; Girolomoni, Giampiero

    2014-04-17

    Adult onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown etiology and pathogenesis characterized by high spiking fever, arthralgia or arthritis, sore throat, lymphadenopathy, hepatosplenomegaly, serositis, and transient cutaneous manifestations. Although more common in children, cases are seen also in adults. Cutaneous involvement is common and may be suggestive for the diagnosis. A case of AOSD in a 35-year-old man is reported here, presenting with urticarial maculopapular rash of trunk, high spiking fever, acute respiratory distress syndrome, and myopericarditis. Skin biopsy showed interstitial and perivascular mature CD15(+) neutrophils. A comprehensive review of literature showed that cutaneous involvement occurs in about 80 % of patients, with various clinical presentations. The most common skin manifestation is an evanescent salmon pink or erythematous maculopapular exanthema, predominantly on the trunk and proximal limbs, with rare involvement of face and distal limbs. Less common manifestations include persistent erythematous plaques and pustular lesions. A constant histopathologic finding is the presence of interstitial dermal neutrophils aligned between the collagen bundles. This pattern may provide an easy accessible clue for the definitive diagnosis of AOSD and exclude other diagnosis such as drug eruptions or infectious diseases. PMID:24737284

  19. Autoimmune bullous skin diseases. Part 1: Clinical manifestations.

    PubMed

    Kneisel, Andrea; Hertl, Michael

    2011-10-01

    Autoimmune bullous skin diseases are characterized by autoantibodies against adhesion molecules of the skin. Pemphigus is a disorder with an intraepidermal loss of adhesion and is characterized by fragile blisters and erosions. Pemphigus vulgaris often shows extensive lesions of the oral mucosa, while pemphigus foliaceus is commonly restricted to cutaneous involvement with puff pastry-like scale formation. Paraneoplastic pemphigus is obligatorily associated with malignancies and often presents as hemorrhagic stomatitis with multiforme-like exanthems. IgA pemphigus typically presents with pustules and annular plaques but not with mucosal involvement. The clinical spectrum of the pemphigoids includes tense blisters, urticarial plaques, and prurigo- like eczematous lesions. Pemphigoid gestationis mostly occurs during the last trimester of pregnancy and mucous membrane pemphigoid primarily involves the oral mucosa and conjunctivae and leads to scarring. Linear IgA bullous dermatosis manifests with tense blisters in a "cluster of jewels"-like pattern in childhood and is more heterogeneous in adulthood. Classical epidermolysis bullosa acquisita shows extensive skin fragility. Dermatitis herpetiformis is associated with gluten-sensitive enteropathy and manifests clinically with severe itching and papulovesicles on the extensor surfaces of the extremities and the lumbosacral area. The intention of the review is to demonstrate the heterogeneous clinical spectrum of autoimmune bullous disorders. PMID:21955378

  20. Prurigo pigmentosa: clinicopathological study and analysis of 50 cases in Korea.

    PubMed

    Kim, Jae Kyung; Chung, Woon Kyong; Chang, Sung Eun; Ko, Joo Yeon; Lee, Jong Hee; Won, Chong Hyun; Lee, Mi Woo; Choi, Jee Ho; Moon, Kee Chan

    2012-11-01

    Prurigo pigmentosa is a recurrent dermatosis with severe pruritus and several peculiar clinical features. Its exact etiology and pathogenesis are unclear. The aim of this study was to investigate the clinical features and chronological changes in the histopathology of prurigo pigmentosa in Korean patients and to assess the etiology of this condition. We reviewed the medical records, clinical photographs and biopsy specimens from 50 patients diagnosed with prurigo pigmentosa. Mean age at diagnosis was 23.7 years (range, 15-61 years). Prurigo pigmentosa started as urticarial papules or plaques, changing first to papulovesicles and then to reticulated brownish macules. The most frequent sites were the back and chest, especially depressed areas such as the central back and inter-mammary area. Dietary change was suspected as a cause of prurigo pigmentosa in 17 patients. Histopathologically, early-stage lesions had dermatitis herpetiformis-like features; fully-developed lesions displayed impetigo-like or acute, generalized, exanthematous, pustulosis-like features; and late lesions presented with post-inflammatory hyperpigmentation-like features. Oral minocycline, with or without dapsone, was effective in inhibiting the appearance of new lesions, but did not prevent recurrence. Prurigo pigmentosa is not rare in Korea, is apparently associated with dietary modification and preferentially involves the depressed regions of the trunk. PMID:22901017

  1. Review: dermatitis herpetiformis.

    PubMed

    Mendes, Fernanda Berti Rocha; Hissa-Elian, Adaucto; Abreu, Marilda Aparecida Milanez Morgado de; Gonçalves, Virgínica Scaff

    2013-01-01

    Dermatitis herpetiformis (DH) or Duhring-Brocq disease is a chronic bullous disease characterized by intense itching and burning sensation in the erythematous papules and urticarial plaques, grouped vesicles with centrifuge growth, and tense blisters. There is an association with the genotypes HLA DR3, HLA DQw2, found in 80-90% of cases. It is an IgA-mediated cutaneous disease, with immunoglobulin A deposits appearing in a granular pattern at the top of the dermal papilla in the sublamina densa area of the basement membrane, which is present both in affected skin and healthy skin. The same protein IgA1 with J chain is found in the small intestinal mucosa in patients with adult celiac disease, suggesting a strong association with DH. Specific antibodies such as antiendomysium, antireticulina, antigliadin and, recently identified, the epidermal and tissue transglutaminase subtypes, as well as increased zonulin production, are common to both conditions, along with gluten-sensitive enteropathy and DH. Autoimmune diseases present higher levels of prevalence, such as thyroid (5-11%), pernicious anemia (1-3%), type 1 diabetes (1-2%) and collagen tissue disease. The chosen treatment is dapsone and a gluten-free diet. PMID:24068131

  2. [Allergic reaction to patent blue dye in breast surgery - case report.

    PubMed

    Maranhão, Marcius Vinícius M; Nóbrega, Dyluzia Kelly Amaral da; Anunciação, Carlos Eduardo Caiado; Maia, Barbara de Alcântara Brito; Mariano, Paulo Virgílio Dantas

    2014-11-20

    We present a case of allergic reaction to patent blue in a patient who underwent excision of sentinel lymph node associated with segmental breast resection. About 20minutes after the dye injection, the patient developed hypotension (BP=70×30mmHg) associated with increased heart frequency. The patient was treated successfully with decreased inspired fraction of inhaled anesthetic and fluid replacement. At the end of the procedure, she presented with bluish urticarial - like plaques on the head, neck, upper limbs, and trunk; hydrocortisone was then used. The patient recovered uneventfully and was discharged from the PACU two hours after the end of surgery without skin changes, and was discharged from hospital on the morning after surgery. The incidence of allergic reactions with the use of patent blue is far superior to the hypersensitivity reactions seen with anesthetic and adjuvant drugs. Therefore, the anesthesiologist must be aware of cardiovascular instability associated with skin changes during the use of patent blue, for early diagnosis and appropriate treatment of this hypersensitivity reaction to this dye. PMID:25458838

  3. Basophil Activation Test with Food Additives in Chronic Urticaria Patients

    PubMed Central

    Kang, Min-Gyu; Song, Woo-Jung; Park, Han-Ki; Lim, Kyung-Hwan; Kim, Su-Jung; Lee, Suh-Young; Kim, Sae-Hoon; Cho, Sang-Heon; Min, Kyung-Up

    2014-01-01

    The role of food additives in chronic urticaria (CU) is still under investigation. In this study, we aimed to explore the association between food additives and CU by using the basophil activation test (BAT). The BAT using 15 common food additives was performed for 15 patients with CU who had a history of recurrent urticarial aggravation following intake of various foods without a definite food-specific IgE. Of the 15 patients studied, two (13.3%) showed positive BAT results for one of the tested food additives. One patient responded to monosodium glutamate, showing 18.7% of CD203c-positive basophils. Another patient showed a positive BAT result to sodium benzoate. Both patients had clinical correlations with the agents, which were partly determined by elimination diets. The present study suggested that at least a small proportion of patients with CU had symptoms associated with food additives. The results may suggest the potential utility of the BAT to identity the role of food additives in CU. PMID:24527415

  4. Analysis of circulating vascular endothelial growth factor and its soluble receptors in patients with different forms of chronic urticaria.

    PubMed

    Jagodzinska, Julia; Polaniak, Renata; Birkner, Ewa; Kasperska-Zajac, Alicja

    2015-01-01

    Background. Vascular endothelial growth factor (VEGF) is a powerful enhancer of vascular permeability and inflammatory response; however its significance in chronic urticaria is poorly recognised. Aim. To compare free circulating levels of VEGF and its soluble receptors (sVEGFR1 and VEGFR2) in patients with different forms of chronic urticaria. Methods. The concentrations of VEGF and its receptors in plateletpoor plasma (PPP)/plasma were measured using enzyme-linked immunosorbent assay in chronic urticaria: (1) chronic spontaneous urticaria (CSU) with positive autologous serum skin test (ASST), (2) CSU with negative response to ASST, (3) CSU with concomitant euthyroid Hashimoto's thyroiditis (CSU/Hashimoto), (4) delayed pressure urticaria (DPU), and the healthy subjects. Results. There were no significant differences in VEGF concentration in PPP between CSU groups and the healthy subjects. Contrary, VEGF concentration was significantly higher in DPU and CSU/Hashimoto patients as compared with the healthy subjects and CSU groups. Furthermore, VEGF value in CSU/Hashimoto patients during the remission was similar to that of the active period and significantly higher than the healthy subjects; VEGF concentration was significantly correlated with TSH. Plasma concentrations of sVEGF1 and sVEGF2 were similar in chronic urticaria patients and the healthy subjects. Conclusions. Increased free circulating VEGF concentration may result from the urticarial process itself as well as concomitant Hashimoto's thyroiditis. PMID:25756047

  5. Prospective study of definite caterpillar exposures.

    PubMed

    Balit, Corrine R; Geary, Merilyn J; Russell, Richard C; Isbister, Geoffrey K

    2003-11-01

    Exposure to caterpillars results in a variety of clinical effects depending on the species involved. The aim of this study was to describe the clinical effects from caterpillar exposures within Australia. Cases were recruited prospectively from calls to a poison information centre. Subjects were included if they had a definite exposure and they had collected the caterpillar or cocoon. The caterpillars were identified to genus and species level where possible. There were 36 included cases: two were contact exposures to caterpillar contents, one was an ingestion of a caterpillar and the remaining 33 patients had definite reactions from caterpillar or cocoon exposure. There were five families of caterpillars identified in the study: Arctiidae, Limacodidae, Anthelidae, Lymantriidae and Sphingidae, many of which occur worldwide. Clinical effects ranged from severe pain to an urticarial response depending on the species involved. There were no adverse effects following ingestion in this study. Treatment consisted primarily of removal of the caterpillar or cocoon. Other treatment measures consisted of symptomatic treatment such as ice packs and antihistamines. This is the first prospective study of caterpillar exposures within Australia and demonstrates that exposures can result in a variety of reactions depending on the family and species involved. PMID:14602121

  6. T cell activity in successful treatment of chronic urticaria with omalizumab

    PubMed Central

    2011-01-01

    Omalizumab, a humanized monoclonal anti-IgE antibody has the potential to alter allergen processing. Recently, it has been postulated the assessment of PHA-stimulated adenosine triphosphate (ATP) activity as maker of CD4+ T cells activity in peripheral blood cells. We present the case report of a 35-year-old woman with a history of chronic idiopathic urticaria and angioedema of 8 years of development with poor response to treatment. The patient was partially controlled with cyclosporine at doses of 100 mg/12 h. However, she was still developing hives daily. Finally treatment with omalizumab was started at dose of 300 mg every 2 weeks. The patient experienced a decrease in urticarial lesions 2 days after starting therapy. We also evaluated the effects of omalizumab therapy on the activity of peripheral blood CD4+ T cells from the patient, in order to determine the potential modification of anti-IgE therapy on the process of antigen presentation-recognition. Activity of CD4+ cells by ATP release was clearly increased demonstrating an enlarged CD4 activity. Omalizumab may be useful in the treatment of severe chronic urticaria. ATP activity of peripheral blood CD4+ T cells might be a non-subjective method to assess Omalizumab activity. PMID:21791043

  7. Combined cold- and heat-induced cholinergic urticaria.

    PubMed

    Farnam, J; Grant, J A; Lett-Brown, M A; Lord, R A; Russell, W L; Henry, D P

    1986-08-01

    A 36-year-old white woman with a 20-year history of cutaneous, respiratory, and cardiovascular symptoms triggered by physical activity and by exposure to either heat or cold was evaluated. A routine evaluation for the cause of her condition was positive only for certain physical factors. Cutaneous testing for dermatographism, ice-cube challenge, and exposure to ultraviolet A and ultraviolet B light were negative. A methacholine skin test was positive. Sitting in a cold room (4 degrees C) induced micropapular wheals on exposed areas similar to those classically associated with cholinergic urticaria. Placing both feet in warm water (44 degrees C) induced similar but more intense cutaneous lesions at sites not exposed to heat, light headedness, and severe asthma. Exercise for 10 minutes caused confluent and punctate urticarial lesions. Simultaneous measurement of plasma histamine during cold and heat challenges revealed increases paralleling the course of symptoms. Repeat challenge with cold, heat, and exercise after beginning treatment with both H1 and H2 histamine antagonists resulted in marked reduction in symptoms; however, significant rises in plasma histamines were still noted. PMID:3734288

  8. Mast Cell Dependent Vascular Changes Associated with an Acute Response to Cold Immersion in Primary Contact Urticaria

    PubMed Central

    Meyer, Joseph; Gorbach, Alexander M.; Liu, Wei-Min; Medic, Nevenka; Young, Michael; Nelson, Celeste; Arceo, Sarah; Desai, Avanti; Metcalfe, Dean D.; Komarow, Hirsh D.

    2013-01-01

    Background While a number of the consequences of mast cell degranulation within tissues have been documented including tissue-specific changes such as bronchospasm and the subsequent cellular infiltrate, there is little known about the immediate effects of mast cell degranulation on the associated vasculature, critical to understanding the evolution of mast cell dependent inflammation. Objective To characterize the microcirculatory events that follow mast cell degranulation. Methodology/Principal Findings Perturbations in dermal blood flow, temperature and skin color were analyzed using laser-speckle contrast imaging, infrared and polarized-light colorimetry following cold-hand immersion (CHI) challenge in patients with cold-induced urticaria compared to the response in healthy controls. Evidence for mast cell degranulation was established by documentation of serum histamine levels and the localized release of tryptase in post-challenge urticarial biopsies. Laser-speckle contrast imaging quantified the attenuated response to cold challenge in patients on cetirizine. We found that the histamine-associated vascular response accompanying mast cell degranulation is rapid and extensive. At the tissue level, it is characterized by a uniform pattern of increased blood flow, thermal warming, vasodilation, and recruitment of collateral circulation. These vascular responses are modified by the administration of an antihistamine. Conclusions/Significance Monitoring the hemodynamic responses within tissues that are associated with mast cell degranulation provides additional insight into the evolution of the acute inflammatory response and offers a unique approach to assess the effectiveness of treatment intervention. PMID:23451084

  9. Anaphylaxis to black widow spider antivenom.

    PubMed

    Hoyte, Christopher O; Cushing, Tracy A; Heard, Kennon J

    2012-06-01

    Black widow spider envenomation is commonly reported to poison centers. Black widow spider envenomation produces a clinical syndrome, known as latrodectism, characterized by headache, nausea, vomiting, several muscle cramping and pain, joint stiffness, hypertension, and regional diaphoresis. Black widow spider antivenom (Merck & Co, Inc, West Point, PA USA) is an effective and relatively safe treatment option. There is 1 clear case of anaphylaxis secondary to black widow spider antivenom reported in the medical literature. Here, we report a case of anaphylaxis to antivenom. A 12-year-old boy presented to the emergency department (ED) with diffuse, severe pain 2 1/2 hours after being bitten by a black widow spider on the right lower extremity. In the ED, the patient failed analgesic therapy with fentanyl and was given black widow spider antivenom. Within 45 minutes, he exhibited signs and symptoms consistent with anaphylaxis, including wheezing, chest tightness, pruritus, and urticarial rash. The patient was given standard therapy for anaphylaxis, and all of his signs and symptoms (including the pain secondary to the black widow envenomation) resolved over 6 hours of observation. Leading experts agree that the use of antivenom is indicated in cases of severe envenomation not responsive to standard therapy. Despite concern that the antivenom is an equine-derived whole IgG and can precipitate early hypersensitivity reactions, there is only 1 other reported case of anaphylaxis to the antivenom in the medical literature. PMID:21641165

  10. Dermatoses of Pregnancy - Clues to Diagnosis, Fetal Risk and Therapy

    PubMed Central

    2011-01-01

    The specific dermatoses of pregnancy represent a heterogeneous group of pruritic skin diseases that have been recently reclassified and include pemphigoid (herpes) gestationis, polymorphic eruption of pregnancy (syn. pruritic urticarial papules and plaques of pregnancy), intrahepatic cholestasis of pregnancy, and atopic eruption of pregnancy. They are associated with severe pruritus that should never be neglected in pregnancy but always lead to an exact work-up of the patient. Clinical characteristics, in particular timing of onset, morphology and localization of skin lesions are crucial for diagnosis which, in case of pemphigoid gestationis and intrahepatic cholestasis of pregnancy, will be confirmed by specific immunofluorescence and laboratory findings. While polymorphic and atopic eruptions of pregnancy are distressing only to the mother because of pruritus, pemphigoid gestationis may be associated with prematurity and small-for-date babies and intrahepatic cholestasis of pregnancy poses an increased risk for fetal distress, prematurity, and stillbirth. Corticosteroids and antihistamines control pemphigoid gestationis, polymorphic and atopic eruptions of pregnancy; intrahepatic cholestasis of pregnancy, in contrast, should be treated with ursodeoxycholic acid. This review will focus on the new classification of pregnancy dermatoses, discuss them in detail, and present a practical algorithm to facilitate the management of the pregnant patient with skin lesions. PMID:21909194

  11. Analysis of Circulating Vascular Endothelial Growth Factor and Its Soluble Receptors in Patients with Different Forms of Chronic Urticaria

    PubMed Central

    Jagodzinska, Julia; Polaniak, Renata; Birkner, Ewa; Kasperska-Zajac, Alicja

    2015-01-01

    Background. Vascular endothelial growth factor (VEGF) is a powerful enhancer of vascular permeability and inflammatory response; however its significance in chronic urticaria is poorly recognised. Aim. To compare free circulating levels of VEGF and its soluble receptors (sVEGFR1 and VEGFR2) in patients with different forms of chronic urticaria. Methods. The concentrations of VEGF and its receptors in plateletpoor plasma (PPP)/plasma were measured using enzyme-linked immunosorbent assay in chronic urticaria: (1) chronic spontaneous urticaria (CSU) with positive autologous serum skin test (ASST), (2) CSU with negative response to ASST, (3) CSU with concomitant euthyroid Hashimoto's thyroiditis (CSU/Hashimoto), (4) delayed pressure urticaria (DPU), and the healthy subjects. Results. There were no significant differences in VEGF concentration in PPP between CSU groups and the healthy subjects. Contrary, VEGF concentration was significantly higher in DPU and CSU/Hashimoto patients as compared with the healthy subjects and CSU groups. Furthermore, VEGF value in CSU/Hashimoto patients during the remission was similar to that of the active period and significantly higher than the healthy subjects; VEGF concentration was significantly correlated with TSH. Plasma concentrations of sVEGF1 and sVEGF2 were similar in chronic urticaria patients and the healthy subjects. Conclusions. Increased free circulating VEGF concentration may result from the urticarial process itself as well as concomitant Hashimoto's thyroiditis. PMID:25756047

  12. The Association between Platelet Count and Acute Phase Response in Chronic Spontaneous Urticaria

    PubMed Central

    Kasperska-Zaj?c, Alicja; Grzanka, Alicja; Jarzab, Jerzy; Misio?ek, Maciej; Wyszy?ska-Ch?ap, Magdalena; Kasperski, Jacek; Machura, Edyta

    2014-01-01

    Background. The platelet parameters and C-reactive protein (CRP) are markers reflecting a systemic inflammatory response. Among those, CRP is one of the major proteins helpful in determination of severity/activity of chronic spontaneous urticaria (CSU). Aim. To determine relationships between platelet activation indices and serum concentration of CRP, the best marker of acute phase response, and their potential clinical use in CSU patients. Methods. Mean platelet volume (MPV), platelet distribution width (PDW), and platelet count as well as serum CRP concentration were measured in CSU patients, showing different degrees of urticarial severity, and in the healthy subjects. Results. No significant differences were found in MPV and PDW between CSU group and the healthy subjects. The platelet count was significantly higher in moderate-severe CSU than that of the controls and mild CSU patients. Serum CRP concentrations were significantly higher in CSU patients as compared with the healthy subjects and significantly correlated with the platelet count in CSU patients. Conclusions. Acute phase response in CSU is associated with the increased number of circulating platelets in patients with more severe symptoms. It seems that simple determination of platelet size indices is not a reliable indicator of CSU severity/activity. PMID:25025065

  13. Early-Onset Stroke and Vasculopathy Associated with Mutations in ADA2

    PubMed Central

    Zhou, Q.; Yang, D.; Ombrello, A.K.; Zavialov, Andrey V.; Toro, C.; Zavialov, Anton V.; Stone, D.L.; Chae, J.J.; Rosenzweig, S.D.; Bishop, K.; Barron, K.S.; Kuehn, H.S.; Hoffmann, P.; Negro, A.; Tsai, W.L.; Cowen, E.W.; Pei, W.; Milner, J.D.; Silvin, C.; Heller, T.; Chin, D.T.; Patronas, N.J.; Barber, J.S.; Lee, C.-C.R.; Wood, G.M.; Ling, A.; Kelly, S.J.; Kleiner, D.E.; Mullikin, J.C.; Ganson, N.J.; Kong, H.H.; Hambleton, S.; Candotti, F.; Quezado, M.M.; Calvo, K.R.; Alao, H.; Barham, B.K.; Jones, A.; Meschia, J.F.; Worrall, B.B.; Kasner, S.E.; Rich, S.S.; Goldbach-Mansky, R.; Abinun, M.; Chalom, E.; Gotte, A.C.; Punaro, M.; Pascual, V.; Verbsky, J.W.; Torgerson, T.R.; Singer, N.G.; Gershon, T.R.; Ozen, S.; Karadag, O.; Fleisher, T.A.; Remmers, E.F.; Burgess, S.M.; Moir, S.L.; Gadina, M.; Sood, R.; Hershfield, M.S.; Boehm, M.; Kastner, D.L.; Aksentijevich, I.

    2014-01-01

    BACKGROUND We observed a syndrome of intermittent fevers, early-onset lacunar strokes and other neurovascular manifestations, livedoid rash, hepatosplenomegaly, and systemic vasculopathy in three unrelated patients. We suspected a genetic cause because the disorder presented in early childhood. METHODS We performed whole-exome sequencing in the initial three patients and their unaffected parents and candidate-gene sequencing in three patients with a similar phenotype, as well as two young siblings with polyarteritis nodosa and one patient with small-vessel vasculitis. Enzyme assays, immunoblotting, immunohistochemical testing, flow cytometry, and cytokine profiling were performed on samples from the patients. To study protein function, we used morpholino-mediated knockdowns in zebrafish and short hairpin RNA knockdowns in U937 cells cultured with human dermal endothelial cells. RESULTS All nine patients carried recessively inherited mutations in CECR1 (cat eye syndrome chromosome region, candidate 1), encoding adenosine deaminase 2 (ADA2), that were predicted to be deleterious; these mutations were rare or absent in healthy controls. Six patients were compound heterozygous for eight CECR1 mutations, whereas the three patients with polyarteritis nodosa or small-vessel vasculitis were homozygous for the p.Gly47Arg mutation. Patients had a marked reduction in the levels of ADA2 and ADA2-specific enzyme activity in the blood. Skin, liver, and brain biopsies revealed vasculopathic changes characterized by compromised endothelial integrity, endothelial cellular activation, and inflammation. Knockdown of a zebrafish ADA2 homologue caused intracranial hemorrhages and neutropenia — phenotypes that were prevented by coinjection with nonmutated (but not with mutated) human CECR1. Monocytes from patients induced damage in cocultured endothelial-cell layers. CONCLUSIONS Loss-of-function mutations in CECR1 were associated with a spectrum of vascular and inflammatory phenotypes, ranging from early-onset recurrent stroke to systemic vasculopathy or vasculitis. (Funded by the National Institutes of Health Intramural Research Programs and others.) PMID:24552284

  14. Modulation of MMP-2 and MMP-9 in Churg-Strauss syndrome respiratory mucosa: potential monitoring parameters.

    PubMed

    Leone, A; Uzzo, M L; Gerbino, A; Tortorici, S; Tralongo, P; Cappello, F; Incandela, S; Spatola, G F; Jurjus, A R

    2014-01-01

    Churg-Strauss (CSS) syndrome is rare and of unknown etiology. It is associated with vasculitis, blood eosinophilia and granulomatosis, and affects multiple organs and systems at various stages of the disease. Specific diagnostic and monitoring tests are not yet available. This study aims to assess the changes in MMP-2 and MMP-9 along with the histopathological alterations in two cases of CSS, as possible potential diagnostic and monitoring criteria. Two adult male patients were diagnosed with CSS in the otorhinolaryngology clinic in the University of Palermo, based on multiple clinical and histopathologic criteria. Biopsies of respiratory mucosa were taken after the consent of the patients, processed for routine histopathology and immunohistochemistry as well as quantitative polymerase chain reaction (qPCR). Similar biopsies were also taken from a non- CSS patient. The Assessment of MMP-2 and MMP-9 was performed using both immunohistochemistry and qPCR techniques. Histopathological alterations in the respiratory mucosa were consistent with vasculitis and granulomatous tissue formation, in addition to inflammatory cell infiltration with abundance of eosinophils. Immunohistochemistry assay performed on the samples derived from the two CSS patients showed a relative and remarkable increase of both MMP-2 and MMP-9 compared to controls. Such an increase was consistent with the qPCR results which depicted a significant increase between 20 and 30% for both MMP-2 and MMP-9, respectively. Since the secretion of MMPs is an essential step in angiogenesis, could these enzymatic factors be used as parameters to diagnose or monitor the evolution of CSS? The small number of samples analyzed in this study does not allow us to suggest a general statement correlating the increase in expression of MMP-2 and MMP-9 to the appearance or evolution of vasculitis; it is only speculative. PMID:25004843

  15. Acute Retinal Necrosis in Childhood

    PubMed Central

    Pikkel, Yoav Y.; Pikkel, Joseph

    2014-01-01

    Background Acute retinal necrosis (ARN) is a viral syndrome consisting of uveitis/vitritis, occlusive vasculitis and peripheral necrosis. Few incidents are reported in children. The etiology is reactivated herpes simplex virus (HSV) or varicella-zoster virus (VZV). Treatment with acyclovir is often used. The administration of oral glucocorticosteroids is of unproven benefit. Prognosis is variable but poor. Methods Three weeks after contracting mild chickenpox, a healthy 4-year-old girl developed blurred vision in her right eye. Severely reduced visual acuity was noted, together with anterior uveitis, ‘mutton-fat’ precipitates and vitral flare. Retinal vasculitis with necrosis was present. Serology for toxoplasma, cytomegalovirus and HIV was negative, while HSV and VZV IgG antibodies were positive. She was treated with 30 mg/kg of intravenous methylprednisolone (3 days), 30 mg of oral prednisone (3 days), and tapering for 8 weeks. Intravenous acyclovir was given for 10 days, followed by oral acyclovir for 4 months. Aspirin (100 mg/day) was given for 4 months. Results At 12 months, the girl felt good. Her right eye acuity was 6/9, with an intraocular pressure of 17 mm Hg. The peripheral retina showed scarring but no detachment. Conclusions This is the first report of a once-daily high-dose methylprednisolone pulse therapy in one of the youngest known ARN cases. Pulsed steroid therapy was based on its known effectiveness in vasculitis, which is the main pathophysiology in ARN. There was no evidence of steroid-related viral over-replication. Our case achieved an excellent clinical and ophthalmic recovery in spite of the poor prognosis. The positive result of this case report provides a basis for further evaluation of high-dose steroid pulse therapy in ARN. PMID:24932179

  16. Clinical Significance of Antiproteinase 3 Antibody Positivity in cANCA-Positive Patients

    Microsoft Academic Search

    V. van Pesch; M. Jadoul; C. Lefèbvre; B. R. Lauwerys; J.-P. Tomasi; J.-P. Devogelaer; F. A. Houssiau

    1999-01-01

    :   We addressed the clinical significance of antiproteinase 3 (anti-PR3) antibody (Ab) positivity by reviewing the files of\\u000a 79 patients whose serum contained antineutrophil cytoplasmic antibodies with a cytoplasmic staining pattern (cANCA) and had\\u000a been tested for anti-PR3 reactivity. Vasculitis was present in most (22\\/35) cANCA+ PR3+ patients but in only a few (5\\/44) cANCA+ PR3+ patients, thereby suggesting that

  17. A case of multiple extrahepatic manifestations in a patient with untreated, chronic hepatitis C virus infection.

    PubMed

    Meillier, Andrew; McGee, Jean; Kartan, Saritha; Baskin, Stuart

    2014-02-01

    Chronic hepatitis C virus (HCV) infection is a complex, multi-organ disorder, not just limited to the liver. Mixed cryoglobulinemia (MC) type 2 is a common extrahepatic complication, in which immunoglobulin complexes deposit in vascular endothelium. This in turn creates a diffuse inflammatory reaction, leading to a variety of disorders involving multiple systems. We report the rare case of a patient with cryoglobulinemia, cutaneous vasculitis, membroproliferative glomerulonephritis, and B cell lymphoma with a variant t(6;10) translocation in the setting of an untreated, chronic HCV infection. This case highlights the challenge associated with diagnosing and managing such a complex presentation. PMID:24315985

  18. Nodular Erythema Elevatum Diutinum Mimicking Kaposi's Sarcoma in a Human Immunodeficiency Virus Infected Patient

    PubMed Central

    Rao, G Raghurama; Joshi, Rajiv; Phaneendra Prasad, A Krishna; Amareswar, A; Sandhya, S; Sridevi, M

    2014-01-01

    Erythema elevatum diutinum (EED) has been emerging as a specific Human Immunodeficiency Virus (HIV) associated dermatosis in recent times. It is an extremely rare chronic disease of unknown origin and part of the spectrum of leukocytoclastic vasculitis. We describe a case of EED simulating Kaposi's sarcoma in a 52-year-old HIV infected female patient with no previous opportunistic infections and CD4+ count of 164/mm3. Therapy with oral dapsone (100 mg/day) for two weeks resulted in resolution of some lesions. PMID:25484391

  19. [Acute hemiparesis revealing a neuroborreliosis in a child].

    PubMed

    Rénard, C; Marignier, S; Gillet, Y; Roure-Sobas, C; Guibaud, L; Des Portes, V; Lion-François, L

    2008-01-01

    We report on a 11-year-old boy who had 2 acute hemiparesis episodes over a period of 1 month. He suffered from headache and fatigue since 1 year. He could not remember neither a tick bite nor a local erythematous skin lesion. The diagnosis of neuroborreliosis was based on intrathecal production of specifics antibodies. Furthermore, the CSF/blood glucose ratio was decreased (0.14), which was rarely described. Cranial MRI showed left capsulothalamic inflammation and a vasculitis. The patient was successfully treated by ceftriaxone. Neuroborreliosis should be considered in all children with stroke-like episode, even in the absence of a history of a tick bite. PMID:18155890

  20. Percutaneous Transcatheter Embolization of Gastrointestinal Bleeding in a Child with Polyarteritis Nodosa

    PubMed Central

    Bas, Ahmet; Samanci, Cesur; Numan, Furuzan

    2014-01-01

    Summary Background Polyarteritis nodosa is a form of necrotizing vasculitis of small and medium-sized arteries. Major gastrointestinal complications are ulceration, perforation, hemorrhage, and obstruction. Case Report We report on a clinical case of a 16-year-old female patient with massive hematemesis, who was successfully treated with embolization with a 1:2 dilution of N-butyl cyanoacrylate glue. Conclusions To the best of our knowledge, this is the youngest child reported on with massive GI bleeding secondary to PAN, treated with successful percutaneous transcatheter embolization under emergency conditions. PMID:25512765

  1. NOAH-New York Online Access to Health: Arthritis and Rheumatoid Disorders

    NSDL National Science Digital Library

    From New York Online Access to Health (NOAH), this website offers links to numerous Internet resources for Arthritis and Rheumatoid Disorders. Care and Treatment resource categories include Diet; Medications; Total Hip Replacement; and Alternative Therapies-to name a few. Websites relating to diagnosis and symptoms, as well as specific concerns like arthritis in children, and rheumatoid diseases and pregnancy are available as well. In addition, the site links to websites for a wide range of Rheumatoid Disorders such as Avascular Necrosis, Gout, Osteoarthritis, Vasculitis, and more. Site visitors can also link to Internet resources for other health issues on the NOAH website including Heart Disease, Infections, Cancer, Asthma, and Dental Care.

  2. Hemorrhagic stroke after naphazoline exposition: case report.

    PubMed

    Zavala, Jorge A A; Pereira, Eduardo R; Zétola, Viviane H F; Teive, Hélio A G; Nóvak, Edison M; Werneck, Lineu C

    2004-09-01

    Ten percent of all strokes are due to spontaneous cerebral hemorrhages. They are associated to drugs (licit and illicit) in 9.5% of all cases in young adults. This is a case report of a 44-year-old man, without previous morbidities, who presented a sudden onset headache and arterial hypertension 24 hours after use of naphazoline as nasal decongestant. Cranial tomography showed right thalamus hemorrhage. Cerebral angiography showed no aneurisms, vascular malformations or vasculitis. No other risk factors were found during investigation in this patient and the stroke was attributed to naphazoline exposition. PMID:15476091

  3. 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.

  4. Recurrent Febrile Neutropenia and Thrombocytopenia in a Chronic Cocaine User: A Case of Levamisole Induced Complications

    PubMed Central

    Martinez, Eduardo; Alvi, Raza; Venkatram, Sindhaghatta; Diaz-Fuentes, Gilda

    2015-01-01

    Cocaine is used by approximately 1.5 million Americans each month and up to 69% of the cocaine seized contains levamisole. The real incidence of cocaine-levamisole induced neutropenia is unclear but probably underestimated. Associated complications include fever, thrombocytopenia, skin-vasculitis disorders, and rarely kidney injury. We present a young male, with chronic active cocaine use presenting with recurrent episodes of febrile neutropenia and thrombocytopenia. He underwent extensive work-up and was treated with many antibiotics and we suspect that his neutropenia and thrombocytopenia were caused by recurrent cocaine-levamisole use.

  5. [Chlamydial infection of the central nervous system. Laboratory diagnosis and clinic and morphological features].

    PubMed

    Va?nshenker, Iu I; Nuralova, I V; Onishenko, L S

    2014-01-01

    The paper presents data on the diagnosis, clinical and pathomorphological changes in the central nervous system (CNS) in neurochlamydiasis according to clinical, autoptic, and experimental evidence. It discusses the possible implication of Ch. trachomatis, Ch. pneumoniae, and Ch. psittaci in the development and course of different diseases with CNS involvement: atherosclerosis, vasculitis, multiple sclerosis, Alzheimer's disease, schizophrenia, autism, vegetative state, sequels of perinatal lesions in childhood and adolescence, HIV infection, etc. Considerable attention is paid to the specific features of diagnosis of Chlamydia-induced CNS lesions. Purposeful pathomorphological investigations are shown to be needed. PMID:24745195

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

    SciTech Connect

    Gueven, Koray, E-mail: korayguven@yahoo.com; Rozanes, Izzet, E-mail: rozanes@superonline.co [Istanbul University, Department of Radiology, Istanbul Faculty of Medicine (Turkey); Kayabali, Murat, E-mail: murat.kayabali@veezy.co [Istanbul University, Section of Vascular Surgery, Department of General Surgery, Istanbul Faculty of Medicine (Turkey); Minareci, Ozenc, E-mail: minareci@doctor.co [Istanbul University, Department of Radiology, Istanbul Faculty of Medicine (Turkey)

    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.

  7. Hyperostosis frontalis interna in a patient with giant cell arteritis.

    PubMed

    Kocabas, Hilal; Sezer, Ilhan; Melikoglu, Meltem Alkan; Gurbuz, Ulku; Illeez, Ozge; Ozbudak, Irem Hicran; Butun, Bulent

    2008-01-01

    Hyperostosis frontalis interna (HFI) is a disorder characterized by progressive symmetric thickening of the inner table of the frontal bone of the human skull. HFI may be accompanied by headache and some neuropsychiatric diseases such as epilepsy and dementia. Giant cell arteritis (GCA), also called temporal arteritis, is a systemic inflammatory vasculitis of unknown etiology that affects medium- and large-sized arteries. It affects elderly people and may result in a wide variety of systemic, neurologic and ophthalmologic complications. As no association of HFI and GCA was encountered in the literature, we found it interesting to report a case with both of these clinical entities. PMID:18250961

  8. Granulomatous orchitis in a pre-pubertal school-aged child: differential diagnosis dilemmas.

    PubMed

    Jesus, Lisieux E; Rocha, Kátia L M; Caldas, Maria L R; Fonseca, Elissa

    2012-10-01

    A 6-year-old male presented with testicular growth and persistent chronic orchiepididymitis with high inflammatory markers (C reactive protein and erythrocyte sedimentation rate). Biopsies of the testes and epididymides showed bilateral epididymal and testicular granulomata, testicular fibrosis and chronic inflammatory infiltration, and the histological diagnosis was granulomatous orchitis. The symptoms receded with oral corticosteroids. Although rare, granulomatous orchitis is a possible diagnosis in children presenting testicular enlargement. It is important to differentiate it from testicular tumors (if necessary with testicular biopsy) and to investigate its association with systemic vasculitis and infectious diseases. PMID:22575712

  9. Diagnostic exercise: suppurative rhombencephalitis and meningitis in a goat.

    PubMed

    Dill, J A; Rissi, D R

    2014-07-01

    A 1-year-old female Boer goat was presented for necropsy following spontaneous death and history of acute recumbency, nystagmus, and sialorrhea. A swollen area was grossly observed in the brainstem at the level of the pons. On cut surface, the right cerebellar peduncles were expanded by a focal, pale, poorly demarcated, slightly depressed, and soft area of malacia. Microscopically, this area contained diffuse edema and necrosis, with microabscesses, neuronal necrosis, neuronophagia, axonal spheroids, vasculitis, and perivascular accumulations of lymphocytes, plasma cells, macrophages, and neutrophils. The diagnosis was based on the morphologic findings, fluorescent antibody test results, and special staining. PMID:23576239

  10. Extreme distal bypass to improve wound healing in Buerger's disease.

    PubMed

    De Caridi, Giovanni; Massara, Mafalda; Villari, Simona; Martelli, Eugenio; Spinelli, Francesco; Grande, Raffaele; Butrico, Lucia; de Franciscis, Stefano; Serra, Raffaele

    2014-02-25

    Thromboangiitis obliterans or Buerger's disease is a rare non-atherosclerotic segmental inflammatory vasculitis that most commonly involves small and medium-sized arteries, veins and nerves of the extremities, and generally affects young tobacco smokers. A 53-year-old man was found to have critical ischaemia of his left lower limb with foot gangrene. He underwent extremely distal surgical revascularisation using a great saphenous vein bypass graft. The choice of a very distal artery as run-off vessel promoted a faster wound healing and pain relief, with improvement in quality of life. PMID:24612761

  11. Eosinophilic Granulomatosis with Polyangiitis and Diffuse Gastrointestinal Involvement

    PubMed Central

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

    2014-01-01

    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

  12. Sarcoidosis presenting as severe renin-dependent hypertension due to kidney vascular injury

    PubMed Central

    Rafat, Cedric; Bobrie, Guillaume; Chedid, Antoine; Nochy, Dominique; Hernigou, Anne; Plouin, Pierre-François

    2014-01-01

    Renal sarcoidosis embraces a wide variety of clinical patterns. Renal vascular involvement has seldom been reported and usually in the setting of systemic vasculitis. We report the case of a 22-year-old patient in whom inaugural manifestation of renal sarcoidosis consisted of severe hypertension associated with bilateral perfusion defects and tumour-like nodules. In the setting of renal sarcoidosis, our case suggests that renin-dependant hypertension may arise from renal ischaemia as a result of extrinsic compression of kidney blood vessels due to severe granulomatous inflammation. PMID:25852913

  13. Multiple cerebral infarctions with severe multi-organ dysfunction following multiple wasp stings

    PubMed Central

    Wani, Mushtaq; Saleem, Sheikh; Verma, Sawan; Yousuf, Irfan; Wani, Maqbool; Asimi, Ravouf; Daga, Riyaz Ahmed; Shah, Irfan; Aejaz

    2014-01-01

    Wasp and bee sting are commonly encountered worldwide. Local reactions are more common, generally are self-limiting and settle within a few hours. Multiple stings can lead to various clinical manifestations like vomiting, diarrhea, dyspnea, generalized edema, hypotension, syncope, acute renal failure, and even death. Rarely, they can cause vasculitis, serum sickness, neuritis, and encephalitis. We are reporting a case of 40-year-old male who presented with stroke, right hemiparesis with severe multi-organ dysfunction due to multiple wasp stings. PMID:24753680

  14. [Spontaneous regression of sclerosing mesenteritis presenting as a huge mass].

    PubMed

    Lee, Hyun Jeong; Kim, Jin Il; Ahn, Ji Won; Kim, Jeong Ho; Cheung, Mo Eun; Park, Soo Heon; Kim, Jae Kwang; Im, Soyoung

    2012-04-01

    Sclerosing mesenteritis is a rare benign disease originated from the mesenteries. It can be related to autoimmune disease, vasculitis, ischemia, infection, trauma and operation, but most of cases are idiopathic. The overall prognosis of sclerosing mesenteritis is usually good with benign, course. However, no consensus of treatment has yet been established. We report a case of spontaneous partial regression of sclerosing mesenteritis presented as a huge mass and diagnosed by finding of contrast enhanced abdominal computed tomography and percutaneous ultrasonography guided needle biopsy. PMID:22544031

  15. Notes on the kidney and its diseases for the neurologist

    PubMed Central

    Zandi, Michael S; Coles, Alasdair J

    2007-01-01

    To save their patients from dialysis and transplantation, neurologists need simply remain alert to the possibility of renal failure, particularly in the context of systemic disease, diabetes, sepsis and drugs. Of the numerous territories shared by our respective specialities, we outline a pragmatic approach to the diagnosis and treatment of the vasculitides, underpinned by knowing which questions to ask, equally importantly when to ask them, and in the art of obtaining a tissue diagnosis. We consider the current evolving trial evidence that directs the usage of a growing arsenal of therapies in the induction and maintenance stages of vasculitis treatment, and extend this consideration to Lupus and Sjogren's. PMID:17435183

  16. Giant aortic arch aneurysm complicating Kawasaki's disease.

    PubMed

    Hakim, Kaouthar; Boussada, Rafik; Chaker, Lilia; Ouarda, Fatma

    2014-09-01

    Kawasaki disease (KD) is a common acute vasculitis in pediatric population that usually involves small- and middle-sized arteries, commonly coronary arteries. Although the incidence and natural course of coronary aneurysms after KD are well documented in studies, related reports on peripheral arterial and aortic aneurysms are scarce. We report the occurrence of a giant aortic aneurysm involving the horizontal part of aortic arch in a 28-month-old boy diagnosed with KD. This complication was managed by steroids therapy in the beginning. Because of mechanical complication and potential risk of rupture, surgery was undertaken. PMID:25298695

  17. Indications for biotherapy in systemic vasculitides

    Microsoft Academic Search

    Loïc Guillevin; Christian Pagnoux; Philippe Guilpain; Boris Bienvenu; Valérie Martinez; Luc Mouthon

    2007-01-01

    Biotherapy now holds a specific place in the therapeutic armamentarium for systemic vasculitides. Such therapy includes cytokines,\\u000a such as (pegylated)?-interferon for hepatitis B virus-related polyarteritis nodosa and hepatitis C virus-related cryoglobulinemic\\u000a vasculitis, and polyvalent immunoglobulin (IVIg), with well-defined indications and pending positive results. More specifically\\u000a targeted monoclonal antibodies include antitumor necrosis factor-? or anti-CD20 for antineutrophil cytoplasmic antibody-associated\\u000a vasculitides or

  18. Acute acalculous cholecystitis complicating systemic lupus erythematosus: case report and review.

    PubMed Central

    Swanepoel, C R; Floyd, A; Allison, H; Learmonth, G M; Cassidy, M J; Pascoe, M D

    1983-01-01

    A case of acalculous cholecystitis presented as an acute abdominal emergency in a 22 year old woman with severe systemic lupus erythematosus. At the time of presentation the patient was receiving high doses of prednisone and cyclophosphamide to control her underlying disease. Histological examination of the biopsy specimen from the gall bladder showed lupus vasculitis. This complication of systemic lupus erythematosus has not been reported before. Laboratory studies and changes in lupus activity may fail to predict the onset of cholecystitis. Images p252-a PMID:6402058

  19. Severe haemoptysis due to subclavian arteritis.

    PubMed

    Lioulias, A; Misthos, P; Kokotsakis, J; Drosos, O; Karagiannidis, N; Pavlopoulos, D; Mitselou, M

    2012-06-01

    Severe haemoptysis due to infective subclavian arteritis has, to our knowledge, never been documented. We report a case of subclavian arterial vasculitis that eroded into the left lung apex, causing a large intraparenchymal mycotic pseudoaneurysm. The patient presented with high fever and blood expectoration. An emergent left lateral thoracotomy was performed. The inflamed segment of the subclavian artery was resected and continuity was restored with a reversed saphenous vein graft. The postoperative course was uneventful and the patient was discharged on the 10th postoperative day. PMID:22732928

  20. Technetium scanning in Kaposi's sarcoma and its simulators.

    PubMed

    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. PMID:6281316

  1. Unfamiliar Manifestations of Anti-tubercular Therapy.

    PubMed

    Aggarwal, Ramesh; Dwivedi, Shridhar; Aggarwal, Meenakshi

    2014-01-01

    Cutaneous drug reactions are frequent in hospitalized patients and vary from simple manifestations like rash and erythema to severe life threatening conditions like angio-oedema, erythroderma, Stevens-Johnson syndrome and toxic epidermal necrolysis. However drug eruptions with antitubercular drugs are largely unknown except few case reports. We highlight here one similar case which presented with pleomorphic cutaneous manifestations after taking anti tubercular therapy and closely mimicked vasculitis. But when the offending drugs were stopped the lesions disappeared and the patient improved. PMID:24791242

  2. [Ocular manifestations of Churg-Strauss syndrome: review article and case report].

    PubMed

    Atili, A; Richter, C; Bahn, E; Rustenbeck, H H; Schittkowski, M

    2013-11-01

    This paper summarizes the possible ocular manifestations of Churg-Straus syndrome (CCS) from the literature and presents an unusual case report from routine clinical practice with conjunctival granuloma, orbital pseudotumor and choroidal folds. The CSS is an ANCA-associated granulomatous vasculitis which can be manifested in various organs and represents a life-threatening situation for the patient. Ocular manifestations are rare and can spread to all segments of the eye and orbit. The most frequent forms of ocular involvement described in the literature are retinal occlusion and orbital pseudotumor with various degrees of expression. PMID:23765370

  3. Indications for /sup 99m/technetium dimercapto-succinic acid scan in children

    SciTech Connect

    Gordon, I.

    1987-03-01

    The /sup 99m/technetium dimercapto-succinic acid scan provides an image of functional renal parenchyma. This static scan has specific indications and cannot be used simply in place of a /sup 99m/technetium diethylenetriaminepentaacetic acid scan. The major clinical indications for this investigation are the detection and/or evaluation of a renal scar, the small or absent kidney, an occult duplex system, certain renal masses, systemic hypertension or suspected vasculitis. The physiology of the /sup 99m/technetium dimercapto-succinic acid scan is reviewed briefly.

  4. Lessons learned from an animal model of Kawasaki disease.

    PubMed

    Yeung, R S M

    2007-01-01

    Kawasaki disease is the most common cause of multisystem vasculitis in childhood. Kawasaki disease has been reported throughout the world and affects children of all ethnicity. Coronary artery damage from Kawasaki disease is the leading cause of acquired heart disease in children in the developed world. Diagnostic tests and prognostic markers are lacking, and questions remain unanswered in our understanding of the etiopathogenesis of the disease, thus limiting our ability to improve therapy and coronary outcome. In this article I will review advances made in an animal model of disease, which has helped advance our understanding of the etiology and pathogenesis of this fascinating clinical syndrome. PMID:17428374

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

    PubMed

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

    2014-11-12

    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

  6. Systemic lupus erythematosus presenting with hyporeninemic hypoaldosteronism in a 10-year-old girl.

    PubMed

    Kozeny, G A; Hurley, R M; Fresco, R; Vertuno, L L; Bansal, V K; Hano, J E

    1986-01-01

    Hyperkalemia has been noted to occur spontaneously in patients with long-standing systemic lupus erythematosus who did not have advanced renal insufficiency. The patients previously described all had relatively normal renin-aldosterone systems, and the hyperkalemia was thus presumed to be secondary to a primary defect in renal tubular potassium secretion. We describe at 10-year-old girl with lupus nephritis, without significant renal insufficiency, who had hyperkalemia from hyporeninemic hypoaldosteronism postulated to be due to vasculitis involving the afferent/efferent arterioles and juxtaglomerular apparatus. PMID:3535507

  7. Amyloidosis in Behcet's disease.

    PubMed

    Ghorbel, I Ben; Feki, N Bel; Salem, T Ben; Lamloum, M; Houman, M H

    2015-01-01

    Behcet's disease (BD) is a multisystem vasculitis with protean manifestations. It is characterized by a heightened state of inflammation, although the factors that initiate and sustain this inflammation are not clear. We report some cases of BD-associated amyloidosis and have similar features. The patients developed nephrotic syndrome due to secondary amyloidosis, which was refractory to the immunosuppressive agents. Two patients expired and the third was lost to follow-up during the course. The BD complicated with amyloidosis is associated with high mortality despite the current aggressive therapy. PMID:25579733

  8. [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

  9. Cerebral complications in juvenile rheumatoid arthritis

    PubMed Central

    Jan, James E.; Hill, Robert H.; Low, Morton D.

    1972-01-01

    A study of 170 patients with juvenile rheumatoid arthritis and a review of the literature indicate that this disease can significantly affect the central nervous system. Signs of CNS dysfunction were observed in 13 children. During the acute toxic stages the EEG is abnormal in many cases. Other manifestations of toxic encephalopathy such as irritability, drowsiness, stupor, convulsions and marked meningismus may be evident in severe cases. Meningitis is often suspected but ruled out by the finding of normal CSF. Steroids can rapidly improve the condition of these children. If `unexplained' seizures occur during the chronic stage, the diagnosis of cerebral vasculitis should be entertained. PMID:4665094

  10. Severe Raynaud's Phenomenon in a Patient With Antinuclear Antibody-Negative Systemic Lupus Erythematosus

    PubMed Central

    Maxwell, Celia J.; Potter, Edger; Townsend, John

    1988-01-01

    There is a well-recognized subset of patients with clinical findings consistent with systemic lupus erythematosus (SLE) but with negative antinuclear antibodies (ANA). Most of these patients have significant cutaneous involvement with little central nervous system or renal pathology. The following case report describes such a patient whose presentation was suggestive of SLE but who was ANA negative despite repeated testing. Additionally, the patient was found to have severe Raynaud's phenomenon and cutaneous vasculitis. This case is noteworthy because of the prominence of vascular insufficiency as a presenting feature of ANA-negative SLE. PMID:3262169

  11. Atypical presentation of acute-onset endophthalmitis after uncomplicated pars plana vitrectomy.

    PubMed

    Reilly, Gayatri S; Garfinkel, Richard A; Melamud, Alexander

    2015-03-01

    A 70-year-old woman presented with signs of acute-onset endophthalmitis within 24 hours of surgery, with retinal hemorrhages and vascular changes in the absence of pain or significant inflammation. She had undergone uncomplicated 25-gauge pars plana vitrectomy for epiretinal membrane with presenting visual acuity of 20/40- and significant distortion. Vitreous culture revealed Staphylococcus epidermidis. Final visual acuity was no light perception with persistent pain, and the patient ultimately underwent enucleation. Although rarely, acute-onset postoperative endophthalmitis can present as retinal vasculitis with intraretinal hemorrhages and mild inflammation. [Ophthalmic Surg Lasers Imaging Retina. 2015;46:393-395.]. PMID:25856829

  12. A clinicopathological classification of granulomatous disorders

    PubMed Central

    James, D

    2000-01-01

    Granulomatous disorders comprise a large family sharing the histological denominator of granuloma formation. A granuloma is a focal compact collection of inflammatory cells, mononuclear cells predominating, usually as a result of the persistence of a non-degradable product and of active cell mediated hypersensitivity. There is a complex interplay between invading organism or prolonged antigenaemia, macrophage activity, a Th1 cell response, B cell overactivity and a vast array of biological mediators. Differential diagnosis and management demand a skilful interpretation of clinical findings and pathological evidence. They are classified into infections, vasculitis, immunological aberration, leucocyte oxidase deficiency, hypersensitivity, chemicals, and neoplasia.???Keywords: granuloma; Th1 cell; cytokines; neoplasia PMID:10908370

  13. Kawasaki Disease has so much to teach us!

    PubMed

    Orenstein, Jan M

    2014-04-01

    Kawasaki Disease (KD) is primarily a childhood vasculitis of mid-size muscular arteries, of which the coronary arteries (CA) are most clinically significant. Although timely treatment with pooled intravenous immunoglobulin (IVIG) has significantly reduced CA pathology, as determined by ECHO cardiology, about 30% of children still develop potentially fatal aneurysms, thrombi, or stenosis. This paper describes several additional pathologies and phenomena of undetermined significance, e.g. myocarditis, endocarditis with fibroelastosis, adrenal medullary necrosis, arterial smooth muscle cell transition into proliferating myofibroblasts, pneumonia in patients dying from KD, and calcification of organizing thrombi. PMID:24460693

  14. Systemic amyloidosis of beta 2-microglobulin type: a complication of long-term haemodialysis.

    PubMed Central

    Theaker, J M; Raine, A E; Rainey, A J; Heryet, A; Clark, A; Oliver, D O

    1987-01-01

    A patient receiving long-term haemodialysis developed systemic amyloidosis, which was shown immunohistochemically to be of beta 2-microglobulin type, a previously unrecognised form of systemic amyloidosis. Histologically, the amyloid deposits were closely associated with foci of acute and granulomatous inflammation and vasculitis, although it was not clear if the amyloid deposits directly caused the inflammatory process, or if amyloid was deposited preferentially in areas of inflammation of uncertain aetiology. Images Fig 1 Fig 2 Fig 3 Fig 4 Fig 5 PMID:3119676

  15. Chronic inflammatory lesions of the placenta.

    PubMed

    Katzman, Philip J

    2015-02-01

    The chronic inflammatory lesions of the placenta often run in the shadows of the better-known acute inflammatory processes of the placenta, such as acute chorioamnionitis and acute funisitis. A heterogeneous population of T-cell lymphocytes, plasma cells, and macrophages is the primary player in chronic villitis, chronic chorioamnionitis, chronic deciduitis, and chronic intervillositis, and eosinophils are an added component of eosinophilic/T-cell chorionic vasculitis. The histologic appearance, sites of occurrence in the placenta, and pathogeneses of these entities are reviewed. PMID:25455621

  16. Generalized vasculitic exanthem following Loxosceles reclusa envenomation.

    PubMed

    Robb, Christopher W; Hayes, Benjamin B; Boyd, Alan S

    2007-06-01

    Brown recluse spider bites (BRSB) cause a myriad of reactions ranging from local necrosis to potentially lethal systemic involvement. Envenomation may induce a generalized exanthem known clinically but not described histologically. We report a 49-year-old female who developed a generalized exanthem 24 hours after BRSB. The histopathology demonstrated a necrotizing vasculitis similar to that seen at an envenomation site but without epidermal necrosis. Loxoscelism should be considered in patients residing in endemic areas who present with a vasculitic exanthem. PMID:17518783

  17. Familial Behçet's disease.

    PubMed

    Yilmaz, Sema; Cimen, Kadriye Akar

    2010-06-01

    Behçet's disease (BD) is a multisystemic vasculitis syndrome characterized by a course of remissions and exacerbations of unpredictable frequency and duration. The disease has a worldwide distribution, but the majority of cases cluster along the ancient Silk Road, which extends from eastern Asia to the Mediterranean basin. The etiopathogenesis of BD is still unknown, but familial aggregation and peculiar geographical distribution have been regarded as evidence supporting genetic influence on the pathogenesis of BD. In this article, we describe a patient with BD, who had four members of his family associated with BD. PMID:19575202

  18. [Neuro-Behçet in a Caucasian Danish patient].

    PubMed

    Karshena, Ali; Koch-Hendriksen, Nils J

    2010-02-22

    Behçet's disease (BD) is a chronic, relapsing multisystem inflammatory disorder, vasculitis being its major pathological feature. It is prevalent in the Orient and areas along the ancient Silk Road, but very rare among North European Caucasians. Neurological involvement is relatively uncommon. The diagnosis of neuro-Behçet can be difficult to establish if the mucocutaneous involvement is incomplete, absent, or unattended. We present an exceptional case of neuro-Behçet in a 38-year-old Caucasian Danish woman. On account of the risk of serious complication, it is important to make the diagnosis as soon as possible. PMID:20184824

  19. Tuberculosis infection causing intestinal perforations in 2 patients with systemic lupus erythematosus.

    PubMed

    González, Luis A; Muñoz, Carolina; Restrepo, Mauricio; Vanegas, Adriana Lucía; Vásquez, Gloria

    2014-08-01

    Patients with systemic lupus erythematosus (SLE) have a higher incidence rate of tuberculosis and a more frequent extrapulmonary involvement than the general population. We present 2 SLE patients who developed gastrointestinal tuberculosis complicated with intestinal perforation, a rare but serious complication that could be confused with lupus-associated intestinal vasculitis. Opportunistic infections such as tuberculosis must be suspected in SLE patients with abdominal symptoms on immunosuppressive therapy because its early recognition could prevent catastrophic complications such as intestinal perforation and subsequent peritonitis. PMID:25036568

  20. Gastrointestinal manifestations of Henoch-Schonlein purpura: A report of two cases

    PubMed Central

    Prathiba Rajalakshmi, Parameswaran; Srinivasan, Kalyanasundaram

    2015-01-01

    Henoch-Schonlein purpura (HSP) is a small vessel vasculitis mediated by type III hypersensitivity with deposition of IgA immune complex in the walls of vessels. It is a multi-system disorder characterized by palpable purpura, arthritis, glomerulonephritis and gastrointestinal manifestations and commonly occurs in children and young adults. The patients with gastrointestinal involvement usually present with colicky abdominal pain, vomiting and melena. The imaging findings include multifocal bowel thickening with mucosal hyperenhancement, presence of skip areas, mesenteric vascular engorgement, with involvement of unusual sites like stomach, duodenum and rectum. These imaging findings in a child or young adult with appropriate clinical findings could suggest HSP.