Sample records for vasculitis

  1. Vasculitis

    MedlinePLUS

    ... patients take lower doses of glucocorticoids. What is vasculitis? Vasculitis refers to inflammation of the blood vessels. ... Most types of vasculitis are rare. What causes vasculitis? We do not know what causes most types ...

  2. Vasculitis

    MedlinePLUS

    Vasculitis is an inflammation of the blood vessels. It happens when the body's immune system attacks the ... or another disease. The cause is often unknown. Vasculitis can affect arteries, veins and capillaries. Arteries are ...

  3. Vasculitis

    PubMed Central

    Ongley, Roberta C.

    1987-01-01

    The vasculitides are a large and diverse group of disorders, often confusing for the clinician in terms of diagnosis, investigation, and treatment. As most patients will have cutaneous lesions, it is useful for the physician to be aware of the dermatologic manifestations that may help in differentiating these diseases. This review covers both the common and not so common form of vasculitis, including both cutaneous and systemic disorders, an approach to investigation, and brief discussion of therapeutic approaches. PMID:21263957

  4. Urticarial Vasculitis

    MedlinePLUS

    Urticarial Vasculitis joseph July 18, 2012 No Comments Urticarial vasculitis is a form of vasculitis that affects the skin, ... complements; it’s called hypocomplementemic vasculitis. What causes Urticarial Vasculitis? The cause of most cases of urticarial vasculitis ...

  5. Allergic vasculitis

    MedlinePLUS

    Vasculitis - allergic; Hypersensitivity vasculitis; Cutaneous leukocytoclastic vasculitis ... Allergic vasculitis is caused by an allergic reaction to a drug, an infection, or other foreign substance. It most ...

  6. Hypersensitivity Vasculitis

    MedlinePLUS

    Hypersensitivity Vasculitis joseph July 18, 2012 No Comments What is Hypersensitivity vasculitis? Hypersensitivity vasculitis (HV) is often used to describe ... vessels, called a leukocytoclastic vasculitis. What causes Hypersensitivity vasculitis? HV may be caused by a specific drug ...

  7. Necrotizing vasculitis

    MedlinePLUS

    Vasculitis - necrotizing ... Necrotizing vasculitis is common with: Polyarteritis nodosa Rheumatoid arthritis Scleroderma Systemic lupus erythematosus Granulomatosis with polyangiitis (formerly called Wegener's ...

  8. Granulomatous Vasculitis.

    PubMed

    Sharma, Aman; Dogra, Sunil; Sharma, Kusum

    2015-07-01

    Vasculitides are uncommon disorders, characterized by inflammation of the blood vessels resulting either in ischemia or hemorrhage. They are commonly classified as small-, medium-, or large-vessel vasculitides. Antineutrophil cytoplasmic antibody-associated vasculitis is an important group of small-vessel vasculitis. This group includes granulomatous vasculitides, namely, granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and nongranulomatous vasculitis, namely, microscopic polyangiitis (MPA). Classic polyarteritis nodosa (PAN) is a granulomatous medium-vessel vasculitis. This review discusses the classification, etiopathogenesis, clinical features, and management of GPA, MPA, EGPA and PAN. PMID:26143427

  9. Pediatric Vasculitis

    PubMed Central

    Weiss, Pamela F.

    2012-01-01

    Synopsis Childhood vasculitis is a challenging and complex group of conditions that are multisystem in nature and often require integrated care from multiple subspecialties including rheumatology, dermatology, cardiology, nephrology, neurology, and gastroenterology. Vasculitis is defined as the presence of inflammation in the blood vessel wall. The site of vessel involvement, size of the affected vessels, extent of vascular injury, and underlying pathology determine the disease phenotype and severity. This review explores the classification and general features of pediatric vasculitis as well as the clinical presentation, diagnostic evaluation, and therapeutic options for the most common vasculitides. PMID:22560577

  10. Pulmonary vasculitis.

    PubMed

    Lally, Lindsay; Spiera, Robert F

    2015-05-01

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

  11. 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

  12. How Is Vasculitis Diagnosed?

    MedlinePLUS

    ... page from the NHLBI on Twitter. How Is Vasculitis Diagnosed? Your doctor will diagnose vasculitis based on your signs and symptoms, your medical ... results. Specialists Involved Depending on the type of vasculitis you have and the organs affected, your doctor ...

  13. Impact of Vasculitis on Employment and Income

    ClinicalTrials.gov

    2015-06-18

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

  14. Central Nervous System Vasculitis

    MedlinePLUS

    ... eyesight seizures encephalopathy (swelling and damage to the brain) sensation abnormalities Diagnosis The diagnosis of vasculitis, including CNS vasculitis, is based on a person’s medical history, symptoms, a complete ...

  15. Granulomatous vasculitis.

    PubMed

    Marzano, A V; Balice, Y; Tavecchio, S; Desimine, C; Colombo, A; Berti, E

    2015-04-01

    Granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Wegener's granulomatosis and Churg-Strauss Syndrome respectively, are systemic granulomatous vasculitides affecting small- and medium-sized blood vessels. Both GPA and EGPA are included within the group of antineutrophilic cytoplasmic antibodies (ANCA)-associated vasculitides, on the basis of the detection of such autoantibodies in a significant proportion of affected patients. Two main settings of GPA, possibly overlapping each other, are recognized: a localized form, which is limited to the upper airways but is highly relapsing and refractory, and a diffuse form, which is initially more severe but then less commonly recurrent. In EGPA, a prodromic phase characterized by asthma and rhino-sinusitis is followed by an eosinophilic phase, marked by peripheral eosinophilia, and then by a vasculitic phase, in which skin lesions are a prominent feature together with peripheral neuropathy and renal involvement. Polymorphic cutaneous manifestations can occur during the course of both GPA and EGPA, and include palpable purpura, livedo reticularis, papules, nodules, vesiculo-bullae and necrotic-ulcerative lesions most commonly involving the lower extremities; pyoderma gangrenosum-like ulcers and lesions resembling erythema multiforme have been described in GPA and EGPA, respectively. Oral involvement is not uncommon in GPA and may manifest as nonspecific erosive lesions or as a hyperplastic gingivitis named strawberry gingivitis. Considering that skin involvement is common in ANCA-associated vasculitides and may also be their presenting sign, the role of dermatologist is crucial in the early diagnosis of these forms as well as of vasculitis in general. PMID:25791629

  16. 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 ...

  17. Olanzapine-induced vasculitis

    Microsoft Academic Search

    Mahesh K. Duggal; Amritpal Singh; Arunabh; James D. Lolis; Howard J. Guzik

    2005-01-01

    Introduction:Elderly patients are particularly vulnerable to adverse drug reactions as a result of polypharmacy and metabolic changes associated with aging. We present a case of leukocytoclastic vasculitis induced by olanzapine, a medication commonly used in elderly patients.

  18. Patients with Retinal Vasculitis Rarely Suffer from Systemic Vasculitis

    PubMed Central

    Rosenbaum, James T.; Ku, Jennifer; Ali, Amro; Choi, Dongseok; Suhler, Eric B.

    2014-01-01

    Objectives Systemic vasculitis is often mistakenly assumed to be a common cause of retinal vasculitis. We sought to determine the relationship between retinal vasculitis and systemic vasculitis. Methods A selected review was performed on 1390 charts of patients attending the uveitis clinic at the Oregon Health & Science University between 1985 and 2010. Included in the review were all patients with diagnoses commonly associated with retinal vasculitis and all patients who were diagnosed with a systemic vasculitis. Retinal vasculitis was identified by perivascular exudates, intraretinal hemorrhage, or cotton wool spots as seen on clinical examination or by vascular occlusion or leakage as identified by fluorescein angiogram. Results 207 or 14.9% of patients with uveitis had retinal vasculitis as a component of the intraocular inflammation. Thirty-five patients had retinal vasculitis which was primary, i.e. not associated with a systemic disease, and the dominant manifestation of the uveitis. Fourteen of the patients with retinal vasculitis had Behcet’s disease. Only 11 of the 1390 patients with uveitis had a systemic vasculitis. Of these 11, four had retinal vasculitis including one secondary to a CMV retinitis. Thus, systemic vasculitis was directly responsible for 1.4% or 3 of 207 cases of retinal vasculitis. No-vasculitic systemic diseases such as sarcoidosis (n=13), syndromes confined to the eye such as pars planitis (n=36), and intraocular infections (n=29) were far more common causes of retinal vasculitis. Conclusion Retinal vasculitis is a relatively common feature of uveitis. Patients with retinal vasculitis, however, rarely suffer from one of the classical systemic vasculitides. PMID:22177107

  19. Small vessel vasculitis

    PubMed Central

    Eleftheriou, Despina; Dillon, Michael

    2009-01-01

    The pediatric small vessel vasculitides reviewed in this article are Henoch–Schönlein purpura (HSP) and the anti-neutrophil cytoplasmic antibody-associated vasculitides (AAV). The new classification criteria for HSP and Wegener’s granulomatosis are now validated and will facilitate the conduct of future epidemiological studies and clinical trials. The clinical manifestations of small vessel vasculitis in children are described, and current therapies discussed. There is a lack of good clinical trial data on which to base therapy for HSP. Similarly, data based on randomized controlled trials (RCTs) for pediatric AAV are lacking, although children with AAV are for the first time now included in a RCT of mycophenolate mofetil versus cyclophosphamide. Significant challenges remain in the field of pediatric small vessel vasculitis, including the development of validated disease outcome measures and biomarkers to be used in clinical trials. Lastly, long-term outcome data are lacking in survivors of pediatric small vessel vasculitis. PMID:19885685

  20. Renal disease and vasculitis

    Microsoft Academic Search

    J. Stewart Cameron

    1988-01-01

    Vasculitis is rare in childhood, apart from Henoch-Schönlein purpura, and paediatric nephrologists see few cases of Wegener's granuloma or microscopic polyarteritis. In contrast, this pattern is reversed in adults. Recently much information about the presentation and longer-term course of both these conditions has been described in adults, and more aggressive treatment of severe cases with renal failure has improved the

  1. Living with Vasculitis

    MedlinePLUS

    ... your quality of life. Joining a patient support group may help you adjust to living with vasculitis. You can ... them. Talk with your doctor about local support groups or check with an area medical center. Support from family and friends also can help relieve stress and anxiety. Let your loved ones ...

  2. Cutaneous vasculitis: diagnosis and management.

    PubMed

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

    2006-01-01

    Vasculitis is histologically defined as inflammatory cell infiltration and destruction of blood vessels. Vasculitis is classified as primary (idiopathic, eg, cutaneous leukocytoclastic angiitis, Wegener's granulomatosis) or secondary, a manifestation of connective tissue diseases, infections, adverse drug eruptions, or a paraneoplastic phenomenon. Cutaneous vasculitis, manifested as urticaria, purpura, hemorrhagic vesicles, ulcers, nodules, livedo, infarcts, or digital gangrene, is a frequent and often significant component of many systemic vasculitic syndromes such as lupus or rheumatoid vasculitis and antineutrophil cytoplasmic antibody-associated primary vasculitic syndromes such as Churg-Strauss syndrome. In most instances, cutaneous vasculitis represents a self-limited, single-episode phenomenon, the treatment of which consists of general measures such as leg elevation, warming, avoidance of standing, cold temperatures and tight fitting clothing, and therapy with antihistamines, aspirin, or nonsteroidal anti-inflammatory drugs. More extensive therapy is indicated for symptomatic, recurrent, extensive, and persistent skin disease or coexistence of systemic disease. For mild recurrent or persistent disease, colchicine and dapsone are first-choice agents. Severe cutaneous and systemic disease requires more potent immunosuppression (prednisone plus azathioprine, methotrexate, cyclophosphamide, cyclosporine, or mycophenolate mofetil). In cases of refractory vasculitis, plasmapheresis and intravenous immunoglobulin are viable considerations. The new biologic therapies that work via cytokine blockade or lymphocyte depletion such as tumor alpha inhibitor infliximab and the anti-B-cell antibody rituximab, respectively, are showing benefit in certain settings such as Wegener's granulomatosis, antineutrophil cytoplasmic antibody-associated vasculitis, Behçet's disease, and cryoglobulinemic vasculitis. PMID:16966021

  3. Cutaneous vasculitis complicating coeliac disease

    Microsoft Academic Search

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

    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

  4. Tubercular retinal vasculitis

    PubMed Central

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

    2013-01-01

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

  5. [Vasculitis and vasculopathy].

    PubMed

    Markovi?, Asjastipi?

    2012-10-01

    Many pathophysiological process components are known to be implicated in lower limb ulcerations, among which vascular lesions have a major role. Vasculitis denotes a heterogeneous group of clinical entities which all are characterized by the inflammatory process of arterial and venous walls of any size and in any organ, quite frequently in the skin. Vasculopathy, on the other hand, refers to vascular and capillary lesions caused by, for example, some medications. The classification of vasculitides according to the size of the blood vessels involved serves for proper understanding the issue among clinicians and researchers, and not as a diagnostic tool. According to histologic finding obtained by examination of blood vessel biopsy specimen, vasculitides are divided into three groups: lymphocytic, leukocytoclastic and granulomatous. Livedoid vasculitis (livedo reticularis) most commonly affects women and is generally localized on lower extremities. The etiology oflivedoid vasculitis may imply autoimmune diseases, capillary obstruction with cryoglobulins, or antiphospholipid syndrome. Livedoid vasculopathy is a hyalinization disease of the vasculature, with thromboses and ulcerations on lower extremities, and of unknown etiology. Livedoid vasculopathy has been singled out as a separate disease that usually does not occur consequentially to other primary diseases. Livedoid vasculopathy typically affects women (71%) at a mean age of 45 (range 10-85) years; bilateral involvement of both lower limbs is present in 80.8%, disease manifested with ulcerations in 68.9%, ulcerations followed by development of atrophie blanche in 71.1%, transcutaneous oximetry reduction is found in 74.1%, factor V mutation (Leiden heterozygotes) in 22.2%, reduced protein C activity in 13.3%, prothrombin gene mutation (G20210A) in 8.3%, positive lupus anticoagulant in 17.9%, positive anticardiolipin antibodies in 28.6%, and elevated homocysteine level in 14.3% cases; blood vessel histology shows intraluminal thrombosis in 97.8% of patients, while direct immunofluorescence of blood vessel specimen shows immunoglobulins and complement components in blood vessels on the surface, in the mid-dermis as well as deep in the dermis. The immunofluorescence pattern differs from that found in immune complex diseases. Some of the agents tried in the treatment of livedoid vasculopathy include pentoxifylline, low-molecular heparin, hyperbaric oxygen therapy, methylprednisolone i.v. with pentoxifylline, recombinant tissue plasminogen activator, intravenous immunoglobulins, phenformin (biguanide) and ethylestrenol (anabolic steroid) combination, warfarin, heparin, systemic photochemotherapy (PUVA with oral psoralen), and low-molecular dextran. Infected ulcerations are treated with antibiotics. Combined therapy with folic acid, vitamin B12 and vitamin B6 can also be used. PMID:23193816

  6. Ultrasound in vasculitis.

    PubMed

    Schmidt, Wolfgang A

    2014-01-01

    Colour Doppler ultrasound displays a pathognomonic circumferential wall thickening in large-vessel vasculitis. Even rather small arteries like the temporal arteries can be easily examined with modern ultrasound equipment. In addition, ultrasound can detect stenoses and acute arterial occlusions. In large-vessel giant cell arteritis, the axillary arteries are most commonly involved. Takayasu arteritis affects particularly the left subclavian and the left common carotid arteries. As ultrasound diagnosis at the temporal arteries becomes more difficult already after a few days of glucocorticoid treatment in some patients, institutions are implementing fast-track clinics for which patients receive an appointment within 24 hours. An experienced rheumatologist is able to establish a definite diagnosis in most cases with standardised history, clinical examination and ultrasound of temporal and axillary arteries. Furthermore, early diagnosis and treatment may prevent blindness. PMID:24529335

  7. Vasculitis of the nervous system

    Microsoft Academic Search

    Aksel Siva

    2001-01-01

    Vasculitis is inflammation of the blood vessels, which may involve either the central nervous system (CNS), or the peripheral\\u000a nervous system (PNS), or both. This involvement may be primary and restricted to the CNS, and rarely to the PNS. Vasculitis\\u000a is inflammation of the blood vessels, which may involve either the central nervous system (CNS), or the peripheral nervous\\u000a system

  8. Hypersensitivity vasculitis induced by cefoperazone/sulbactam

    PubMed Central

    Islek, Ismail; Baris, Sancar; Katranci, Ali O; Ariturk, Ender; Gurses, Nuran

    2003-01-01

    Background Cefoperazone has not been reported to cause vasculitic complications before. Here, we report a case of hypersensitivity vasculitis associated with cefoperazone/sulbactam. Case presentation A 13-year-old girl with appendicitis developed hypersensitivity vasculitis on the fifth day of cefoperazone/sulbactam therapy. Hypersensitivity vasculitis resolved gradually after removal of the agent on the seventh day and did not recur. Although hypersensitivity vasculitis has multiple causes, coexistence of hypersensitivity vasculitis and cefoperazone treatment, and the quite resolution of the disease after removal of the drug, strongly favours a causative relationship. Conclusion To our knowledge, this is the first report of a hypersensitivity vasculitis associated with cefoperazone. PMID:12556245

  9. Developments in large and midsize vasculitis.

    PubMed

    Terreri, Maria Teresa; Clemente, Gleice

    2013-11-01

    Childhood vasculitis is a complex and fascinating area in pediatric rheumatology that has experienced an unprecedented surge in research, leading to new knowledge over the past several years. Vasculitis is defined as the presence of inflammatory cell infiltration in blood vessel walls, usually with multisystemic involvement. The most frequent forms of vasculitis in childhood are the small-size vasculitides, of which Henoch-Schoenlein Purpura and other leucocytoclastic vasculitis are the best examples, followed by Kawasaki disease, a midsize vasculitis, and Takayasu arteritis, a large-size vasculitis, both of which are topics in this article. PMID:24182858

  10. Cerebral vasculitis associated with cocaine abuse

    Microsoft Academic Search

    B. R. Kaye; M. Fainstat

    1987-01-01

    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.

  11. Disseminated histoplasmosis and necrotizing vasculitis.

    PubMed Central

    Small, P.; Levitt, P.

    1976-01-01

    A 74-year-old man with congestive heart failure was found to have Histoplasma capsulatum in a lesion of the right nasal septum. His initial treatment with amphotericin B was inadequate because of severe intolerance to the drug. Three months after initial presentation H. capsulatum was detected in his blood and bone marrow. Slightly elevated purpuric lesions on the arms, lower legs and trunk showed the typical features of necrotizing vasculitis. Cutaneous anergy was reversed after treatment with transfer factor. Skin involvement in disseminated histoplasmosis is unusual and there are no previous reports of vasculitis associated with this infection. Images FIG. 1 FIG. 2 PMID:1253047

  12. Medium-size-vessel vasculitis

    PubMed Central

    Eleftheriou, Despina; Brogan, Paul A.

    2009-01-01

    Medium-size-artery vasculitides do occur in childhood and manifest, in the main, as polyarteritis nodosa (PAN), cutaneous PAN and Kawasaki disease. Of these, PAN is the most serious, with high morbidity and not inconsequential mortality rates. New classification criteria for PAN have been validated that will have value in epidemiological studies and clinical trials. Renal involvement is common and recent therapeutic advances may result in improved treatment options. Cutaneous PAN is a milder disease characterised by periodic exacerbations and often associated with streptococcal infection. There is controversy as to whether this is a separate entity or part of the systemic PAN spectrum. Kawasaki disease is an acute self-limiting systemic vasculitis, the second commonest vasculitis in childhood and the commonest cause of childhood-acquired heart disease. Renal manifestations occur and include tubulointerstitial nephritis and renal failure. An infectious trigger and a genetic predisposition seem likely. Intravenous immunoglobulin (IV-Ig) and aspirin are effective therapeutically, but in resistant cases, either steroid or infliximab have a role. Greater understanding of the pathogenetic mechanisms involved in these three types of vasculitis and better long-term follow-up data will lead to improved therapy and prediction of prognosis. PMID:19946711

  13. 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

  14. Vasculitis associated with connective tissue disorders

    Microsoft Academic Search

    Mittie K. Doyle

    2006-01-01

    Vasculitis associated with connective tissue disorders is an important cause of secondary vasculitis about which little is\\u000a written. When vasculitis occurs in the setting of a preexisting connective tissue disorder, it often correlates with disease\\u000a severity and portends a poorer prognosis. It may involve virtually any organ system and present in a myriad of ways. Prompt\\u000a recognition and treatment of

  15. 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 ...

  16. Vasculitis Syndromes of the Central and Peripheral Nervous Systems

    MedlinePLUS

    Vasculitis Syndromes of the Central and Peripheral Nervous Systems Fact Sheet See a list of all NINDS ... Where can I get more information? What is vasculitis? Vasculitis is an inflammation of blood vessels, which ...

  17. Ischemic Neuropathy Associated with Livedoid Vasculitis

    PubMed Central

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

    2011-01-01

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

  18. 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

  19. Challenging mimickers of primary systemic vasculitis.

    PubMed

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

    2015-01-01

    The need to distinguish true primary systemic vasculitis from its multiple potential mimickers is one of the most challenging diagnostic conundrums in clinical medicine. This article reviews 9 challenging vasculitis mimickers: fibromuscular dysplasia, calciphylaxis, segmental arterial mediolysis, antiphospholipid syndrome, hypereosinophilic syndrome, lymphomatoid granulomatosis, malignant atrophic papulosis, livedoid vasculopathy, and immunoglobulin G4-related disease. PMID:25399945

  20. Fulminant Vasculitis: A Rare Fatal Complication of Lymphoma

    Microsoft Academic Search

    Eyal Leshem; Yaron Davidovitz; Eyal Meltzer; Paul Fefer; Efrat Ofek; Yechezkel Sidi

    2006-01-01

    Vasculitis is rarely associated with lymphoma; however, most cases associated with lymphoma are cutaneous. Systemic vasculitis in association with lymphoma is usually an indolent and non-fatal complication. Two patients presented to our department with fulminant vasculitis with a fatal course and were later diagnosed with lymphoma. A search of the literature for systemic fulminant vasculitis in association with lymphoma disclosed

  1. Skin involvement in cutaneous and systemic vasculitis.

    PubMed

    Marzano, Angelo V; Vezzoli, Pamela; Berti, Emilio

    2013-02-01

    Cutaneous vasculitides are a heterogeneous group of inflammatory disorders affecting skin blood vessels. They may be triggered by several factors, such as infection or drug, or may be related to underlying disease, notably connective tissue or malignancies. However, vasculitis occurs without any demonstrable triggering agents in a relevant number of patients. On the other hand, vasculitic skin lesions may manifest as a component of vasculitis affecting also internal organs; in someone of these patients, skin involvement occurs initially as the sole sign of disease, leading to consider cutaneous vasculitis a diagnosis of exclusion. In this review, we have focused on the most common variants of cutaneous vasculitis, including cutaneous small vessel vasculitis and urticarial vasculitis as well as Henoch-Schönlein purpura, a systemic form in which however skin involvement often predominates. We have also argued on livedoid vasculopathy, a cutaneous entity which, although nonfrankly vasculitic in origin, is frequently associated with connective tissue disease. Finally, we have analyzed the variety of cutaneous manifestations that may develop during the course of the main systemic vasculitides, such as Wegener's granulomatosis, Churg-Strauss syndrome and polyarteritis nodosa. PMID:22959234

  2. Biological drugs in ANCA-associated vasculitis.

    PubMed

    Lutalo, Pamela M K; D'Cruz, David P

    2015-08-01

    The anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitides granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA) and microscopic polyangiitis (MPA) constitute a wide spectrum of clinical manifestations of systemic vasculitis which may range from limited disease to organ or life-threatening disease. The introduction of biologics for the management of severe ANCA-associated vasculitis and severe, relapsing disease refractory to conventional immunosuppressants, has significantly improved the clinical prognosis of these autoimmune disorders. Rituximab, an anti-CD20 monoclonal antibody is licenced for remission induction in severe GPA and MPA and the management of severe relapsing, refractory GPA and MPA. Belimumab, an anti-B lymphocyte stimulatory monoclonal antibody is in clinical trials for the management of the ANCA-associated vasculitis GPA. Mepolizumab and Omalizumab are biologics which have been reported to be efficacious in refractory asthma associated with EGPA. The role of anti-TNF therapy and T cell targeting drugs in ANCA-associated vasculitis is less clear due to limited study data. This review will summarise the clinical trials and clinical practice use of biologic treatment strategies for the management of ANCA-associated vasculitis. PMID:25907243

  3. 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

  4. Antineutrophil Cytoplasmic Antibodies, Autoimmune Neutropenia, and Vasculitis

    PubMed Central

    Grayson, Peter C.; Sloan, J. Mark; Niles, John L.; Monach, Paul A.; Merkel, Peter A.

    2011-01-01

    Objectives Reports of an association between antineutrophil cytoplasmic antibodies (ANCA) and autoimmune neutropenia have rarely included cases of proven vasculitis. A case of ANCA-associated vasculitis (AAV) with recurrent neutropenia is described and relevant literature on the association between ANCA, neutropenia, and vasculitis is reviewed. Methods Longitudinal clinical assessments and laboratory findings are described in a patient with AAV and recurrent episodes of profound neutropenia from December 2008 – October 2010. A PubMed database search of the medical literature was performed for papers published from 1960 through October 2010 to identify all reported cases of ANCA and neutropenia. Results A 49 year-old man developed recurrent neutropenia, periodic fevers, arthritis, biopsy-proven cutaneous vasculitis, sensorineural hearing loss, epididymitis, and positive tests for ANCA with specificity for antibodies to both proteinase 3 and myeloperoxidase. Antineutrophil membrane antibodies were detected during an acute neutropenic phase and were not detectable in a post-recovery sample, whereas ANCA titers did not seem to correlate with neutropenia. An association between ANCA and neutropenia has been reported in 74 cases from 24 studies in the context of drug/toxin exposure, underlying autoimmune disease, or chronic neutropenia without underlying autoimmune disease. In these cases, the presence of atypical ANCA patterns and other antibodies were common; however, vasculitis was uncommon and when it occurred was usually limited to the skin and in cases of underlying toxin exposure. Conclusions ANCA is associated with autoimmune neutropenia, but systemic vasculitis rarely occurs in association with ANCA and neutropenia. The interaction between neutrophils and ANCA may provide insight into understanding both autoimmune neutropenia and AAV. PMID:21507463

  5. Plasmapheresis in a Patient With "Refractory" Urticarial Vasculitis

    PubMed Central

    Gulec, Mustafa; Caliskaner, Zafer; Nevruz, Oral; Cetin, Turker; Sener, Osman

    2012-01-01

    Immune complexes are found in the circulation of 30%-75% of patients with urticarial vasculitis and much evidence supports the role of these immune complexes in the pathogenesis of urticarial vasculitis. Plasmapheresis is effective for removing these immune complexes; however, there are few reports on the use of plasmapheresis in the treatment of urticarial vasculitis. We describe a case of "refractory" urticarial vasculitis in which the symptoms improved after plasmapheresis treatment. We suggest that plasmapheresis be considered as an option in patients with severe or treatment-resistant urticarial vasculitis. PMID:22754719

  6. MICROANGIOPATHIC HAEMOLYTIC ANAEMIA AND SYSTEMIC VASCULITIS

    Microsoft Academic Search

    C. N. ROSS; H. REUTER; D. SCOTT; D. V. HAMILTON

    1996-01-01

    SUMMARY Two cases of systemic vasculitis complicated by microangiopathic haemolytic anaemia (MAHA) are described: this association has not previously been reported. Both patients had atypical presentations of their primary disease, one with parotitis and one with a Guillain-Barri syndrome. Other causes of MAHA were excluded and a possible link with macromolecular von Willebrands factor is speculated upon. HAEMATOLOGICAL abnormalities in

  7. Cerebral vasculitis associated with Schistosoma mansoni infection

    PubMed Central

    2012-01-01

    Background Cerebral involvement in schistosomiasis is not rare, but it is underdiagnosed because of the lack of clinical suspicion and the frequency of asymptomatic forms. Neurologic complications are generally supported by granuloma formation around ectopic eggs which have migrated to the brain. Moreover, vascular lesions and cerebral arteritis have been well documented in histopathological studies. Nevertheless, cerebral vasculitis in later stages of the Schistosoma mansoni infection have not yet been described in living subjects. Case presentation A 28-year-old french woman had a stroke linked with cerebral vasculitis, 6 monthes after returning from Burkina-Faso. At the same time, a S. mansoni disseminated infection was diagnosed. She suffered from a new stroke after undertaking praziquantel therapy, which lead us to associate the S. mansoni infection and cerebral vasculitis. Conclusion This is the first report of such association, since cerebral vasculitis has never been described in later stages of the S. mansoni infection. Although the causal link between the two pathologies could not be proved, we suggest that S. mansoni is able to cause severe vascular damage in cerebral vessels. Schistosomiasis must be investigated in the event of a brain infarct in young people, particularly in patients originating or returning from an endemic area. PMID:22978371

  8. Leucocytoclastic vasculitis in severe ulcerative colitis.

    PubMed

    Martin, Donald; Handler, Tristan; McDermott, Joseph

    2011-05-01

    Ulcerative colitis may be associated with a number of extraintestinal skin manifestations including erythema nodosum and pyoderma gangrenosum. We describe an unusual case of a 26-year-old military pilot with ulcerative colitis and skin lesions diagnosed as leucocytoclastic vasculitis. The skin lesions occurred twice during the severe flare. The first occurrence was treated successfully with corticosteroids. When the lesions recurred several weeks later, concomitant with the ongoing flare, treatment with mesalamine and infliximab resolved the lesions. Ultimately, the patient required total colectomy for flare unresponsive to maximum medical therapy. The leucocytoclastic vasculitis did not recur after colectomy. We propose a potential immunopathophysiologic mechanism linking the 2 conditions based on recent biochemical and clinical research. PMID:21634306

  9. CHILDHOOD CUTANEOUS VASCULITIS: A COMPREHENSIVE APPRAISAL

    PubMed Central

    Palit, Aparna; Inamadar, Arun C

    2009-01-01

    Cutaneous vasculitides in childhood are rare and often present with clinical features distinct from adults. Diagnosis of cutaneous vasculitides in children was difficult because of lack of a satisfactory classification systems for this age group. A new international classification system for childhood vasculitis has been discussed in the following section along with important clinical features, diagnostic modalities, and recent therapeutic developments of important vasculitides in children. PMID:20101304

  10. [Myocardial infarction in a young patient--with underlying vasculitis].

    PubMed

    Romppanen, Hannu; Niemelä, Matti; Kervinen, Kari; Ylitalo, Kari; Huikuri, Heikki

    2009-01-01

    Myocardial infarction in a young patient is rare, and its diagnosis and treatment may be delayed. Vasculitis can cause inflammation in the coronary artery and cause a myocardial infarction even in a young person. We describe a sudden myocardial infarction caused by vasculitis in two young adults having chest pain symptoms. PMID:19860082

  11. Treatment of Recalcitrant Idiopathic Leukocytoclastis Vasculitis with PUVA

    Microsoft Academic Search

    Surangkana Nawapuntu; Rutsanee Akaraphanth; Pimonpun Gritiyarangsan

    Leukocytoclastic vasculitis (LCV) is a small-vessel inflammatory disease mediated by deposition of immune complexes. Idiopathic LCV is defined as LCV with negative or normal laboratory findings. Some of these patients were recalcitrant to conventional therapy especially patients who showed livedo reticularis and livedoid vasculitis clinically. Several studies reported good response to PUVA treating conditions which showed evidence of LCV in

  12. Low-Dose Danazol in the Treatment of Livedoid Vasculitis

    Microsoft Academic Search

    G.-H. Hsiao; H.-C. Chiu

    1997-01-01

    Background: Livedoid vasculitis is characterized clinically by smooth or depressed ivory-white scars surrounded by hyperpigmentation and telangiectasia with or without preceding purpuric infiltrated papules and plaques and histologically by intravascular deposition of fibrin. Its etiology remains obscure and therapy very difficult. Objective: Our purpose was to test the efficacy of low-dose danazol in the treatment of livedoid vasculitis. Methods: Seven

  13. Livedo reticularis and livedoid vasculitis responding to PUVA therapy

    Microsoft Academic Search

    Hyun-Joo Choi; Seung-Kyung Hann

    1999-01-01

    Livedo reticularis is a mottled blue discoloration of the skin, which occurs in a netlike pattern. Livedoid vasculitis is a chronic disorder clinically manifested by recurrent painful ulcerations of the lower extremities and is characterized by the presence of smooth or depressed ivory-white lesions surrounded by hyperpigmentation and telangiectasia. We describe two patients with livedo reticularis and livedoid vasculitis who

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

    PubMed

    Holl-Ulrich, K

    2014-11-01

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

  15. Examination of disease severity in systemic vasculitis from the novel perspective of damage using the vasculitis damage index (VDI)

    Microsoft Academic Search

    A. R. EXLEY; P. A. BACON; R. A. LUQMANI; G. D. KITAS; D. M. CARRUTHERS

    1998-01-01

    SUMMARY Assessment of disease severity in systemic vasculitis encompasses mortality, which is now uncommon, and morbidity, which is increasing in significance. Morbidity includes permanent scars or damage, an evolving concept oÄering a novel perspective which may be particularly valuable in chronic disease. We have developed a method for assessing damage in systemic vasculitis, but the relationship between damage and disease

  16. Leukocytoclastic vasculitis after pneumococcal pneumonia in an elderly adult.

    PubMed

    Nakamura, Shigeki; Yanagihara, Katsunori; Izumikawa, Koichi; Seki, Masafumi; Miyazaki, Yoshitsugu; Hirakata, Yoichi; Soejima, Yoshifumi; Mizuta, Yohei; Kohno, Shigeru

    2007-01-01

    Hypersensitivity vasculitis (HSV) has been used to describe several forms of vasculitis of small blood vessels, including Henoch-Schönlein purpura (HSP), mixed cryoglobulinemia, and allergic vasculitis, etc. HSP is a disease occasionally seen in childhood, and is characterized by dermatological and abdominal symptoms. Here, we report a rare case of HSV which showed a clinical course similar to HSP after pneumococcal pneumonia in an elderly adult. Generally, Streptococcus pneumoniae is the most common pathogen in adult community-acquired pneumonia. Therefore, it is critical to recognize HSV as one of the important complications after bacterial infection, especially Streptococcus pneumoniae. PMID:17443040

  17. Antineutrophilic Cytoplasmic Antibody Positive Vasculitis Associated with Methimazole Use

    PubMed Central

    Shikha, Deep; Harris, Jonathan; Resta, Christine; Park, Patricia

    2015-01-01

    ANCA-associated vasculitis (AAV) is a rare and potentially life threatening complication associated with antithyroid drug use. It is more commonly reported with propylthiouracil, with fewer cases reported with methimazole use. We present the case of a 55-year-old man with toxic multinodular goiter which was treated with methimazole for 6 months. He developed ANCA positive leukocytoclastic vasculitis with hemorrhagic and necrotic bullous lesions of lower extremities. The vasculitis was initially thought to be secondary to recent cephalosporin use; however, the skin lesions progressed despite stopping the cephalosporin and treatment with steroids, and he developed osteomyelitis. His vasculitis resolved after cessation of methimazole use. This case highlights the importance of careful monitoring for variable manifestations of AAV in patients treated with methimazole. PMID:26060588

  18. Lung vasculitis and alveolar hemorrhage: pathology.

    PubMed

    Fishbein, Gregory A; Fishbein, Michael C

    2011-06-01

    Pulmonary vasculitides are a diverse group of limited and systemic disorders associated with inflammation of pulmonary vessels and parenchyma. These diseases often have distinctive clinical, serological, and histopathological features-extrapulmonary sites of involvement, circulating autoantibodies, predispositions for small or large vessels, and others. Some have characteristic inflammatory lesions; others are characterized by the absence of such lesions. Frequently pathological findings overlap, rendering classification, and diagnosis a challenge. The anti-neutrophil cytoplasmic antibody (ANCA)-associated small-vessel diseases constitute the major pulmonary vasculitides. These include Wegener granulomatosis (WG), Churg Strauss syndrome (CSS), and microscopic polyangiitis (MPA). Less frequently, diseases such as polyarteritis nodosa, Takayasu arteritis, Behçet syndrome, and connective tissue diseases may involve pulmonary vessels, but these entities are better associated with extrapulmonary disease. Diffuse alveolar hemorrhage (DAH) is a severe manifestation of pulmonary vasculitis. DAH is most commonly seen in small-vessel vasculitides, specifically MPA and WG. Other syndromes associated with DAH include Goodpasture syndrome, Henoch-Schönlein purpura, and systemic lupus erythematosus. Less commonly, DAH may be secondary to infection or drugs/toxins. Furthermore, in the absence of discernable systemic disease, DAH may be idiopathic-referred to as isolated pulmonary capillaritis (IPC) or idiopathic pulmonary hemosiderosis (IPH), depending on the presence of capillaritis. PMID:21674412

  19. Mycoplasmal Upper Respiratory Infection Presenting as Leukocytoclastic Vasculitis

    PubMed Central

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

    2015-01-01

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

  20. Granulomatosis with polyangiitis and associated pulmonary emphysema: Breathtaking vasculitis.

    PubMed

    Gadre, Shruti K; Stoller, James K; Mehta, Atul C

    2015-01-01

    Pulmonary emphysema occasionally occurs in the absence of smoking or noxious exposures. Other than through a known association with alpha-1 antitryspin deficiency, to our knowledge, no reports implicate granulomatosis with polyangiitis (GPA) in causing airflow obstruction with small airway involvement and severe air trapping. To extend available experience, we report a 51-year-old male with biopsy-proven cytoplasmic-antineutrophilic cytoplasmic antibody proteinase 3 (ANCA PR3)-positive GPA who developed centrilobular emphysema and airflow obstruction during a phase of active vasculitis. He was a lifelong non-smoker and had a normal alpha-1 antitrypsin level and a PI*MM phenotype. Treatment with corticosteroids and cyclophosphamide caused clinical remission of his vasculitis which was associated with improvement in his respiratory symptoms. However, to date, structural changes of emphysema have persisted for over 9 years of follow-up. Clinicians should remain vigilant to the possibility of emphysema in patients with pulmonary vasculitis. PMID:26180387

  1. Leukemic vasculitis: an unusual manifestation of leukemia cutis.

    PubMed

    Seckin, Dilek; Senol, Asli; Gurbuz, Oya; Demirkesen, Cuyan

    2009-09-01

    Leukemia cutis is frequently observed as papules, nodules, and plaques, but unusual clinical manifestations rarely occur. We report a 64-year-old woman with acute myeloid leukemia M1 who presented with erythematous papules and vesiculobullous lesions limited to the arms, hands, and neck in addition to purpuric papules on the legs. Because of the symmetric distal involvement and vesiculobullous nature of the skin lesions, the differential diagnosis included erythema multiforme and vasculitis. However, a skin biopsy specimen revealed infiltration with a striking predominance of blast cells in the dermis. These cells were also observed within the walls of the venules along with fibrin. Leukemia cutis associated with vasculitis was diagnosed. A few blast cells can be observed in many reactive dermatoses in patients with leukemia. However, in this patient a predominance of blast cells in the dermis and infiltration of vessel walls by these cells favored leukemic vasculitis. PMID:19481293

  2. Cutaneous polyarteritis nodosa: A rare isolated cutaneous vasculitis

    PubMed Central

    Subbanna, Praveen Kumar A.; Singh, Negi Vir; Swaminathan, Rathinam P.

    2012-01-01

    Cutaneous polyarteritis nodosa (CPAN) is a rare form of cutaneous vasculitis that involves small and medium sized arteries of the dermis and subcutaneous tissue without systemic involvement. It presents with tender subcutaneous nodules, digital gangrene, livedo reticularis and subcutaneous ulcerations. The diagnosis is by skin biopsy and characteristic pathologic feature is a leukocytoclastic vasculitis in the small to medium-sized arterioles of the dermis. We report a rare case of benign cutaneous PAN in a 14-year-old girl who presented with history of fever, subcutaneous nodules with cutaneous ulcer and digital gangrene. The skin biopsy showed leukocytoclastic vasculitis with fibrinoid necrosis in the dermal vessels. She received treatment with steroids and lesions resolved completely over a period of month. PMID:23130255

  3. Giant cell arteritis: A rare cause of posterior vasculitis

    PubMed Central

    Moschos, Marilita M; Guex-Crosier, Yan

    2009-01-01

    Purpose: To report three cases of posterior vasculitis associated with subacute giant cell arteritis (GCA). Methods: Three patients with decreased vision underwent complete ophthalmologic examination and fluorescein angiography. Results: All patients presented posterior vasculitis. Patient 1 had an erythrocyte sedimentation rate (ESR) of 38 mm/hr and a C-reactive protein (CRP) of 28mg/L. Patient 2 and 3 had an ESR of 104 and 95 mm/hr and a CRP of 42 and 195 mg/L accordingly. Diagnosis was established by temporal artery biopsy. Resolution was observed after systemic prednisolone therapy. Conclusion: GCA should be suspected when posterior vasculitis and relatively high ESR and CRP are present. PMID:19668553

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

    E-print Network

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

    2015-01-01

    Biomarkers in Rheumatology Keywords$ gene expression; biomarker; interferon; Systemic Lupus Erythematosus; vasculitis 2 Abbreviations$ Systemic Lupus Erythematosus (SLE) ANCA-associated vasculitis (AAV) Peripheral blood mononuclear cells (PBMC... -expression based biomarkers in Systemic Lupus Erythematosus (SLE) and systemic vasculitis. The type 1 interferon inducible gene signature remains by far the most studied in autoimmune rheumatic disease. While initially presented as an objective, blood...

  5. 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

  6. Leukemic vasculitis: a rare pattern of leukemia cutis.

    PubMed

    Cañueto, Javier; Meseguer-Yebra, Carmen; Román-Curto, Concepción; Santos-Briz, Angel; Fernández-López, Emilia; Fraile, Carmen; Unamuno, Pablo

    2011-04-01

    Although non-specific skin lesions are quite common in patients with leukemia, the specific infiltration of the skin by blast cells, known as leukemia cutis, is rare. Its incidence ranges from 1 to 50% and depends on the specific type of leukemia. Leukemic vasculitis represents a rare form of leukemia cutis consisting of the involvement and destruction of vessel walls by leukemic cells, which in themselves cause the vascular injury. To date, only few cases of leukemic vasculitis have been described. Here, we report two cases of this rare skin condition, one of which mimicked cutaneous polyarteritis nodosa. PMID:20860730

  7. Cryptogenetic multifocal ulcerous stenosing enteritis: an atypical type of vasculitis or a disease mimicking vasculitis

    PubMed Central

    Perlemuter, G; Guillevin, L; Legman, P; Weiss, L; Couturier, D; Chaussade, S

    2001-01-01

    BACKGROUND/AIMS—Cryptogenetic multifocal ulcerous stenosing enteritis (CMUSE) is a rare disease whose origin is unknown. The aim of this study was to describe the clinical spectrum of CMUSE, to determine the origin and pathophysiology of the disease, and to propose a treatment strategy.?METHODS—A total of 220 French gastroenterology departments were contacted to review patients with unexplained small bowel strictures. Of 17 responses, 12 corresponded to a diagnosis of CMUSE. These patients were hospitalised between 1965 and 1993 and their medical records were reviewed.?RESULTS—All patients (mean age 42.1 (4.4) years) had intestinal and five had extraintestinal symptoms (peripheral neuropathy, buccal aphthae, sicca syndrome, polyarthralgia, Raynaud's phenomenon, arterial hypertension). One patient had heterozygous type I C2 deficiency (28 base pair gene deletion). Two to 25 (mean 8.3 (1.9)) small intestine strictures were found. Stenoses of the large jejunoileal arteries were observed on two and aneurysms on three of five mesenteric angiograms. Despite surgery, symptoms recurred in seven of 10 patients and strictures in four. Steroid therapy was effective but caused dependence. One untreated patient died. Small bowel pathology showed superficial ulceration of the mucosae and submucosae, and an inflammatory infiltrate made of neutrophils and eosinophils.?CONCLUSIONS—CMUSE is an independent entity characterised by steroid sensitive inflammation of the small bowel which often recurs after surgery. CMUSE may be related to a particular form of polyarteritis nodosa with mainly intestinal expression or with an as yet unclassified vasculitis.???Keywords: cryptogenetic multifocal ulcerous stenosing enteritis; vasculitis; small intestine PMID:11171822

  8. Incidence of selective IgG2 deficiency in patients with vasculitis.

    PubMed Central

    Jiménez, A; López-Trascasa, M; Fontán, G

    1989-01-01

    IgG2 deficiency has been recently associated with the appearance of vasculitis. None of the 42 patients with IgG2 deficiency whom we studied has a history of vasculitis. We have also studied 56 patients with vasculitis, and found four of them with IgG2 levels below the normal range for their age. As a group, the vasculitis patients did not differ in the incidence of low IgG2 levels from a normal population. Three of the four vasculitis patients with low IgG2 levels had a Henoch-Schonlein syndrome, and the association between both features was statistically significant. PMID:12412740

  9. Digital artery thrombosis and vasculitis in juvenile rheumatoid arthritis

    PubMed Central

    Carson, R. Annan

    1973-01-01

    A case of juvenile rheumatoid arthritis with vasculitis is presented. Sixteen months after the onset of the disease the patient developed digital artery thrombosis with incipient gangrene. Both the latter and the skin lesions resolved during treatment with azathioprine. PMID:4742476

  10. Prolonged prodrome, systemic vasculitis, and deafness in Cogan's syndrome

    Microsoft Academic Search

    S Van Doornum; G McColl; M Walter; I Jennens; P Bhathal; I P Wicks

    2001-01-01

    Cogan's syndrome is a rare, multisystem disease which occurs predominantly in children and young adults. It was originally described as the combination of interstitial keratitis and audiovestibular disturbance, but other forms of ocular disease, as well as systemic vasculitis, have since been recognised as part of the syndrome. Diagnosis can be difficult if the various manifestations occur separately, but early

  11. Acute neuroschistosomiasis: two cases associated with cerebral vasculitis.

    PubMed

    Jauréguiberry, Stéphane; Ansart, Séverine; Perez, Lucia; Danis, Martin; Bricaire, François; Caumes, Eric

    2007-05-01

    Encephalitis and focal neurologic deficits can occur during the acute phase of schistosomiasis. We report two cases in which cerebral imaging showed cerebral vasculitis located in arterial junctional territories. These neurologic complications may be caused by eosinophil-mediated toxicity. Immediate treatment should consist of corticosteroids rather than specific antischistosomal drugs, which may aggravate the disorders. PMID:17488923

  12. VASCULITIS LIVEDOIDE: REPORTE DE CASO Y REVISIÓN DE LITERATURA

    Microsoft Academic Search

    S. García; F. Cortez; L. Rengifo; Sandra García-Salas; Florencio Cortez-Franco; Lizbeth Rengifo-Pinedo; COMUNICACIONES B REVES

    The livedoid vasculitis is an uncommon chronic disorder that usu- ally presents with painful slowly healing ulcerations of the lower limbs. The precise pathophysiology of this relatively rare disease remains obscure. Therapeutic strategies usually include antiinflam- matory or immunosuppressive agents. However, no continuing benefit has been reported in any of these modalities. We report the case of young woman with

  13. URTICARIA AND ARTHRALGIAS AS MANIFESTATIONS OF NECROTIZING ANGIITIS (VASCULITIS)

    Microsoft Academic Search

    Nicholas A. Soter; Frank Austen; Irma Gigli

    1974-01-01

    Although necrotizing angiitis (vasculitis) of the superficial venules and capillaries of the skin is usually appreciated visually as a purpuric papule, a group of patients has been defined in whom all of the skin lesions were urticarial. Microscopic examination of skin biopsy specimens showed fibrinoid necrosis of the blood-vessel walls, an infiltrate containing polymorphonuclear leukocytes, fragmentation of cell nuclei, and

  14. Single-organ gallbladder vasculitis: characterization and distinction from systemic vasculitis involving the gallbladder. An analysis of 61 patients.

    PubMed

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

    2014-11-01

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

  15. Immunoregulatory gene polymorphisms are associated with ANCA-related vasculitis

    Microsoft Academic Search

    Marjan C. Slot; Milena G. Sokolowska; Kim G. Savelkouls; Rob G. J. H. Janssen; Jan G. M. C. Damoiseaux; Jan Willem Cohen Tervaert

    2008-01-01

    T cell activation is regulated by inhibitory molecules such as PD-1 and CTLA-4, whose expression may be affected by gene polymorphisms. Increased T cell activation is present in patients with ANCA-associated vasculitis (AAV). We investigated two single-nucleotide polymorphisms (SNPs) in PDCD1 and five polymorphisms in CTLA4 in 102 patients with AAV and 188 healthy controls (HC). The distributions of the

  16. [Livedoid vasculitis in a patient with antiphospholipid syndrome].

    PubMed

    Serra, Sara; Saavedra, M João; Salvador, M João; Reis, J Pedro; Malcata, A

    2010-01-01

    The authors present a clinical case of a 30 year old male patient admitted to the hospital for recurrent cyanosis and feet pain lasting for one year. In addition he presented bilateral purpuric lesions in the lateral maleolar region, one of which with ulceration. The finding of anticardiolipin antibodies associated with intraluminal thrombosis in the dermal vessels, allowed to conclude for Antiphospholipid syndrome. The cutaneous changes identified are named livedoid vasculitis. PMID:20711098

  17. Challenges in diagnosis of isolated central nervous system vasculitis

    PubMed Central

    Amara, Amy W; Bashir, Khurram; Palmer, Cheryl A; Walker, Harrison C

    2011-01-01

    Isolated central nervous system (CNS) vasculitis is a rare and complicated disorder. Patients typically present with nonspecific neurologic symptoms such as headache and encephalopathy, and have variable progression and severity of the disease. Challenges to definitive diagnosis include the limitations of currently available diagnostic modalities with high likelihood of false-positive or false-negative findings. Imaging, serologic, and cerebrospinal fluid (CSF) evaluation, and even angiography can fail to establish the diagnosis. Often, brain biopsy is required. In order to illustrate these challenges, we report the case of a patient who presented with subacute cognitive decline and was ultimately diagnosed with isolated CNS eosinophilic vasculitis. Initial work-up included CSF and serologic analyses, magnetic resonance imaging (MRI), and cerebral angiography, but definitive diagnosis required brain biopsy. Immunosuppressive therapy resulted in clinical improvement and stabilization. To our knowledge, only one other case of isolated CNS eosinophilic vasculitis has been reported in the literature. We discuss the importance of a high index of clinical suspicion in cases of progressive nonspecific neurologic symptoms. PMID:22398982

  18. Severe leukocytoclastic vasculitis secondary to the use of a naproxen and requiring amputation: a case report

    PubMed Central

    2010-01-01

    Introduction Leukocytoclastic vasculitis (also known as hypersensitivity vasculitis and cutaneous necrotizing vasculitis) can present with various manifestations, which often delays the diagnosis and treatment. In order to show the importance of the early recognition of leukocytoclastic vasculitis, we present a case which occurred secondary to the use of a common pharmaceutical, naproxen. We were unable to find a case of leukocytoclastic vasculitis secondary to naproxen in the literature. Case presentation We present the case of a 33-year-old African American woman with below the knee and bilateral digital gangrene from hypersensitivity vasculitis secondary to the non-steroidal anti-inflammatory medication naproxen. Conclusion This is an original case report focusing on the rheumatologic management of leukocytoclastic vasculitis. However, other specialties, such as internal medicine, dermatology, infectious disease, general surgery and pathology, can gain valuable information by reviewing this case report. Reporting a case of leukocytoclastic vasculitis secondary to treatment with naproxen will advance our understanding of this disease etiology by adding yet another non-steroidal anti-inflammatory drug to the list of potential causes of leukocytoclastic vasculitis. PMID:20594300

  19. Livedo (livedoid) vasculitis and the factor V Leiden mutation: Additional evidence for abnormal coagulation

    Microsoft Academic Search

    Kenneth T. Calamia; Maria Balabanova; Charles Perniciaro; John S. Walsh

    2002-01-01

    We report the case of a patient with livedo vasculitis associated with the factor V Leiden mutation. This association provides additional support for abnormalities of coagulation in patients with this disorder. The spectrum of platelet, coagulation, and fibrinolytic disorders reported with livedo vasculitis is reviewed. (J Am Acad Dermatol 2002;46:133-7.)

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

    PubMed Central

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

    2015-01-01

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

  1. Allergic vasculitis: most common of the necrotizing vasculitides.

    PubMed

    Sams, W M

    1981-08-01

    Allergic vasculitis presents with purpuric lesions that are distinguishable by their palpability and by their distribution primarily on the lower extremities. The disease can affect any organ system but most often involves the kidneys, joints, gastrointestinal tract, lungs, or nervous system. Histopathologic examination shows characteristic destruction of the vessel wall by polymorphonuclear leukocytes. Numerous etiologic agents have been implicated, but streptococcal infection and drug ingestion are the most common. Presumptive evidence suggests that the disease is due to immune complexes. The workup is aimed at establishing the cause and extent of systemic involvement, and treatment is tailored accordingly. PMID:7019874

  2. A central role for the mast cell in early phase vasculitis in the Brown Norway rat model of vasculitis: a histological study

    PubMed Central

    Vinen, Catherine S; Turner, David R; Oliveira, David B G

    2004-01-01

    Administration of mercuric chloride (HgCl2) to Brown Norway rats causes Th2-dominated autoimmunity with raised immunoglobulin E concentrations and gut vasculitis, both of which are T-cell dependent, peak at 14 days after starting HgCl2 and then spontaneously resolve. If animals are re-challenged with HgCl2 6 weeks after initial exposure, they are resistant to autoimmunity, developing only attenuated disease. Recently, a separate phase of early caecal vasculitis was described beginning 24 h after initiating HgCl2 and prior to caecal entry of T cells. Previous work suggested this early vasculitis was ?? T-cell independent and implied a role for mast cells. We further tested this hypothesis by performing a histological study during the first 93 h following HgCl2 challenge defining the precise relationship between gut mast cell degranulation and appearing caecal vasculitis. We also studied whether early caecal vasculitis enters a resistant phase upon re-challenge with HgCl2. We show a direct correlation between mast cell degranulation and early caecal vasculitis following initial HgCl2 challenge. We demonstrate resistance to re-challenge in this phase of injury, with results at re-challenge also showing a correlation between mast cell degranulation and early caecal injury. PMID:15255970

  3. Cutaneous vasculitis in ulcerative colitis mimicking Henoch-Schönlein purpura.

    PubMed

    de Oliveira, Guilherme Trudes; Martins, Sofia Simão; Deboni, Mariana; Picciarelli, Patrícia; Campos, Lúcia Maria Arruda; Jesus, Adriana Almeida; Koda, Yu Kar Ling; Silva, Clovis Artur

    2013-03-01

    To the best of our knowledge, no cases of ulcerative colitis (UC) mimicking Henoch-Schönlein purpura (HSP) have been reported so far. During a 28-year period 5635 patients were followed up at our Pediatric Rheumatology Unit and 357 had HSP according to the European League Against Rheumatism, the Paediatric Rheumatology International Trials Organisation and the Paediatric Rheumatology European Society validated classification criteria. At the same period, 148 patients with IBD according to the European Society for Paediatric Gastroenterology, Hepatology and Nutrition criteria were followed up at the Pediatric Gastroenterology Unit in our University Hospital. Only two of them had vasculitis, as an extra intestinal manifestation of UC mimicking HSP, and fulfilled both disease criteria. A 2-year old girl had bloody diarrhoea, severe abdominal pain, arthritis in ankles, petechiae and palpable purpura not related to thrombocytopenia in lower limbs. A 5-year old boy had bloody diarrhoea, palpable purpura in buttocks, lower limbs, penis and scrotum associated with arthritis in knees, orchitis in right testicle and periarticular swelling in hands and feet. Their ileocolonoscopy showed diffuse mucosal erythema, oedema, friability and multiple irregular ulcers, and histopathological examination of colonic specimen revealed diffuse chronic mucosal inflammation, crypt distortion and crypt abscesses suggesting ulcerative colitis. There were no signs of intestinal vasculitis in both cases. In conclusion, this is the first study in a paediatric population that evidenced palpable purpura associated with UC mimicking HSP. PMID:22626504

  4. 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

  5. Causal Attributions about Disease-Onset and Relapse in Patients with Systemic Vasculitis

    PubMed Central

    Grayson, Peter C.; Amudala, Naomi A.; McAlear, Carol A.; Leduc, Renée L.; Shereff, Denise; Richesson, Rachel; Fraenkel, Liana; Merkel, Peter A.

    2014-01-01

    Objectives Patients vary in their beliefs related to the cause of serious illness. The impact of these beliefs among patients with systemic vasculitis is not known. This study aimed to describe causal attributions about disease-onset and relapse in systemic vasculitis and to examine whether causal beliefs a) differ by type of vasculitis; and b) are associated with negative health outcomes. Methods Patients with vasculitis were recruited to complete an online questionnaire. Categories of causal beliefs were assessed with the Revised Illness Perception Questionnaire (IPQ-R). Differences in beliefs about disease-onset versus relapse were compared across different forms of vasculitis. Causal beliefs were assessed in association with several health outcomes including fatigue, functional impairments, and personal understanding of the condition. Results 692 patients representing 9 forms of vasculitis completed the questionnaire. The majority (90%) of patients had beliefs about the cause of their illness. Causal attributions were highly variable, but altered immunity and stress were the most commonly agreed upon causal beliefs. Frequencies of causal beliefs were strikingly similar across different forms of vasculitis, with few notable exceptions primarily in Behçet’s disease. Beliefs differed about causes of disease-onset versus relapse. Specific beliefs about disease-onset and relapse were weakly associated with fatigue, functional impairments, and understanding of the condition. Conclusion Patient beliefs related to the cause of systemic vasculitis are highly variable. Patterns of causal beliefs are associated with important negative health outcomes. Clinicians who care for patients with vasculitis should be mindful of these associations and consider asking about patients’ causal beliefs. PMID:24634202

  6. [Necrotizing vasculitis of the cerebral and spinal leptomeninges in a Bernese mountain dog].

    PubMed

    Gerhardt, A; Risse, R; Meyer-Lindenberg, A

    1998-04-01

    A case of necrotizing vasculitis involving the central nervous system in a seven month old Bernese mountain dog is reported. The clinical signs include apathy, fever and increased head and cervical pain. The cerebrospinal fluid was unusual viscous and bloody. In the EEG high activity and spikes were found. A hydrocephalus internus was confirmed by CT scan. On histopathological examination a necrotizing vasculitis on the medulla oblongata and spinal cord leptomeninges with perivascular granulomatous inflammation were detected. The neuropathological lesions are consistent with those reported for the rare disease of necrotizing vasculitis in the central nervous system of Bernese mountain dogs, beagles and boxers. The cause is unknown. PMID:9618983

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

    SciTech Connect

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

    1986-06-01

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

  8. Inflammatory myopathy as the initial presentation of cryoglobulinaemic vasculitis

    PubMed Central

    Rodríguez-Pérez, Noelia; Rodríguez-Navedo, Yerania; Font, Yvonne M; Vilá, Luis M

    2013-01-01

    Cryoglobulinaemic vasculitis is characterised by immunoglobulin deposition at low temperatures. The most common manifestations are cutaneous involvement, arthralgias, Raynaud's phenomenon, peripheral neuropathy and renal disease. Myopathy is unusual and only a few cases have been reported. Here, we present a 31-year-old woman who developed progressive muscle weakness involving upper and lower extremities, dysphagia, paraesthesias and palpable purpura. Diagnostic studies revealed elevated creatine kinase, diffuse myopathic and sensorimotor axonal neuropathy on electromyography and nerve conduction studies, and inflammatory myopathy on muscle biospsy. Cryoglobulin levels were elevated on two occasions. She responded favourably to cyclophosphamide and high-dose corticosteroids. Cyclophosphamide was continued for 1?year followed by methotrexate. Prednisone was gradually tapered and discontinued 1?year later. She remained in clinical remission after 4?years of follow-up. This case suggests that cryoglobulinaemia should be considered in the differential diagnosis of a patient presenting with inflammatory myopathy. PMID:23737595

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

    PubMed

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

    2015-04-01

    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

  10. Comparison of cell adhesion molecule expression in cutaneous leucocytoclastic and lymphocytic vasculitis.

    PubMed Central

    Burrows, N P; Molina, F A; Terenghi, G; Clark, P K; Haskard, D O; Polak, J M; Jones, R R

    1994-01-01

    AIMS--To compare the expression of the cell adhesion molecules intercellular adhesion molecule-1 (ICAM-1), ELAM-1 (E-selectin), and vascular cell adhesion molecule-1 (VCAM-1) in cutaneous leucocytoclastic and lymphocytic vasculitis. METHODS--Immunohistochemical analysis was performed on early lesional skin biopsy specimens of leucocytoclastic vasculitis (n = 14), lymphocytic vasculitis (n = 10), non-lesional skin (n = 12), and normal skin (n = 5). A standard immunoperoxidase technique was used to detect expression of ICAM-1, E-selectin, VCAM-1, and the cell markers CD11a, CD11b, CD11c, von Willebrand factor, CD3, CD68, and neutrophil elastase (NP57). RESULTS--Basal keratinocyte intercellular adhesion molecule-1 was expressed in eight (80%) cases of lymphocytic and in only one (7%) case of leucocytoclastic vasculitis, and not in non-lesional skin or control biopsy specimens from normal subjects. E-selectin was expressed on vascular endothelium in eight (57%) cases of leucocytoclastic and in seven (70%) cases of lymphocytic vasculitis. Endothelial vascular cell adhesion molecule-1 expression was seen in three (21%) biopsy specimens of leucocytoclastic and five (50%) of lymphocytic vasculitis. There were increased numbers of cells in the dermal infiltrate stained for NP57, CD11b, and CD11c in leucocytoclastic compared with lymphocytic vasculitis (p < 0.001, p = 0.013, p = 0.009, respectively); immunoreactive positive cells for CD3 and CD11a were increased in lymphocytic compared with leucocytoclastic vasculitis (p < 0.001, p = 0.011, respectively). CONCLUSIONS--These observations indicate that upregulation of adhesion molecule expression occurs in both leucocytoclastic and lymphocytic vasculitis. The different patterns of adhesion molecule expression in the two groups of vasculitis may reflect differences in the local release of cytokines. In particular, detection of intercellular adhesion molecule-1 expression by keratinocytes in lymphocytic vasculitis is consistent with an active role for mediators derived from T lymphocytes in the pathogenesis of the lesion. Images PMID:7525658

  11. The contribution of genetic variation and infection to the pathogenesis of ANCA-associated systemic vasculitis

    PubMed Central

    2010-01-01

    The aetiology of anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis has not been well defined. Here we review two factors which may play a role in the pathogenesis of the disease: genetics and infection. In particular, we discuss the role of autoantibodies to LAMP-2, which may arise following infection with Gram-negative bacteria, and may contribute to the development of ANCA-associated systemic vasculitis in genetically susceptible individuals. PMID:20236493

  12. Membranoproliferative glomerulonephritis presenting as arthropathy and cardiac valvulopathy in hypocomplementemic urticarial vasculitis: a case report

    PubMed Central

    2014-01-01

    Introduction Hypocomplementemic urticarial vasculitis syndrome is a rare disorder characterized by chronic urticarial vasculitis, arthralgia, arthritis, and hypocomplementemia. Previously, only six patients with concomitant hypocomplementemic urticarial vasculitis syndrome, Jaccoud’s arthropathy, and valvular heart disease have been reported. Case presentation A 30-year-old Korean man presented with hypocomplementemic urticarial vasculitis syndrome. In addition to urticarial cutaneous lesions, he experienced polyarthralgia and arthritis that resulted in progressive deformity of the joints of both hands, cardiac valvulopathy with mitral, tricuspid, and aortic regurgitation, and intermittent neck swelling with laryngeal edema. He also developed nephritis with azotemia. His renal biopsy results revealed membranoproliferative glomerulonephritis, type I. He showed a partial response to a combination therapy of steroid, cyclophosphamide, and mycophenolate mofetil. Conclusions We describe, to the best of our knowledge, the first case of glomerulonephritis presenting a arthropathy and cardiac valvulopathy in hypocomplementemic urticarial vasculitis syndrome. A combination of corticosteroids, cyclophosphamide, and mycophenolate mofetil appear to be a safe and effective treatment for nephropathy, however are less effective for cutaneous vasculitis, cardiac valvulopathy, and arthropathy. PMID:25339233

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

    PubMed

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

    2014-01-01

    Antineutrophil cytoplasmic antibody (ANCA)-related vasculitis is a systemic small-vessel vasculitis, including 3 clinical syndromes: granulomatosis with polyangiitis, known as Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and the Churg-Strauss syndrome (CSS). ANCA-related vasculitis usually presents with severe kidney or pulmonary disease, has a mortality of 28% at 5 years, and also contributes to increased morbidity in vasculitis patients. Cardiac involvement in this entity may have different forms, including coronary vessels, pericarditis, myocarditis, endocarditis, myocardial infarction and subendocardial vasculitis that can contribute to reduced life expectancy. Cardiovascular magnetic resonance using oedema and fibrosis imaging can early reveal, noninvasively and without radiation, heart involvement during vasculitis, undetected by other imaging techniques and guide further risk stratification and treatment of these patients. PMID:25256145

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

    PubMed

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

    2014-01-01

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

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

    PubMed

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

    2015-05-01

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

  16. Cutaneous leukocytoclastic vasculitis. Serial histopathologic evaluation demonstrates the dynamic nature of the infiltrate.

    PubMed

    Zax, R H; Hodge, S J; Callen, J P

    1990-01-01

    Data from experimentally induced cutaneous vasculitis have suggested that the inflammatory infiltrate is dynamic. In contrast, data from humans with cutaneous vasculitis have suggested that two distinct patterns of cellular infiltrate exist, a mononuclear-predominant and a neutrophilic-predominant type. There are little data regarding the temporal evolution of spontaneously occurring cutaneous vasculitis in humans. A patient with a cutaneous leukocytoclastic vasculitis manifest as palpable purpura had four lesions encircled on the day of presentation. Biopsies of these lesions were obtained sequentially at 0, 24, 48, and 120 hours. The histopathologic specimens were graded without knowledge of the timing of the biopsy. The character of the infiltrate progressively changed from a neutrophilic-predominant to a mononuclear-predominant infiltrate supporting the theory of a dynamic process in cutaneous vasculitis. The previous reports that suggest that there are two distinct inflammatory cell types may be the result of performing the biopsy at one point in time during this transitory process. PMID:2297253

  17. 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

  18. A review of pediatric vasculitis with a focus on juvenile polyarteritis nodosa.

    PubMed

    Kawakami, Tamihiro

    2012-12-01

    Systemic polyarteritis nodosa (PAN) is a vasculitis characterized and defined by necrotizing inflammatory changes in medium and/or small arteries. Children and adults with vasculitis differ in the relative frequency of some clinical manifestations and concomitant diseases. The European League against Rheumatism (EULAR)/Pediatric Rheumatology European Society (PRES) working group has proposed a classification of childhood vasculitis. With support from EULAR, the Pediatric Rheumatology International Trials Organization (PRINTO), and PRES, a formal statistical validation process, which included large-scale, web-based data collection, was undertaken. I now propose a set of criteria for systemic juvenile PAN that combines a modified mix of the EULAR/PRES criteria and the EULAR/PRINTO/PRES criteria. Cutaneous juvenile PAN is characterized by the presence of cutaneous features with no systemic involvement. The common cutaneous manifestations include cutaneous nodules and livedo racemosa. Our research group previously established an algorithm for the differential diagnosis of primary cutaneous vasculitis. We have recently developed a new version of that algorithm to diagnose vasculitis with cutaneous manifestations from a dermatologic point of view. Treatment of systemic juvenile PAN is based on a combination of corticosteroids and immunosuppressant agents. The clinical course of cutaneous juvenile PAN is generally benign. PMID:22845169

  19. Leukocytoclastic Vasculitis in Subacute Cutaneous Lupus Erythematosus: Clinicopathologic Study of Three Cases and Review of the Literature

    Microsoft Academic Search

    J. Sánchez-Pérez; P. E Peñas; L. Ríos-Buceta; J. Fernández-Henera; J. Fraga; A. García-Díez

    1996-01-01

    Background: Leukocytoclastic vasculitis associated with subacute cutaneous lupus erythematosus (SCLE) was observed by Sontheimer et al. in their first clinical series of patients with SCLE, although recent reports have suggested that its frequency was rare. Objective: To evaluate the prevalence of cutaneous leukocytoclastic vasculitis (CLV) in patients with SCLE and to describe the clinicopathologic manifestations, response to treatment and prognosis

  20. Vasculitic emergencies in the intensive care unit: a special focus on cryoglobulinemic vasculitis

    PubMed Central

    2012-01-01

    Vasculitis is characterized by the infiltration of vessel walls by inflammatory leukocytes with reactive damage and subsequent loss of vessel integrity. The clinical course of systemic vasculitis may be punctuated by acute life-threatening manifestations that require intensive care unit (ICU) admission. Furthermore, the diagnosis may be established in the ICU after admission for a severe inaugural symptom, mostly acute respiratory failure. Among the systemic vasculitides, cryoglobulinemic vasculitis (CV) has been rarely studied in an ICU setting. Severe CV-related complications may involve the kidneys, lungs, heart, gut, and/or central nervous system. The diagnosis of CV in the ICU may be delayed or completely unrecognized. A high level of suspicion is critical to obtain a timely and accurate diagnosis and to initiate appropriate treatment. We describe severe acute manifestations of CV based on six selected patients admitted to our ICU. That all six patients survived suggests the benefit of prompt ICU admission of patients with severe CV. PMID:22812447

  1. 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

  2. Transduction of a Foreign Histocompatibility Gene into the Arterial Wall Induces Vasculitis

    NASA Astrophysics Data System (ADS)

    Nabel, Elizabeth G.; Plautz, Gregory; Nabel, Gary J.

    1992-06-01

    Autoimmune vasculitis represents a disease characterized by focal inflammation within arteries at multiple sites in the vasculature. Therapeutic interventions in this disease are empirical and often unsuccessful, and the mechanisms of immune injury are not well-defined. The direct transfer of recombinant genes and their expression in the arterial wall provides an opportunity to explore the pathogenesis and treatment of vascular disease. In this report, an animal model for vasculitis has been developed. Inflammation has been elicited by direct gene transfer of a foreign class I major histocompatibility complex gene, HLA-B7, to specific sites in porcine arteries. Transfer and expression of this recombinant gene was confirmed by a polymerase chain reaction and immunohistochemistry, and cytolytic T cells specific for HLA-B7 were detected. These findings demonstrate that expression of a recombinant gene in the vessel wall can induce a focal immune response and suggest that vessel damage induced by cell-mediated immune injury can initiate vasculitis.

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

    PubMed Central

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

    2015-01-01

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

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

    PubMed

    Berlit, P; Kraemer, M

    2014-03-01

    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

  5. Clinical analysis of patients with myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitis.

    PubMed

    Shuai, Z W; Lv, Y F; Zhang, M M; Hu, Z Y

    2015-01-01

    This prospective study analyzed the clinical characteristics of myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA)-associated vasculitis and explored the relationship between MPO-ANCA and clinical manifestations of the associated vasculitis in 132 p-ANCA and MPO-ANCA-positive patients (average age, 62.3 ± 14.8 years) who were initially diagnosed with ANCA-associated vasculitis. The p-ANCA and MPO-ANCA levels in peripheral blood were detected in all patients. Among these, 128 (97%) had microscopic polyangiitis (MPA), 3 (2.3%) had granulomatous polyangiitis, and 1 (0.7%) had eosinophilic granulomatous vasculitis. The average time of diagnosis was 10.2 ± 18 months; only 14 (10.6%) patients were diagnosed within 1 month. The main organs involved and the corresponding number of patients were: renal, 95 (72%); lung, 89 (67.4%); joints, 35 (26.5%); heart, 26 (19.7%); peripheral nerve, 23 (17.4%); skin rash, 14 (10.6%); and CNS, 13 (9.8%). Older patients were more likely to show lung involvement in the early disease stage, whereas the joints were involved mostly in the younger patients. The p-ANCA levels (mean titers, 1:60) were not correlated with disease activity and extent of organ involvement, and the MPO-ANCA levels were positively correlated with disease activity, but had no correlation with the extent of organ involvement. MPO-ANCA vasculitis is a common occurrence in China; it mainly involves the elderly and presents as clinical manifestations of MPA. However, the multiple organ damage is not specific leading to delay in diagnosis. MPO-ANCA may play a pathogenic role in the associated vasculitis, and the diverse clinical manifestations might be related with the different characteristics of MPO-ANCA. PMID:26125725

  6. Pachymeningitis as a manifestation of ANCA-associated vasculitis: a care report and literature review

    PubMed Central

    Li, Sheyu; Tang, Honghu; Rong, Xia; Huang, Xiangyang; Li, Qianrui

    2015-01-01

    Pachymeningitis is a rare cause of headache characterized by dura mater thickening with various origins. We present a 67-year-old male with cranial and spinal pachymeningitis and MPO-ANCA-associated vasculitis, which is considered as the cause of pachymeningitis of this patient. After revision of other 32 reported cases, our study suggested cranial and spinal pachymeningitis could be a manifestation of ANCA-associated vasculitis. Thus, it is important to screen other manifestations of ANCA-associated vasulitis as soon as pachymeningitis was suspected.

  7. Evaluation of the Sorensen diagnostic criteria in the classification of systemic vasculitis

    Microsoft Academic Search

    R. A. Watts; T. H. W. Barker; D. G. I. Scott

    2002-01-01

    Objectives. To evaluate the use of the diagnostic criteria for Wegener's granulomatosis (WG) and microscopic polyangiitis (mPA) proposed by Sørensen et al. in the classification of primary systemic vasculitis (PSV). Methods. We applied to our cohort of PSV patients the American College of Rheumatology (ACR) criteria for WG, Churg-Strauss syndrome (CSS) and polyarteritis nodosa (PAN), the Chapel Hill Consensus Conference

  8. 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...

  9. IgA vasculitis (Henoch-Shönlein purpura) in adults: Diagnostic and therapeutic aspects.

    PubMed

    Audemard-Verger, Alexandra; Pillebout, Evangeline; Guillevin, Loïc; Thervet, Eric; Terrier, Benjamin

    2015-07-01

    Immunoglobulin A (IgA) vasculitis, formerly called Henoch-Schönlein purpura, is an immune complex vasculitis affecting small vessels with dominant IgA deposits. Clinical manifestations mainly involve cutaneous purpura, arthralgias and/or arthritis, acute enteritis and glomerulonephritis. IgA vasculitis is more common among children than adults, with more severe disease in adults. Gastrointestinal and renal involvements represent the principal causes of morbidity and mortality in adults. Factors associated with long-term end-stage renal disease (ESRD) include baseline renal function impairment and baseline proteinuria >1 or 1.5 g/day, and on renal biopsy degree of interstitial fibrosis, sclerotic glomeruli and fibrinoid necrosis. Management of IgA vasculitis in adults is rendered difficult for clinicians because of the absence of correlation between initial presentation and long-term renal outcome, and the possible occurrence of spontaneous remission in patients with severe presentation or, in contrast, possible evolution to ESRD in patients with mild symptoms. Treatment is often symptomatic because disease course is usually benign. Treatment of severe involvement, including severe gastrointestinal complications or proliferative glomerulonephritis, remains controversial, with no evidence that corticosteroids or immunosuppressive agents improved long-term outcome. Prospective, randomized, controlled trials are thus needed to analyze the benefit-risk ratio of such treatments. PMID:25688001

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

    PubMed Central

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

    2015-01-01

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

  11. The role of KIR2DL3/HLA-C*0802 in Brazilian patients with rheumatoid vasculitis

    PubMed Central

    Nishimura, Wester Eidi; Sachetto, Zoraida; Costallat, Lilian Teresa Lavras; Yazbek, Michel Alexandre; Londe, Ana Carolina Santos; Guariento, Edilaine Gildo; Marques, Silvia Barbosa Dutra; Bertolo, Manoel Barros

    2015-01-01

    OBJECTIVES: Rheumatoid arthritis is a polygenically controlled systemic autoimmune disease. Rheumatoid vasculitis is an important extra-articular phenotype of rheumatoid arthritis that can result in deep cutaneous ulcers. The objective of this study was to establish a correlation between the frequency of major histocompatibility complex class I/II alleles and killer immunoglobulin-like receptor genotypes in patients with cutaneous rheumatoid vasculitis. METHODS: Using the Scott & Bacon 1984 criteria to diagnose rheumatoid vasculitis and after excluding any other causes such as diabetes, atherosclerosis, adverse drug reactions, infection, and smoking, patients who met the criteria were selected. All of the selected rheumatoid vasculitis patients presented deep cutaneous ulcers. Identification of the major histocompatibility complex class I/II and killer immunoglobulin-like receptor genotypes was performed by polymerase chain reaction assays of samples collected from the 23 rheumatoid vasculitis patients as well as from 80 controls (40 non-rheumatoid vasculitis RA control patients and 40 healthy volunteers). RESULTS: An association between the presence of the HLA-DRB1*1402 and HLA-DRB1*0101 alleles and cutaneous lesions in rheumatoid vasculitis patients and a correlation between the inhibitor KIR2DL3 and the HLA-C*0802 ligand in rheumatoid vasculitis patients were found. CONCLUSION: An association was found between the presence of the HLA-DRB1*1402 and HLA-DRB1*0101 alleles and the development of cutaneous lesions in rheumatoid vasculitis patients. Additionally, the HLA-C*0802 ligand protects these individuals from developing cutaneous lesions. PMID:26106958

  12. Surrogate markers of B cell non-Hodgkin's lymphoma in patients with hepatitis C virus-related cryoglobulinaemia vasculitis

    Microsoft Academic Search

    Guillaume Geri; Benjamin Terrier; Oren Semoun; David Saadoun; Damien Sène; Frédéric Charlotte; Hélène Merle-Béral; Lucile Musset; Matthieu Resche-Rigon; Patrice Cacoub

    2010-01-01

    ObjectiveTo evaluate clinical and biological surrogate markers associated with the presence of B cell non-Hodgkin's lymphoma (B-NHL) in patients with hepatitis C virus (HCV) with mixed cryoglobulinaemia (MC) vasculitis.MethodsA total of 104 patients with HCV-MC vasculitis (including 20 with B-NHL) were included. The main clinical and biological markers associated with the presence of B-NHL were evaluated.ResultsPatients with B-NHL compared to

  13. Clinicopathological features of antineutrophil cytoplasmic antibodies-associated vasculitis in Japanese patients with IgA nephropathy

    Microsoft Academic Search

    Miho Shimizu; Takashi Wada; Norihiko Sakai; Yoshiaki Izumiya; Kengo Furuichi; Tsugiho Misaki; Ken-ichi Kobayashi; Satoshi Goshima; Shin-ichi Takeda; Hitoshi Yokoyama

    2000-01-01

    Antineutrophil cytoplasmic antibodies (ANCA) have been reported to be associated with systemic vasculitis. However, the roles\\u000a of ANCA subtypes in patients with IgA nephropathy remain to be fully investigated. We describe three Japanese patients with\\u000a IgA nephropathy complicated by ANCA-associated vasculitis. Two patients with IgG class ANCA developed rapidly progressive\\u000a renal failure and demonstrated mesangial proliferation with extensive extracapillary proliferation

  14. Hypocomplementemic Urticarial Vasculitis Syndrome in an 8-year-old Boy: A Case Report and Review of Literature

    PubMed Central

    Al Mosawi, Zakiya Saleh Adnan; Al Hermi, Badriya Ebrahim Ahmed

    2013-01-01

    Hypocomplementemic urticarial vasculitis syndrome is an immune complex-mediated disease of unknown etiology. The clinical course is characterized by urticaria, conjunctivitis, joint pain, and hypocomplementemia. We here report a case of a child with hypocomplementemic urticarial vasculitis syndrome that progressed to nephritis. Renal biopsy was consistent with diffuse proliferative glomerulonephritis with diffuse subendothelial immune deposits. He responded well to a combination of steroid and mofetil micofenolate. PMID:23904922

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

    Microsoft Academic Search

    Benjamin Wilde; Marielle Thewissen; Jan Damoiseaux; Pieter van Paassen; Oliver Witzke; Jan Willem Cohen Tervaert

    2010-01-01

    ABSTRACT: Anti-neutrophil cytoplasmic antibody (ANCA) - associated vasculitis (AAV) is a life-threatening autoimmune disease characterized by an antibody-mediated glomerulonephritis and necrotizing vasculitis. Apart from antibodies, T cells are also involved in disease pathogenesis. This review stresses the hallmarks of T cell-mediated pathology in AAV and highlights the characteristics of lesional and circulating T cells in the immune response in AAV.

  16. Fulminant amoebic colitis mimicking intestinal vasculitis in a patient with systemic lupus erythematosus.

    PubMed

    Lee, J; Jung, H-S; Nam, H-C; Kwok, S-K; Ju, J H; Park, K-S; Kim, H-Y; Park, S-H

    2012-10-01

    Colitis in patients with systemic lupus erythematosus (SLE) is quite rare. It can be caused by intestinal vasculitis, mesenteric vascular thrombosis, concomitant inflammatory bowel disease or infectious colitis. It is important to make an accurate and early diagnosis as the treatments for each condition differ and a delayed diagnosis can result in life-threatening complications. However, non-specific gastrointestinal symptoms make a timely diagnosis challenging. Amoebic colitis is a rare condition in patients with SLE. Here we present a case of fulminant amoebic colitis in a patient with SLE which was initially misdiagnosed as ischemic colitis due to intestinal vasculitis. Her colitis was complicated with multiple intestinal perforations, disseminated intravascular coagulation and acute respiratory distress syndrome; but in the end, the patient was successfully treated with metronidazole and paromomycin. PMID:22570337

  17. Stroke-like Phenomena Revealing Multifocal Cerebral Vasculitis in Pediatric Lyme Neuroborreliosis.

    PubMed

    Kurian, Mary; Pereira, Vitor Mendes; Vargas, Maria Isabel; Fluss, Joel

    2014-10-14

    Stroke-like presentation in Lyme neuroborreliosis is rare in the pediatric age group. We report a previously healthy 12-year-old boy who presented with acute left hemiparesis and meningeal signs. Neuroimaging failed to reveal any cerebral infarction but demonstrated a multifocal cerebral vasculitis involving small, medium and large-sized vessels affecting both the anterior and posterior circulation. Concentric contrast enhancement of the basilar artery was also observed. Further investigations and laboratory findings were consistent with Lyme neuroborreliosis. A rapidly favorable clinical outcome was obtained with appropriate antibiotic treatment along with antiaggregants and steroids. Lyme neuroborreliosis should be considered in the diagnostic differential, not only in adults but also among children, especially in the context of an unexplained cerebral vasculitis. PMID:25316727

  18. Effects of imatinib mesylate on pulmonary allergic vasculitis in a murine model

    PubMed Central

    Suzuki, Naomi; Sasaki, Nobuhito; Utsumi, Yu; Nagashima, Hiromi; Nakamura, Yutaka; Yamashita, Masahiro; Yamauchi, Kohei; Sawai, Takashi

    2013-01-01

    Objectives Imatinib mesylate (IM) is a potent and specific tyrosine inhibitor and has been reported to inhibit mesenchymal cell proliferation in pulmonary fibrosis. In the present study, we examine the effects of IM on vascular remodeling in a murine model of allergic vasculitis with eosinophil infiltration. Methods C57BL/6 mice were sensitized with ovalbumin (OVA) and alum. The positive controls were exposed to aerosolized OVA daily for 7 days. IM treated mice with exposure to OVA were administered IM in parallel with daily exposure to aerosolized OVA for 7 days. On the 7th day, bronchoalveolar lavage (BAL) was performed and the lungs were excised for pathological analysis. Cell differentials were determined and the concentrations of cytokines in the BAL fluid (BALF) were measured. Semi-quantitative analysis of pathological changes in the pulmonary arteries was evaluated according to the criteria of severity of vasculitis. Immunohistochemistry for Ki-67 to detect proliferating cells was performed. Results The number of eosinophils in BALF was reduced significantly in the IM-treated group compared to the positive control. There was no significant difference in the concentrations of interleukin (IL)-2, IL-4, IL-5, interferon (IFN)-?, tumor necrosis factor (TNF)-?, tumor growth factor (TGF)-? or platelet-derived growth factor in the BAL fluid between the positive control and the IM-treated group. The pathological scores of vasculitis and the ratio of Ki-67-positive intra-luminal cells were reduced significantly in the IM-treated group compared to the control group after OVA exposure. Conclusion IM-suppressed pulmonary vascular remodeling in a murine model of allergic vasculitis with eosinophil infiltration. PMID:23992268

  19. Preliminary Results of Intracranial Angioplasty for Vascular Stenosis Caused by Atherosclerosis and Vasculitis

    Microsoft Academic Search

    John D. McKenzie; Robert C. Wallace; Bruce L. Dean; Richard A. Flom; Mazen H. Khayata

    PURPOSE: To evaluate the results of balloon angioplasty of 17 stenoses resulting from intracranial atherosclerosis and vasculitis. METHODS: Seventeen skull-base and intracranial lesions were dilated with a microballoon angioplasty catheter.RESULTS:Initially, 16 of the 17 stenoses showed improvement at angiography. Moderate residual stenosis was found in 2 of 12 atherosclerotic lesions, both in the distal vertebral artery. Angioplasty in 1 of

  20. 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

  1. Circulating Endothelial Progenitor Cells in Patients with ANCA-Associated Vasculitis

    Microsoft Academic Search

    Jakub Závada; Linda Kideryová; Robert Pytlík

    2008-01-01

    Background\\/Aims: Bone marrow-derived endothelial progenitor cells (EPCs) are believed to contribute to endothelial repair after vascular damage. To investigate the potential for microvascular repair in patients with ANCA-associated vasculitis (AAV), we conducted a cross-sectional study determining the number of circulating EPCs in patients with AAV, chronic uremia, atherosclerosis, and in healthy volunteers. Methods: The number of circulating EPCs was determined

  2. 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

  3. Diffuse dermal angiomatosis: a clinical mimicker of vasculitis associated with calciphylaxis and monoclonal gammopathy.

    PubMed

    Ferreli, C; Atzori, L; Pinna, A L; Pau, M; Aste, N; Ricotti, C; Rongioletti, F

    2015-02-01

    Diffuse dermal angiomatosis is a form of cutaneous reactive angiomatosis characterized clinically by painful erythematous or violaceous lesions with ulcers that may mimic cutaneous vasculitis/vasculopathy. Histologically it shows a benign, diffuse proliferation of endothelial cells with tiny blood vessels in the papillary and reticular dermis. Herein, we report four patients with diffuse dermal angiomatosis in the setting of calciphylaxis and monoclonal gammopathy and review the cases previously published in the literature. Comorbidities and management will also be discussed. PMID:25502367

  4. Levamisole-Induced Occlusive Necrotizing Vasculitis of the Ears After Use of Cocaine Contaminated with Levamisole

    Microsoft Academic Search

    Jennie A. Buchanan; Jody A. Vogel; Aaron M. Eberhardt

    2011-01-01

    Based on the best available data, approximately 2.1 million Americans use illicit cocaine each month; for the last several\\u000a months, 30% of that cocaine has been “cut” with a veterinary pharmaceutical, levamisole. Levamisole can cause agranulocytosis,\\u000a leaving patients susceptible to fulminate and opportunistic infections and also can cause a debilitating cutaneous necrotizing\\u000a vasculitis. In this manuscript, we describe a case

  5. Primary central nervous system vasculitis mimicking brain tumour: case report and literature review

    Microsoft Academic Search

    Song-Bin Qu; Sofia Khan; Hua Liu

    2009-01-01

    Primary central nervous system vasculitis (PCNSV) is a rare inflammatory disease causing significant morbidity and mortality.\\u000a We present a detailed history and clinical course of a patient with PCNSV along with a literature review. A 50-year-old Chinese\\u000a female presented with a 6-month history of mild to moderate headache and sudden onset of visual loss. Early computed tomography\\u000a of the brain

  6. 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

    and neurological manifestations. AAV can be divided into three clinical syndromes (Wegener's granulomatosis, microscopic polyangiitis and Churg-Strauss syndrome). All are characterised by inflammation of medium to small calibre blood vessels, and the presence... Biosystems) for each SNP, with fluorescence data captured using an ABI 7900HT (Applied Biosystems) after 40 cycles of PCR. Assessment of disease activity Birmingham Vasculitis Activity Scores (BVAS) were calcu- lated for AAV patients seen by the Lupus...

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

    PubMed

    Flossmann, Oliver

    2015-06-01

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

  8. Successful treatment of cerebral large vessel vasculitis in systemic lupus erythematosus with intravenous pulse cyclophosphamide.

    PubMed

    Kato, R; Sumitomo, S; Kawahata, K; Fujio, K; Yamamoto, K

    2015-07-01

    A 39-year-old woman with a six-year history of systemic lupus erythematosus (SLE) was admitted because of a prolonged high fever, discoid rash, and multiple lymphadenopathies. She also developed pericarditis, and was treated with intravenous methylprednisolone pulse therapy followed by prednisolone 50?mg daily and cyclosporine 100?mg daily. Meanwhile, she had a progressive headache, and a brain MRI revealed right pons infarction, although she did not have any abnormal neurological findings. An MRA revealed obvious irregular narrowing in the basilar, right vertebral and right posterior cerebral artery. There was no evidence of antiphospholipid syndrome. We concluded that the cause of the asymptomatic brain infarction was cerebral large vessel vasculitis associated with neuropsychiatric SLE. Intravenous cyclophosphamide pulse therapy was started, and two months later, we confirmed that the irregular arterial narrowing had markedly ameliorated.Cerebral large vessel vasculitis in neuropsychiatric SLE is very rare, and a marked amelioration has not been reported to date. Here, we present a rare case of cerebral large vessel vasculitis treated successfully with a clear visual presentation. PMID:25661835

  9. 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

  10. Recurrent ALK-negative anaplastic large T-cell lymphoma presenting as necrotizing vasculitis.

    PubMed

    Nambudiri, Vinod E; Aboutalebi, Amir; Granter, Scott R; Saavedra, Arturo

    2013-06-01

    Anaplastic large cell lymphoma (ALCL) is a T-cell lymphoma histologically characterized by expression of CD30, a cell surface receptor present on activated T cells and B cells. ALCL may occur in a primary cutaneous form or as systemic ALCL with lymph node involvement. Anaplastic lymphoma kinase (ALK) is a tyrosine kinase that induces neoplastic transformation as a result of translocational fusion with an activating promoter. The presence of ALK can be used to distinguish between primary cutaneous ALCL and systemic nodal ALCL in certain cases. Primary cutaneous and systemic ALCL metastatic to the skin are histologically indistinguishable. "Leukemic vasculitis"--an uncommon finding in cases of cutaneous leukemia and even more exceptional in cutaneous lymphoma--refers to a pattern of vasculitis occurring as a direct result of infiltrating neoplastic cells. We report a fatal case of recurrent ALK-negative ALCL presenting as ulcerating skin lesions in a patient previously treated with the new anti-CD30 agent brentuximab vedotin. Biopsy revealed a necrotizing vasculitis resulting from the infiltration of neoplastic cells reminiscent of the patient's primary malignancy. We review the clinical and pathological findings of ALCL and present this case to highlight a subtle diagnostic clue in assessing recurrence of cutaneous lymphoma. PMID:23291583

  11. 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.

  12. 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

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

    PubMed Central

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

    2013-01-01

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

  14. Anti-neutrophil cytoplasmic antibody vasculitis presenting with bilateral renal vein thrombosis

    PubMed Central

    Robson, Michael Gregory

    2012-01-01

    We report a case of anti-neutrophil cytoplasmic antibody (ANCA)-associated necrotizing crescentic glomerulonephritis presenting with bilateral renal vein thrombosis and pulmonary emboli in a patient who also had a lupus anticoagulant and anti-cardiolipin antibodies. Although the link between venous thrombosis and ANCA vasculitis is well established, the coexistence of renal vein thrombosis is unusual. Furthermore, despite the positive ANCA, he was initially negative for antibodies to myeloperoxidase (MPO) and proteinase-3 (PR3), illustrating that a positive ANCA may be significant despite a negative test for antibodies to MPO and PR3.

  15. 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

  16. Nafcillin Implicated in A Case of Cutaneous and Gastrointestinal Leukocytoclastic Vasculitis

    PubMed Central

    Xie, Changqing; Pancholi, Suchita S

    2015-01-01

    Leukocytoclastic vasculitis (LV) is a rare hypersensitive reaction involving the small vessels, which is usually mediated by drugs. Very few cases of nafcillin -associated LV have been reported. Here, we reported a case of LV with the presentation of skin rashes and gastrointestinal bleeding after receiving nafcillin, evidenced by endoscopy and skin biopsy. The symptoms resolved after withdrawal of nafcillin and the addition of prednisone treatment. LV should be considered in the differential diagnosis of erythematous rash, especially with gastrointestinal symptoms after the exposure.

  17. Mixed Connective Tissue Disease Associated with Skin Defects of Livedoid Vasculitis

    Microsoft Academic Search

    Y. B. Oh; J.-B. Jun; C. K. Kim; C. W. Lee; C. K. Park; T.-Y. Kim; D.-H. Yoo; S. Y. Kim

    2000-01-01

    :   A 21-year-old woman who had a 2-year history of mixed connective tissue disease (MCTD) developed rapidly evolving ulcers\\u000a consistent with livedoid vasculitis (LV) in all distal extremities. She presented clinically with Raynaud’s phenomenon, polyarthritis\\u000a and swollen hands; serologically with high titres of ANA and anti-nRNP; and immunogenetically with HLA-DR4 and HLA-DR53. Although\\u000a there was initial success in treatment except

  18. Lymphocytic vasculitis associated with the anthrax vaccine: case report and review of anthrax vaccination.

    PubMed

    Muñiz, Antonio E

    2003-10-01

    Anthrax is caused by the spore-forming bacteria Bacillus anthracis. It occurs naturally, but recently has been manufactured as a biological warfare agent. This makes prophylaxis for anthrax an urgent concern and efforts are ongoing for the production of an efficient and safe vaccine. Side effects to the current anthrax vaccine are usually minor and mainly consist of local skin reactions. Occasionally an unusual complication may occur; a case of a patient with lymphocytic vasculitis temporally associated with the anthrax vaccine is reported. PMID:14585454

  19. Occurrence of relapsing polychondritis with a rising cANCA titre in a cANCA-positive systemic and cerebral vasculitis patient.

    PubMed

    Mattiassich, Georg; Egger, Markus; Semlitsch, Georg; Rainer, Franz

    2013-01-01

    Relapsing polychondritis (RP) is a relatively rare disorder, with a high death rate that affects cartilaginous structures. RP can be either primary or secondary as part of autoimmune syndromes. We present a case of RP in a 49-year-old man suffering from cytoplasmic antineurophil cytoplasmic antibodies (cANCA) positive vasculitis, admitted to our hospital with red swollen left ear and painful sternoclavicular joint and larynx. The patient was in remission from the vasculitis but manifested a high cANCA titre indicating vasculitis activity. With his high cANCA titre vasculitis, full manifestation of RP concomitantly occurred. After a successful cortisone treatment for RP, the patient received cyclophosphamide treatment for his vasculitis that resulted in a decrease in cANCA titre and full remission of his RP symptoms. PMID:23417970

  20. [Orbital apex syndrome without MRI lesion caused by ANCA-associated vasculitis].

    PubMed

    Iwanami, Hiroaki; Katoh, Hirotaka; Ohnaka, Youhei; Nakajima, Masashi; Kawamura, Mitsuru

    2014-01-01

    A 73-year-old man developed double vision and a progressive loss of visual acuity of the left eye over one week. Examination showed disturbances of the left II, III, IV, and VI cranial nerves, that is, an orbital apex syndrome. A brain MRI showed abnormal T2-high signals in the right maxillary sinus and the left mastoid cells without abnormalities in the left orbital apex and the surroundings. Laboratory examination showed an elevated erythrocyte sedimentation rate and a positive perinuclear anti-neutrophil cytoplasmic antibody (MPO-ANCA). After two courses of methyl-prednisolone pulse treatment, his external ophthalmoplegia fully recovered and he regained his left eye's sight. MPO-ANCA was negative and MRI abnormalities were disappeared after treatment. Two years later, the patient developed upper respiratory symptoms associated with an elevation of MPO-ANCA titer, and rapidly progressive renal failure. Renal biopsy specimen showed fibrinoid necrosis with periarteriolar neutrophil infiltration, which suggested that the patient suffered from ANCA-associated vasculitis probably of Wegener's granulomatosis or microscopic polyarteritis. ANCA-associated vasculitis may present with a focal neurological syndrome such as the orbital apex syndrome without a lesion detectable with MRI. PMID:24583592

  1. [Case of CNS-limited ANCA-associated vasculitis presenting as recurrent ischemic stroke].

    PubMed

    Wakisaka, Kayo; Hagiwara, Noriko; Kanazawa, Yuka; Arakawa, Shuji; Ago, Tetsuro; Kitazono, Takanari

    2014-01-01

    A 73-year-old man was admitted to our hospital because of a decrease in spontaneity. His medical history included two stroke episodes, probably related to hypertension. Brain MRI on admission demonstrated acute infarction in the right caudate nucleus and left putamen. Intravenous infusion of a low molecular-weight heparin added to oral antiplatelets was started. Following admission, he developed a low grade fever and severe inflammatory reaction. The focus of infection was not evident, and none of the antibiotics tried were effective. Ten days after admission, he developed right hemiparesis, and an additional brain MRI showed new multiple infarctions. We also determined the presence of a high MPO-ANCA titer (57 EU), and we diagnosed the patient's condition to be ANCA-associated vasculitis (AAV). Steroid therapy improved his inflammatory reaction and stroke recurrence was not observed. We suggest that vasculitis should be considered as a potential risk factor for repeated small infarctions with fever of unknown origin, especially those of perforating artery territories. PMID:24943081

  2. An overview of pharmacotherapy for anti-neutrophil cytoplasmic antibody-associated vasculitis.

    PubMed

    Stasi, R

    2010-12-01

    A number of recently published prospective clinical trials have enabled the definition of the type, duration and intensity of immunosuppressive treatment in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Patients with early or localized nonrenal disease should receive an induction therapy consisting of oral prednisone in combination with oral or parenteral methotrexate. Patients with generalized or severe disease, including those with renal vasculitis, should receive 6-10 pulses of cyclophosphamide over 3-6 months or 3-6 months of daily oral cyclophosphamide plus tailing doses of prednisone. Remission therapy consists of azathioprine plus low-dose prednisone given for at least 18-24 months after the achievement of remission. Despite their efficacy, treatment with conventional agents is associated with a number of problems, including a high number of relapses and considerable morbidity. To overcome these issues, research has focused on novel immunosuppressive drugs, such as mycophenolate mofetil and leflunomide, and on molecular targeted therapy. B-cell depletion with rituximab has been shown to be at least as effective as cyclophosphamide in inducing remission in AAV, but it is not safer. TNF-? blockade with infliximab is a promising strategy for refractory disease, but evidence from controlled trials is still lacking. With increased understanding of the disease, novel agents and therapy principles are rapidly emerging and undergoing clinical testing. PMID:21589949

  3. Spontaneous coronary artery thrombosis in the setting of active lupus mesenteric vasculitis.

    PubMed

    Reddy, Yogesh N V; Sundaram, V; Tam, M; Parikh, S A

    2015-07-01

    A 33-year-old male with systemic lupus erythematosus (SLE) presented with acute abdominal pain and was found to have lupus mesenteric vasculitis on imaging and during exploratory laparotomy. Post laparotomy he continued to have persistent nausea and dyspepsia and an electrocardiogram showed evidence of an inferior ST elevation myocardial infarction (STEMI). Emergency cardiac catheterization showed evidence of thrombotic right coronary artery occlusion. His coronaries were otherwise normal with no evidence of underlying coronary artery disease. Extensive workup with trans-esophageal echo, serologies for antiphospholipid antibody syndrome (APS) and bubble study was negative. This effectively ruled out Libman-Sacks endocarditis, APS-induced arterial thrombus and paradoxical emboli as potential causes of his STEMI. By exclusion of other causes, the etiology of his STEMI was felt to be secondary to in-situ coronary artery thrombosis in the setting of active SLE. To the best of our knowledge, this is the first report of a patient with SLE presenting with both lupus mesenteric vasculitis and in-situ coronary arterial thrombosis in the absence of APS. PMID:25672373

  4. 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

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

    PubMed

    Tesar, Vladimir

    2015-07-01

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

  6. Management of ANCA-associated vasculitis: Current trends and future prospects

    PubMed Central

    Hamour, Sally; Salama, Alan D; Pusey, Charles D

    2010-01-01

    The antineutrophil cytoplasm antibody (ANCA)-associated vasculitides are a spectrum of heterogeneous autoimmune diseases characterized by necrotizing small vessel vasculitis and the presence of ANCA. These chronic multisystem disorders may be life-threatening if there is major organ involvement, such as acute renal failure or pulmonary hemorrhage, and require significant initial immunosuppression and long-term maintenance treatment. Long-established protocols using cyclophosphamide and prednisolone have resulted in dramatically improved outcomes for patients since the 1970s. Subsequently, international collaboration has contributed to a growing evidence base and consensus in the management of these rare disorders. Modifications to traditional treatment protocols by the use of azathioprine or methotrexate rather than cyclophosphamide, and the introduction of newer agents, such as rituximab, has maintained outcomes whilst decreasing toxicity. However, the treatment limitations of incomplete efficacy, infection, and cumulative toxicity persist. These issues have continued to drive the search for safer and more effective modulation of the immune system using targeted immunotherapy. This review will explore the current evidence base for management of ANCA-associated vasculitis and future treatment prospects. PMID:20596502

  7. Necrotizing mycotic vasculitis with cerebral infarction caused by Aspergillus niger in a horse with acute typholocolitis.

    PubMed

    Tunev, S S; Ehrhart, E J; Jensen, H E; Foreman, J H; Richter, R A; Messick, J B

    1999-07-01

    An 18-year-old Morgan mare was presented to the Veterinary Medical Teaching Hospital, University of Illinois, with a 10-day history of watery diarrhea, depression, and dysphagia. On admission, the animal was severely dehydrated, depressed, and unable to swallow and had no clinical signs of diarrhea. The respiratory and heart rate and body temperature were within normal limits. Following fluid therapy, the mare developed severe watery diarrhea and continued to be depressed, incoordinated, and dysphagic. The animal died on the fourth day after admission and was sent to the Laboratories of Veterinary Diagnostic Medicine for necropsy. Gross postmortem findings were consistent with an acute cerebral infarction in the right cerebral hemisphere, an acute necrotizing typhlocolitis, multifocal petechial and ecchymotic hemorrhages, enlarged and congested pars intermedia of the pituitary gland, and marked bilateral adrenocortical hyperplasia with multifocal areas of necrosis and hemorrhage. Histologic evaluation of the affected brain demonstrated an area of coagulative necrosis of the gray matter, with hemorrhage, vasculitis, and thrombosis. There were many fungal hyphae 3.5-6.0 microm, pale basophilic, septate, and occasionally branching at 45 degrees present in the arterial walls and throughout the necrotic tissue. Immunohistochemical analysis revealed Aspergillus niger as the etiologic agent responsible for the mycotic vasculitis and infarction in the brain. Bacteria culture and immunohistochemical staining of the colon and cecum failed to demonstrate specific pathogens. PMID:10421105

  8. No difference in the incidences of vasculitides between north and south Germany: first results of the German vasculitis register

    Microsoft Academic Search

    E. Reinhold-Keller; K. Herlyn; R. Wagner-Bastmeyer; J. Gutfleisch; H. H. Peter; H. H. Raspe; W. L. Gross

    2002-01-01

    Objective. To register all newly diagnosed patients with primary systemic vasculitis (PSV) in two large regions in north and south Germany. Methods. Between 1 January 1998 and 31 December 1999, all newly diagnosed cases of PSV, as defined by the Chapel Hill Consensus Conference 1992, were identified in two large mixed ruraluurban regions in north and south Germany with a

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

    E-print Network

    Mascaro, Stephen A.

    Wet Shape Memory Alloy Actuators for Active Vasculated Robotic Flesh Stephen A. Mascaro and H is presented where Shape Memory Alloy (SMA) wires are embedded within artificial "blood vessels." Fluid flowing: Biologically Inspired Actuators The human body is filled with a variety of fluids and extensive networks

  10. 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

  11. 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

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

    PubMed

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

    2012-01-01

    This article attempts to perform an evaluation of the state of the art of the economic and societal burden of systemic vasculitis (VAs). Due to the rarity of these diseases and their variable clinical picture, few data are available in the literature on their health economic issues, and only some papers have been published that marginally examine the problem. Since VAs are severe conditions with a high medical and societal impact and determine high healthcare resource consumption, studies able to define societal, quality of life and economic burden of these pathologies are needed. Policy makers, private and public organisations involved in the care of VAs need data to programme future investment or make cost-effectiveness analysis for introducing new drugs or protocols. PMID:23072925

  13. Treatment of vasculitis and dermatitis in a 59-yr-old Nile hippopotamus (Hippopotamus amphibius).

    PubMed

    Spriggs, Maria; Reeder, Chris

    2012-09-01

    A 59-yr-old female Nile hippopotamus (Hippopotamus amphibius) was diagnosed and treated for severe dermatitis. Lesions included large areas of depigmentation, erosions, and ulcerations on glabrous skin areas, limbs, and perineal region. Histopathologic lesions included a markedly edematous, focally eroded, ulcerative to necrotic epidermis; foci of keratinocyte apoptosis; and a mixed suppurative dermatitis. Most of the dermal vessels had variable hyalinized walls with plump endothelial cells and frequent intramural neutrophils, and some vessels had vascular thrombi consistent with vasculitis. Culture of the lesions yielded beta-hemolytic Streptococcus, Morganella morgannii, and Enterococcus sp. The hippopotamus was successfully treated with sulfamethoxazole and trimethoprim, amoxicillin, and pentoxifylline for more than 2 mo, and the condition did not recur over the subsequent 16 mo. PMID:23082536

  14. [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

  15. Anti-Plasminogen Antibodies Compromise Fibrinolysis and Associate with Renal Histology in ANCA-Associated Vasculitis

    PubMed Central

    Berden, Annelies E.; Nolan, Sarah L.; Morris, Hannah L.; Bertina, Rogier M.; Erasmus, Dianhdra D.; Hagen, E. Christiaan; Hayes, Donal P.; van Tilburg, Nico H.; Bruijn, Jan A.; Savage, Caroline O.S.; Bajema, Ingeborg M.

    2010-01-01

    Antibodies recognizing plasminogen, a key component of the fibrinolytic system, associate with venous thrombotic events in PR3-ANCA vasculitis. Here, we investigated the prevalence and function of anti-plasminogen antibodies in independent UK and Dutch cohorts of patients with ANCA-associated vasculitis (AAV). We screened Ig isolated from patients (AAV-IgG) and healthy controls by ELISA. Eighteen of 74 (24%) UK and 10/38 (26%) Dutch patients with AAV had anti-plasminogen antibodies compared with 0/50 and 1/61 (2%) of controls. We detected anti-plasminogen antibodies in both PR3-ANCA– and MPO-ANCA–positive patients. In addition, we identified anti-tissue plasminogen activator (tPA) antibodies in 13/74 (18%) patients, and these antibodies were more common among patients with anti-plasminogen antibodies (P = 0.011). Eighteen of 74 AAV-IgG (but no control IgG) retarded fibrinolysis in vitro, and this associated with anti-plasminogen and/or anti-tPA antibody positivity. Only 4/18 AAV-IgG retarded fibrinolysis without harboring these antibodies; dual-positive samples retarded fibrinolysis to the greatest extent. Patients with anti-plasminogen antibodies had significantly higher percentages of glomeruli with fibrinoid necrosis (P < 0.05) and cellular crescents (P < 0.001) and had more severely reduced renal function than patients without these antibodies. In conclusion, anti-plasminogen and anti-tPA antibodies occur in AAV and associate with functional inhibition of fibrinolysis in vitro. Seropositivity for anti-plasminogen antibodies correlates with hallmark renal histologic lesions and reduced renal function. Conceivably, therapies that enhance fibrinolysis might benefit a subset of AAV patients. PMID:20847144

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

    PubMed

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

    2015-02-01

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

  17. Administration of human immunoglobulin suppresses development of murine systemic vasculitis induced with Candida albicans water-soluble fraction: an animal model of Kawasaki disease

    Microsoft Academic Search

    Kei Takahashi; Toshiaki Oharaseki; Yuki Yokouchi; Noriko N. Miura; Naohito Ohno; Akiko I. Okawara; Hisao Murata; Shiro Naoe; Kazuo Suzuki

    2010-01-01

    We investigated the inhibitory effect of human immunoglobulin (h-Ig) on the development of coronary arteritis in a murine\\u000a model of vasculitis induced with a Candida albicans water-soluble fraction (CAWS). CAWS was intraperitoneally injected to C57BL\\/6 mice for 5 days. Then h-Ig was administered\\u000a according to various schedules. The animals were sacrificed in week 5, and the status of vasculitis in the coronary

  18. Oxaliplatin-Induced Leukocytoclastic Vasculitis under Adjuvant Chemotherapy for Colorectal Cancer: Two Cases of a Rare Adverse Event.

    PubMed

    Quack, Henriette; Erpenbeck, Luise; Wolff, Hendrik A; Sprenger, Thilo; Seitz, Cornelia S; Schön, Michael P; Neumann, Steffen; Stanek, Kathrin; Ghadimi, B Michael; Michels, Beate; Middel, Peter; Schaefer, Inga-Marie; Liersch, Torsten; Conradi, Lena-Christin

    2013-09-01

    Leukocytoclastic vasculitis is a multicausal systemic inflammatory disease of the small vessels, histologically characterized by inflammation and deposition of both nuclear debris and fibrin in dermal postcapillary venules. The clinical picture typically involves palpable purpura of the lower legs and may be associated with general symptoms such as fatigue, arthralgia and fever. Involvement of the internal organs, most notably the kidneys, the central nervous system or the eyes, is possible and determines the prognosis. Oxaliplatin-induced leukocytoclastic vasculitis is a very rare event that limits treatment options in affected patients. We report 2 patients who developed the condition under chemotherapy for advanced rectal and metastatic colon carcinoma, respectively; a termination of the therapy was therefore necessary. While current therapies for colorectal cancer include the combination of multimodal treatment with new and targeted agents, rare and unusual side effects elicited by established agents also need to be taken into account for the clinical management. PMID:24474925

  19. Oxaliplatin-Induced Leukocytoclastic Vasculitis under Adjuvant Chemotherapy for Colorectal Cancer: Two Cases of a Rare Adverse Event

    PubMed Central

    Quack, Henriette; Erpenbeck, Luise; Wolff, Hendrik A.; Sprenger, Thilo; Seitz, Cornelia S.; Schön, Michael P.; Neumann, Steffen; Stanek, Kathrin; Ghadimi, B. Michael; Michels, Beate; Middel, Peter; Schaefer, Inga-Marie; Liersch, Torsten; Conradi, Lena-Christin

    2013-01-01

    Leukocytoclastic vasculitis is a multicausal systemic inflammatory disease of the small vessels, histologically characterized by inflammation and deposition of both nuclear debris and fibrin in dermal postcapillary venules. The clinical picture typically involves palpable purpura of the lower legs and may be associated with general symptoms such as fatigue, arthralgia and fever. Involvement of the internal organs, most notably the kidneys, the central nervous system or the eyes, is possible and determines the prognosis. Oxaliplatin-induced leukocytoclastic vasculitis is a very rare event that limits treatment options in affected patients. We report 2 patients who developed the condition under chemotherapy for advanced rectal and metastatic colon carcinoma, respectively; a termination of the therapy was therefore necessary. While current therapies for colorectal cancer include the combination of multimodal treatment with new and targeted agents, rare and unusual side effects elicited by established agents also need to be taken into account for the clinical management. PMID:24474925

  20. Cerebral vascular findings in PAPA syndrome: cerebral arterial vasculopathy or vasculitis and a posterior cerebral artery dissecting aneurysm.

    PubMed

    Khatibi, Kasra; Heit, Jeremy J; Telischak, Nicholas A; Elbers, Jorina M; Do, Huy M

    2015-01-01

    A young patient with PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne) syndrome developed an unusual cerebral arterial vasculopathy/vasculitis (CAV) that resulted in subarachnoid hemorrhage from a ruptured dissecting posterior cerebral artery (PCA) aneurysm. This aneurysm was successfully treated by endovascular coil sacrifice of the affected segment of the PCA. The patient made an excellent recovery with no significant residual neurologic deficit. PMID:26109622

  1. CAWS administration increases the expression of interferon ? and complement factors that lead to severe vasculitis in DBA/2 mice

    PubMed Central

    2013-01-01

    Background Candida albicans water-soluble fraction (CAWS), a mannoprotein-?-glucan complex obtained from the culture supernatant of C. albicans NBRC1385, causes CAWS-mediated vasculitis (CAWS-vasculitis) in B6 and DBA/2 mice with mild and lethal symptoms, respectively. Why CAWS is lethal only in DBA/2 mice remains unknown. Results We performed DNA microarray analyses using mRNA obtained from peripheral blood mononuclear cells (PBMCs) of B6 and DBA/2 mice and compared their respective transcriptomes. We found that the mRNA levels of interferon-? (Ifng) and several genes that regulate the complement system, such as C3, C4, Cfb, Cfh, and Fcna, were increased dramatically only in DBA/2 mice at 4 and 8 weeks after CAWS administration. The dramatic increase was confirmed by quantitative real-time polymerase chain reactions (qRT-PCR). Moreover, mRNA levels of immune-related genes, such as Irf1, Irf7, Irf9, Cebpb, Ccl4, Itgam, Icam1, and IL-12rb1, whose expression levels are known to be increased by Ifng, were also increased, but only in DBA/2 mice. By contrast, the mRNA level of Dectin-2, the critical receptor for the ?-mannans of CAWS, was increased slightly and similarly in both B6 and DBA/2 mice after CAWS administration. Conclusions Taken together, our results suggest that CAWS administration induces Dectin-2 mediated CAWS-vasculitis in both B6 and DBA/2 mice and the expression of Ifng, but only in DBA/2 mice, which led to increased expression of C3, C4, Cfb, Cfh, and Fcna and an associated increase in lethality in these mice. This model may contribute to our understanding of the pathogenesis of severe human vasculitis. PMID:24063402

  2. 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

  3. 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

  4. A Randomized Controlled Trial of Rituximab Following Failure of Antiviral Therapy for Hepatitis C-Associated Cryoglobulinemic Vasculitis

    PubMed Central

    Sneller, Michael C.; Hu, Zonghui; Langford, Carol A.

    2011-01-01

    OBJECTIVES To report on the results of a randomized controlled trial of rituximab in hepatitis C virus (HCV)-associated mixed cryoglobulinemic vasculitis. METHODS We conducted an open-label single center randomized controlled trial of rituximab (375 mg/m2 per week for 4 weeks) compared to best available therapy for treatment of patients with HCV-associated cryoglobulinemic vasculitis in whom antiviral therapy failed to induce remission. The primary endpoint was remission at 6 months from study entry. RESULTS A total of 24 patients were enrolled. Baseline disease activity and organ involvement were similar in the two groups. Ten patients in the rituximab group (83%) were in remission at study month 6, compared with 1 patient in the control group (8%), a result that met criterion for stopping the study (P<0.001). The median duration of remission for rituximab-treated patients reaching the primary endpoint was 7 months. No adverse effect of rituximab on HCV plasma viremia or hepatic transaminase levels was observed. CONCLUSIONS Therapy with rituximab was well tolerated and effective treatment for patients with HCV-associated cryoglobulinemic vasculitis in whom antiviral therapy fails to induce remission. PMID:22147444

  5. An Unusual Presentation of Childhood Vasculitis Presenting in Adulthood: A Challenging Diagnosis of Henoch-Schönlein Purpura

    PubMed Central

    Thongprayoon, Charat; Cheungpasitporn, Wisit; Thamcharoen, Natanong; Bruminhent, Jackrapong

    2014-01-01

    Context: Henoch-Schönlein purpura (HSP), a systemic IgA vascultitis, is uncommon in adults, with an incidence rate of 0.1 to 1.2 per million in adults over 20 years old. This vasculitic syndrome can present as an uncommon cause of intestinal obstruction in older patients. We report a case of an older woman with HSP presenting with small bowel obstruction and vasculitic rash. Case Report: We report a 67-year-old woman who presented with small bowel obstruction and skin rash. Skin biopsy revealed leukocytoclastic vasculitis with +IgA granular deposition within the walls of superficial dermal vessels. Kidney biopsy confirmed the diagnosis of HSP with mild mesangial proliferative IgA nephropathy. Her abdominal pain and small bowel obstruction were improved with conservative treatment. She continued to do well with normal kidney function at a 3-month follow-up visit. Conclusion: HSP, a systemic IgA vasculitis, is a predominantly pediatric vasculitis and is uncommon in adults. In adults, the disease process is identical to that in children. However, gastrointestinal manifestation is less common in older patients, and bowel perforation and obstruction are rare. Intestinal obstruction with skin rash and renal involvement should raise suspicions of HSP. PMID:25489569

  6. Anti-oxidized low-density lipoprotein antibodies in myeloperoxidase–positive vasculitis patients preferentially recognize hypochlorite-modified low density lipoproteins

    PubMed Central

    Slot, M C; Theunissen, R; van Paassen, P; Damoiseaux, J G M C; Cohen Tervaert, J W

    2007-01-01

    Many patients surviving vasculitis are prone to accelerated atherosclerosis and often have enhanced levels of antibodies to oxidized low-density lipoprotein (oxLDL). To measure anti-oxLDL antibodies, oxidation of LDL is achieved with copper (Cu) or malondialdehyde (MDA). Because, in vivo, LDL may be oxidized with myeloperoxidase (MPO) or its product hypochlorite, we measured anti-hypochlorite LDL antibodies in patients with vasculitis, haemodialysis patients and healthy controls. A newly developed enzyme-linked immunosorbent assay (ELISA) was used to detect antibodies to oxLDL as modified by hypochlorite. Results are compared with data obtained by standard LDL oxidation using MDA–LDL or Cu–LDL as substrate. Results were compared between anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) patients (n = 93), haemodialysis (HD) patients (n = 59) and healthy controls (HC; n = 43). Furthermore, patients with MPO–ANCA-associated vasculitis (n = 47) were compared with patients with proteinase 3 (PR3)–ANCA associated vasculitis (n = 46). Optimal cut-off points were determined by receiver operator characteristic (ROC) curve analysis. Anti-oxLDL antibodies are enhanced in AAV patients (MDA–LDL and hypochlorite–LDL) and in HD patients (hypochlorite–LDL), when compared to HC. Furthermore, patients with MPO–ANCA-associated vasculitis had higher levels of antibodies to hypochlorite–LDL than patients with PR3–ANCA-associated vasculitis. Our newly developed assay, in which hypochlorite–LDL is used as substrate, seems a more sensitive assay than traditional assays to measure oxLDL antibodies. Furthermore, our results suggest that enhanced MPO-mediated LDL oxidation occurs in patients with MPO–ANCA. PMID:17521320

  7. Neutrophil and recombinant myeloperoxidase as antigens in ANCA positive systemic vasculitis.

    PubMed Central

    Short, A K; Lockwood, C M; Bollen, A; Moguilevsky, N

    1995-01-01

    Myeloperoxidase (MPO) is one of the major autoantigens recognized by anti-neutrophil cytoplasm antibodies. The association of this antigen with specific disease entities requires that there is a source of pure antigen present in large quantities. Further delineation of the molecular mechanisms involved in the antigen-antibody interaction requires the ability to manipulate the molecule. The expression of recombinant MPO in Chinese hamster ovary cells has produced a source of pure protein, suitable for molecular studies. We have shown that this protein is an antigen recognized by 95% of anti-MPO antibodies from patients with systemic vasculitis. This recombinant molecule will be of use in providing an additional specific solid-phase assay for these antibodies and further forms of this protein which mirror the antigenicity of native MPO more exactly may replace chemically purified antigen. It will also be of great value in studies examining the epitopes recognized by anti-MPO antibodies and in studies of immunoregulation and T cell activation. Images Fig. 2 PMID:7554375

  8. 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

  9. 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

  10. 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

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

    PubMed Central

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

    2014-01-01

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

  12. Classification, epidemiology and clinical subgrouping of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis.

    PubMed

    Watts, Richard A; Mahr, Alfred; Mohammad, Aladdin J; Gatenby, Paul; Basu, Neil; Flores-Suárez, Luis Felipe

    2015-04-01

    It is now 25 years since the first European studies on vasculitis--the anti-neutrophil cytoplasmic antibody (ANCA) standardization project. Over that period of time, there have been major developments in the classification of the vasculitides, which has permitted the conduct of high-quality epidemiology studies. Studying the epidemiology of rare diseases such as the ANCA-associated vasculitides (AAV) poses considerable challenges to epidemiologists. The first is the need for a clear definition of a case with good differentiation from similar disorders. The second is case capture. The vasculitides are rare, and therefore, a large population is required to determine the incidence and prevalence, and this poses questions of feasibility. A large population increases the risk of incomplete case detection but permits a reasonable number of cases to be collected in a practicable time frame, whereas a smaller population requires a much longer time frame to collect the necessary cases, which may also not be feasible. Statistical methods of capture-recapture analysis enable estimates to be made of the number of missing cases. The third is case ascertainment. The AAV are virtually always managed in secondary care, and therefore, hospital-based case ascertainment may be appropriate. Fourthly, the rarity of the conditions makes prospective case-control studies investigating risk factors difficult to conduct because the population size required to achieve statistical confidence is in excess of that which is readily available. Thus, much of the data on risk factors are derived from retrospective studies with inherent potential bias. PMID:25805746

  13. Interaction between CX3CL1 and CX3CR1 regulates vasculitis induced by immune complex deposition.

    PubMed

    Morimura, Sohshi; Sugaya, Makoto; Sato, Shinichi

    2013-05-01

    A type III hypersensitivity reaction induced by an immune complex, such as leukocytoclastic vasculitis, is mediated by inflammatory cell infiltration that is highly regulated by multiple adhesion molecules. CX3CL1, a ligand for CX3C chemokine receptor 1 (CX3CR1), has recently been identified as a key mediator of leukocyte adhesion that functions without the recruitment of integrins or selectin-mediated rolling. To elucidate the role of CX3CL1 and CX3CR1 in the development of leukocytoclastic vasculitis, the cutaneous and peritoneal reverse Arthus reactions, classic experimental models for immune complex-mediated tissue injury, were examined in mice lacking CX3CR1. CX3CL1 expression in sera and lesional skin of patients with polyarteritis nodosa (PN) and healthy controls was also examined. Edema and hemorrhage were significantly reduced in CX3CR1(-/-) mice compared with wild-type mice. Infiltration of neutrophils and mast cells and expression of IL-6 and tumor necrosis factor-? were also decreased in CX3CR1(-/-) mice. CX3CL1 was expressed in endothelial cells during the cutaneous reverse Arthus reactions. Furthermore, serum CX3CL1 levels were significantly higher in patients with PN than in healthy controls. Endothelial cells in lesional skin of patients with PN strongly expressed CX3CL1. These results suggest that interactions between CX3CL1 and CX3CR1 may contribute to the development of leukocytoclastic vasculitis by regulating neutrophil and mast cell recruitment and cytokine expression. PMID:23470165

  14. High Prevalence of Autoantibodies to hLAMP-2 in Anti–Neutrophil Cytoplasmic Antibody–Associated Vasculitis

    PubMed Central

    Tadema, Henko; McKinney, Eoin F.; Benharkou, Alexandra; Brandes, Ricarda; Peschel, Andrea; Hubert, Virginie; Feenstra, Tjerk; Sengölge, Gürkan; Stegeman, Coen; Heeringa, Peter; Lyons, Paul A.; Smith, Kenneth G.C.; Kallenberg, Cees; Rees, Andrew J.

    2012-01-01

    The involvement of autoantibodies to human lysosome-associated membrane protein-2 (hLAMP-2) in anti–neutrophil cytoplasmic antibody (ANCA)–associated vasculitis is controversial because of the absence of confirmatory data subsequent to the initial reports of their high prevalence in this disease. We characterized three assays for anti-hLAMP-2 antibodies: ELISA and Western blotting assays using unglycosylated recombinant hLAMP-2 expressed in Escherichia coli, and an indirect immunofluorescence assay using stably transfected ldlD cells that expressed glycosylated full-length hLAMP-2 on the plasma membrane. The assays detected autoantibodies to hLAMP-2 in human sera reproducibly and with comparable sensitivity and the assays gave the same results in 80.5% of the test panel of 40 selected positive and negative sera. In untreated patients at presentation, the frequencies of autoantibodies to LAMP-2 were 89%, 91%, and 80%, respectively, among three groups of patients with ANCA-associated vasculitis from Vienna, Austria (n=19); Groningen, the Netherlands (n=50) and Cambridge, United Kingdom (n=53). Prevalence of LAMP-2 autoantibodies was similar in both those with myeloperoxidase-ANCA and proteinase 3-ANCA. Furthermore, we detected LAMP-2 autoantibodies in two ANCA-negative patients. LAMP-2 autoantibodies rapidly became undetectable after the initiation of immunosuppressive treatment and frequently became detectable again during clinical relapse. We conclude that when robust assays are used, circulating autoantibodies to hLAMP-2 can be detected in most European patients with ANCA-associated vasculitis. Large-scale prospective studies are now needed to determine whether they are pathogenic or merely an epiphenomenon. PMID:22323643

  15. Con: Should all patients with anti-neutrophil cytoplasmic antibody-associated vasculitis be primarily treated with rituximab?

    PubMed

    Kronbichler, Andreas; Jayne, David R W

    2015-07-01

    Rituximab has enriched our armamentarium in the treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Two randomised controlled trials have shown that rituximab is non-inferior compared with cyclophosphamide followed by azathioprine for the induction of remission. The newly diagnosed patients in the Rituximab in ANCA-Associated Vasculitis (RAVE) and Rituximab Versus Cyclophosphamide in ANCA-Associated Vasculitis (RITUXVAS) trials had a numerically higher response rate in the cyclophosphamide/azathioprine arm, and the number of such patients treated with rituximab numbered <90. We are arguing that in newly diagnosed patients, the evidence for rituximab requires further confirmation and the length of experience with a cyclophosphamide-based induction therapy supports it continuing as the preferred first choice for induction. Also, there is an absence of information regarding rituximab as 'sole' remission induction along with steroids in patients with advanced renal presentations or lung haemorrhage. Reported side effects were similar in both trials; however, the number of participants with at least one serious adverse event following rituximab induction treatment was numerically higher in the RAVE trial. In addition, hypogammaglobulinaemia with the need of substitution in some cases and late-onset neutropaenia are complications not seen with cyclophosphamide. Over the longer term it is unclear what relapse prevention strategy should be employed after rituximab, and there was a trend to a higher relapse risk after rituximab in the RITUXVAS trial at 2 years. Further health economic studies are required to understand all the costs associated with rituximab. In the context of concomitant underlying infectious complications, in terms of fertility concerns, especially in young patients, and when malignancy is underlying we would recommend the use of rituximab as first-line therapy. PMID:25999374

  16. [Clinical Characteristics of antineutrophil cytoplasmic antibody-associated vasculitis in a respiratory diseases referral center in Mexico (1982-2010)].

    PubMed

    Flores-Suárez, Luis Felipe; Alba, Marco A

    2015-01-01

    Introducción: A pesar de que las manifestaciones respiratorias en las vasculitis asociadas a ANCA son comunes, existen pocos reportes derivados de centros especializados en enfermedades respiratorias. Objetivo: Describir las manifestaciones clínicas, radiológicas, de exámenes de laboratorio y el pronóstico de los casos con vasculitis asociadas a ANCA de un centro de tercer nivel especializado en enfermedades respiratorias de la Ciudad de México. Materiales y métodos: Estudio retrospectivo que incluyó a todos los pacientes con vasculitis asociada a ANCA diagnosticados entre 1982 y 2010. Los casos fueron identificados en una base de datos de egresos y se clasificaron mediante los criterios del Colegio Americano de Reumatología (ACR) o las definiciones de la Conferencia de Consenso de Chapel Hill. Resultados: Se describen las características de 74 pacientes con granulomatosis con poliangitis (GPA), de 10 con poliangitis microscópica (MPA) y de 6 con granulomatosis eosinofílica con poliangitis (GEPA). El tiempo promedio entre la sospecha inicial y el diagnóstico definitivo fue de 30 meses. Al comparar esta serie con otras, las manifestaciones de las vías aéreas fueron semejantes, pero la estenosis subglótica (ESG) fue más frecuente. Durante el tiempo de seguimiento (promedio: 22 meses), la supervivencia en esta serie fue del 83%; el 87% de pacientes alcanzaron la remisión y el 9%, respuesta. Siete pacientes murieron, principalmente por infecciones. Conclusiones: Este estudio documenta que, en general, las manifestaciones de las vías aéreas de las VAA en mexicanos son semejantes a lo descrito en la literatura, pero el tiempo para arribar al diagnóstico es largo. Con los datos presentados, insistimos en las manifestaciones respiratorias principales de estos pacientes, particularmente para facilitar el diagnóstico a quienes se dedican a la medicina respiratoria. PMID:25946528

  17. What you should know about PR3-ANCA: Evidence for the role of T cells in the pathogenesis of systemic vasculitis

    PubMed Central

    Clayton, Abigail R; Savage, Caroline OS

    2000-01-01

    The pathogenesis of systemic vasculitis is complex and is likely to involve many mechanisms. There is a growing body of evidence that T cells may contribute to the pathogenesis of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Predominantly, T cells and monocytes are found in inflammatory infiltrates in patients with Wegener's granulomatosis (WG). The production of ANCA appears to be T-cell-dependent. T lymphocytes from the peripheral blood of patients with ANCA-associated vasculitis have been shown to proliferate in response to proteinase 3 (PR3). These and other findings outlined in this review indicate T-cell involvement, although further studies are still needed to elucidate the exact contribution of T cells to the pathogenesis of systemic vasculitis. PMID:11094438

  18. Development of anti-neutrophil cytoplasmic antibody-associated vasculitis in a patient with Graves' disease independent of anti-thyroid drug therapy.

    PubMed

    Khan, Nasim Ahmed; Singh, Manisha

    2012-09-01

    Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) in patients with Graves' disease (GD) is linked with the use of anti-thyroid drugs (ATDs). We report the co-occurrence of AAV and GD in a patient that was independent of ATD therapy. A 38-year-old white male presented with systemic symptoms, palpitations, tremors, purpuric skin lesions, and digital pain. Physical examination and biological tests confirmed GD. He quickly developed multiple digital gangrenes and testicular pain/mass. Skin and testicular biopsies showed granulomatous vasculitis of the small- and medium-sized vessels, while his serum contained anti-proteinase-3 antibody. PMID:22167487

  19. The Role of 18F-FDG PET/CT in Large-Vessel Vasculitis: Appropriateness of Current Classification Criteria?

    PubMed Central

    Balink, H.; Bennink, R. J.; van Eck-Smit, B. L. F.; Verberne, H. J.

    2014-01-01

    Patients with clinical suspicion of large-vessel vasculitis (LVV) may present with nonspecific signs and symptoms and increased inflammatory parameters and may remain without diagnosis after routine diagnostic procedures. Both the nonspecificity of the radiopharmaceutical 18F-FDG and the synergy of integrating functional and anatomical images with PET/CT offer substantial benefit in the diagnostic work-up of patients with clinical suspicion for LVV. A negative temporal artery biopsy, an ultrasonography without an arterial halo, or a MRI without aortic wall thickening or oedema do not exclude the presence of LVV and should therefore not exclude the use of 18F-FDG PET/CT when LVV is clinically suspected. This overview further discusses the notion that there is substantial underdiagnosis of LVV. Late diagnosis of LVV may lead to surgery or angioplasty in occlusive forms and is often accompanied by serious aortic complications and a fatal outcome. In contrast to the American College of Rheumatology 1990 criteria for vasculitis, based on late LVV effects like arterial stenosis and/or occlusion, 18F-FDG PET/CT sheds new light on the classification of giant cell arteritis (GCA) and Takayasu arteritis (TA). The combination of these observations makes the role of 18F-FDG PET/CT in the assessment of patients suspected for having LVV promising. PMID:25328890

  20. 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

  1. Development of outcome measures for large-vessel vasculitis for use in clinical trials: opportunities, challenges, and research agenda.

    PubMed

    Direskeneli, Haner; Aydin, Sibel Z; Kermani, Tanaz A; Matteson, Eric L; Boers, Maarten; Herlyn, Karen; Luqmani, Raashid A; Neogi, Tuhina; Seo, Philip; Suppiah, Ravi; Tomasson, Gunnar; Merkel, Peter A

    2011-07-01

    Giant cell (GCA) and Takayasu's arteritis (TAK) are 2 forms of large-vessel vasculitis (LVV) that involve the aorta and its major branches. GCA has a predilection for the cranial branches, while TAK tends to affect the extracranial branches. Both disorders may also cause nonspecific constitutional symptoms. Although some clinical features are more common in one or the other disorder and the ages of initial presentation differ substantially, there is enough clinical and histopathologic overlap between these disorders that some investigators suggest GCA and TAK may be 2 processes within the spectrum of a single disease. There have been few randomized therapeutic trials completed in GCA, and none in TAK. The lack of therapeutic trials in LVV is only partially explained by the rarity of these diseases. It is likely that the lack of well validated outcome measures for LVV and uncertainties regarding trial design contribute to the paucity of trials for these diseases. An initiative to develop a core set of outcome measures for use in clinical trials of LVV was launched by the international OMERACT Vasculitis Working Group in 2009 and subsequently endorsed by the OMERACT community at the OMERACT 10 meeting. Aims of this initiative include: (1) to review the literature and existing data related to outcome assessments in LVV; (2) to obtain the opinion of experts and patients on disease content; and (3) to formulate a research agenda to facilitate a more data-based approach to outcomes development. PMID:21724719

  2. Life-threatening allergic vasculitis after clipping an unruptured aneurysm: Case report, weighing the risk of nickel allergy

    PubMed Central

    Grande, Andrew; Grewal, Sanjeet; Tackla, Ryan; Ringer, Andrew J.

    2014-01-01

    Background: This case report represents one of the estimated 17,000 aneurysms clipped annually in the United States, often with nickel-containing clips. The authors highlight the development of life-threatening allergic vasculitis in a 33-year-old woman after aneurysm clipping. Case Description: After suffering subarachnoid hemorrhage, the patient had coil embolization at another facility for rupture of a right internal carotid artery (ICA) aneurysm. An incidental finding, an unruptured left posterior communicating artery aneurysm unamenable to coiling, was then successfully clipped via a left pterional craniotomy. Arriving in our emergency department 11 days later, she progressively declined during the next weeks, facing deteriorating clinical status (i.e. seizures) and additional infarctions in the left frontal lobe, midline shift, and new infarctions in the bilateral frontal lobe, right sylvian, right insular regions, and posterior cerebral artery distribution. During decompressive surgery, biopsy findings raised the possibility of lymphocytic vasculitis; consultations with rheumatology, allergy, and immunology specialists identified that our patient had a nickel allergy. After reoperation to replace the nickel-containing clip with one of a titanium alloy, the patient had an uncomplicated postoperative course and was discharged 6 days later to a rehabilitation facility. Conclusions: Nickel-related allergies are more common than appreciated, affecting up to 10% of patients. Fortunately, severe reactions are rare; nevertheless, vascular neurosurgeons should be aware of this potential complication when using cobalt alloy aneurysms clips. The use of titanium alloy clips eliminates this risk. PMID:25071940

  3. Detection rate and antigenic specificities of antineutrophil cytoplasmic antibodies in chinese patients with clinically suspected vasculitis.

    PubMed

    Xin, Gang; Zhao, Ming-Hui; Wang, Hai-Yan

    2004-05-01

    The detection rate of antineutrophil cytoplasmic antibodies (ANCA) in Chinese patients with clinically suspected small vessel vasculitis was investigated, and their antigen specificity and demographic features were analyzed. A number of sera (n = 5,604) sent to our referral laboratory for ANCA screening were tested by indirect immunofluorescence (IIF), enzyme-linked immunosorbent assays (ELISAs) for myeloperoxidase (MPO)- and proteinase 3 (PR3)-ANCA. Then the IIF-ANCA-positive sera that were negative for MPO- and PR3-ANCA were further tested by antigen-specific ELISA by using other five highly purified known ANCA antigens as solid-phase ligands. The known antigens included bactericidal/permeability-increasing protein (BPI), human leukocyte elastase (HLE), lactoferrin, cathepsin G, and azurocidins. Of the 5,604 sera, 267 (4.76%) sera were IIF-ANCA positive and 390 (7%) were antinuclear antibody (ANA) positive in the IIF assay. Of the IIF-positive samples, 213 were anti-MPO positive, 32 were anti-PR3 positive, and five cases were positive for both. Of the 48 sera positive for IIF-ANCA but negative for MPO- and PR3-ANCA, 13 sera (27%) recognized other target antigens, 7 sera recognized BPI, 5 recognized HLE, 1 recognize cathepsin G, and 1 recognized azurocidin. None of the sera recognized lactoferrin, and one serum sample recognized both BPI and HLE. The majority of ANCA-positive patients presented in summer or winter. There was no difference in gender (male/female ratio, 1:1.12) in ANCA-positive patients with a mean age of 53.1 years. The male/female ratio was 1.17:1 for patients over 60 years of age; however, it was 1:4 for patients under 20 years of age. We conclude that ANCA-related diseases are not rare in China, and the major antigens are MPO and PR3. When the IIF technique is used to detect ANCA, ANA should be carefully distinguished. PMID:15138182

  4. Leg ulcer as a manifestation of eosinophilic vasculitis in a patient with hepatitis C virus infection, medicated with pegylated interferon/ribavirin.

    PubMed

    Resende, Cristina; Pereira, Teresa; Ventura, Filipa; Brito, Celeste

    2015-01-01

    Cryoglobulinaemic vasculitis is a complication of hepatitis C virus (HCV) infection, responding to treatment with pegylated interferon (peg-IFN)/ribavirin (RIB), but vasculitis may first appear after treatment with peg-IFN/RIB. A 35-year-old man with HCV infection presented to our department with a 2-month history of a 3.3×3?cm ulcer localised on the right shin, with a regular border, on a violaceous base. Histopathological examination revealed a leucocytoclastic vasculitis, rich in eosinophils. The patient had been treated with peg-IFN/RIB 10?months prior and treatment was discontinued after 2?months because of the appearance of arthralgias and neuropathy. Laboratory investigations revealed positive cryoglobulins, elevation of rheumatoid factor and reduction of C4 after treatment with peg-IFN/RIB. Dressings with a hydrocellular foam were placed and after 2?months the ulcer resolved. We presented this case because of the rarity of development of a cryoglobulinaemic vasculitis in a patient with HCV infection, previously treated with peg-IFN/RIB. PMID:26065548

  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. [Rapidly progressive ANCA-negative glomerulonephritis in the course of pauci immune microscopic vasculitis with hemolytic anemia probable in the course of Wilson's disease].

    PubMed

    Wieczorek-Surdacka, Ewa; Kaczmarczyk, Ireneusz; Jasik, Piotr; Przepiórkowska-Hoyer, Bernadetta; Su?owicz, W?adys?aw

    2011-01-01

    Pauci-immune glomerulonephritis, i.e., with no evidence of immune deposits in the blood vessel, is the most prevalent form of rapidly progressive glomerulonephritis (RPGN). In the pathogenesis of pauci-immune renal disease inflammation of blood vessels in the presence of circulating anti-neutrophil cytoplasm antibodies (ANCA) takes place. However the lack of ANCA (about 5-30% of patients) does not exclude pauci-immune vasculitis. The patients without circulating ANCA might have fewer extrarenal symptoms than those who are ANCA-positive. We describe a case of a 40-year old women with ANCA-negative renal limited pauci-immune small-vessel vasculitis with rapidly decreasing kidney function. She was ineffectively treated with plasmapheresis combined with a puls of cyclophosphamide (i.v.) and 3 pulses of methyloprednisolone (i.v.). The patient progressed to end-stage renal disease and should be treated with renal replacement therapy. In differential diagnosis we excluded other causes of pauci-immune vasculitis (Churg-Strauss syndrome, Wegener's granulomatosis), vasculitis with immune complexes deposition (systemic lupus erythematosus, Schoenlein-Henoch purpura, post-infection RPGN), Goodpasture disease, haemolytic-uremic syndrome (HUS), disseminated intravascular coagulation (DIC) and Wilson's disease. PMID:21751523

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

    PubMed Central

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

    2015-01-01

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

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

    PubMed Central

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

    2015-01-01

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

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

    PubMed

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

    1992-01-01

    Two clinical cases of female patients with hepatic cirrhosis and autoimmune multisystemic involvement with infectious intercurrent are reported. Case 1 presented infective endocarditis and erysipelas on the left thigh. In the course of the clinical picture a cutaneous vasculitis developed in the same place together with autoimmune thrombocytopenia, leukopenia and pulmonary restrictive picture with inflammatory pattern. There are also elevate immune complexes and complement consumption. Case 2 presented erysipelas on the left thigh cutaneous vasculitis and complement consumption. In Case 1 the infective endocarditis was treated with antibiotic therapy during 4 weeks followed by 1 mg/kg corticoid (Prednisone) with thrombocytopenia and leukopenia reversion. Case 2 presented an improvement with antibiotic only. The relation between chronic liver diseases and systemic autoimmune phenomena is commented, special attention being paid to the cutaneous, hematological and pulmonary affection. PMID:1307215

  10. What you should know about PR3ANCA: Evidence for the role of T cells in the pathogenesis of systemic vasculitis

    Microsoft Academic Search

    Abigail R Clayton; Caroline OS Savage

    2000-01-01

    The pathogenesis of systemic vasculitis is complex and is likely to involve many mechanisms. There is a growing body of evidence that T cells may contribute to the pathogenesis of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Predominantly, T cells and monocytes are found in inflammatory infiltrates in patients with Wegener's granulomatosis (WG). The production of ANCA appears to be T-cell-dependent. T

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

    PubMed Central

    2012-01-01

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

  12. Vasculitis as part of the fetal response to acute chorioamnionitis likely plays a role in the development of necrotizing enterocolitis and spontaneous intestinal perforation in premature neonates.

    PubMed

    Ducey, Jonathan; Owen, Anthony; Coombs, Robert; Cohen, Marta

    2015-06-01

    Background?Necrotizing enterocolitis (NEC) and spontaneous intestinal perforation (SIP) are causes of bowel perforation in premature neonates. Studies have demonstrated that both are associated with acute chorioamnionitis (ACA) of the placenta. Aim?The aim of our study was to identify any histopathological links between placental histopathological abnormalities and the later development of NEC and/or SIP in premature patients presenting at our institution. Patients and Methods?Cases with a diagnosis of NEC/SIP were identified. Entry criteria were the diagnosis of NEC/SIP was confirmed clinically and/or histologically, had been made within the first 7 days of life, neonates were premature, and the placenta had been submitted for histological examination. In those cases with ACA, CD34 immunohistochemistry and Martius scarlet blue staining was performed. Medical records were reviewed for demographics, clinical variables, and clinical outcomes. Statistical analysis was performed using Fisher exact test. Results?In total, 21 cases met defined inclusion criteria (12 NEC, 8 SIP, and 1 clinically indeterminate). Mean gestational age was 27 weeks. Median age of presentation was 5 days. Placental histology showed ACA in 16 of 21 cases (76.2%). Of those with ACA, 13 of 16 (81.3%) had umbilical phlebitis, 12 of 16 (75.0%) had umbilical arteritis, 6 of 16 (37.5%) funisitis, and 12 of 16 (75.0%) had chorionic vasculitis. No differences (p?>?0.05) were seen between ACA and diagnosis or clinical outcome (Fisher exact test). Of the 16 cases, 14 with ACA that later developed either NEC or SIP showed vasculitis in the umbilical cord and/or chorionic plate and/or stem villi vasculature. The association between ACA and vasculitis was highly significant (p?vasculitis. Conclusion?NEC or SIP shows a significant association with ACA with presence of vasculitis as part of the FIR (p?

  13. Systemic injection of products of activated neutrophils and H2O2 in myeloperoxidase-immunized rats leads to necrotizing vasculitis in the lungs and gut.

    PubMed Central

    Heeringa, P.; Foucher, P.; Klok, P. A.; Huitema, M. G.; Tervaert, J. W.; Weening, J. J.; Kallenberg, C. G.

    1997-01-01

    The strong association of anti-neutrophil cytoplasmic antibodies with various forms of systemic vasculitis suggests a role for these autoantibodies in the pathophysiology of systemic vasculitis. In the present study, we tested the hypothesis that release of neutrophil lysosomal enzymes in the presence of an anti-myeloperoxidase (anti-MPO) immune response may underlie the development of systemic vasculitis. Brown Norway rats were immunized with MPO in complete Freund's adjuvant or complete Freund's adjuvant alone. Two weeks after immunization, rats bad developed antibodies to human and rat MPO as measured by enzyme-linked immunosorbent assay. Next, rats were intravenously infused with 400 micrograms of a human neutrophil lysosomal extract containing 200 micrograms of MPO followed by 0.5 ml of a 1 mmol/L solution of H2O2 through a cannula inserted into the right jugular vein. Rats were sacrificed at 4 hours, 24 hours, 7 days, or 14 days, and several organs (lungs, heart, liver, spleen, gut, and kidneys) were examined for vasculitic lesions and inflammatory cell infiltrates. Macroscopically, patchy hemorrhagic spots were observed in the lungs and gut of MPO-immunized rats at days 7 and 14 after systemic infection of the neutrophil lysosomal extract and H2O2. Such changes were not observed at earlier time points or in control immunized rats. Histologically, the lungs of MPO-immunized rats sacrificed at days 7 and 14 showed patchy inflammatory cell infiltrates associated with vasculitis, granuloma formation, giant cells, and foci of hemorrhage. At 14 days, early signs of fibrosis were found with deposition of collagen and proliferation of fibroblasts. Furthermore, a prominent leukocytoclastic vasculitis was found in the small intestine of these rats characterized by fibrinoid necrosis and an extensive neutrophilic infiltrate. No inflammatory changes were found in the other organs studied (heart, liver, spleen, and kidneys). Control immunized rats, sacrificed at days 7 and 14 showed only some small foci of inflammatory infiltrates in the lungs whereas no inflammatory changes were found in the gastrointestinal tract. These studies show that release of products from activated neutrophils in the presence of anti-MPO autoantibodies may be relevant to the pathogenesis of anti-MPO-associated vasculitides. Images Figure 1 Figure 3 Figure 4 Figure 6 PMID:9212739

  14. ANCA-positive vasculitis induced by levamisole-adulterated cocaine and nephrotic syndrome: The kidney as an unusual target

    PubMed Central

    Álvarez Díaz, Hortensia; Mari?o Callejo, Ana Isabel; García Rodríguez, José Francisco; Rodríguez Pazos, Laura; Gómez Buela, Inmaculada; Bermejo Barrera, Ana María

    2013-01-01

    Patient: Male, 36 Final Diagnosis: Levamisole-induced vasculopathy Symptoms: Purpuric skin lesions Medication: Levamisole Clinical Procedure: — Specialty: Internal Medicine Objective: Unusual clinical course Background: Levamisole has been detected in seized cocaine samples and a levamisole-induced vasculopathy (LIV) has been described, mainly focused on skin. Case Report: A 36-year-old Caucasian man with history of antibodies to hepatitis C infection (negative hepatitis C virus RNA and negative HIV serology), smoking, and intravenous use of cocaine and brown heroin, presented to the hospital with purpuric skin lesions on extremities and earlobes. One month before the current presentation, a skin punch biopsy of one of these lesions was performed, showing histopathologic findings suggestive of mixed cryoglobulinemia. Laboratory testing revealed leukopenia, renal failure, and nephrotic syndrome. Antimyeloperoxidase antineutrophil cytoplasmic antibodies (MPO-ANCA) were positive. The previous skin punch biopsy was revised and demonstrated pathologic findings consistent with leukocytoclastic vasculitis. An analysis of a cocaine sample for personal use, provided by the patient, was performed using mass spectrometry-gas chromatography and levamisole was detected. Three boluses of intravenous methylprednisolone were administered, followed by oral prednisone 1 mg/Kg per day. Skin lesions and renal function improved. Conclusions: To our knowledge, this is the first report of nephrotic syndrome induced by levamisole-adulterated cocaine, proven by cocaine sample toxicology. Lack of renal biopsy is a limitation of this report. PMID:24478818

  15. Features of renal vasculitis in autoimmune MRL lpr/lpr mice: phenotypes and functional properties of infiltrating cells.

    PubMed Central

    Tarkowski, A; Jonsson, R; Sanchez, R; Klareskog, L; Koopman, W J

    1988-01-01

    MRL lpr/lpr (MRL/1) mice spontaneously develop a widespread renal vasculitis. The majority of the cells in vasculitic lesions are bright Ly-1, L3T4 and la-positive in contrast to the cells found in lymph nodes and spleens of the old MRL/1 mice. However, despite differences in phenotypical patterns, B and T cells from arteritic lesions do not differ from mononuclear cells (MNC) eluted from MRL/1 lymph nodes with regard to the frequency of IgG secreting cells and the proliferative responses to Concanavalin A (Con A). Co-culture experiments with congeneic MRL+/+ (MRL/n) spleen cells indicate that the poor response to Con A of the MNC eluted from vasculitic lesions is, unlike the case of lymph node MNC, due to suppressive action of vasculitic cells on the indicator cell population. Further support for the activation status of infiltrating MHC in kidney vasculitic lesions, expressed by high in vivo uptake of 3H-thymidine, was obtained by autoradiography performed on frozen sections. The observed differences in phenotypic patterns and functional features between lymph node MNC and infiltrating vasculitic MNC indicate that different immune mechanisms may be responsible for the development of lymphadenopathy and vasculopathy, respectively in MRL/1 mouse. Images Fig. 2 Fig. 3 Fig. 4 PMID:3293853

  16. Eosinophilic/T-cell chorionic vasculitis and chronic villitis involve regulatory T cells and often occur together.

    PubMed

    Katzman, Philip J; Oble, Darryl A

    2013-01-01

    Eosinophilic/T-cell chorionic vasculitis (ETCV) is characterized by mixed T-cell, eosinophilic, and histiocytic infiltrates within the chorionic vessel wall. We sought to better characterize this lesion with respect to other pathologic correlates and the T-cell populations involved. Epidemiologic data and other pathologic diagnoses, including concurrent chronic villitis (CV), were tabulated for each case of ETCV diagnosed at our institution over a 6-year period. CD3, CD25, FOXP3, and dual FOXP3-CD3 immunostains were used to identify regulatory T-cell populations in ETCV and CV. Cells positive for CD3, FOXP3, and CD25 were quantitated by manual counts of ×40 fields at the sites of ETCV and CV, and FOXP3?CD3 and CD25?CD3 ratios were calculated. Digital analysis of ETCV and CV using the dual FOXP3-CD3 immunostain was also performed on select cases. Of 31 ETCV cases, 10 (32%) were accompanied by CV and 13 (42%) by a thrombus in the vessel affected by ETCV. The mean Treg cell marker?CD3 ratios in ETCV ranged from 0.18 to 0.26 by manual count and digital analysis, but the counts did not statistically differ by method. The mean Treg cell marker?CD3 ratios in CV ranged from 0.37 to 0.39 by manual count and 0.19 by digital analysis, but these counts also did not statistically differ by method. Chronic villitis was seen in one-third of ETCV cases. FOXP3+ and CD25+ regulatory T cells represent a significant subpopulation of T cells in ETCV and CV, suggesting that they may play a role in these entities. PMID:23600955

  17. Pro: Should all patients with anti-neutrophil cytoplasmic antibody-associated vasculitis be primarily treated with rituximab?

    PubMed

    Specks, Ulrich

    2015-07-01

    Randomized controlled trials have shown that rituximab is non-inferior to cyclophosphamide followed by azathioprine (CYC/AZA) for remission induction in severe granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). The efficacy of rituximab is on par with CYC/AZA for 18 months, for patients with GPA and MPA alike, and for patients with any degree of renal impairment. The Rituximab in ANCA-associated Vasculitis (RAVE) trial also showed superiority of rituximab for patients presenting with a severe disease relapse. An exploratory analysis of the RAVE data further suggests that rituximab may be preferable for PR3-ANCA-positive patients as superiority was also achieved in that subset. When considering treatment options for patients with disease presentations for which only non-inferiority has been documented, safety concerns, compliance issues, the overall cost of each treatment approach to the patient, to society and to insurers, as well as individual patient preferences all should affect the decision-making process. The trials failed to uncover any difference in adverse events between rituximab and CYC/AZA. However, daily oral cyclophosphamide given for 3-6 months has measurable negative effects on fertility. Rituximab has certain compliance and convenience advantages. When assessing cost, the overall cost of a treatment, the societal context of the individual patient and not merely the sticker price of the drug should be considered. For all of these reasons, the author believes that CYC/AZA should be reserved for patients with newly diagnosed, MPO-ANCA-positive disease who raise no fertility, compliance or malignancy concerns. PMID:25999375

  18. 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

  19. A rare association of localized scleroderma type morphea, vitiligo, autoimmune hypothyroidism, pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case report

    PubMed Central

    2012-01-01

    Background The localized scleroderma (LS) known as morphea, presents a variety of clinical manifestations that can include systemic involvement. Current classification schemes divide morphea into categories based solely on cutaneous morphology, without reference to systemic disease or autoimmune phenomena. This classification is likely incomplete. Autoimmune phenomena such as vitiligo and Hashimoto thyroiditis associated with LS have been reported in some cases suggesting an autoimmune basis. To our knowledge this is the first case of a morphea forming part of a multiple autoimmune syndrome (MAS) and presenting simultaneously with autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case presentation We report an uncommon case of a white 53?year old female patient with LS as part of a multiple autoimmune syndrome associated with pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis presenting a favorable response with thrombopoietin receptor agonists, pulses of methylprednisolone and cyclophosphamide. Conclusion Is likely that LS have an autoimmune origin and in this case becomes part of MAS, which consist on the presence of three or more well-defined autoimmune diseases in a single patient. PMID:23256875

  20. 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

  1. Intermediate monocytes in ANCA vasculitis: increased surface expression of ANCA autoantigens and IL-1? secretion in response to anti-MPO antibodies

    PubMed Central

    O’Brien, Eóin C.; Abdulahad, Wayel H.; Rutgers, Abraham; Huitema, Minke G.; O’Reilly, Vincent P.; Coughlan, Alice M.; Harrington, Mark; Heeringa, Peter; Little, Mark A.; Hickey, Fionnuala B.

    2015-01-01

    ANCA vasculitis encompasses several autoimmune conditions characterised by destruction of small vessels, inflammation of the respiratory tract and glomerulonephritis. Most patients harbour autoantibodies to myeloperoxidase (MPO) or proteinase 3 (PR3). Clinical and experimental data suggest that pathogenesis is driven by ANCA-mediated activation of neutrophils and monocytes. We investigated a potential role for distinct monocyte subsets. We found that the relative proportion of intermediate monocytes is increased in patients versus control individuals, and both MPO and PR3 are preferentially expressed on these cells. We demonstrate that MPO and PR3 are expressed independently of each other on monocytes and that PR3 is not associated with CD177. MPO expression correlates with that of Fc receptor CD16 on intermediate monocytes. Monocyte subsets respond differently to antibodies directed against MPO and PR3, with anti-MPO but not anti-PR3 leading to increased IL-1?, IL-6 and IL-8 production. In concordance with the observed higher surface expression of MPO on intermediate monocytes, this subset produces the highest quantity of IL-1? in response to anti-MPO stimulation. These data suggest that monocytes, specifically, the intermediate subset, may play a role in ANCA vasculitis, and also indicate that substantial differences exist between the effect of anti-MPO and anti-PR3 antibodies on these cells. PMID:26149790

  2. Intermediate monocytes in ANCA vasculitis: increased surface expression of ANCA autoantigens and IL-1? secretion in response to anti-MPO antibodies.

    PubMed

    O'Brien, Eóin C; Abdulahad, Wayel H; Rutgers, Abraham; Huitema, Minke G; O'Reilly, Vincent P; Coughlan, Alice M; Harrington, Mark; Heeringa, Peter; Little, Mark A; Hickey, Fionnuala B

    2015-01-01

    ANCA vasculitis encompasses several autoimmune conditions characterised by destruction of small vessels, inflammation of the respiratory tract and glomerulonephritis. Most patients harbour autoantibodies to myeloperoxidase (MPO) or proteinase 3 (PR3). Clinical and experimental data suggest that pathogenesis is driven by ANCA-mediated activation of neutrophils and monocytes. We investigated a potential role for distinct monocyte subsets. We found that the relative proportion of intermediate monocytes is increased in patients versus control individuals, and both MPO and PR3 are preferentially expressed on these cells. We demonstrate that MPO and PR3 are expressed independently of each other on monocytes and that PR3 is not associated with CD177. MPO expression correlates with that of Fc receptor CD16 on intermediate monocytes. Monocyte subsets respond differently to antibodies directed against MPO and PR3, with anti-MPO but not anti-PR3 leading to increased IL-1?, IL-6 and IL-8 production. In concordance with the observed higher surface expression of MPO on intermediate monocytes, this subset produces the highest quantity of IL-1? in response to anti-MPO stimulation. These data suggest that monocytes, specifically, the intermediate subset, may play a role in ANCA vasculitis, and also indicate that substantial differences exist between the effect of anti-MPO and anti-PR3 antibodies on these cells. PMID:26149790

  3. Management of large-vessel vasculitis with FDG-PET: a systematic literature review and meta-analysis.

    PubMed

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

    2015-04-01

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

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

    Technology Transfer Automated Retrieval System (TEKTRAN)

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

  5. Epidemiology of systemic vasculitis

    Microsoft Academic Search

    Richard Watts; David G. I. Scott

    2005-01-01

    The systemic vasculitides are heterogeneous conditions of unknown etiology characterized by inflammation and necrosis of different\\u000a sized blood vessels. Wegener’s Granulomatosis, microscopic polyangiitis, and Churg Strauss syndrome are associated with anti-neutrophil\\u000a cytoplasmic antibodies and affect small and medium blood vessels. They are very rare in childhood and peak in the 65 to 70\\u000a year old age group. Wegener’s Granulomatosis appears

  6. Large vessel vasculitis

    PubMed Central

    Gulati, Ashima

    2009-01-01

    Takayasu arteritis is a chronic granulomatous disease of the aorta and its major branches that usually affects women during the second and third decades of life, but it has been reported in young children. This review details the clinical, pathological and radiological features, differential diagnoses and management of the condition, focusing chiefly on the disease in children. The recent definition of Takayasu arteritis is discussed. The condition should be considered in patients with unexplained arterial hypertension or unexplained inflammatory syndromes without signs of localization. Since the disease may be life-threatening and progressive, early recognition is necessary to initiate appropriate therapy. Patients with persistent ischaemic symptoms including hypertension might benefit from revascularization procedures. PMID:19844748

  7. Vasculitis: report from Mexico.

    PubMed

    Flores-Suárez, Luis Felipe

    2013-10-01

    Although primary systemic vasculitides (PSV) are infrequent diseases, basic and clinical research have increased the knowledge of these autoimmune conditions substantially. Some PSV seem to be more frequent in certain countries. Here we present a brief history of the modest, but important contributions made in Mexico in this area of research. PMID:23229651

  8. 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

  9. Glossary of Vasculitis Terms

    MedlinePLUS

    ... in cross-sectional images, permitting doctors to examine “slices” through different organs. Catheterization: The insertion of a ... within a body tissue; for example, in the lung. Pulmonary infiltrates are visible on chest x-rays ...

  10. Pathogenesis of lung vasculitis.

    PubMed

    Nachman, Patrick H; Henderson, Ashley G

    2011-06-01

    Vasculitides that affect the lung represent a diverse group of diseases with various systemic clinical manifestations, and include microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA, formerly Wegener granulomatosis), Churg-Strauss syndrome (CSS), and anti-glomerular basement membrane (anti-GBM) disease (Goodpasture syndrome). The etiologies of these diseases remain largely unknown. Although the pathogenic mechanisms of each differ, these diseases overlap by the presence of anti-neutrophil cytoplasmic autoantibodies in the vast majority of patients with MPA and GPA, and a substantial minority of patients with CSS and anti-GBM disease. This article reviews the current understanding of the pathogenesis of these four disease entities. PMID:21674411

  11. Antineutrophil cytoplasmic antibody-associated vasculitis associated with Epstein-Barr virus infection: a case report and review of the literature.

    PubMed

    Xu, P; Lin, S; Wei, L; Shang, W

    2014-06-01

    A 16-year-old female with fever was admitted to our hospital. On admission, her serum IgM antibody against Epstein-Barr virus (EBV) was positive. Then, the disease aggravated and acute kidney injury occurred gradually. Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) was confirmed by serum test and kidney biopsy. The patient was treated with oral methylprednisolone. Along with the disappearance of the IgM anti-EBV antibody, the AAV also relieved without relapse during follow-up for half a year. Although a previous study indicated that there was a high positive rate of ANCA in the sera positive for IgM antibodies to EBV and EBV infection might trigger the relapse of AAV, this is the first case of incipient AAV associated with acute EBV infection. One possible explanation might be that EBV infection stimulated the production of ANCA. PMID:24610176

  12. An improved quantitative method for the study of fibrinolysis in the skin as exemplified in patients with leukocytoclastic vasculitis and erysipelas.

    PubMed

    Hammar, H; Blombäck, M; Sverdrup, B; Borglund, E

    1980-01-01

    The activity of plasminogen activator has been measured in tissue sections with the aid of a modified Todd technique. Frozen skin biopsies were sectioned and the tissue covered with fibrin-plasminogen film. After incubation at 37 degrees C fibrinolysis was studied for a period of 30 min and was graded into six exponentially increasing steps. During this period, grades were linear with the llogarithm of time of incubation. The rate of fibrinolysis is a measure of the activity of the plasminogen activator and is expressed by the slope of the linear regression of grades versus the ogarithm of time; the value of the slope is proportional to the common logarithm of the activity of the plasminogen activator present in the section. Using urokinase as a specific plasminogen activator, the same linear expression was shown with time, indicating the validity of our grading and experimental system. Eleven healthy subjects served as controls to 6 patients with leukocytoclastic vasculitis and 4 patients with erysipelas and 4 with necrotizing fascilitis. The controls showed values similar to the non-involved skin in the patients. The activity was higher in the arm than in the thigh sites. The activity in the thighs was lower in women than in men. A decrease in the plasminogen activator activity was found in the three disorders. PMID:6163272

  13. 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

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

    PubMed

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

    2015-01-01

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

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

    PubMed

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

    2015-07-01

    Monocyte subsets with differing functional properties have been defined by their expression of CD14 and CD16. We investigated these subsets in anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) and determined their surface expression of ANCA autoantigens. Flow cytometry was performed on blood from 14 patients with active AAV, 46 patients with AAV in remission and 21 controls. The proportion of classical (CD14(high) CD16(neg/low) ), intermediate (CD14(high) CD16(high) ) and non-classical (CD14(low) CD16(high) ) monocytes and surface expression levels of CD14 and CD16 were determined, as well as surface expression of proteinase 3 (PR3) and myeloperoxidase (MPO) on monocyte subsets. There was no change in the proportion of monocytes in each subset in patients with AAV compared with healthy controls. The expression of CD14 on monocytes from patients with active AAV was increased, compared with patients in remission and healthy controls (P?

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

    PubMed

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

    2015-05-01

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

  17. Interferon-gamma-producing T cells in giant cell vasculitis represent a minority of tissue-infiltrating cells and are located distant from the site of pathology.

    PubMed Central

    Wagner, A. D.; Björnsson, J.; Bartley, G. B.; Goronzy, J. J.; Weyand, C. M.

    1996-01-01

    Giant cell vasculitis is an arteritis that predominantly affects medium- and large-sized arteries. Genetic risk factors and clonal expansion of selected CD4+ T cell specificities in the vascular lesions support the model that giant cell arteritis is a T-cell-driven disease. Interferon (IFN)-gamma production in the tissue is intimately associated with the formation of the inflammatory infiltrates. Antigens inducing stimulation of T cells are unknown. To provide indirect evidence for the type and the tissue localization of the antigen, we examined CD4+ T cells in the lesions that secrete IFN-gamma. Temporal artery specimens from patients with giant cell arteritis were analyzed bu two-color immunohistochemistry applying antibodies to T cell markers. IFN-gamma, the interleukin-2 receptor alpha-chain (CD25) and talin, a cytoskeletal protein that is reorganized in T cells interacting with antigen-presenting cells. Proliferating cells in the lesions were identified through the expression of the Ki-67 nuclear antigen. More than 90% of tissue-infiltrating IFN-gamma-producing cells were CD4+ CD45RO+. They represented a minute subset (2 to 4%) of tissue-infiltrating T cells. IFN-gamma+ T cells aggregated in the adventitial layer of the inflamed artery where they were either diffusely distributed or arranged in clusters. The majority of IFN-gamma-secreting T cells expressed CD25. IFN-gamma+ T cells included a fraction of cells that had reorganized the cytoskeletal protein talin, indicating an interaction of the T cell receptor and an antigen-presenting cell. A subset of IFN-gamma-expressing T cells was undergoing proliferation in the tissue. IFN-gamma-producing T cells in vasculitic lesions of giant cell arteritis express several markers that identify them as T cells that have recently been stimulated through their antigen-specific receptor. These putatively disease-relevant T cells represent only a very minor fraction of tissue-infiltrating cells. Their preferential accumulation in the adventitia is most compatible with the model that they contact the relevant antigen primarily in this particular region of the artery. Their regulatory function appears to extend into the inner media and intima where pathological changes in giant cell arteritis are most pronounced. Images Figure 1 Figure 3 Figure 4 Figure 5 Figure 6 PMID:8669478

  18. Vasculitis Terms A to Z

    MedlinePLUS

    ... in cross-sectional images, permitting doctors to examine “slices” through different organs. Chlorambucil: A medication used to ... within a body tissue; for example, in the lung. Pulmonary infiltrates are visible on chest x-rays ...

  19. New horizons in renal vasculitis

    Microsoft Academic Search

    J. S. Cameron

    1991-01-01

    Summary After a long period during which few new data on the vasculitides emerged, the past decade has seen a real explosion of data on the subject. This began with basic clinical descriptions and the deliniation of long term outcome as survival improved to a major extent under the influence of improved treatment regimes. In parallel, better understanding of the

  20. Roadmap to vasculitis: a rheumatological treasure hunt

    Microsoft Academic Search

    Yrjö T. Konttinen; Tom Pettersson; Marco Matucci-Cerinic; Jolanta Dadoniene; Praseet Poduval

    2007-01-01

    In the third part of this four part review, we already have the stop sign and our three road signs pointing to secondary vasculitides, pseudovasculitides and primary vasculitides behind our back and we have also passed the first milestone, where “patient history and physical examination” was written with large black block letters. GP can get far with simple blood, urine

  1. 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

  2. Emerging concepts in the pathogenesis of ANCA-associated vasculitis

    E-print Network

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

    -PR3 ANCA in otherwise healthy individuals or individuals with a range of non-vasculitic diseases (e.g. systemic lupus erythematosus (SLE), ulcerative colitis), which demonstrates that ANCA are not always pathogenic.[10,11] In addition, up to a third...

  3. Midaortic syndrome and celiac disease: a case of local vasculitis

    Microsoft Academic Search

    E. Stadlmaier; A. Spary; M. Tillich; E. Pilger

    2005-01-01

    Midaortic syndrome is a variety of aortic coarctation, located in the distal thoracic aorta, the abdominal aorta or both, involving the intestinal and renal vessels, usually presenting with renovascular arterial hypertension. Underlying conditions are thought to be Takayasu’s arteritis, von Recklinghausen’s disease, and connate hypoplasia. Celiac disease is an inflammation in the small intestine, triggered by an allergic reaction to

  4. 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...

  5. Rapidly progressive cutaneous vasculitis in a patient with chronic myelomonocytic leukemia.

    PubMed

    Rosen, A M; Haines, K; Tallman, M S; Hakimian, D; Ramsey-Goldman, R

    1995-12-01

    This case report reviews the unique development of a vasculitic syndrome involving medium-sized arteries in a man with chronic myelomonocytic leukemia (CMMoL). This case has many features in common with cutaneous polyarteritis nodosa (CPAN), and this may represent the first instance in which CPAN developed in the setting of CMMoL. PMID:7485111

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

    PubMed Central

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

    2015-01-01

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

  7. 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

    cases or controls. Two loci, CTLA4 and PTPN22, marked by rs3087243 and rs2476601 respectively, were associated with disease (tables 1 &2). rs3087243 in CTLA4 was associated with disease using both genotype and allele tests (allelic P = 6.4 × 10-3; table... , Lynch WP, Bluestone JA, Sharpe AH: Loss of CTLA-4 leads to massive lymphoprolifer- ation and fatal multiorgan tissue destruction, revealing a critical negative regulatory role of CTLA-4. Immunity 1995, 3(5):541-547. 21. Stamper CC, Zhang Y, Tobin JF...

  8. Neutrophil extracellular trap components in fibrinoid necrosis of the kidney with myeloperoxidase-ANCA-associated vasculitis.

    PubMed

    Yoshida, Masaharu; Sasaki, Mariko; Sugisaki, Kentaro; Yamaguchi, Yutaka; Yamada, Michiyuki

    2013-06-01

    In response to antineutrophil cytoplasmic antibody (ANCA), activated neutrophils release decondensed chromatin associated with cytoplasmic granular proteins composed of proteolytic enzymes and myeloperoxidase (MPO); these complexes are named neutrophil extracellular traps (NETs). NET formation requires peptidylarginine deiminase 4 (PAD4) to citrullinate chromatin histones and also MPO and neutrophil elastase to aid in decondensation and release of chromatin. Immunostaining of renal biopsy revealed that NET components of citrullinated histone, MPO and PAD4 were concurrently deposited both around fibrinoid necrosis in necrotizing glomerulonephritis in the early focal phase of ANCA-associated polyangiitis and along an interlobular arterial wall. PMID:26064491

  9. Chronic bacterial-viral vasculitis as manifestation of systemic inflammatory response syndrome in females.

    PubMed

    Kvantaliani, T; Tsiklauri, P; Pargalava, N

    2005-09-01

    Study involved 52 women (mean age 43+/-1,3) without any evidence of CHD, suffering from dull or acute prolonged non-anginal chest pain, with undergoing chronic bacterial-viral infection--Chlamydia pneumonia in combination with HSV-I, CMV and/or EBV. 30--patients serum-positive to plasma markers (IgG antibodies) of previous infection were enclosed in group I, 22--with plasma consumption of IgA+IgG antibody complex demonstrating re-infection/reactivation phase of disease--in group II and 20 healthy serum-negative females (median age 47+/-2,8 years)--controls (group III). The intergroup analysis revealed the complex of disturbances in some plasma parameters of II group patients, namely significant elevation of CRP, F and LPO activity vs. I and III group data, along with reduced parameters of immune status in both groups of infected persons. The patients with mixed infections showed the high frequency of specific re-polarization phase abnormalities, cardiac rhythm and conduction disturbances. These changes together with intimate-medial wall injuries of inflammatory origin disclosed by DS technique enables us to suggest that in women with previous chronic untreated or insufficiently treated infection, in cases of their overload activity, recurrent or super-infection may provide deep immunosuppressive conditions leading to farther cardio-vascular abnormalities. PMID:16234591

  10. [Case of trimethoprim-induced hyperkalemia complicating ANCA-associated vasculitis].

    PubMed

    Shishido, Takashi; Ryuzaki, Munekazu; Futatsugi, Koji; Takimoto, Chie; Kobayashi, Emi; Handa, Michiko; Itoh, Hiroshi

    2012-01-01

    A 76-year-old man was admitted to our hospital because of severe anemia. Routine screening revealed a sigmoid adenocarcinoma, and he underwent sigmoidectomy. Post-operatively, he developed rapidly progressive glomerulonephritis. He was positive for myeloperoxidase anti-neutrophil cytoplasmic antibody. A renal biopsy revealed idiopathic crescentic glomerulonephritis of the pauci-immune type. He was treated with methylprednisolone semi-pulse therapy with clinical improvement. After the steroid pulse therapy, he was given oral prednisolone, 40 mg per day, and oral trimethoprim (TMP), 160 mg, and sulfamethoxazole (SMX), 800 mg twice weekly for chemoprophylaxis against pneumocystis pneumonia. One month after the initiation of TMP/SMX, he developed hyperkalemia and hyponatremia. His transtubular K gradient was low, and urinary potassium excretion was decreased. On the other hand, plasma renin activity and plasma aldosterone concentrations were within normal limits. These results suggested that TMP acted similarly to a potassium-sparing diuretic amiloride and reduced renal potassium excretion. Administration of calcium polystyrene sulfonate resulted in correction of the hyperkalemia without discontinuation of TMP/SMX. We emphasize that patients with impaired renal function are at the significant risk of developing trimethoprim-induced hyperkalemia even with chemoprophylaxis. PMID:22991842

  11. Therapy Insight: the recognition and treatment of retinal manifestations of systemic vasculitis

    Microsoft Academic Search

    Petros Aristodemou; Miles Stanford

    2006-01-01

    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,

  12. 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

  13. Management of heparin-induced thrombocytopenia (HIT) in patients with systemic vasculitis and pulmonary haemorrhage

    PubMed Central

    Thong, Kah Mean; Toth, Peter; Khwaja, Arif

    2013-01-01

    Heparin-induced thrombocytopenia (HIT) is a relatively uncommon but potentially fatal complication of the use of heparin in haemodialysis. It is associated with a risk of venous and arterial thrombosis due to the formation of a heparin-platelet factor 4 antibody. Early recognition and immediate treatment of HIT are crucial to reduce the morbidity and mortality rate. Here, we report two patients with acute kidney injury due to anti-glomerular membrane (GBM) glomerulonephritis and granulomatosis with polyangiitis respectively who developed haemoptysis and pulmonary haemorrhage complicated by HIT. We discuss the diagnostic and management challenges of such patients.

  14. Renal transplantation in anti-neutrophil cytoplasmic antibody-associated vasculitis.

    PubMed

    Hruskova, Zdenka; Geetha, Duvuru; Tesar, Vladimir

    2015-04-01

    Despite major advances in the management of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) achieved in the last decades, a large proportion of AAV patients still develop end-stage renal disease. The survival of AAV patients dependent on dialysis is significantly worse compared with dialysis-independent AAV patients, but is comparable to other non-diabetic patients requiring dialysis. Renal transplantation (RTx) is the method of choice among renal replacement therapies and there has been increasing evidence that it is a suitable method with favorable patient- and graft-survival also in AAV patients. It is recommended to perform RTx after ?12 months of remission, and ANCA positivity at the time of RTx is generally not considered a contraindication. Even though the risk of relapse after RTx is relatively low with current post-transplant immunosuppressive regimens, disease recurrence may occur. Besides cyclophosphamide, rituximab might become a therapeutic alternative for post-transplant AAV recurrence in the near future but its efficacy and safety in this setting needs to be confirmed in larger studies. PMID:25324359

  15. Maintenance therapy in antineutrophil cytoplasmic antibody-associated vasculitis: who needs what and for how long?

    PubMed

    de Joode, Anoek A E; Sanders, Jan Stephan F; Rutgers, Abraham; Stegeman, Coen A

    2015-04-01

    Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV) are severe chronic auto-immune diseases in which the small vessels are inflamed. Nowadays, in the majority of patients disease can be brought into remission with cyclophosphamide and corticosteroids. However, depending upon disease characteristics patients with AAV have a risk of 29-60% to experience relapses of disease within 5 years despite maintenance therapy after induction of remission with less toxic agents, such as azathioprine, methotrexate or mycophenolate mofetil. More recently, rituximab has been found effective in both induction and maintenance of remission in AAV. This review discusses the different aspects of maintenance therapy in AAV based on reported cohorts and studies, including the different agents, therapy duration, efficacy or lack thereof and future directions. Finally, recommendations are made who to treat and for how long. PMID:25609739

  16. Vasculitis, Atherosclerosis, and Altered HDL Composition in Heme-Oxygenase-1-Knockout Mice

    PubMed Central

    Ishikawa, Kazunobu; Navab, Mohamad; Lusis, Aldons J.

    2012-01-01

    To elucidate roles of heme oxygenase-1 (HO-1) in cardiovascular system, we have analyzed one-year-old HO-1-knockout mice. Homozygous HO-1-knockout mice had severe aortitis and coronary arteritis with mononuclear cellular infiltration and fatty streak formation even on a standard chow diet. Levels of plasma total cholesterol and HDL were similar among the three genotypes. However, homozygous HO-1-knockout mice had lower body weight and plasma triglyceride. HO-1-deficiency resulted in alteration of the composition of HDL. The ratio of apolipoprotein AI to AII in HO-1-knockout mice was reduced about 10-fold as compared to wild-type mice. In addition, paraoxonase, an enzyme against oxidative stress, was reduced less than 50% in HO-1-knockout mice. The knockout mice also exhibited significant elevation of plasma lipid hydroperoxides. This study using aged HO-1-knockout mice strengthened the idea that HO-1 functions to suppress systemic inflammation in artery wall and prevents plasma lipid peroxidation. PMID:22518297

  17. Lymphocytic vasculitis associated with the anthrax vaccine: case report and review of anthrax vaccination

    Microsoft Academic Search

    Antonio E Muñiz

    2003-01-01

    Anthrax is caused by the spore-forming bacteria Bacillus anthracis. It occurs naturally, but recently has been manufactured as a biological warfare agent. This makes prophylaxis for anthrax an urgent concern and efforts are ongoing for the production of an efficient and safe vaccine. Side effects to the current anthrax vaccine are usually minor and mainly consist of local skin reactions.

  18. Parvovirus B19-induced multisystemic vasculitis and acute endocapillary proliferative glomerulonephritis in a child.

    PubMed

    Sakalli, Hale; Baskin, Esra; Bayrakçi, Umut Selda; Melek, Engin; Cengiz, Nurcan; Ozdemir, Binnaz Handan

    2010-05-01

    A 10-year-old girl was admitted with fever, cough, maculopapular rash, hemoptysis, dark-colored urine, edema, multiple lymphadenopathies, and hepatosplenomegaly. She developed acute glomerulonephritis during the course of these complex clinical features. Laboratory data showed hematuria, proteinuria, and hypocomplementemia. Serological tests showed positive human parvovirus B19 (HPVB19)-specific immunoglobin M (IgM) and HPVB19 DNA was detected in the patient's serum using polymerase chain reaction (PCR). Renal biopsy revealed acute endocapillary proliferative glomerulonephritis (AEPGN) with coarse granular C3 depositions in a "starry sky pattern," which is more peculiar to poststreptococcal glomerulonephritis. Electron microscopy showed subendothelial and small hump-shaped subepithelial electron-dense deposits in glomerular capillary walls. There was no evidence of either any mycobacterial, staphylococcal, or streptococcal infection, and the diagnosis of Goodpasture syndrome and connective tissue disorders was excluded during clinical and laboratory investigations. A diagnosis of HPVB19-induced pleuropneumonitis and glomerulonephritis was made. Through a literature search there was no documented pediatric case of AEPGN induced by HPVB19, and this case represents, to our knowledge, the first time that a direct relationship between parvovirus infection and AEPGN has been demonstrated in a child. PMID:20446792

  19. Nerve granulomas and vasculitis in sarcoid peripheral neuropathy: A clinicopathological study of 11 patients

    Microsoft Academic Search

    Gerard Said; Catherine Lacroix; V Plante-Bordeneuve; L Le Page; F Pico; O Presles; J Senant; P Remy; P Rondepierre; J. Mallecourt

    2002-01-01

    Summary Peripheral neuropathy is a rare, yet treatable manifest- ation of sarcoidosis, a multisystem disorder character- ized by the presence of non-caseating granulomas that are seldom found in nerve biopsy specimens. In order to learn more about the subject, we reviewed our clin- ical and pathological findings in a series of 11 patients (six men and five women aged 26-83

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

    E-print Network

    Palczewski, Krzysztof

    right eye since childhood, and had had peripheral retinal vascular occlusion. Although the exact visual acuity in her left eye, and minimal orbital involvement. Our patient had a pale optic disk in her. Kohnen EM. Retinal ischemia. In: Schahat AP, Murphy RB, eds. Retina. St. Louis: Mosby, 1994:1009­1018. 8

  1. Neutrophil Membrane Expression of Proteinase 3 (PR3) Is Related to Relapse in PR3-ANCA-Associated Vasculitis

    Microsoft Academic Search

    AGNIESZKA A. RAROK; COEN A. STEGEMAN; PIETER C. LIMBURG; CEES G. M. KALLENBERG

    Wegener granulomatosis (WG) is strongly associated with the presence of antineutrophil cytoplasm autoantibodies (ANCA) with specificity for proteinase 3 (PR3). Relapses of WG are frequently preceded by a rise of autoantibody titer and PR3-ANCA are able to activate primed neutrophils in vitro. Except being stored intracellularly and translocated to the cell surface upon neutrophil stimulation, PR3 can also be detected

  2. Takayasu's Arteritis

    MedlinePLUS

    ... This disease is one of many types of vasculitis. Vasculitis refers to inflammation of blood vessels, and arteries ... causes Takayasu’s arteritis? As with most types of vasculitis, the cause of TAK is not known. It ...

  3. A New Etiology for Visual Impairment and Chronic Headache: The Tolosa-Hunt Syndrome may be Only One Manifestation of Venous Vasculitis

    Microsoft Academic Search

    Jan Hannerz; Kaj Ericson; Gustaf Bergstrand

    1986-01-01

    The Tolosa-Hunt syndrome is characterized by recurrent periods of painful ophthalmoplegia, dramatically ameliorated by steroid treatment. In half of the cases orbital phlebography shows characteristic changes. Ninety-six patients with orbital pain characteristic of the Tolosa-Hunt syndrome were submitted to orbital phlebography regardless of the existence of other symptoms. A pathologic phlebogram with changes typical of the Tolosa-Hunt syndrome was found

  4. Vasculitides with cutaneous expression in children: clinico-pathological correlations.

    PubMed

    Borroni, G; Grassi, S; Carugno, A

    2015-02-01

    The most recent pediatric vasculitis classifications (EULAR/PRINTO/PRES) have proposed the use of an integration of clinical signs and symptoms, laboratory data, imaging and pathologic data. Pediatric vasculitis represent a peculiar clinical-diagnostic model, compared to the corresponding adult pathology chapter, and in particular, dermatopathologic aspects of these diseases identify more specific issues, made contingent by crucial variables such as duration of vasculitis lesion, site of the biopsy, proper biopsy depth, and possibility to correlate histopathological findings with immunopathological results. Possible additional diagnostic difficulties may arise from the fact that, in children, the same systemic disease, such as lupus erythematosus, may present with different clinical manifestations, with histopathological features of a precise type of vasculitis specific for that type of clinical manifestation. Examples are provided by hypocomplementemic urticarial vasculitis, cryoglobulinemic purpura, lymphocytic vasculitis of livedoid lesions. This paper describes the cutaneous histopathological findings of some vasculitis related pediatric diseases, be they pertaining to a systemic vasculitis with corresponding cutaneous vasculitis, to a systemic vasculitis with sporadic cutaneous vasculitic involvement, and to a systemic vasculitis without cutaneous vasculitic involvement. Type and level of histopathological vasculitic involvement, caliber of the vessel, type of vasculitis associated infiltrate, are likewise reliable integration in the complex diagnostic path of vasculitis in childhood. On the basis of these criteria dermatopathologists should be confident in identifying the type of the vasculitis and relate them to a specific pediatric disease. PMID:25516220

  5. 75 FR 41501 - Government-Owned Inventions; Availability for Licensing

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-07-16

    ...such as autoimmune diseases (e.g., lupus, scleroderma, psoriasis, vasculitis...Interferon-alpha in auto-immune diseases such as lupus, scleroderma, psoriasis, and vasculitis...in part) by Interferon-alpha include lupus, scleroderma, psoriasis, and...

  6. Cryoglobulinemia

    MedlinePLUS

    ... No Comments What is Cryoglobulinemia? Cryogloblinemia is a vasculitis of small blood vessels that is caused by ... they are bound to. Some other types of vasculitis are also caused by immune complexes, but cryoglobulinemia ...

  7. Cyclophosphamide (Cytoxan)

    MedlinePLUS

    ... of immunologically–mediated diseases, including some forms of vasculitis. In general, much lower doses of cyclophosphamide are used to treat vasculitis than are used to treat cancer. However, the ...

  8. Behcet's Disease

    MedlinePLUS

    ... Behcet’s is one of the few forms of vasculitis in which there is a known genetic predisposition. ... the patient’s symptom, and if possible, proof of vasculitis by biopsy of an involved organ all would ...

  9. Prednisone

    MedlinePLUS

    ... are a cornerstone of treating most types of vasculitis, and are often used in combination with other ... ago revolutionized the treatment of many diseases, including vasculitis. When used properly, these drugs save lives and ...

  10. Genetics Home Reference: Granulomatosis with polyangiitis

    MedlinePLUS

    ... feature of GPA is inflammation of blood vessels (vasculitis), particularly the small- and medium-sized blood vessels ... of vessels, such as small arteries and veins. Vasculitis causes scarring and tissue death in the vessels ...

  11. Methotrexate

    MedlinePLUS

    Methotrexate (MTX) is used to treat forms of vasculitis which, although serious, do not require therapy with ... decreases patients’ needs for steroids. Specific forms of vasculitis treated with MTX include Wegener’s granuloma t o s ...

  12. Henoch-Schonlein Purpura

    MedlinePLUS

    ... resolved. HSP is the most common form of vasculitis in children, with an annual incidence on the ... can be mimicked by other forms of systemic vasculitis that are more often life-threatening. Granulomatosis with ...

  13. Granulomatosis with Polyangiitis (GPA)

    MedlinePLUS

    ... under the microscope to confirm the presence of vasculitis and granulomas (a specific type of inflammation), which ... maintaining remission. NIAID news release: Therapy for Severe Vasculitis Shows Long-Term Effectiveness Other medicines During the ...

  14. ESR (Erythrocyte Sedimentation Rate) Test

    MedlinePLUS

    ... diagnose certain specific inflammatory diseases, temporal arteritis , systemic vasculitis and polymyalgia rheumatica . A significantly elevated ESR is ... individual has symptoms that suggest polymyalgia rheumatica , systemic vasculitis , or temporal arteritis , such as headaches, neck or ...

  15. Buerger's Disease

    MedlinePLUS

    ... involve other organs, unlike many other forms of vasculitis. Even as ulcers and gangrene develop in the ... the lining of the heart), other types of vasculitis, severe Raynaud’s phenomenon associated with connective tissue disorders ( ...

  16. Kawasaki Disease

    MedlinePLUS

    ... Topics Cardiac Catheterization Chest X Ray Echocardiography Electrocardiogram Vasculitis Send a link to NHLBI to someone by ... disease. It's a form of a condition called vasculitis (vas-kyu-LI-tis). This condition involves inflammation ...

  17. Isolated Aortitis

    MedlinePLUS

    ... causes are rheumatic diseases including various forms of vasculitis, as well as infections. Noninfectious causes of aortitis are forms of large vessel vasculitis, most commonly giant cell arteritis and Takayasu arteritis. ...

  18. Henoch-Schonlein purpura

    MedlinePLUS

    Anaphylactoid purpura; Vascular purpura; Leukocytoclastic vasculitis ... Ardoin SP, Fels E. Vasculitis syndromes. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics . 19th ed. Philadelphia, Pa: Saunders ...

  19. U.S. DEPARTMENT OF HEALTH AND HUMAN SERVICES

    E-print Network

    Vasculitis Syndromes of the Central and Peripheral Nervous Systems #12;#12;1 Vasculitis Syndromes, including the central (CNS) and peripheral (PNS) nervous systems. Vasculitic disorders, or syndromes, or capillaries. #12;What are the symptoms? A vasculitis syndrome may begin suddenly or develop over time

  20. Glomerulonephritis

    MedlinePLUS

    ... as vasculitis or polyarteritis Focal segmental glomerulosclerosis Goodpasture syndrome Heavy use of pain relievers, especially NSAIDs Henoch-Schonlein purpura IgA nephropathy Lupus nephritis Membranoproliferative GN

  1. 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

  2. Pregnancy and Medically Assisted Conception in Rare Diseases

    ClinicalTrials.gov

    2015-05-20

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

  3. 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

  4. Diseases associated with hidranitis suppurativa: part 2 of a series on hidradenitis

    E-print Network

    Scheinfeld, Noah

    2013-01-01

    lichen  planus   porokeratosis   granulomatosis  vasculitis   pyoderma  faciale   acne  fulminans   neutrophilic  dermatosis  of  malar  region   vitiligo   erythema  multiforme   pyostomatitis  vegetans  (pyoderma  vegetans)   psoriasis  

  5. Vasculitic Peripheral Ulcerative Keratitis

    Microsoft Academic Search

    Elisabeth M Messmer; C. Stephen Foster

    1999-01-01

    The onset of peripheral ulcerative keratitis in the course of a connective tissue disorder, such as rheumatoid arthritis, relapsing polychondritis, or systemic lupus erythematosus, may reflect the presence of potentially lethal systemic vasculitis. Moreover, peripheral ulcerative keratitis may be the first sign of systemic necrotizing vasculitis in patients with Wegener’s granulomatosis, polyarteritis nodosa, microscopic polyangiitis, or Churg-Strauss syndrome. Although the

  6. Matrix metalloproteinase haplotypes associated with coronary artery aneurysm formation in patients with Kawasaki disease

    Microsoft Academic Search

    Chisato Shimizu; Tomoyo Matsubara; Yoshihiro Onouchi; Sonia Jain; Shelly Sun; Caroline M Nievergelt; Hiroko Shike; Victoria H Brophy; Tsuyoshi Takegawa; Susumu Furukawa; Teiji Akagi; Jane W Newburger; Annette L Baker; David Burgner; Martin L Hibberd; Sonia Davila; Michael Levin; Manju Mamtani; Weijing He; Sunil K Ahuja; Jane C Burns

    2010-01-01

    Aneurysms of the vascular wall represent a final common pathway for a number of inflammatory processes, including atherosclerosis and idiopathic vasculitis syndromes. Kawasaki disease (KD) is an acute, self-limited vasculitis in children and the leading cause of acquired coronary artery aneurysms. We sought to identify shared molecular mechanisms of aneurysm formation by genotyping eight polymorphisms in matrix metalloproteinase (MMP)-1, 3,

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

    PubMed

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

    2010-08-01

    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

  8. 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

  9. Giant Cell Arteritis

    Microsoft Academic Search

    Aki Kawasaki

    ? Giant cell arteritis (GCA) is the most common vasculitis in Caucasian adults older than 50 years. \\u000a \\u000a ? There are three predominant clinical subtypes of GCA: systemic inflammatory syndrome, cranial arteritis, and largevessel\\u000a vasculitis. ? Patients with large-vessel vasculitis are likely to have negative temporal artery biopsy results; thus, diagnosis\\u000a rests on clinical suspicion, corroborative laboratory tests, and imaging. \\u000a \\u000a \\u000a \\u000a ?

  10. Cocaine-induced pseudovasculitis.

    PubMed

    Friedman, Daphne R; Wolfsthal, Susan D

    2005-05-01

    Pseudovasculitis is a disease process that mimics the presentation and possibly the laboratory findings of true vasculitis. However, biopsy specimens do not reveal the typical histopathologic findings expected in vasculitis. One often overlooked cause of pseudovasculitis is cocaine use, which has been described in case reports to cause aggressive nasal destruction and various skin lesions and thus has been confused with Wegener granulomatosis or leukocytoclastic vasculitis. Unfortunately, serologic tests such as antinuclear antibody or antineutrophil cytoplasmic antibody cannot reliably differentiate between these entities. We describe a patient who presented with what was believed to be Wegener granulomatosis affecting the skin and upper airway. However, findings from repeated biopsies did not support this diagnosis, and the only unifying diagnosis was cocaine-induced pseudovasculitis. The ability to recognize and differentiate between true vasculitis and pseudovasculitis is essential for the clinician because treatment options are radically disparate. PMID:15887436

  11. Fingers that change color

    MedlinePLUS

    Blanching of the fingers; Fingers - pale; Toes that change color; Toes - pale ... Necrotizing vasculitis Peripheral artery disease Raynaud's phenomenon - sudden change in the finger color ranges from pale to ...

  12. The effect of feeding various levels of energy and phosphorus on blood glucose and blood phosphorus in heifers pre- and post-partum 

    E-print Network

    Fugger, Edward Floyd

    1970-01-01

    as to the apparent cause of death. One calf was sent to the Texas A&M University Veterinary College for necropsy, virology, bacteriology and pathology tests. The report concluded focal cortical malacia and cerebral cortex with vasculities, etiology undetermined...

  13. Bronchoscopy

    MedlinePLUS

    ... the bronchoscope to take very small tissue samples (biopsies) from your lungs. The doctor can also place a stent in ... the trachea or bronchi Sarcoidosis Vasculitis Other inflammatory lung disease

  14. Takayasu's Arteritis

    MedlinePLUS

    ... in addition to prednisone (as “steroid sparing agents”), methotrexate, azathioprine, and even cyclophosphamide are sometimes used. There ... symptoms and can thwart progression of the vasculitis. Methotrexate may be an effective corticosteroid-sparing agent. Angioplasty ...

  15. 38 CFR 4.115b - Ratings of the genitourinary system-diagnoses.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ...cortical necrosis: Rate as renal dysfunction. 7541Renal involvement in diabetes mellitus, sickle cell anemia, systemic lupus erythematosus, vasculitis, or other systemic disease processes. Rate as renal dysfunction. 7542Neurogenic...

  16. 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.

  17. 38 CFR 4.115b - Ratings of the genitourinary system-diagnoses.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ...cortical necrosis: Rate as renal dysfunction. 7541Renal involvement in diabetes mellitus, sickle cell anemia, systemic lupus erythematosus, vasculitis, or other systemic disease processes. Rate as renal dysfunction. 7542Neurogenic...

  18. 38 CFR 4.115b - Ratings of the genitourinary system-diagnoses.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ...cortical necrosis: Rate as renal dysfunction. 7541Renal involvement in diabetes mellitus, sickle cell anemia, systemic lupus erythematosus, vasculitis, or other systemic disease processes. Rate as renal dysfunction. 7542Neurogenic...

  19. 38 CFR 4.115b - Ratings of the genitourinary system-diagnoses.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ...cortical necrosis: Rate as renal dysfunction. 7541Renal involvement in diabetes mellitus, sickle cell anemia, systemic lupus erythematosus, vasculitis, or other systemic disease processes. Rate as renal dysfunction. 7542Neurogenic...

  20. 38 CFR 4.115b - Ratings of the genitourinary system-diagnoses.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ...cortical necrosis: Rate as renal dysfunction. 7541Renal involvement in diabetes mellitus, sickle cell anemia, systemic lupus erythematosus, vasculitis, or other systemic disease processes. Rate as renal dysfunction. 7542Neurogenic...

  1. 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

  2. THE VASCULAR LESIONS OF A COW AND BISON WITH SHEEP-ASSOCIATED MALIGNANT CATARRHAL FEVER CONTAIN OVINE HERPESVIRUS 2-INFECTED CD8+ T LYMPHOCYTES

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Malignant catarrhal fever (MCF) is a serious herpesviral disease syndrome of certain ruminant species. The microscopic pathology of MCF is characterized by lymphoid proliferation and infiltration, necrotizing vasculitis, and necrosis of lymphoid and epithelial tissues. Previous attempts to detect vi...

  3. Undifferentiated Connective Tissue Disease

    MedlinePLUS

    ... vessels. Examples of connective tissue diseases include lupus , scleroderma , rheumatoid arthritis , Sjögren's syndrome , myositis , and vasculitis . There ... connective tissue diseases, such as lupus, Sjögren's or scleroderma. More UCTD Information Causes Diagnosis Symptoms Treatment Print ...

  4. The CARRA Registry

    ClinicalTrials.gov

    2015-04-22

    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

  5. 21 CFR 312.32 - IND safety reports.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

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

  6. 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

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

    MedlinePLUS

    You are here: Home / Types of Vasculitis / Eosinophilic Granulomatosis with Polyangiitis, formerly Churg-Strauss Syndrome (EGPA) Eosinophilic Granulomatosis with Polyangiitis, formerly Churg-Strauss Syndrome (EGPA) First Description Who gets ...

  8. Chemical Sensitivities

    MedlinePLUS

    ... 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 dioxide has ...

  9. ANCA-associated diffuse alveolar hemorrhage due to benzylthiouracil.

    PubMed

    Thabet, Farah; Sghiri, Rim; Tabarki, Brahim; Ghedira, Ibtissem; Yacoub, Moncef; Essoussi, Ahmed Sahloul

    2006-07-01

    Benzylthiouracil has been recently observed to be associated with antineutrophil cytoplasmic antibody-positive vasculitis, resulting in crescentic glomerulonephritis. We report an 8-year-old girl treated with benzylthiouracil for Graves's disease who developed an ANCA-positive vasculitis with pulmonary hemorrhage. She responded to corticosteroids and discontinuation of benzylthiouracil. This represents the first pediatric case of benzylthiouracil-induced diffuse alveolar hemorrhage. PMID:16622664

  10. ANCA-associated diffuse alveolar hemorrhage due to benzylthiouracil

    Microsoft Academic Search

    Farah Thabet; Rim Sghiri; Brahim Tabarki; Ibtissem Ghedira; Moncef Yacoub; Ahmed Sahloul Essoussi

    2006-01-01

    Benzylthiouracil has been recently observed to be associated with antineutrophil cytoplasmic antibody-positive vasculitis, resulting in crescentic glomerulonephritis. We report an 8-year-old girl treated with benzylthiouracil for Graves’s disease who developed an ANCA-positive vasculitis with pulmonary hemorrhage. She responded to corticosteroids and discontinuation of benzylthiouracil. This represents the first pediatric case of benzylthiouracil-induced diffuse alveolar hemorrhage.

  11. The safety and efficacy of a thymidine kinase negative equine herpesvirus 1 vaccine 

    E-print Network

    Cornick, Janyce Louise

    1988-01-01

    -mortem examination, small focal areas of hemorrhage are found randomly throughout the meninges and parenchyma. The most consistent microscopic lesion is vasculitis of small cerebral blood vessels with secondary hypoxic degeneration and resultant areas of malacia... the CNS. The neurologic disease does not appear to be due to a productive viral infection, but is believed to be a result of the ischemia and inflammation secondary to an immune-mediated vasculitis. The histologic lesions closely resemble those caused...

  12. Wegener's granulomatosis presenting as temporal arteritis.

    PubMed

    Small, P; Brisson, M L

    1991-02-01

    A granulomatous giant cell vasculitis of the temporal artery was observed in a biopsy specimen from a patient with corresponding clinical symptoms. Within weeks, the new onset of pulmonary infiltrates and renal failure prompted biopsy of the patient's kidney. A necrotizing glomerulonephritis, compatible with a diagnosis of Wegener's granulomatosis, was present. Vasculitis of the temporal artery may be a feature of Wegener's granulomatosis. PMID:1994920

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

    E-print Network

    Parks, Harrison Lee

    2014-04-28

    and hypergammaglobulinemia are often concomitant, while palpable purpura is associated with dermal vasculitis. Dermal vasculitis is linked with SS-related extraglandular manifestations and lymphoma 20. In a recent study of pSS, 89 of 559 patients (16%) presented... hypothyroidism among any who have common autoimmune diseases 34. Approximately 5% of SS develop lymphoproliferative disorders. B-cell hyperactivity explains increased prevalence of these diseases 35.The odds ratio of 6 lymphoma was 4.75 in pSS and 9...

  14. Renal Involvement in Wegener’s Granulomatosis

    Microsoft Academic Search

    Y Renaudineau; Y Le Meur

    2008-01-01

    The term pauci-immune glomerulonephritis with vasculitis encompasses a group of auto-immune disorders, which includes Wegener’s\\u000a granulomatosis, microscopic polyangiitis, Churg–Strauss syndrome, and renal-limited vasculitis. Over the past few years, progress\\u000a has been made in understanding the epidemiology and environmental and genetic risk factors of the role of antineutrophil cytoplasmic\\u000a antibodies (ANCA) in kidney pathogenesis and the utilization of ANCA in diagnosis.

  15. Livedoid Vasculopathy and Mononeuritis Multiplex, with a Fulminant Hepatic Failure which was caused by Herpes Simplex Hepatitis: A Case Report.

    PubMed

    Pai B, Sathish; Pai, Kanthilatha

    2013-05-01

    Livedoid vasculopathy with mononeuritis multiplex is a rare association. We are presenting a case of an unusual association of livedoid vasculopathy with mononeuritis multiplex, who developed fulminant hepatic failure which was secondary to Herpes simplex virus (HSV) hepatitis, while she was on treatment with immunosuppressants. Her skin biopsy and immunofluorescence studies showed the features of vasculitis. A biopsy from the sural nerve showed the features of chronic vasculitis. PMID:23814745

  16. Livedoid Vasculopathy and Mononeuritis Multiplex, with a Fulminant Hepatic Failure which was caused by Herpes Simplex Hepatitis: A Case Report

    PubMed Central

    Pai B, Sathish; Pai, Kanthilatha

    2013-01-01

    Livedoid vasculopathy with mononeuritis multiplex is a rare association. We are presenting a case of an unusual association of livedoid vasculopathy with mononeuritis multiplex, who developed fulminant hepatic failure which was secondary to Herpes simplex virus (HSV) hepatitis, while she was on treatment with immunosuppressants. Her skin biopsy and immunofluorescence studies showed the features of vasculitis. A biopsy from the sural nerve showed the features of chronic vasculitis. PMID:23814745

  17. 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.

  18. Epidemiology of Vasculitides in Khorasan Province, Iran

    PubMed Central

    Jokar, Mohammadhassan; Mirfeizi, Zahra

    2015-01-01

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

  19. Epidemiology of Vasculitides in Khorasan Province, Iran.

    PubMed

    Jokar, Mohammadhassan; Mirfeizi, Zahra

    2015-07-01

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

  20. Respiratory disease in systemic lupus erythematosus: correlation with results of laboratory tests and histological appearance of muscle biopsy specimens.

    PubMed Central

    Evans, S A; Hopkinson, N D; Kinnear, W J; Watson, L; Powell, R J; Johnston, I D

    1992-01-01

    BACKGROUND: In systemic lupus erythematosus, certain laboratory tests and evidence from muscle biopsy specimens of lymphocytic vasculitis reflect disease activity. A study was designed to determine if such indices predict respiratory lesions, and in particular whether the presence of vasculitis in quadriceps muscle reflects respiratory muscle function. METHODS: Twenty seven 27 patients with systemic lupus erythematosus were studied, ten of whom were consecutive untreated patients and 17 having clinically active disease and being treated. They were prospectively evaluated on the basis of erythrocyte sedimentation rate, lymphocyte count, C3 degradation products, quadriceps muscle biopsy, spirometry, lung volumes, carbon monoxide transfer factor, and mouth pressure during a maximal sniff. RESULTS: Lung function test results were abnormal in 12 patients. Vital capacity was reduced in seven, carbon monoxide transfer factor capacity in five, and mouth pressure was low (< 70% predicted) in ten. Lymphocytic vasculitis was seen in the muscle biopsy specimens of ten patients. No correlation was found between laboratory tests and lung function or mouth pressure, or between the presence of lymphocytic vasculitis and mouth pressure. In untreated patients, those with lymphocytic vasculitis had lower spirometric values. CONCLUSIONS: In systemic lupus erythematosus, evidence from muscle biopsy specimens of lymphocytic vasculitis is not predictive of impaired inspiratory muscle function as measured by mouth pressure. In untreated patients there were relationships between some laboratory test results and respiratory function, but this was not the case for the whole group. In systemic lupus erythematosus, laboratory tests and evidence from muscle biopsy specimens of lymphocytic vasculitis are therefore unlikely to be helpful in the assessment of respiratory disease. Images PMID:1465755

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

    PubMed Central

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

    2015-01-01

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

  2. [Antineutrophil cytoplasmic antibodies and associated diseases].

    PubMed

    Sghiri, R; Meddeb, H; Bouguila, J; Jarray, M; Bahri, F; Nouira, R; Zellama, D; Achour, A; Essoussi, A S; Harbi, A; Ghedira, I

    2009-07-01

    Antineutrophil cytoplasmic antibodies are classical serological markers of small-vessels vasculitis. However, they have been described in many other pathological situations. The aim of this study was to determine through our experience, the main antineutrophil cytoplasmic antibodies-associated diseases and to investigate antigen targets of these antibodies. Forty complete observations of antineutrophil cytoplasmic antibodies (ANCA) positive patients either by indirect immunofluorescence or by enzyme immunoassay were analysed. Only five (12.5%) patients have small-vessels vasculitis. Among these, antineutrophil cytoplasmic antibodies were detected only by Elisa in one patient and they were exclusively directed against bactericidal permeability increasing protein in another one. Our study confirms the presence of antineutrophil cytoplasmic antibodies in different diseases. It demonstrates that antineutrophil cytoplasmic antibodies should be investigated by Elisa when indirect immunofluorescence is negative. In small-vessels vasculitis, Proteinase 3 and myeloperoxidase are mainly but not exclusively the antigenic targets of antineutrophil cytoplasmic antibodies. PMID:18834675

  3. Pre-operative radiological diagnosis of acute necrotizing enteritis in systemic lupus erythematodes.

    PubMed

    Decrop, E; Ponette, E; Baert, A L; Verberckmoes, R; Kerremans, R; Geboes, K

    1990-01-01

    Acute abdominal complications of systemic lupus erythematodes requiring laparatomy are not frequent. A 30-year-old man with systemic lupus erythematodes developed a necrotizing enteritis of the small bowel with pneumatosis intestinalis. Only one case of intestinal vasculitis associated with systemic lupus erythematodes has previously been reported. Presence of intramural gas in the small bowel and of gas in the portal vein was detected on plain abdominal film and confirmed by subsequent CT examination. Since corticosteroids often mask the severity of the disease, radiological evaluation is essential to the overall management of the patient with intestinal vasculitis. Due to the observed radiological signs, emergency laparotomy was performed. Histologic examination revealed necrotizing enteritis due to vasculitis. PMID:2318798

  4. Henoch-Schonlein Purpura—A Case Report and Review of the Literature

    PubMed Central

    Sohagia, Amit B.; Gunturu, Srinivas Guptha; Tong, Tommy R.; Hertan, Hilary I.

    2010-01-01

    We describe a case of an adolescent male with Henoch-Schonlein purpura (HSP), presenting with cutaneous and gastrointestinal manifestations. Endoscopy revealed diffuse ulcerations in the stomach, duodenum, and right colon. Biopsies revealed a leukocytoclastic vasculitis in the skin and gastrointestinal tract. Steroid therapy led to complete resolution of the symptoms. HSP is the most common childhood vasculitis, and is characterized by the classic tetrad of nonthrombocytopenic palpable purpura, arthritis or arthralgias, gastrointestinal and renal involvement. It is a systemic disease where antigen-antibody (IgA) complexes activate the alternate complement pathway, resulting in inflammation and small vessel vasculitis. Mild disease resolves spontaneously, and symptomatic treatment alone is sufficient. Systemic steroids are recommended for moderate to severe HSP. The prognosis depends upon the extent of renal involvement, which requires close followup. Early recognition of multiorgan involvement, especially outside of the typical age group, as in our adolescent patient, and appropriate intervention can mitigate the disease and limit organ damage. PMID:20508739

  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. Neurologic manifestations of Henoch-Schönlein purpura.

    PubMed

    Bérubé, Maxime D; Blais, Normand; Lanthier, Sylvain

    2014-01-01

    Henoch-Shönlein purpura (HSP) is a systemic small vessel vasculitis. Most patients present during childhood. The characteristic association of purpura, arthralgia, abdominal pain, and nephritis reflects the predominant distribution of vasculitis. Headaches and mild behavioral changes suggest CNS involvement in one-third of HSP patients. Salient central nervous system (CNS) manifestations are rarer and mostly reported in adults and patients with a severe disease course. Diagnosis of CNS vasculitis is rarely confirmed by histopathology and generally relies on "suggestive" imaging showing brain hemorrhages, infarcts and edema, predominantly located in the parieto-occipital regions. Vessel wall friability and thrombogenicity of active vasculitis, antiphospholipid antibody synthesis, and other hemostatic disturbances may contribute to hemorrhagic and thrombotic complications of HSP. Posterior reversible encephalopathy syndrome and hypertensive encephalopathy occur in HSP and can be difficult to differentiate from CNS vasculitis. Some 53% of patients with neurologic complications experience seizures. Cerebral venous thrombosis, subdural hematoma, subarachnoidal hemorrhage, neuro-ophthalmologic complications, myelopathy, and diverse neuromuscular manifestations are also reported. In contrast with other systemic small vessel vasculitides, peripheral nervous system involvement is infrequent in HSP. Systemic involvement of HSP and homeostatic disorders such as hypertension, uremia, and electrolyte disturbances, as well as superimposed infections can affect the nervous system secondarily. Identification of nervous system complications of HSP is often challenging due to prominent systemic manifestations. HSP is usually a self-limiting disease that requires only supportive care. Patients with CNS vasculitis are commonly treated with corticosteroids. One-fifth of patients with CNS involvement remain with sequelae. PMID:24365374

  7. [Pulmonary-renal syndrome].

    PubMed

    Risso, Jorge A; Mazzocchi, Octavio; De All, Jorge; Gnocchi, César A

    2009-01-01

    The pulmonary-renal syndrome is defined as a combination of diffuse alveolar hemorrhage and glomerulonephritis. The coexistence of these two clinical conditions is due to diseases with different pathogenic mechanisms. Primary systemic vasculitis and Goodpasture syndrome are the most frequent etiologies. Systemic lupus erythematosus, connective tissue diseases, negative anti neutrophil cytoplasmic antibody vasculitis and those secondary to drugs are far less common causes. An early diagnosis based on clinical, radiologic, laboratory and histologic criteria enables early treatment, thus diminishing its high morbidity-mortality rate. Therapy is based on high doses of corticosteroids, immunosuppressants, tumor necrosis factor inhibitors and plasmapheresis. PMID:20053612

  8. Dyspnea due to pulmonary vessel arteritis

    PubMed Central

    Gilmour, Suzana M; Dominelli, Giulio S; Leipsic, Jonathon A; Levy, Robert D

    2014-01-01

    Pulmonary arteritis is a rare cause of pulmonary hypertension. Causes of pulmonary arteritis can be divided into primary and secondary, as well as classified according to vessel size. Only large vessel vasculitis is associated with pulmonary hypertension; primary forms include Takayasu arteritis and giant cell arteritis. The diagnosis of pulmonary arteritis can be challenging and the associated morbidity is serious without prompt, directed treatment. The authors present a case involving a 48-year-old First Nations man presenting with a six-month history of exertional dyspnea and severe stenosis of the left pulmonary artery, who was ultimately diagnosed with pulmonary arteritis related to large vessel vasculitis. PMID:24524110

  9. Old drug new trick: levamisole-adulterated cocaine causing acute kidney injury.

    PubMed

    Ammar, Abeer T; Livak, Mark; Witsil, Joanne C

    2015-02-01

    Levamisole is an agent previously used in humans and later withdrawn from the US drug market due to concerns of agranulocytosis.It is currently used as an adulterating agent in cocaine, bringing to light toxicities typically manifested by vasculitis and skin necrosis.We report a case of a 36-year-old crack cocaine user who presented with a purpuric rash on her face and limbs. Levamisole-induced vasculitis was suspected, and she therefore underwent an extensive work-up. In addition to these findings, she also presented with acute kidney injury of unknown etiology, which was later attributed to levamisoleadulterated cocaine. PMID:25195046

  10. [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

  11. Takayasu's or tuberculous arteritis?

    PubMed

    Moura, Carlos; Aquino, Mauricio Antonio; Rocha Filho, Jose; Santiago, Mittermayer

    2015-01-01

    Takayasu's arteritis (TA) is a large vessel vasculitis of unknown aetiology characterised by involvement of the aorta and its major branches. Tuberculous arteritis of the aorta is an uncommon condition usually secondary to the dissemination of Mycobacterium tuberculosis infection from the mediastinum and/or lung to the adjacent aorta; this may mimic TA. We report a case of a 23-year-old woman with cutaneous granulomatous vasculitis and saccular aneurysmal dilation of the aorta and large vessels, and highlight the findings shared by TA and tuberculous arteritis. PMID:26106169

  12. The first case of Henoch-Schonlein purpura associated with rosuvastatin: colonic involvement coexisting with small intestine.

    PubMed

    Gonen, Korcan Aysun; Erfan, Gamze; Oznur, Meltem; Erdogan, Cuneyt

    2014-01-01

    Henoch-Schönlein purpura (HSP) is a systemic vasculitis affecting small vessels. It is the most common systemic vasculitis in children, and is rare in adults. Serious gastrointestinal complications are more common in childhood. Infections and drugs are the most prominent factors in the aetiology. Wall thickening in segments of the small intestine is commonly seen in imaging studies in gastrointestinal system (GIS) involvement. Simultaneous involvement of small intestine and colon is rare. An HSP case involving small intestine and colon in an adult patient due to the use of rosuvastatin, an antihyperlipidaemic agent, is presented, and is first of its kind reported in the literature. PMID:24648473

  13. 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

  14. 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

  15. [Skin necrosis: report of eleven cases].

    PubMed

    Molgó, Montserrat N; Arriagada, Camila E; Salomone, Claudia B; Vera, Cristián K; Giesen, Laura F; Solar, Antonieta G; González, Sergio B

    2014-01-01

    Skin necrosis must be considered as a syndrome, because it is a clinical manifestation of different diseases. An early diagnosis is very important to choose the appropriate treatment. Therefore, its causes should be suspected and confirmed quickly. We report eleven patients with skin necrosis seen at our Department, caused by different etiologies: Warfarin-induced skin necrosis, loxoscelism, diabetic microangiopathy, ecthyma gangrenosum, disseminated intravascular coagulation, necrotizing vasculitis, paraneoplastic extensive necrotizing vasculitis, livedoid vasculopathy, necrotizing fasciitis, necrosis secondary to the use of vasoactive drugs and necrosis secondary to the use of cocaine. We also report the results of our literature review on the subject. PMID:24861125

  16. Drug-Induced Glomerular Disease: Immune-Mediated Injury.

    PubMed

    Hogan, Jonathan J; Markowitz, Glen S; Radhakrishnan, Jai

    2015-07-01

    Drug-induced autoimmune disease was initially described decades ago, with reports of vasculitis and a lupus-like syndrome in patients taking hydralazine, procainamide, and sulfadiazine. Over the years, multiple other agents have been linked to immune-mediated glomerular disease, often with associated autoantibody formation. Certain clinical and laboratory features may distinguish these entities from their idiopathic counterparts, and making this distinction is important in the diagnosis and management of these patients. Here, drug-induced, ANCA-associated vasculitis, drug-induced lupus, and drug-associated membranous nephropathy are reviewed. PMID:26092827

  17. Infection of Human Endothelial Cells with Spotted Fever Group Rickettsiae Stimulates Cyclooxygenase 2 Expression and Release of Vasoactive Prostaglandins

    Microsoft Academic Search

    Elena Rydkina; Abha Sahni; Raymond B. Baggs; David J. Silverman; Sanjeev K. Sahni

    2006-01-01

    Rickettsiae, a diverse group of obligately intracellular gram-negative bacteria, include etiologic agents of the spotted fever and typhus groups of diseases. Rocky Mountain spotted fever and boutonneuse fever, due to Rickettsia rickettsii and R. conorii, respectively, are characterized by widespread infection of the vascular endothelium, microvascular injury, and vasculitis. Cultured human endothelial cells (EC) are highly suscep- tible to infection

  18. Secondary Erythermalgia Associated with an Autoimmune Disorder of Undetermined Significance

    Microsoft Academic Search

    J. P. H. Drenth; J. J. M. Michiels; T. Van Joost; V. D. Vuzevski

    1995-01-01

    A 50-year-old female patient is described with an acquired, persisting and yet incurable erythermalgia featured by symmetric burning pain and red congestion of the extremities secondary to cutaneous vasculitis. A weakly positive antinuclear antibody titer and high titers of antibodies against gastric parietal mucosa cells pointed to an underlying but unclassifiable autoimmune disorder. It is concluded that histopathology of lesional

  19. Autoantigen complementarity: a new theory implicating complementary proteins as initiators of autoimmune disease

    Microsoft Academic Search

    William F. Pendergraft; Barrak M. Pressler; J. Charles Jennette; Ronald J. Falk; Gloria A. Preston

    2005-01-01

    Autoimmune diseases affect approximately 1 in 21 persons in the United States. Treatment often requires long-term cytotoxic therapy. How and why these deleterious diseases occur is unclear. A serendipitous finding in our laboratory using serum from patients with autoimmune vasculitis led us to develop the theory of autoantigen complementarity, a novel concept that may elucidate the etiological and pathogenetic mechanisms

  20. Anticorps anticytoplasme des polynucléaires neutrophiles et pathologies associées

    Microsoft Academic Search

    R. Sghiri; H. Meddeb; J. Bouguila; M. Jarray; F. Bahri; R. Nouira; D. Zellama; A. Achour; A. S. Essoussi; A. Harbi; I. Ghedira

    2009-01-01

    Antineutrophil cytoplasmic antibodies are classical serological markers of small-vessels vasculitis. However, they have been described in many other pathological situations. The aim of this study was to determine through our experience, the main antineutrophil cytoplasmic antibodies – associated diseases and to investigate antigen targets of these antibodies. Forty complete observations of antineutrophil cytoplasmic antibodies (ANCA) positive patients either by indirect

  1. 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

  2. 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

  3. 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

  4. Cytotoxicity to Endothelial Cells by Sera from Aged MRL\\/ lpr\\/ lpr Mice Is Associated with Autoimmunity to Cell Surface Heparan Sulfate

    Microsoft Academic Search

    Alexandra Dimitriu-Bona; Maja Matic; Wanhong Ding; Chang Pei Yang; Howard Fillit

    1995-01-01

    Vasculitis is an common clinical feature of systemic lupus erythematosus (SLE) in humans and in animal models of this disease. Humoral autoimmunity against endothelial cells has been previously demonstrated in SLE and other autoimmune disorders, but the precise cell surface antigenic targets involved in the initiation and progression of vascular injury are still essentially unknown. In the current studies, we

  5. Bilateral posterior circulation stroke secondary to a crotalid envenomation: case report.

    PubMed

    Vale, Thiago Cardoso; Leite, Alysson Ferreira; Hora, Priscila Ribeiro da; Coury, Marayra Inês França; Silva, Ricardo Cipriano da; Teixeira, Antônio Lúcio

    2013-01-01

    Snake bite envenoming is a disease with potential serious neurological complications. We report a case of an adolescent who was bitten by a rattlesnake and developed bilateral posterior circulation stroke. The rattlesnake was later identified as being Crotalus durissus terrificus. Stroke was probably due to toxic vasculitis or toxin-induced vascular spasm and endothelial damage. PMID:23740059

  6. Detection of Antiendothelial Cell Antibodies by an Enzyme-Linked Immunosorbent Assay Using Antigens from Cell Lysate: Minimal Interference with Antinuclear Antibodies and Rheumatoid Factors

    Microsoft Academic Search

    Christian Drouet; Marie-France Nissou; Denise Ponard; Josiane Arvieux; Chantal Dumestre-Perard; Philippe Gaudin; Bernard Imbert; Christian Massot; Francoise Sarrot-Reynauld

    2003-01-01

    Antiendothelial cell antibodies (AECAs), a heterogeneous group of autoantibodies, are associated with several diseases characterized by immune-mediated vascular damage (for re- views, see references 2 and 13), including systemic lupus ery- thematosus (19), systemic sclerosis (17), Wegener's granulo- matosis (6), and rheumatoid arthritis complicated by vasculitis (7). Even if they recognize poorly characterized targets, they may be valuable as markers

  7. C3-containing serum immune complexes in patients with systemic lupus erythematosus: correlation to disease activity and comparison with other rheumatic diseases

    Microsoft Academic Search

    C. Huber; A. Riiger; M. Herrmann; F. Krapf; J. R. Kalden

    1989-01-01

    Although testing for circulating immune complexes (CIC) is regarded as a useful, complementary, laboratory parameter in the differential diagnosis and management of immune complex-induced vasculitis syndromes, there is still an uncertainty with regard to assay systems used for the demonstration of soluble immune complexes. This is partly due to difficulties in the reproducibility, handling and principle limitations of available test

  8. 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

  9. Henoch-Schönlein purpura with intracerebral haemorrhage in an adult patient: a case report

    Microsoft Academic Search

    Lazarus Karamadoukis; Linmarie Ludeman; Anthony J Williams

    2008-01-01

    INTRODUCTION: Henoch-Schönlein purpura is a small vessel vasculitis that affects mainly the skin, joints, gastrointestinal tract and kidneys. The central nervous system is also occasionally affected, although the majority of patients experience only mild symptoms such as headaches and behavioural changes. Intracerebral haemorrhage is a rare complication of Henoch-Schönlein purpura that so far has mainly been described in children and

  10. Henoch- Schönlein Purpura Associated with Major Complications

    Microsoft Academic Search

    Mohammad Zahidul Islam

    2010-01-01

    Henoch Schönlein Purpura is a vasculitis syndrome involving small vessels and characterized by deposition of IgA1 in the renal mesangium and in the blood vessels. It can occur at any age but occurs mostly between the ages of 3 to 15 years, more commonly in boys. The onset of HSP maybe preceded by a upper respiratory tract infection. HSP is

  11. cANCA-associated aortitis

    PubMed Central

    Amos, Liv A.; Roberts, Matthew A.; Blair, Susan

    2012-01-01

    Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is traditionally viewed as a small vessel disease. We report a patient with cANCA antibodies directed against proteinase-3 with asymptomatic aortic involvement, in combination with diffuse alveolar haemorrhage and pauci-immune, necrotizing crescentic glomerulonephritis. A review of the literature is discussed.

  12. Mucormycosis associated with carotid cavernous fistula and cavernous carotid mycotic aneurysm.

    PubMed

    Saff, G; Frau, M; Murtagh, F R; Silbiger, M L

    1989-10-01

    Mucormycosis has not been described previously as a cause of carotid-cavernous sinus fistula or mycotic aneurysm, certainly not coexisting in the same patient. CT and angiographic findings are presented in a patient who had a fistula, mycotic aneurysm, and associated cerebral vasculitis. PMID:2809556

  13. Cogan's syndrome: an oculo-audiovestibular disease

    Microsoft Academic Search

    J R García Berrocal; J A Vargas; M Vaquero; S Ramón y Cajal; R A Ramírez-Camacho

    1999-01-01

    Typical Cogan's syndrome is a rare disease of young adults consisting of flares of interstitial keratitis and sudden onset of Ménière-like attacks (nausea, vomiting, tinnitus, vertigo and hearing loss). Life-threatening aortic insufficiency develops in 10% of reported cases. Atypical Cogan's syndrome (audiovestibular dysfunction with other types of inflammatory eye disease) is associated with vasculitis in 20% of cases and has

  14. Rift Valley fever ocular manifestations: observations during the 1977 epidemic in Egypt

    Microsoft Academic Search

    A L Siam; J M Meegan; K F Gharbawi

    1980-01-01

    Ocular manifestations resulting from Rift Valley fever (RVF) virus infection were studied during an extensive RVF epidemic in Egypt during 1977. Colour photography and fluorescein angiography of 7 serologically diagnosed patients showed the commonest manifestations to be macular, paramacular, and\\/or extramacular retinal lesions, often occurring bilaterally. Haemorrhage and oedema were frequently associated with the lesions, and vasculitis, retinitis, and vascular

  15. Aortic aneurysm and orchitis due to Wegener's granulomatosis.

    PubMed

    Minnee, R C; van den Berk, G E L; Groeneveld, J O; van Dijk, J; Turkcan, K; Visser, M J; Vahl, A C

    2009-01-01

    We present a patient with Wegener's granulomatosis (WG) with involvement of the abdominal aorta, testis, peripheral nerve system, and skin. A 51-year-old man presented at our outpatient clinic with lower back pain. He had a history of smoking, hypertension, and an embryonal carcinoma of the left testis, treated 13 years ago with orchidectomy and chemotherapy. One month earlier, he underwent a partial orchidectomy of the right testis due to testicular swelling. Abdominal computed tomography showed a 3.8 cm wide aneurysm of the distal part of the aorta with inflammation. One week later he was admitted to the hospital with numbness of his hands and feet. Physical examination showed signs of peripheral microemboli. Serological laboratory tests revealed elevated antineutrophil cytoplasmic antibody titers with positive reactions against proteinase-3, indicating Wegener's disease. The chest X-ray was normal. Pathological examination of the right testis showed necrotizing vasculitis of a small artery. He was treated with cyclophosphamide and prednisolone. WG with extrapulmonary involvement occurs infrequently, and reports of manifestations of WG in aorta, testis, the peripheral nerve system, and skin are even more uncommon. Small- and medium-vessel vasculitis can precede large-vessel vasculitis or occur in the absence of small-vessel involvement. Therefore, WG should be included in the work-up of large-vessel vasculitis, which can give rise to periaortic inflammation. PMID:19748223

  16. A case of Behcet's disease with abdominal aortic aneurysm

    Microsoft Academic Search

    Mansoor Karimifar; Mansour Salesi; Mozhgan Karimifar; Peyman Mottaghi

    2009-01-01

    Behcet's disease is a multisystem disease that is mainly classified as vasculitis and may involve many organs like skin, mucosa, eyes, joints, vessels, nervous system, gastrointestinal tract, testis, heart and lungs. This case depicts a rare presentation of Behcet's disease with multiple saccular aneurysms in a 33 year old man. A patient with a persistent pain in the back and

  17. Abdominal angina: an unusual presentation of Takayasu’s arteritis

    Microsoft Academic Search

    S C Chaudhary; A Gupta; D Himanshu; S P Verma; R Khanna; D K Gupta

    2011-01-01

    Takayasu’s arteritis (TA) is an idiopathic large vessel vasculitis of young adults that affects the aorta and its major branches. The authors hereby present a case of TA that presented with abdominal aorta thrombosis. She was put on low-molecular weight heparin, antiplatelets, corticosteroids, cyclophosphamide along with haematinics and was referred to Department of Cardiothoracic and Vascular Surgery for further management.

  18. Lupus 101Lupus 101 Division of Allergy, Immunology & Rheumatology

    E-print Network

    Goldman, Steven A.

    Lupus 101Lupus 101 Division of Allergy, Immunology & Rheumatology University of Rochester Medical myositis Multiple sclerosis Vasculitis Myasthenia gravis Lupus is a SystemicLupus is a Systemic Autoimmune DiseaseAutoimmune Disease #12;Systemic Lupus Erythematosus · Inflammatory multisystem disease primarily

  19. Thrombosis in systemic lupus erythematosus: striking association with the presence of circulating lupus anticoagulant

    Microsoft Academic Search

    M L Boey; C B Colaco; A E Gharavi; K B Elkon; S Loizou; G R Hughes

    1983-01-01

    The lupus anticoagulant was found in the plasma of 31 of 60 patients with systemic lupus erythematosus and other connective tissue disorders (mixed connective tissue disease, systemic vasculitis, polyarteritis nodosa, primary sicca syndrome, discoid lupus, Behcet's syndrome, and systemic sclerosis). Strong associations were found with biological false positive seroreaction for syphilis and thrombocytopenia. The most striking association, however, was with

  20. Fluoxetin-induced pulmonary granulomatosis.

    PubMed

    de Kerviler, E; Trédaniel, J; Revlon, G; Groussard, O; Zalcman, G; Ortoli, J M; Espié, M; Hirsch, A; Frija, J

    1996-03-01

    A patient treated with fluoxetin for a manic depressive disorder developed pulmonary inflammatory nodules with noncaseating giant cell granulomas, interstitial pneumonia and non-necrotizing vasculitis, whilst remaining asymptomatic. A progressive resolution of pulmonary nodules occurred after withdrawal of the offending agent, and the chest radiograph returned to normal in 9 months. The diagnosis was assessed by an open lung biopsy. PMID:8730028

  1. Relapsing polychondritis: An autoimmune disease with many faces

    Microsoft Academic Search

    Tobias Lahmer; Matthias Treiber; Alexander von Werder; Frauke Foerger; Andreas Knopf; Uwe Heemann; Klaus Thuermel

    2010-01-01

    Relapsing polychondritis (RPC) is a rare immune mediated disease which is associated with inflammation in cartilaginous tissue throughout the body. Especially the cartilaginous structures of ear, nose, joints and respiratory tract are affected. In around 30% of the cases an association with other diseases especially systemic vasculitis or myelodysplatic syndrome can be detected.The relative rarity of RPC has not permitted

  2. POLICONDRITIS RECIDIVANTE Y COMPROMISO NEUROLOGICO

    Microsoft Academic Search

    MARIA A. REQUENA; DANIEL A. PALMA; BODGAN M. POPESCU

    Neurologic involvement in relapsing polychondritis. Relapsing polychondritis is a multysistemic in- flammatory disease, of unknown etiology. It is characterized by recurrent episodes of inflammation with progressive destruction of cartilaginous structures and connective tissue. It may also affect tissues that con- tain proteoglycans like eyes, heart, kidney, and blood vessels causing a picture of vasculitis. We report here the case of

  3. Quand penser à une maladie systémique en réanimation ?

    Microsoft Academic Search

    B. Godeau

    2005-01-01

    Severe organ dysfunction related to systemic rheumatic diseases (SRD) occasionnaly can be the first manifestation and justify an admission in intensive care unit. It mainly concerns systemic necrotizing vasculitis, systemic lupus erythematosus and inflammatory myositis with pulmonary, renal or central nervous system involvements. This article reviews the main visceral and biological complications which can reveal SRD. The main autoantibodies (i.e.

  4. Muscle biopsy abnormalities in systemic lupus erythematosus: correlation with clinical and laboratory parameters

    Microsoft Academic Search

    K L Lim; R Abdul-Wahab; J Lowe; R J Powell

    1994-01-01

    OBJECTIVES--To investigate the incidence and significance of Type II fibre atrophy, vessel wall thickening, lymphocytic vasculitis and myositis in needle quadriceps muscle biopsies from patients with systemic lupus erythematosus (SLE) and their correlations with clinical and laboratory parameters. METHODS--Needle quadriceps muscle biopsies from 55 patients with SLE and 26 controls were prospectively examined. Clinical and laboratory parameters recorded at the

  5. An atypical case of Henoch-Shonlein purpura in a young patient: treatment of the skin lesions with hyaluronic acid-based dressings.

    PubMed

    Carella, Sara; Maruccia, Michele; Fino, Pasquale; Onesti, Maria Giuseppina

    2013-01-01

    Henoch-Shönlein purpura (HSP) is an acute, self-limited, systemic, small vessel vasculitis, that induces skin lesions, arthritis and abdominal pain. Palpable purpura is the most common manifestation in pediatric patients with HSP. We present an atypical case of HSP in a young patient and report successful treatment of the atypical skin lesions, while avoiding surgery. PMID:23239864

  6. Toxocariasis After Slug Ingestion Characterized by Severe Neurologic, Ocular, and Pulmonary Involvement

    PubMed Central

    Fellrath, Jean-Marc; Magnaval, Jean-François

    2014-01-01

    Human toxocariasis is generally a benign, self-curing disease, and neurologic involvement is quite exceptional. In this study, we report a case of toxocariasis caused by ingestion of an unusual transport host, namely live slugs. The clinical picture comprised eosinophilic lung involvement with severe neurologic disorders in relation to vasculitis as well as retinal detachment. PMID:25734133

  7. Adalimumab-associated antiphospholipid syndrome: a case report and review of the literature.

    PubMed

    Hemmati, Iman; Kur, Jason

    2013-07-01

    This study aims for the presentation of the first reported case of adalimumab-associated antiphospholipid syndrome (APS) and review of the literature on adalimumab-induced vasculitis and APS. A case of APS associated with adalimumab use in a 67-year-old woman is reported. The English medical literature was reviewed for antitumor necrosis factor (TNF) agents and their association with APS and vasculitis. Adalimumab is a fully humanized monoclonal antibody targeted against TNF alpha that is widely used in the treatment of rheumatoid arthritis, juvenile idiopathic arthritis, ankylosing spondylitis, psoriatic arthritis, psoriasis, and Crohn's disease. Literature review reveals several cases of anti-TNF-induced vasculitis including cases associated with adalimumab. We report the first case of adalimumab-induced APS in a 67-year-old woman who developed APS and vasculitis associated with de novo positive anti-cardiolipin (aCL) antibody following the third dose of adalimumab therapy for the treatment of spondyloarthropathy. This is the first case demonstrating that a short course of adalimumab therapy may induce immunoglobulin M aCL autoantibodies leading to APS. With the growing use of anti-TNF medications in immune-mediated and inflammatory diseases, adalimumab and other anti-TNF medications should be considered as a possible explanation for APS. PMID:23559388

  8. The Fix Issue 3 

    E-print Network

    Multiple Contributors

    2013-11-27

    SS 1. Cutaneous manifestations of SS include xerosis, angular cheilitis, eyelid dermatitis, prutitus, cutaneous vasculitis (often as palpable purpura), and erythema annulare 17. Cutaneous rashes of SS often cannot be distinguished clinically... syndrome’ symptoms may be mimicked by amyloidosis, trauma, and scaring. Chronic blepharitis or conjunctivitis, neurogenic 7 impairment of eyelid function or lacrimal gland dysfunction may result in xerophthalmia. Diabetes mellitus, hypercalcaemia...

  9. The relationship of asthma therapy and Churg-Strauss syndrome: NIH workshop summary report

    Microsoft Academic Search

    Peter F. Weller; Marshall Plaut; Virginia Taggart; Anne Trontell

    2001-01-01

    The Churg-Strauss syndrome (CSS) is a distinct form of vasculitis that is notable for its eosinophilia and frequent associations with asthma and sinusitis. Because there has been an increasing recognition that CSS can develop in patients with asthma and that CSS might be associated with specific asthma treatments, the National Heart, Lung, and Blood Institute, the National Institute of Allergy

  10. 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

  11. Behcet's disease: evaluation of a new instrument to measure clinical activity

    Microsoft Academic Search

    B. B. Bhakta; P. Brennan; T. E. James; M. A. Chamberlain; B. A. Noble; A. J. Silman

    1999-01-01

    Objective. Behcet's disease (BD) is a rare multisystem disorder characterized by vasculitis. At present, there are no laboratory markers that correlate well with the clinical activity in BD. This has led to the development of an instrument (BD Current Activity Form) to measure activity. Scoring is based on the history of new clinical features present over the preceding 4 weeks

  12. Current Concepts in the Etiology and Treatment of Behçet Disease

    Microsoft Academic Search

    Cem Evereklioglu

    2005-01-01

    Behçet disease is an idiopathic, multisystem, chronic, and recurrent disease characterized by exacerbations alternating with phases of quiescence, episodic panuveitis, and aggressive non-granulomatous occlusive vasculitis of the arteries and veins of any size with explosive ocular inflammatory attacks that primarily affect the retinal and anterior segment vasculature of the eye. The disease is characterized by endothelial dysfunction and may produce

  13. Ocular immunopathology of Behçet's disease

    Microsoft Academic Search

    Roger K. George; Chi-Chao Chan; Scott M. Whitcup; Robert B. Nussenblatt

    1997-01-01

    A patient developed progressive, severe, recurrent bilateral iridocyclitis, retinal vasculitis, and hemorrhagic infarction of the retina that led to blindness despite immunosuppressive therapy. Histopathology of an enucleated blind and painful eye revealed marked nongranulomatous uveitis with a predominantly CD4 + T-lymphocytic infiltration, as well as B-cell and plasma cell aggregation. Extensive expression of adhesion molecules on vascular endothelial cells were

  14. Do synovial biopsies help to support evidence for involvement of innate immunity in the immunopathology of Behçet's disease?

    PubMed

    van Laar, Jan A M; Kappen, Jasper H; van Daele, Paul L A; van Hagen, P Martin

    2009-01-01

    Behçet's disease is a complex vasculitis of unknown etiology. Abundant neutrophils suggest the involvement of innate immunity. Cytokines are skewed to the T-helper-1 pattern. Few sterile organs are easily accessible for analysis in Behçet's disease. Cañete and coworkers identify inflamed joints as a feasible model and suggest the involvement of innate immunity in Behçet's disease. PMID:19439057

  15. 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

  16. A Single-Base Deletion in the Thrombopoietin (TPO) Gene Causes Familial Essential Thrombocythemia Through a Mechanism of More Efficient Translation of TPO mRNA

    Microsoft Academic Search

    Fabio Stagno; Patrizia Guglielmo; Ugo Consoli; Paolo Fiumara; Mario Russo; Rosario Giustolisi

    37 months), we noticed the appearance of painful perimalleolar skin ulcers. Ulcers appeared with an erythematous border, whereas the epidermidis was replaced by a fibrinous exudate and the dermis scattered by necrotic areas. Skin biopsies of the ulcerated lesions showed in all cases an histologic picture compatible with small vessel vasculitis. Circulating immune complexes were not detectable and doppler-fluximetry was

  17. Advances in the Pathology of Glomerular Diseases Jan J. Weening, Pierre Ronco and Giuseppe Remuzzi

    E-print Network

    of many forms of glomerulonephritis. This new knowledge has facilitated classification and treatment of the renal biopsy in predicting longterm outcome in lupus nephritis, vasculitis and IgA nephropathy and pathogenesis of several forms of renal disease and their evolution over time [1]. The introduction of the renal

  18. Primary glomerular disease

    Microsoft Academic Search

    Peter Mathieson

    2011-01-01

    This article reviews the clinical features, pathogenesis, investigation and management of glomerulonephritis (GN). This can occur as a primary isolated renal disease, as a manifestation of systemic diseases such as vasculitis or lupus, or secondary to drugs, infections or tumours. It is an important cause of morbidity and mortality and a potentially preventable cause of end-stage renal disease, so early

  19. Primary glomerular disease

    Microsoft Academic Search

    Momir Macanovic; Peter Mathieson

    2007-01-01

    This article reviews the clinical features, pathogenesis, investigation and management of glomerulonephritis (GN). This can occur as a primary isolated renal disease, as a manifestation of systemic diseases such as vasculitis or lupus, or secondary to drugs, infections or tumours. It is an important cause of morbidity and mortality and a potentially preventable cause of end-stage renal disease, so early

  20. Anti-neutrophil antibodies in inflammatory bowel disease: prevalence and diagnostic role

    Microsoft Academic Search

    G Cambridge; D S Rampton; T R Stevens; D A McCarthy; M Kamm; B Leaker

    1992-01-01

    Anti-neutrophil antibodies have been shown in sera from patients with a variety of inflammatory diseases. Those reacting with components of neutrophil cytoplasm are associated with systemic vasculitis. Both nuclear and perinuclear staining patterns on human neutrophils have been reported using sera from patients with inflammatory bowel disease. We have evaluated the reactivity against human neutrophils of sera from 100 patients

  1. Genetic polymorphisms in Kawasaki disease

    Microsoft Academic Search

    Ho-chang Kuo; Wei-chiao Chang

    2011-01-01

    Kawasaki disease (KD) is an acute febrile systemic vasculitis, and the cause of KD is not well understood. It is likely due to multiple interactions between genes and environmental factors. The development of genetic association and genome-wide association studies (GWAS) has opened an avenue to better understanding the molecular mechanisms underlying KD. A novel ITPKC signaling pathway was recently found

  2. 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 ...

  3. 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

  4. 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...

  5. Phenomics in Autoimmune and Inflammatory Diseases

    ClinicalTrials.gov

    2015-06-04

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

  6. 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...

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

    Technology Transfer Automated Retrieval System (TEKTRAN)

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

  8. www.nature.com/reviews Leadingthe field

    E-print Network

    Cai, Long

    * Nature Reviews Nephrology* Nature Reviews Neurology* Nature Reviews Rheumatology* Nature Reviews Urology to classification, ANCA detection and trial design focus Vasculitis RHEUMATOLOGY nrrheum_OFC_AUG14.indd 1 15. www.nature.com/reviews/neurol Nature Reviews Rheumatology Chief Editor: Jenny Buckland, PhD Impact

  9. 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.

  10. 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

  11. 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

  12. Three Linked Vasculopathic Processes Characterize Kawasaki Disease: A Light and Transmission Electron Microscopic Study

    PubMed Central

    Orenstein, Jan Marc; Shulman, Stanford T.; Fox, Linda M.; Baker, Susan C.; Takahashi, Masato; Bhatti, Tricia R.; Russo, Pierre A.; Mierau, Gary W.; de Chadarévian, Jean Pierre; Perlman, Elizabeth J.; Trevenen, Cynthia; Rotta, Alexandre T.; Kalelkar, Mitra B.; Rowley, Anne H.

    2012-01-01

    Background Kawasaki disease is recognized as the most common cause of acquired heart disease in children in the developed world. Clinical, epidemiologic, and pathologic evidence supports an infectious agent, likely entering through the lung. Pathologic studies proposing an acute coronary arteritis followed by healing fail to account for the complex vasculopathy and clinical course. Methodology/Principal Findings Specimens from 32 autopsies, 8 cardiac transplants, and an excised coronary aneurysm were studied by light (n=41) and transmission electron microscopy (n=7). Three characteristic vasculopathic processes were identified in coronary (CA) and non-coronary arteries: acute self-limited necrotizing arteritis (NA), subacute/chronic (SA/C) vasculitis, and luminal myofibroblastic proliferation (LMP). NA is a synchronous neutrophilic process of the endothelium, beginning and ending within the first two weeks of fever onset, and progressively destroying the wall into the adventitia causing saccular aneurysms, which can thrombose or rupture. SA/C vasculitis is an asynchronous process that can commence within the first two weeks onward, starting in the adventitia/perivascular tissue and variably inflaming/damaging the wall during progression to the lumen. Besides fusiform and saccular aneurysms that can thrombose, SA/C vasculitis likely causes the transition of medial and adventitial smooth muscle cells (SMC) into classic myofibroblasts, which combined with their matrix products and inflammation create progressive stenosing luminal lesions (SA/C-LMP). Remote LMP apparently results from circulating factors. Veins, pulmonary arteries, and aorta can develop subclinical SA/C vasculitis and SA/C-LMP, but not NA. The earliest death (day 10) had both CA SA/C vasculitis and SA/C-LMP, and an “eosinophilic-type” myocarditis. Conclusions/Significance NA is the only self-limiting process of the three, is responsible for the earliest morbidity/mortality, and is consistent with acute viral infection. SA/C vasculitis can begin as early as NA, but can occur/persist for months to years; LMP causes progressive arterial stenosis and thrombosis and is composed of unique SMC-derived pathologic myofibroblasts. PMID:22723916

  13. Cardiac manifestations in Behcet's disease

    PubMed Central

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

    2015-01-01

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

  14. Paediatric Behçet's disease presenting with recurrent papillitis and episcleritis: a case report

    PubMed Central

    2011-01-01

    Introduction Behçet's disease is a chronic multisystem vasculitis characterized by mucocutaneous, articular, neurological, gastrointestinal and ophthalmological lesions. Ocular involvement is mainly represented by recurrent uveitis, especially posterior uveitis; however, iridocyclitis, retinal and choroidal vasculitis, optic neuritis and retinal vascular occlusion can also occur. Case presentation A 12-year-old Caucasian boy with a history of recurrent buccal aphthosis and nonspecific gastrointestinal symptoms was admitted to our hospital with blurred vision associated with acute episcleritis and papillitis. The patient's pathergy test was positive, suggesting a diagnosis of Behçet's disease. Corticosteroid and cyclosporine therapy was started, but further episodes were noted in both eyes. The patient was then switched to intravenous infliximab, with complete resolution of the inflammation after the second infusion. Conclusion Episcleritis and papillitis should be added to the list of uncommon manifestations of pediatric Behçet's disease. Infliximab is an effective, new therapeutic approach for Behçet's disease that is refractory to the conventional corticosteroid and immunosuppressive therapy. PMID:21352523

  15. Omalizumab treatment in patient with severe asthma and Eosinophilic Granulomatosis with Polyangiitis . A case report.

    PubMed

    Graziani, A; Quercia, O; Girelli, F; Martelli, A; Mirici Cappa, F; Stefanini, G F

    2014-11-01

    Eosinophilic Granulomatosis with Polyangiitis (EGPA), formerly named Churg Strauss Syndrome, is a multisystem disorder characterized by chronic rhinosinusitis, asthma, and prominent peripheral blood eosinophilia; it is classified as a vasculitis of the small and medium sized arteries, although the vasculitis is often not clinically apparent in the initial phases of the disease. We present the case of a woman with EGPA who was frequently treated with high dose steroid therapy during hospital admission for refractory asthma. After December 2008, the date when we started Omalizumab, we observed a significative reduction of circulating eosinophils and IgE serum level, and the patient was no more hospitalized for respiratory failure or asthma attacks. PMID:25398166

  16. 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

  17. 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

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

    SciTech Connect

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

    1988-01-01

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

  19. Granulomatosis with polyangiitis presenting as Henoch-Schönlein purpura in children.

    PubMed

    Bui, Tuan; Chandrakasan, Shanmuganathan; Poulik, Janet; Fathalla, Basil M

    2013-06-01

    Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, in children is an uncommon chronic organ- and life-threatening systemic vasculitis that may share at time of initial presentation a number of clinical features in common with Henoch-Schönlein purpura (HSP), a very common and comparatively benign form of childhood vasculitis. Diagnosis of GPA requires a high index of suspicion, and antineutrophil cytoplasmic antibody tests along with tissue biopsy are helpful tools for diagnosis. We report 2 patients with GPA masqueraded as HSP at time of initial presentation. Both patients presented with nonthrombocytopenic purpura on lower extremities, in addition to abdominal pain, and/or microscopic hematuria and fulfilled both the American College of Rheumatology and the Pediatric Rheumatology European Society classification criteria for HSP. Both patients eventually developed significant renal and pulmonary disease and were diagnosed with GPA. We aim to raise awareness of such atypical presentations of GPA to avoid delayed management. PMID:23669806

  20. 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

  1. Cardiac manifestations in Behcet's disease.

    PubMed

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

    2015-05-01

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

  2. Managing Behçet's disease: An update on current and emerging treatment options.

    PubMed

    van Daele, P La; Kappen, J H; van Hagen, P M; van Laar, J Am

    2009-04-01

    Behçet's disease is an autoinflammatory vasculitis of unknown origin characterized by recurrent oral and genital ulcers, uveitis, arthritis and skin lesions. Additionally, involvement of the gastrointestinal tract, central nervous system and large vessels may occur. The disease is prevalent in countries along the ancient Silk Road from Eastern Asia to the Mediterranean Basin. Many treatment modalities are currently available. The choice of treatment depends on organ involvement and severity of disease. Topical treatment with corticosteroids is often sufficient for mucocutaneous involvement, however for more severe disease with vasculitis or neurological involvement a more aggressive approach is warranted. Newer drugs (biologicals) influencing cytokines and thereby T-cell function are promising with an acceptable side effect profile. Unfortunately, reimbursement of the costs of biologicals for rare disease is still a problem in various countries. In this report we discuss the current treatment modalities for Behçet's disease. PMID:19536320

  3. Managing Behçet’s disease: An update on current and emerging treatment options

    PubMed Central

    van Daele, P LA; Kappen, J H; van Hagen, P M; van Laar, J AM

    2009-01-01

    Behçet’s disease is an autoinflammatory vasculitis of unknown origin characterized by recurrent oral and genital ulcers, uveitis, arthritis and skin lesions. Additionally, involvement of the gastrointestinal tract, central nervous system and large vessels may occur. The disease is prevalent in countries along the ancient Silk Road from Eastern Asia to the Mediterranean Basin. Many treatment modalities are currently available. The choice of treatment depends on organ involvement and severity of disease. Topical treatment with corticosteroids is often sufficient for mucocutaneous involvement, however for more severe disease with vasculitis or neurological involvement a more aggressive approach is warranted. Newer drugs (biologicals) influencing cytokines and thereby T-cell function are promising with an acceptable side effect profile. Unfortunately, reimbursement of the costs of biologicals for rare disease is still a problem in various countries. In this report we discuss the current treatment modalities for Behçet’s disease. PMID:19536320

  4. [Dermatologic manifestations of the antiphospholipid syndrome].

    PubMed

    Francès, C; Barete, S; Soria, A

    2012-04-01

    A wide variety of dermatologic manifestations has been described in the antiphospholipid syndrome (APS). The most frequent skin lesion is livedo reticularis, present not only on the limbs but also on the trunk, with a fine irregular pattern. It belongs to the arterial subset of APS. Circumscribed ulcerations, resembling livedoid vasculitis, may be the first manifestation of APS. Ulcerations may also occur as a late complication of recurrent venous thrombosis. Extensive skin necrosis is a classic manifestation of catastrophic APS. Pseudo-vasculitis lesions are misdiagnosed if a skin biopsy is not performed, especially in the context of systemic lupus erythematosus. In systemic lupus erythematosus, primary anetoderma is always associated with antiphospholipid antibodies. PMID:22078802

  5. Severe hemorrhagic bullous lesions in Henoch Schonlein purpura: three pediatric cases and review of the literature.

    PubMed

    Trapani, Sandra; Mariotti, Paola; Resti, Massimo; Nappini, Lido; de Martino, Maurizio; Falcini, Fernanda

    2010-08-01

    Henoch Schonlein purpura (HSP) is a small-sized blood vessels vasculitis, resulting from immunoglobulin A-mediated inflammation. It is the most common acute systemic vasculitis in childhood and mainly affects skin, gastrointestinal tract, joints and kidney. Skin lesions, usually presenting as erythematous maculopapules, petechiae, and purpura, often involve lower extremities and buttocks, but may also extend to the upper extremities, face and trunk. Conversely to adults, hemorrhagic bullous evolution has been seldom described in childhood. The pressure is likely a factor into the pathogenesis of bullae. We report on three new pediatric cases of HSP with hemorrhagic bullous skin lesions, and a review of the literature. Bullous evolution represents an unusual, but well-recognized cutaneous manifestation that may be a source of diagnostic dilemma, but does not seem to have any prognostic value in the outcome of HSP. PMID:19609528

  6. ANCA-Negative Wegener's Granulomatosis with Multiple Lower Cranial Nerve Palsies

    PubMed Central

    Kim, Sung-Hee; Park, Jin; Bae, Jung Ho; Cho, Min-Sun; Park, Kee Duk

    2013-01-01

    Wegener's granulomatosis (WG) is a systemic vasculitis affecting small and medium-sized vessels with granulomatous formation. Though it is known for respiratory tract and kidney involvement, neurologic manifestation has been also reported. Herein we report a patient who suffered pansinusitis with multiple lower cranial nerve palsies but reached remission by immunosuppressant after the diagnosis of WG. A 54-yr-old female visited with headache, hearing difficulty, and progressive bulbar symptoms. She experienced endoscopic sinus surgeries due to refractory sinusitis. Neurologic examination revealed multiple lower cranial nerve palsies. Vasculitic markers showed no abnormality. Nasal biopsy revealed granulomatous inflammation and vasculitis involving small vessels. Given cyclophosphamide and prednisolone, her symptoms were prominently improved. WG should be considered in the patient with multiple cranial nerve palsies, especially those with paranasal sinus disease. Because WG can be lethal if delayed in treatment, prompt immunosuppressant is warranted after the diagnostic tissue biopsy. PMID:24265538

  7. Diagnostic value and limitations of orbital biopsy in Wegener's granulomatosis.

    PubMed

    Kalina, P H; Lie, J T; Campbell, R J; Garrity, J A

    1992-01-01

    Patients with Wegener's granulomatosis may present with ophthalmic involvement in either the classic or limited forms. Although the overall clinical picture and serologic testing for antineutrophil cytoplasmic antibody (ANCA) are important, biopsy is often necessary for a definitive diagnosis. Accurate interpretation of these orbital biopsies is essential. The authors reviewed the histopathologic features of 13 orbital biopsies in patients with well-documented Wegener's granulomatosis. The classic triad of vasculitis, tissue necrosis, and granulomatous inflammation was seen in 7 of 13 biopsies (54%). Vasculitis in combination with other microscopic findings was seen in 4 of 13 (31%) biopsies. Giant cells were seldom seen. Two biopsies showed only perivascular infiltrates. Based on this study, it appears that a spectrum of histopathologic features can be seen in orbital biopsies in Wegener's granulomatosis. This study underscores the importance of clinical correlation, the application of the ANCA test, and comparison with extraorbital biopsies, if available, when interpreting orbital biopsies in the diagnosis of Wegener's granulomatosis. PMID:1741123

  8. Advances in the pathology of glomerular diseases.

    PubMed

    Weening, Jan J; Ronco, Pierre; Remuzzi, Giuseppe

    2013-01-01

    Glomerular injury can be caused by numerous insults including hemodynamics, infections and immunity, hereditary and metabolic diseases, and toxicity. Basic and translational experimental studies in combination with clinical research in patients with renal disease have advanced our understanding of the etiology and pathogenesis of many forms of glomerulonephritis. This new knowledge has facilitated classification and treatment and has contributed to a better outcome of patients with renal disease. Since renal disease almost without exception leads to systemic cardiovascular complications, these advances are also of general health interest. Here, we shall briefly review general principles in the pathology of glomerular injury and discuss recent developments in the study of podocytopathies; membranous glomerulopathy; ANCA-associated vasculitis; C3 glomerulopathies and the role of complement in endothelial injury; and the prognostic value of the renal biopsy in predicting long-term outcome in lupus nephritis, vasculitis and IgA nephropathy. PMID:23689563

  9. [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

  10. Pulsed-laser therapy (GA-As) in combined treatment of post-traumatic swellings and some dermatological disorders

    NASA Astrophysics Data System (ADS)

    Antipa, Ciprian; Dona, Dumitru; Podoleanu, Adrian Gh.

    1994-02-01

    The effect of a pulsed gallium arsenide infrared laser radiation was studied on 64 patients with post traumatic swellings, allergic vasculitis and varicose crural ulcers, therapy resistant. The soft laser therapy was conducted in combination with classical therapy and was compared with a non irradiated control group of 52 patients treated only by classical therapy. Laser irradiation was directed to the skin damage by laser scanning. Segmental and dermatomic areas of the skin lesions were irradiated by laserpuncture. Therapeutic protocol included an average of nine sessions. The statistical analysis shows a significant difference of the efficiency between Ga-As pulsed laser treated group and the control group, especially in the case of post-traumatic swellings and less in the case of allergic vasculitis. The effects reported by this study are relevant for clinical application of infrared pulsed low lasers in dermatology.

  11. Crescentic glomerulonephritis: new aspects of pathogenesis.

    PubMed

    Tarzi, Ruth M; Cook, H Terence; Pusey, Charles D

    2011-07-01

    This review provides a summary of recent advances in the understanding of crescentic glomerulonephritis, focusing on antineutrophil cytoplasm antibody (ANCA)-associated vasculitis and anti-glomerular basement membrane (anti-GBM) antibody disease. In ANCA-associated vasculitis (AAV), four main conceptual advances are discussed as follows: (1) evidence for the pathogenicity of ANCA, (2) molecular mimicry and the role of infection in AAV, (3) evidence for aberrant T-cell responses and T-cell regulation in AAV, and (4) advances in understanding of genetic predisposition to AAV. In relation to anti-GBM disease we discuss the following: (1) the nature of the Goodpasture autoantigens, (2) T-cell responses and regulation in anti-GBM disease, and (3) human leukocyte antigen and non-human leukocyte antigen genetic associations. PMID:21839369

  12. 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

  13. Neuro-behçet’s syndrome: Differential diagnosis and management

    Microsoft Academic Search

    Erdal Diri; Louis R. Espinoza

    2006-01-01

    Behçet’s syndrome (BS) is a multisystem disease of unknown etiology, characterized by chronic relapsing cardinal symptoms\\u000a of orogenital ulcers, uveitis, and different skin lesions. Its major pathologic feature is vasculitis. Neuro-Behçet’s syndrome\\u000a (NBS) is defined as a constellation of neurologic manifestations with characteristic neuropathologic findings, usually confirmed\\u000a by ancillary investigations, in patients who meet the diagnostic criteria for BS. Neurologic

  14. Painful ophthalmoplegia: a retrospective study of 23 cases

    Microsoft Academic Search

    M. Curone; V. Tullo; A. Proietti-Cecchini; C. Peccarisi; M. Leone; G. Bussone

    2009-01-01

    Painful ophthalmoplegia is a rare pathologic condition caused by non-specific inflammation of the cavernous sinus, but other\\u000a causes such as tumours, vasculitis, basal meningitis, neurosarcoidosis, diabetes can be responsible for the syndrome. Aim\\u000a of this study is a review of the cases of painful ophthalmoplegia admitted to our Institute in the last 20 years in order\\u000a to verify the incidence of

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

    PubMed

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

    2014-01-01

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

  16. [Fever, nosebleeding and myalgic abdominal pain].

    PubMed

    Leopold, M; Siepmann, T

    2008-02-20

    A 38 year old patient presented with fever, myalgic abdominal pain, nose bleeding and acute renal failure since five days. A combination of thrombocytopenia, proteinuria, elevated CrP and creatinin is common in hemorrhagic fever with renal syndrome (HFRS) due to Hantavirus infection. The benigne form is called Nephropathia epidemica. Dialysis is infrequently required by patients with the Puumala virus. Other infection (e.g. malaria, leptospirosis, yellow fever) and systemic diseases (e.g. collagenosis or vasculitis) are considered. PMID:18548800

  17. [Wegener's granulomatosis of the nasal fossa].

    PubMed

    Ibáñez-Bermúdez, F; Castillo Ceballos, A; Gallardo Avila, A; Pérez Benítez, T; Criado Montilla, J; Martínez García, R C

    1995-01-01

    A 62-year-old female patient consulted for a suppurative-hemorrhagic nasal condition 18 months evolution. Exploration found granulomatous rhinitis which was producing septal perforation and histology compatible with necrotizing granulomatous vasculitis, fulfilling the diagnostic criteria for focal Wegener's granulomatosis, in view of the absence of lung and renal damage. Treatment with trimethoprim-sulfamethoxazole alone described in the medical literature as an option for this process, yielded excellent results with remission of symptoms and normalization of the exploration. PMID:8554806

  18. Acute hemorrhagic edema of infancy: case report.

    PubMed

    Babi?, Sanja; Murat-Susi?, Slobodna; Husar, Karmela; Skerlev, Mihael; Rados, Jaka

    2008-01-01

    Acute hemorrhagic edema of infancy (AHEI) is a benign form of leukocytoclastic vasculitis that typically affects children between 4 and 24 months of age. The etiology remains unknown. The potential triggers of AHEI include preceding bacterial or viral infections, immunizations and drugs. The onset of AHEI is often dramatic with petechiae, ecchymoses, and annular, nummular or targetoid purpuric lesions usually appearing on the extremities, face, or ears. We report on a case of AHEI that occurred after upper respiratory tract infection. PMID:18541105

  19. Clinical and surgical therapeutic approach in Erithema Elevatum Diutinum - case report*

    PubMed Central

    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

  20. 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. PMID:25883825

  1. Coronary insufficiency in children: Review of literature and report of a rare case with unknown aetiology

    Microsoft Academic Search

    Ahmed A. Arifi; Munir Ahmad; Ahmed Al Assal; Imad Naja; Hani K. Najm

    2010-01-01

    Myocardial ischemia due to coronary artery disease is a rare condition in children. The reported causes of this condition include vasculitis; commonest being Kawasaki’ disease, pre-mature atherosclerosis due to familial dyslipidemias, congenital coronary artery anomalies and post-operative complications of procedures requiring coronary artery re-implantation in children, e.g. arterial switch procedure and Ross procedure. Allograft arteriopathy after heart transplantation is a

  2. Role of neutrophils in systemic autoimmune diseases

    PubMed Central

    2013-01-01

    Neutrophils have emerged as important regulators of innate and adaptive immune responses. Recent evidence indicates that neutrophils display marked abnormalities in phenotype and function in various systemic autoimmune diseases, and may play a central role in initiation and perpetuation of aberrant immune responses and organ damage in these conditions. This review discusses the putative roles that neutrophils and aberrant neutrophil cell death play in the pathogenesis of various systemic autoimmune diseases, including systemic lupus erythematosus, small vessel vasculitis and rheumatoid arthritis. PMID:24286137

  3. 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. PMID:25861545

  4. Characterization of murine monoclonal anti-endothelial cell antibodies (AECA) produced by idiotypic manipulation with human AECA

    Microsoft Academic Search

    Y. Levy; B. Gilburd; J. George; N. Del Papa; R. Mallone; M. Damianovich; M. Blank; A. Radice; Y. Renaudineau; P. Youinou; A. Wiik; F. Malavasi; P. L. Meroni; Y. Shoenfeld

    1998-01-01

    The IgG fraction of human anti-endothelial cell antibodies (AECA) obtained from a patient with Wegener's granulomatosis was used as immunogen to raise AECA mAb in mice selected among those which developed vasculitis-like lesions after immunization. Three mAb (BGM, 3C8 and 7G2), selected by cyto-ELISA and flow cytometry analyses, featured a specific reactivity with human umbilical vein endothelial cells (HUVEC) and

  5. An interesting perioperative rendezvous with a case of Henoch-Schonlein purpura

    PubMed Central

    Kurdi, Madhuri S.; Deva, Radhika S.; Theerth, Kaushic A.

    2014-01-01

    Henoch-Schonlein purpura (HSP) is an autoimmune, multisystem, acute vasculitis of childhood commonly involving the skin, gut, joints and the kidneys. Fatal complications involving various systems can occur in this disease and careful perioperative management is advocated. We report here the occurrence of postoperative bradycardia and the successful perioperative management of a 12-year-old boy with HSP for diagnostic laparoscopy. PMID:25886345

  6. Use of infliximab and other biologics in Behçet disease.

    PubMed

    Li, J; Fernando, S; Herkes, G

    2014-01-01

    Behçet disease is a multisystem vasculitis characterised by recurrent oral ulceration in conjunction with other manifestations. Neurological involvement or neuro-Behçet disease is not common, but typically affects young men at its onset between the ages of 20 and 40 with significant long-term morbidity and mortality. There is substantial case literature to support the use of tumour necrosis factor antagonists, notably infliximab, in the treatment of neuro-Behçet disease. PMID:24450526

  7. Symptomatic Myocarditis in Kawasaki Disease

    Microsoft Academic Search

    Pooja Aggarwal; Deepti Suri; Nidhi Narula; Rohit Manojkumar; Surjit Singh

    Kawasaki disease (KD) is an acute febrile illness of infants and young children that is characterized by a medium vessel vasculitis,\\u000a most commonly involving the coronary arteries. Though subclinical myocarditis is rather common in KD, symptomatic congestive\\u000a heart failure is extremely uncommon. The authors report a 9-y-old boy who developed heart failure (ejection fraction 28%)\\u000a in the acute phase of

  8. Severe renal impairment in the case of classic polyarteritis nodosa

    Microsoft Academic Search

    S. A. Bakkalo?lu; Mesiha Ekim; Necmiye Tümer; Özden Tulunay; Tülay Özer

    2001-01-01

    A 14-year-old boy with classic polyarteritis nodosa (cPAN) and a clinical picture resembling rapidly progressive glomerulonephritis\\u000a (RPGN) is described. He had severe hypertension, malaise, weight loss, fever, myalgia, and rapid deterioration of renal function.\\u000a Renal biopsy revealed acute necrotizing vasculitis. Angiography showed small saccular aneurysmatic dilatations in the intrarenal\\u000a branches of the right renal artery and the intrahepatic branches of

  9. Brainstem infarcts as an early manifestation of streptococcus anginosus meningitis

    Microsoft Academic Search

    Sung B. Lee; Lyell K. Jones; Caterina Giannini

    2005-01-01

    Background and Purpose: Vasculitis and infarcts are well-established sequelae of bacterial meningitis. However, early large-vessel involvement is\\u000a rare, particularly within the brainstem. There has been one previous case report of a young male who presented with pontine\\u000a infarct as an early manifestation of Streptococcus milleri meningitis. We present another case of brainstem infarction associated with meningitis caused by Streptococcus anginosus

  10. The role and use of recombinant receptors in the investigation and control of antibody-induced inflammation

    Microsoft Academic Search

    M. S. Powell; P. M. Hogarth

    \\u000a The interaction of immune complexes with cell surface Fc receptors is a potent stimulus for the activation of inflammatory\\u000a cells. In autoimmune diseases this activation process can generate widespread tissue destruction, such as vasculitis associated\\u000a with rheumatoid arthritis or glomerulonephritis induced by auto-antibody deposition in systemic lupus erythematosus. Whilst\\u000a the cause of autoimmunity is unknown, it is clear that immune

  11. Differences in CCR5 expression on peripheral blood CD4 +CD28 ? T-cells and in granulomatous lesions between localized and generalized Wegener’s granulomatosis

    Microsoft Academic Search

    Peter Lamprecht; Hilke Bruhl; Anika Erdmann; Konstanze Holl-Ulrich; Elena Csernok; Ulrike Seitzer; Matthias Mack; Alfred C Feller; Eva Reinhold-Keller; Wolfgang L Gross; Antje Muller

    2003-01-01

    Wegener’s granulomatosis (WG) is an autoimmune disease characterized by granulomatous lesions and a necrotizing vasculitis. Th1-type-cells lacking CD28 are expanded independent of age and immunosuppressive therapy in WG. To address their migratory properties of CD4+CD28? T-cells we studied the expression of the inducible inflammatory Th1-type chemokine receptor CCR5 in localized WG and generalized WG. Expansion of CD4+CD28? T-cells was more

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

    PubMed

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

    2012-01-01

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

  13. 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

  14. [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

  15. A case of idiopathic tracheal stenosis

    PubMed Central

    Apostolova, M.; Zeidan, B.

    2013-01-01

    Acquired tracheal stenosis is a condition that often results from trauma, neoplasm, infection, vasculitis, inflammatory or infiltrative processes. Idiopathic tracheal stenosis is a rare entity and represents a diagnosis of exclusion. We report a case of severe localized extrathoracic tracheal stenosis in a 35 year old female who was initially suspected to have asthma as the cause of several months of chronic dyspnea. PMID:26029504

  16. Treatment of relapsing polychondritis with the TNF-alpha antagonist adalimumab

    Microsoft Academic Search

    Tobias Lahmer; Andreas Knopf; Matthias Treiber; Uwe Heemann; Klaus Thuermel

    2010-01-01

    Relapsing polychondritis (RP) is a rare immune-mediated disease which is associated with inflammation in cartilaginous tissue\\u000a throughout the body. Especially, the cartilaginous structures of ear, respiratory tract, nose, and joints are affected. Around\\u000a 30% of the cases are associated with other diseases especially systemic vasculitis. Onset of RP is most likely between the\\u000a ages of 40–60 years. This case reports the

  17. [Neurologic manifestations in rheumatic diseases].

    PubMed

    Kawakami, Atsushi

    2013-01-01

    Among varying organ involvements, neurologic as well as musculoskeletal involvement are important toward the quality of life and prognosis in the patients of rheumatic diseases. In general, these occur as the increment of disease activity and influence the choice of therapeutic regime. In this review, neurologic and musculoskeletal involvements, especially the former, found in systemic lupus erythematosus, dermatomyositis/polymyositis, Sjögren's syndrome, Behçet's disease and vasculitis syndrome are discussed. PMID:23892960

  18. Low levels of interleukin-1 receptor antagonist coincide with kidney involvement in systemic lupus erythematosus

    Microsoft Academic Search

    G. STURFELT; P. ROUX-LOMBARD; F. A. WOLLHEIM; J.-M. DAYER

    1997-01-01

    SUMMARY The objective was to study the relationship between the levels of interleukin-1 receptor antagonist (IL-1Ra) and disease activ- ity and the acute-phase response in SLE patients with and without renal involvement. Twenty SLE patients who had distinct active clinical manifestations (eight glomerulonephritis, four systemic vasculitis without kidney involvement, nine skin rash, 12 arthritis, five serositis, four neuropsychiatric manifestations, three

  19. [Mycophenolate mofetil in the treatment of selected connective tissue diseases].

    PubMed

    Batko, Bogdan; Krawiec, Piotr; Osieleniec, Jolanta; Su?owicz, W?adys?aw

    2013-01-01

    Mycophenolate mofetil (MMF) is an inhibitor of inosine monophosphate dehydrogenase, which affects de novo purine synthesis and T- and B-cell proliferation. So far its efficacy and safety as an immunosuppressive treatment have been proven in organ transplantations and also in various autoimmune diseases. A literature search was conducted by using PubMed and the Cochrane library. This review focuses primarily on current treatment with MMF for systemic lupus erythematosus, systemic sclerosis, vasculitis and idiopathic inflammatory myopathies. PMID:24455833

  20. [Henoch-Schönlein purpura resulting in an obstructive bladder mass].

    PubMed

    Comploj, E; Cassar, W; Trenti, E; Palermo, S; Reinstadler, P; Ladurner, C; Dechet, C; Pycha, A

    2013-11-01

    Henoch-Schönlein purpura (HSP) is the most common form of an immunological systemic vasculitis of childhood. The classic clinical symptoms include purpuric rash, abdominal pain, arthralgias, and haematuria, but the spectrum of HSP may vary to very rare forms. This article reports on an 8-year-old girl with a Henoch-Schönlein purpura (HSP) which resulted in an obstructive bladder mass and subsequent urinary retention. This is the first case reported in the literature, describing such a course. PMID:23824929

  1. Henoch-schönlein purpura associated with gangrenous appendicitis: a case report.

    PubMed

    Semeena, Nk; Adlekha, Shashikant

    2014-03-01

    Henoch-Schönlein Purpura (HSP) is a leucocytoclastic vasculitis of unclear aetiology characterised by symmetrical, non-traumatic, nonthrombocytopenic purpura mostly involving the lower limbs and buttocks, as well as arthritis, gastrointestinal manifestations, and occasional nephritis. A 35 years old male presented with purpuric rash on the lower extremities, abdominal pain, fever, arthralgia, and melaena. A diagnosis of HSP with appendicitis was made, which is an exceedingly rare phenomenon. PMID:24876811

  2. Paroxysmal drastic abdominal pain with tardive cutaneous lesions presenting in Henoch-Schönlein purpura.

    PubMed

    Chen, Xiao-Liang; Tian, Hong; Li, Jian-Zhong; Tao, Jin; Tang, Hua; Li, Yang; Wu, Bin

    2012-04-28

    Henoch-Schönlein purpura (HSP) is a small-vessel vasculitis mediated by IgA-immune complex deposition. It is characterized by the clinical tetrad of non-thrombocytopenic palpable purpura, abdominal pain, arthritis and renal involvement. The diagnosis of HSP is difficult, especially when abdominal symptoms precede cutaneous lesions. We report a rare case of paroxysmal drastic abdominal pain with gastrointestinal bleeding presented in HSP. The diagnosis was verified by renal damage and the occurrence of purpura. PMID:22563183

  3. Parvovirus B19: The Causative Agent of Dilated Cardiomyopathy or a Harmless Passenger of the Human Myocard?

    Microsoft Academic Search

    S. Modrow

    Parvovirus B19 infections may cause a widespread benign and self-limiting disease in children and adults known as erythema\\u000a infectiosum (fifth disease). Several further manifestations are associated with B19 infections, such as arthralgias, arthritis,\\u000a leucopenia and thrombocytopenia, anaemia and vasculitis and spontaneous abortion and hydrops fetalis in pregnant women. Persistent\\u000a infections with continuous virus production may occur in immunocompetent as well

  4. Rupture of the abdominal aorta in a 13-year-old girl secondary to Behçet disease: a case report

    Microsoft Academic Search

    Veysel Kutay; Cevat Yakut; Hasan Ekim

    2004-01-01

    Behçet disease is a multisystemic vasculitis of unknown origin. The vascular complications as a result of the disease are rare in the pediatric age group. We report a 13-year-old patient with vasculo–Behçet disease with a ruptured abdominal aortic aneurysm without a formerly known history of Behçet disease. Urgent aortoiliac bypass with a polytetrafluoroethylene graft was performed with success, and the

  5. Pathological haemostasis and ‘prothrombotic state’ in Behçet's disease

    Microsoft Academic Search

    Sedat Kiraz; ?hsan Ertenli; M. Akif Öztürk; ?brahim C. Haznedaro?lu; ?smail Çelik; Meral Çalgüneri

    2002-01-01

    Behçet's disease (BD) is a widespread occlusive-type vasculitis with life-threatening manifestations. The vasculopathy of BD is unique and any type of vessel can be involved. Moreover, vascular lesions in BD represent an occlusive nature suggesting a hypercoagulable\\/prothrombotic state. The data concerning the genetic defects of the coagulation cascade are expanding. There is evidence of universal activation of haemostatic system in

  6. Infectious Serologies and Autoantibodies in Hepatitis C and Autoimmune Disease-Associated Mixed Cryoglobulinemia

    Microsoft Academic Search

    Merav Lidar; Noga Lipschitz; Nancy Agmon-Levin; Pnina Langevitz; Ori Barzilai; Maya Ram; Bat-Sheba Porat-Katz; Nicola Bizzaro; Jan Damoiseaux; Jan Willem Cohen Tervaert; Salvatore deVita; Stefano Bombardieri; Yehuda Shoenfeld

    Mixed cryoglobulinemia (MC) syndrome is an immune complex-mediated vasculitis characterized by the clinical triad of purpura,\\u000a weakness, and arthralgias, the morbidity of which is mainly related to kidney and peripheral nervous system dysfunction as\\u000a well as to the development of a secondary lymphoma (Ferri et al. Autoimmun Rev 7:114–120, 2007, Lidar et al. Ann N Y Acad Sci 1173:649–657, 2009,

  7. 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

  8. Atherosclerosis associated with vasculopathic lesions in a golden retriever with hypercholesterolemia

    PubMed Central

    Boynosky, Nicole A.; Stokking, Laura

    2014-01-01

    A 2-year-old neutered male golden retriever dog presented for lameness secondary to ulcerations of multiple digital paw pads was diagnosed with vasculitis and hypercholesterolemia. Despite treatment, ischemic necrosis progressed to include all distal extremities and the dog eventually expired due to myocardial infarction secondary to severe atherosclerosis. The rapid demise and the dermatologic lesions may have been secondary to cholesterol embolism syndrome which has never before been reported in a dog. PMID:24790237

  9. Vasculopathie livédoïde et thrombophilie combinée : efficacité de l’iloprost

    Microsoft Academic Search

    N Magy; M. P Algros; E Racadot; H Gil; B Kantelip; J. L Dupond

    2002-01-01

    Introduction. – Livedoid vasculopathy is characterized by early, focal painful purpuric lesions of the lower skin extremities without histologic finding of small vessel vasculitis.Exegesis. – A 38-year-old man was seen in our unit for painful purpuric lesions of both feet localized on toes and external sides. Skin biopsy showed dermic vessel thrombosis and endothelial cell proliferation. Lupus anticogulant antibody was positive in

  10. Kawasaki disease: a review with emphasis on cardiovascular complications

    Microsoft Academic Search

    Ricardo Duarte; Silvia Cisneros; Gabriel Fernandez; Daniel Castellon; Cesar Cattani; Cíntia A Melo; Asier Apocada

    2010-01-01

    Kawasaki disease (KD) is an acute systemic vasculitis that is currently the leading cause of acquired heart disease in childhood\\u000a in the United States. Cardiovascular complications are the major cause of morbidity, are responsible for virtually all deaths\\u000a from KD and should be evaluated as soon as possible after the acute phase to establish the baseline status, in order to

  11. Sustained activation of neutrophils in the course of Kawasaki disease: an association with matrix metalloproteinases

    Microsoft Academic Search

    M. H. Biezeveld; Mierlo van G; R. Lutter; I. M. Kuipers; T. Dekker; C. E. Hack; J. W. Newburger; T. W. Kuijpers

    2005-01-01

    Kawasaki disease (KD) is an acute febrile syndrome of childhood, characterized by vasculitis of the medium-sized arteries. White blood cell counts and the inflammatory parameter C-reactive protein (CRP) are known to be elevated in the acute phase of the disease. In this study we investigated the course of inflammatory cell type-specific parameters in KD over a longer period of time.

  12. Lack of Association between ORAI1\\/CRACM1 Gene Polymorphisms and Kawasaki Disease in the Taiwanese Children

    Microsoft Academic Search

    Ho-Chang Kuo; Ying-Jui Lin; Suh-Hang Hank Juo; Yu-Wen Hsu; Wei-Chiao Chen; Kuender D. Yang; Chi-Di Liang; Shengyu Yang; Mei-Chyn Chao; Hong-Ren Yu; Shouyan Wang; Li-Yan Lin; Wei-Chiao Chang

    Objective  Kawasaki disease (KD) is characterized by systemic vasculitis of an unknown cause. A previous study has indicated that a polymorphism\\u000a of the inositol 1,4,5-trisphosphate 3-kinase C (ITPKC) gene is involved in the susceptibility to KD. ORAI (also known as CRACM1) is one of the components of store-operated calcium\\u000a channels involved in regulating immune and inflammatory reactions. This study was conducted

  13. 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

  14. THE COMPLEMENT SYSTEM IN NECROTIZING ANGIITIS OF THE SKIN. ANALYSIS OF COMPLEMENT COMPONENT ACTIVITIES IN SERUM OF PATIENTS WITH CONCOMITANT COLLAGEN-VASCULAR DISEASES

    Microsoft Academic Search

    Nicholas A. Soter; K. Frank Austen; Irma Gigli

    1974-01-01

    Profiles of serum complement components were studied in patients with necrotizing angiitis (vasculitis) of the skin and concomitant rheumatoid arthritis, Sjögren's syndrome, or systemic lupus erythematosus. In patients with either rheumatoid arthritis or Sjögren's syndrome, the early components—C1, C4, and C2—were depressed. When cryoglobulins containing mainly IgG and IgM were present in the patients with Sjögren's syndrome, there was further

  15. 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.

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

    PubMed Central

    2015-01-01

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

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

    PubMed Central

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

    2015-01-01

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

  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. PMID:25893028

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

    PubMed

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

    2013-07-01

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

  20. 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

  1. 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

  2. Warfarin-induced skin necrosis diagnosed on clinical grounds and treated with maggot debridement therapy.

    PubMed

    Biscoe, Anna Louise; Bedlow, Alison

    2013-01-01

    A patient with a history of deep vein thrombosis presented with painful bruising and blistering on his left leg 7-10 days after warfarin treatment. A complicated 2-month treatment followed, where vasculitis was originally diagnosed from histological findings before the final diagnosis of warfarin-induced skin necrosis (WISN) was made on clinical grounds. Warfarin was stopped, reversed and low molecular weight heparin started but, the lesions had progressed to full thickness necrosis. This was originally treated with conventional surgical debridement before introducing maggot debridement therapy (MDT) in an effort to try to salvage the limb. PMID:23362073

  3. An Unusual Association between Sweet's Syndrome and Metastatic Papillary Follicular Thyroid Carcinoma

    PubMed Central

    Benson, Ariel; Patel, Payal; Friedman, Adam; Broadway, Kameelah

    2013-01-01

    Sweet's syndrome (SS), or acute febrile neutrophilic dermatosis, is marked by fever, leukocytosis, and painful erythematous papules/plaques resulting from neutrophil migration and accumulation in the dermis. This condition has been associated with underlying hematologic as well as solid malignancies. We describe a unique case of SS in a patient with metastatic papillary follicular thyroid carcinoma and group A streptococcal pharyngitis. The distribution of the patient's SS rash was similar to the rash of neutrophilic dermatosis (pustular vasculitis) of the dorsal hands. PMID:23467309

  4. Linking classification and therapeutic management of vasculitides.

    PubMed

    Baldwin, Corisande; Carette, Simon; Pagnoux, Christian

    2015-01-01

    Vasculitides are classified by the size, type and location of the predominantly involved vessels and by their primary or secondary nature. Their treatment depends on the type of vasculitis, its etiology (when known), and its severity and must be further adjusted by the individual characteristics and comorbidities of patients. In this paper, we review how the classification and definition of vasculitides have evolved over the past years and how it has affected therapeutic changes. As new genetic markers are being discovered and the pathogenesis of vasculitides continues to be elucidated, further modifications in classification and treatment can be expected. PMID:26031766

  5. [Diagnosis of isolated angiitis of the central nervous system].

    PubMed

    Totolian, N A; Totovchikov, A A; Kodzaeva, A Iu; Krasnov, V S; Lapin, S V; Skoromets, A A

    2013-01-01

    We have analyzed clinical presentations, neuroimaging data, blood and cerebrospinal fluid diagnostic tests in isolated angiitis of the central nervous system on the basis of data of recent publications and 22 our own cases. Due to the insufficient diagnostic value of invasive methods including biopsy of leptomeningeal and intracerebral vessels, we considered practical approaches to the diagnosis of cerebral vasculitis and ways of its optimization. We suggested the stratification of clinical, neuroimaging and laboratory criteria for the evaluation of their sensitivity and specificity and for the development of diagnostic algorithms. PMID:23528592

  6. Warfarin-induced skin necrosis diagnosed on clinical grounds and treated with maggot debridement therapy

    PubMed Central

    Biscoe, Anna Louise; Bedlow, Alison

    2013-01-01

    A patient with a history of deep vein thrombosis presented with painful bruising and blistering on his left leg 7–10?days after warfarin treatment. A complicated 2-month treatment followed, where vasculitis was originally diagnosed from histological findings before the final diagnosis of warfarin-induced skin necrosis (WISN) was made on clinical grounds. Warfarin was stopped, reversed and low molecular weight heparin started but, the lesions had progressed to full thickness necrosis. This was originally treated with conventional surgical debridement before introducing maggot debridement therapy (MDT) in an effort to try to salvage the limb. PMID:23362073

  7. 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. PMID:25825636

  8. 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

  9. [Baby hamster kidney cells as antigen for demonstration of antinuclear antibodies (author's transl)].

    PubMed

    Pernice, W; Scherer, R; Lüben, G; Sodomann, C P; Sedlacek, H H

    1978-09-15

    Baby hamster kidney cells fixed in acetone on glass slides were used as antigen for demonstration of antinuclear antibodies. Where certain storage conditions were observed (drying agent, 4 degrees C) they have kept for 12 months up to now. As regards specificity, sensitivity, reproducibility, and differentiation of fluorescent types the baby hamster kidney cell test appears superior to other immunofluorescence methods used (chicken erythrocytes, rat liver sections, and crithidiae). These results were obtained in 73 sera from patients with disseminated lupus erythematodes, drug-induced lupus erythematodes, discoid erythematodes, allergic vasculitis, progressive scleroderma, dermatomyositis, and 36 control sera. PMID:308447

  10. Systemic infection with Mortierella wolfii following abortion in a cow

    PubMed Central

    Davies, Jennifer L.; Ngeleka, Musangu; Wobeser, Gary A.

    2010-01-01

    Severe meningoencephalitis and endometritis associated with necrotizing vasculitis, thrombosis, and infarction were found at necropsy of a 4-year-old Aberdeen Angus cow with a history of abortion and neurological signs. Focal pyogranulomatous pneumonia and nephritis were also present. Fungal hyphae typical of zygomycetes were abundant within lesions, and Mortierella wolfii was cultured from multiple tissues. This is believed to be the first report of systemic mortierellosis following abortion in North America, and the second reported instance of encephalitis caused by M. wolfii in a cow. PMID:21358934

  11. Systemic infection with Mortierella wolfii following abortion in a cow.

    PubMed

    Davies, Jennifer L; Ngeleka, Musangu; Wobeser, Gary A

    2010-12-01

    Severe meningoencephalitis and endometritis associated with necrotizing vasculitis, thrombosis, and infarction were found at necropsy of a 4-year-old Aberdeen Angus cow with a history of abortion and neurological signs. Focal pyogranulomatous pneumonia and nephritis were also present. Fungal hyphae typical of zygomycetes were abundant within lesions, and Mortierella wolfii was cultured from multiple tissues. This is believed to be the first report of systemic mortierellosis following abortion in North America, and the second reported instance of encephalitis caused by M. wolfii in a cow. PMID:21358934

  12. Anterior ischemic optic neuropathy due to biopsy-proven giant cell arteritis in Thai patients

    PubMed Central

    Attaseth, Taweevat; Vanikieti, Kavin; Poonyathalang, Anuchit; Preechawat, Pisit; Jindahra, Panitha; Wattanatranon, Duangkamon

    2015-01-01

    Giant cell arteritis is a systemic granulomatous vasculitis affecting medium to large arteries. An arteritic anterior ischemic optic neuropathy is the most common cause of permanent visual loss. Giant cell arteritis is very rare among Asians. We report six patients with biopsy-proven arteritic anterior ischemic optic neuropathy. Demographic data, clinical manifestations, laboratory findings, treatment, and visual outcome are described in detail and compared with Caucasian patients. We found no differences in any clinical features except for sex preference. Moreover, perioptic nerve sheath enhancement was observed in half of our patients. PMID:26109841

  13. 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.

  14. Granulomatosis with Polyangiitis Presenting as a Choroidal Tumor

    PubMed Central

    Masuda, Taro; Izumi, Yasumori; Takeshita, Hayato; Kawahara, Chieko; Tsuji, Yoshika; Kurohama, Hirokazu; Iwanaga, Nozomi; Inamoto, Miwako; Kase, Keiichi; Ito, Masahiro; Kawakami, Atsushi; Migita, Kiyosi

    2015-01-01

    Granulomatosis with polyangiitis (GPA) sometimes involves the eye orbit; however, choroidal involvements in GPA had been rarely reported. We report a rare case presenting with a choroidal mass in an 83-year-old Japanese woman who presented with left eye pain. Diagnostic biopsy revealed necrotizing vasculitis with infiltrates of inflammatory cells. Diagnosis was localized granulomatosis with polyangiitis. Combined treatments with corticosteroid plus azathioprine resolved the choroidal mass region. Although treatment with corticosteroid and immunosuppressive agents improves the prognosis of the disease, ocular morbidity is still well recognized. Clinicians should consider a differential diagnosis of GPA in patients with inflammatory choroidal tumors. PMID:25949841

  15. Duodenal Perforation Precipitated by Scrub Typhus

    PubMed Central

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

    2015-01-01

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

  16. Bilateral renal infarction: an uncommon presentation of fibromuscular dysplasia

    PubMed Central

    Ayach, Taha; Kazory, Amir

    2013-01-01

    While fibromuscular dysplasia (FMD) is an established cause of secondary hypertension, its association with renal infarction is less well recognized. We report a middle-aged man who presented with complaints of loin pain and severe hypertension. Computed tomography angiography of the abdomen revealed bilateral renal infarction with multiple short-segment arterial dissection compatible with FMD in the absence of systemic vasculitis and other risk factors for thromboembolic events. Bilateral renal infarction complicating FMD is extremely rare and has so far been reported only in a handful of cases. Physicians encountering cases of otherwise unexplained renal infarction/ischemia need to be aware of this complication.

  17. [Malignant infarct of the middle cerebral artery in a patient with bacterial meningitis].

    PubMed

    López, Mirta G; Mellado, Patricio T; Huete, Isidro L

    2004-10-01

    The mortality of acute bacterial meningitis (BM) has remained stable in the last decades in spite of the use of new antibiotics, probably due to vascular complications. We report a 68-year-old woman with BM who had a malignant infarction of left middle cerebral artery territory 72 hours after admission to the hospital. The patient experienced a bad evolution and died four days after admission. The arterial involvement in patients with BM is explained by vasospasm of large arteries and vasculitis of small arteries. The medical treatment of a malignant middle cerebral artery infarct has a high mortality. PMID:15631210

  18. Behçet's disease

    PubMed Central

    Kontogiannis, V; Powell, R

    2000-01-01

    Behçet's disease is a systemic vasculitis of unknown aetiology characteristically affecting venules. Onset is typically in young adults with recurrent oral and genital ulceration, uveitis, skin manifestations, arthritis, neurological involvement, and a tendency to thrombosis. It has a worldwide distribution but is prevalent in Japan, the Middle East, and some Mediterranean countries. International diagnostic criteria have been proposed, however diagnosis can be problematical, particularly if the typical ulcers are not obvious at presentation. Treatment is challenging, must be tailored to the pattern of organ involvement for each patient and often requires combination therapies.???Keywords: Behçet's disease; oral ulcers; uveitis; immunosuppressants PMID:11009577

  19. [Cytomegalovirus colitis in patients with acquired immunodeficiency syndrome. Histopathologic, endoscopic, and clinical correlation].

    PubMed

    Reyes, E; Gamboa-Domínguez, A

    1993-01-01

    We describe the frequency of cytomegalovirus (CMV) infection in colon and rectum biopsies obtained endoscopically in patients with AIDS. In 44 of 199 biopsies, CMV was the only opportunistic infection. Cytomegaly, intracytoplasmic inclusions, and vasculitis with endothelial CMV-cytopathic damage were the histological changes that prompted the diagnosis. CMV-infected cells were located primary in the stromal and endothelial cells. The vascular damage was observed only in submucosal vessels. Histological findings were correlated with the endoscopic mucosal appearance and with the clinical symptoms, founding no significative correlation. PMID:8165398

  20. Henoch-Schönlein purpura without systemic involvement beginning with acute scrotum and mimicking torsion of testis.

    PubMed

    Verim, Levent; Cebeci, Filiz; Erdem, M Remzi; Somay, Adnan

    2013-03-01

    Henoch-Schönlein purpura (HSP) is the most common systemic vasculitis in children. Typical presentations of HSP are palpable purpura of the small vessels in the hips and lower limbs, abdominal pain, arthritis, and hematuria. Scrotal involvement manifested by the presence of scrotal pain and swelling during the course of the disease is rarely seen. HSP without systemic involvement with acute scrotum mimicking testicular torsion is even rare in the medical literature. In most cases, patients with this disease achieve complete recovery. Herein, we report an interesting HSP case with skin symptoms but without systemic involvement and then progression to acute scrotum resembling torsion of testis. PMID:23695408

  1. Imaging Characteristics of Takayasu Arteritis

    SciTech Connect

    Canyigit, Murat, E-mail: mcanyigit@yahoo.com; Peynircioglu, Bora; Hazirolan, Tuncay; Dagoglu, Merve Gulbiz; Cil, Barbaros Erhan; Haliloglu, Mithat; Balkanci, Ferhun; Besim, Aytekin [Faculty of Medicine, Hacettepe University, Department of Radiology (Turkey)

    2007-07-15

    Takayasu arteritis is a rare, large-vessel vasculitis in which the nonspecific systemic inflammatory symptoms are followed by inflammation of the aorta and its major branches. The inflammation of this vessel leads to progressive luminal stenosis or aneurysm formation resulting in limb or organ ischemia. Although conventional angiography is still accepted as the gold standard modality, the information obtained is limited to the vessel lumen. Multidetector computed tomographic angiography and magnetic resonance angiography can provide valuable information not only regarding intraluminal pathologies but also concerning the thickening of the vessel wall, which may be the earliest manifestation of the disease.

  2. Mechanical Thrombectomy with Rotarex System in Buerger's Disease

    PubMed Central

    Kilickesmez, Ozgur; Oguzkurt, Levent

    2015-01-01

    We report the case of a patient with 2-month history of chronic thromboembolism of the distal superficial femoral and popliteal arteries with diagnostic features of thromboangiitis obliterans disease. The occlusion could not be crossed by antegrade approach and was achieved retrogradely via dorsalis pedis artery puncture followed by mechanical removal of the thrombus with Rotarex system (Straub Medical AG, Wangs, Switzerland). Subsequent ballooon angioplasties achieved exclusion of the thrombus, and straight-line flow established to the foot through the anterior tibial Artery. The present case report demonstrates the success of mechanical thrombectomy in a patient with Buerger's vasculitis. PMID:25861548

  3. Recent Advances in the Immunopathogenesis of Systemic Lupus Erythematosus

    PubMed Central

    Bardana, Emil J.; Pirofsky, Bernard

    1975-01-01

    Systemic lupus erythematosus (SLE) is a chronic multisystem inflammatory disease having definite etiologic associations with ethnic, genetic, viral and immunologic factors. Its pathologic hallmark, vasculitis, is currently felt to be the end result of an immune-complex mechanism. Several clinical and serologic variants of SLE are recognized including discoid lupus erythematosus (DLE), mixed connective tissue disease (MCTD) and drug-induced equivalents—such as procainamide-induced lupus (PIL). The distinguishing features of these variants as well as their prognosis and therapy are discussed in relation to recent developments in the immunopathogenesis of SLE. PMID:46657

  4. 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

  5. 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

  6. Ocular manifestations of the potentially lethal rheumatologic and vasculitic disorders.

    PubMed

    Foster, C Stephen

    2013-06-01

    Vision threatening ocular inflammation may occur in patients with any of the acquired connective tissue disorders and vasculitic diseases. Additionally, the ocular inflammation may be the presenting manifestation of the disease, which leads the patient to seek medical care. Other manifestations of the potentially lethal disease may be subtle or absent, presenting the thoughtful ophthalmologist with the opportunity to make life saving discoveries. Necrotizing scleritis, peripheral ulcerative keratitis, and retinal vasculitis are the ocular findings which should prompt the ophthalmologist to initiate very aggressive measures aimed at discovering any evidence of extra-ocular abnormalities, laboratory or otherwise. Appropriate therapy will be sight saving and may be life saving. PMID:23688612

  7. Clinicoepidemiological manifestations of RPGN and ANCA-associated vasculitides: an 11-year retrospective hospital-based study in Japan

    Microsoft Academic Search

    Yusuke SuzukiYukihiko; Yukihiko Takeda; Daisuke Sato; Yasuhiko Kanaguchi; Yuichi Tanaka; Shigeto Kobayashi; Kazuo Suzuki; Hiroshi Hashimoto; Shoichi Ozaki; Satoshi Horikoshi; Yasuhiko Tomino

    2010-01-01

    Antineutrophil cytoplasmic antibody (ANCA)-associated small-vessel vasculitides are major causes of rapidly progressive glomerulonephritis\\u000a (RPGN). Although recent papers suggest differences in clinicoepidemiological manifestations of ANCA-associated vasculitis\\u000a between Japan [microscopic polyangiitis (MPA) ? Wegener’s granulomatosis (WG)] and Europe (WG ?MPA), little is known about\\u000a the prevalence and serological pattern. We retrospectively analyzed 27 RPGN patients who were admitted in our hospital over\\u000a the past 11 years

  8. Henoch-Schönlein purpura with uveitis: an unusual case and review of literature.

    PubMed

    Kaur, Sharandeep; Maheshwari, Anu; Aneja, Satinder; Seth, Anju; Beri, Sarita; Agarwal, Shilpi; Garg, Taru

    2012-12-01

    Henoch-Schönlein purpura (HSP) is a small vessel vasculitis with IgA dominant immune complex deposition. It is characterized by a triad of palpable purpura (without thrombocytopenia), abdominal pain and arthritis. Uveitis is rarely associated with HSP with only 3 cases reported in literature. All these cases were in adult population and were associated with nephritis. However, this association is not reported in paediatric age group. We are reporting a case of an 11-year-old child of recurrent HSP with uveitis. PMID:21918898

  9. Gastrointestinal manifestations of Henoch-Schonlein purpura: A report of two cases.

    PubMed

    Prathiba Rajalakshmi, Parameswaran; Srinivasan, Kalyanasundaram

    2015-03-28

    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. PMID:25825636

  10. Stroke and skin rash: A rare case of Henoch-Schonlein purpura.

    PubMed

    Ghosh, Kaushik; Chatterjee, Atri; Sau, Jyoti Tanmay; Dey, Sandip

    2012-10-01

    Henoch-Schonlein purpura (HSP) is a small vessel vasculitis that is rare in adults. Here, we present a case of a woman who presented with palpable purpura, abdominal pain, arthritis and ischemic stroke. The patient met the diagnostic criteria of HSP. However, cerebrovascular disease is reported as an uncommon, yet fatal, complication of HSP. The patient responded to aggressive immunosuppression with pulses of corticosteroids and cyclophosphamide. In the absence of an established protocol of treatment of such neurologic emergency in HSP patients, this report demonstrates a successful outcome. PMID:23349600

  11. A case of polyarteritis nodosa with periurethralaseptic abscesses and testicular lesions.

    PubMed

    Watanabe, K; Nanki, T; Sugihara, T; Miyasaka, N

    2008-01-01

    We describe a 54-year-old man presenting with cutaneous ulcerations, livedo reticularis, numbness of the legs, and skin histological findings compatible with the diagnosis of polyarteritis nodosa (PAN). Initial treatment with 50 mg/day of prednisolone (PSL) was effective. However, the symptoms and signs recurred, and the patient developed multiple periurethral aseptic abscesses, urethra-cutaneous fistula, and testicular lesions after tapering of PSL therapy. The condition improved with PSL and cyclophosphamide administration. Since penile and testicular vasculitis could be associated with PAN, although rarely, we should carefully distinguish such an involvement from infection and malignancy. PMID:19210882

  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. [Mixed type-II cryoglobulinemia associated with a chronic hepatitis C virus infection].

    PubMed

    Suárez, A; Vallina, E; Navascués, C A; Rodríguez, M; Otero, L; Sotorrío, N G; San Román, F; Rodrigo, L

    1993-04-01

    We describe the case of a patient with non A-non B post-transfusional cirrhosis and type-II mixed cryoglobulinemia, who showed in relation with said processes several acute symptoms of vasculitis, polyarthritis, pericarditis and autoimmune hemolytic anemia, finally dying due to an advanced hepatocellular insufficiency. In this patient the determination of antibodies against hepatitis C virus (anti-HCV) was positive, that is why we assume a possible relationship between both processes and the first literature references, after the clonation of the hepatitis C virus (HCV) genome, are reviewed. PMID:8388574

  14. Refractory PMR with aortitis: life-saving treatment with anti-IL6 monoclonal antibody (tocilizumab) and surgical reconstruction of the ascending aorta

    PubMed Central

    Ashraf, Fahd Adeeb Mohamed; Anjum, Shakeel; Hussaini, Abid; Fraser, Alexander

    2013-01-01

    Aortitis is uncommon but well described in patients with polymyalgia rheumatica (PMR). While glucocorticoid remains the mainstay therapy for large-vessel vasculitis, there have been cases where tocilizumab therapy led to clinical and serological improvement in patients with relapsing or refractory disease. We report a case of life-threatening PMR with aortitis in the absence of manifestations related to giant cell arteritis, which, having failed to respond to corticosteroid therapy, was successfully treated with tocilizumab and emergency reconstruction of the ascending aorta. This case adds to the literature supporting the potential value of interleukin-6 inhibition in rare rheumatological conditions such as inflammatory aortitis. PMID:23780768

  15. Eosinophilic granulomatosis with polyangiitis and diffuse gastrointestinal involvement.

    PubMed

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

    2014-09-01

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

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

    PubMed Central

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

    2015-01-01

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

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

    PubMed

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

    2015-01-01

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

  18. Meningoencephalitis in commercial meat turkeys associated with Mycoplasma gallisepticum.

    PubMed

    Chin, R P; Daft, B M; Meteyer, C U; Yamamoto, R

    1991-01-01

    Mycoplasma gallisepticum (MG) infection was diagnosed in three different flocks of 12-to-16-week-old commercial meat turkeys displaying torticollis and/or opisthotonos. MG was isolated from the brain, air sacs, trachea, and sinus of one bird with neurological signs. Histological examination of brains in all three cases revealed moderate-to-severe encephalitis with lymphoplasmacytic cuffing of vessels, fibrinoid vasculitis, focal parenchymal necrosis, and meningitis. Birds with neurological signs were seropositive for MG by the serum-plate agglutination and hemagglutination-inhibition tests. The encephalitic form of MG has been described previously but is rarely mentioned in the current literature. PMID:1786029

  19. Odocoileus hemionus deer adenovirus is related to the members of Atadenovirus genus

    Microsoft Academic Search

    A. Zakhartchouk; A. Bout; L. W. Woods; H. D. Lehmkuhl; M. J. E. Havenga

    2002-01-01

    Summary.  ?The Odocoileus hemionus deer adenovirus (OdAdV-1) causes systemic and local vasculitis and proves extremely lethal for mule deer. To characterize\\u000a the virus, part of the genome flanking the fiber gene was cloned and sequenced. The sequence revealed two open-reading frames\\u000a that mapped to pVIII hexon-associated protein precursor and fiber protein of several other adenoviruses. The highest amino\\u000a acid homology for

  20. Malignant catarrhal fever associated with ovine herpesvirus-2 in free-ranging mule deer in Colorado.

    PubMed

    Schultheiss, Patricia C; Van Campen, Hana; Spraker, Terry R; Bishop, Chad; Wolfe, Lisa; Podell, Brendan

    2007-07-01

    Malignant catarrhal fever (MCF) was diagnosed in four free-ranging mule deer (Odocoileus hemionus) in January and February of 2003. Diagnosis was based on typical histologic lesions of lymphocytic vasculitis and PCR identification of ovine herpesvirus-2 (OHV-2) viral genetic sequences in formalin-fixed tissues. The animals were from the Uncompahgre Plateau of southwestern Colorado. Deer from these herds occasionally resided in close proximity to domestic sheep (Ovis aries), the reservoir host of OHV-2, in agricultural valleys adjacent to their winter range. These cases indicate that fatal OHV-2 associated MCF can occur in free-ranging mule deer exposed to domestic sheep that overlap their range. PMID:17699095

  1. [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

  2. Livedoid vasculopathy: thrombotic or inflammatory disease?

    PubMed

    Khenifer, S; Thomas, L; Balme, B; Dalle, S

    2010-10-01

    The pathogenesis of livedoid vasculopathy (LV) is still unclear. However, with increasing knowledge of disorders of coagulation over the past few years, the cause of some cases of LV has been elucidated. LV has now been described in association with hyperhomocysteinaemia, activated protein C resistance, and prothombin gene mutations in the absence of significant underlying inflammatory disease. When LV is seen in association with systemic lupus erythematosus or polyarteritis nodosa, it is probably due to the pro-coagulable state induced by these diseases rather than being true vasculitis. We review recent insights into LV provided by published clinical cases and discuss its pathogenesis. PMID:19874344

  3. Technetium scanning in Kaposi's sarcoma and its simulators

    SciTech Connect

    Gunnoe, R.; Kalivas, J.

    1982-04-01

    The clinical picture of ulcerated purple plaques on the legs often suggests several diagnoses: Kaposi's sarcoma, stasis dermatitis, atrophie blanche (livedoid vasculitis), and a poorly understood condition called acroangiodermatitis of Favre-Chaix (pseudo-Kaposi's sarcoma). Even the skin biopsy may not always be conclusive. We describe our experience with three patients with pseudo-Kaposi's sarcoma, one with true Kaposi's sarcoma and two with atrophie blanche. Clinical and histopathologic similarities among these three conditions pointed up the need for additional confirmatory studies, i.e., isotope scanning. The technetium scan was positive in both Kaposi's sarcoma and pseudo-Kaposi's sarcoma but negative in atrophie blanche.

  4. [Livedoid vasculopathy. Pathogenesis, diagnosis and treatment of cutaneous infarction].

    PubMed

    Goerge, T

    2011-08-01

    Livedo vasculopathy is a chronic recurrent disease of the cutaneous circulation and is characterized by episodic occurrence of painful ulcerations of the lower leg. These heal slowly leaving small porcelain-white scars called atrophie blanche. Recent research has shown that livedoid vasculopathy is a coagulation disorder classified as a vasculopathy different from inflammatory vasculitis. Distinguishing between the disorders enhances the pathophysiologic understanding and supports the therapeutic rationale. The prevention of irreversible residual scarring is the main goal in treating cutaneous infarction; prompt treatment is required. PMID:21786003

  5. Transient Oculomotor Palsy after Influenza Vaccination: Short Report

    PubMed Central

    de Almeida, Diogo Fraxino; Teodoro, Adilson Teixeira; Radaeli, Rafael de Figueiredo

    2011-01-01

    Several neurological complications have been described after influenza vaccination. Oculomotor palsy has not been yet related with influenza vaccine. We report a 79-year-old man who developed an acute transient right oculomotor palsy two days after a routine influenza vaccination, for which no other cause was identified. There was no evidence of diabetes, glucose intolerance, high blood pressure, hipercholesterolemia, smoking, obesity, systemic vasculitis, or other risk factor for ischemic neuropathy. The cerebrospinal fluid was normal as well as the MRI and MRA scans. The differential diagnosis and the possible relationship between the vaccine and the oculomotor palsy are discussed. PMID:22389831

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

    PubMed

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

    1988-03-01

    Bacillus cereus, which used to be considered non-pathogenic, was isolated from the blood of a patient with acute leukemia who was receiving intensive chemotherapy. Fatal bacteremia developed with a clinical syndrome of acute gastroenteritis, followed by both meningoencephalitis with subarachnoid hemorrhage and multiple liver abscesses probably caused by infective vasculitis. Surveillance stool cultures revealed colonization with the organism prior to the onset of diarrhea, and repetitive blood cultures were found to be positive. Thus, this case suggested some new important clinicopathologic features of true B. cereus bacteremia complicating acute leukemia. PMID:3127617

  7. [Autoimmune and lymphoproliferative HCV-correlated manifestations: example of mixed cryoglobulinaemia (review)].

    PubMed

    Ghinoi, A; Mascia, M T; Puccini, R; Ferri, C

    2004-01-01

    Mixed cryoglobulinaemia (MC) is a systemic vasculitis involving small vessels (arterioles, capillaries, venules). The histological hallmark of the disease is the leukocytoclastic vasculitis secondary to the vascular deposition of circulating immune-complexes (CIC), mainly cryoglobulins and complement. The immune-mediated vasculitic lesions are responsible for different MC clinical features, including cutaneous and visceral organ involvement. Hepatitis C virus (HCV) represents the triggering factor in the large majority of MC patients (>90%). Moreover, several epidemiological, clinico-pathological and laboratory investigations suggested a possible role for HCV in a wide spectrum of immuno-lymphoproliferative disorders; namely, porphyria cutanea tarda, diabetes, polyarthritis, lung fibrosis, poly-dermatomyositis, thyroiditis, thyroid cancer, B-cell non-Hodgkin's lymphomas (B-NHL), etc. Renal involvement with or without MC syndrome can be observed in HCV-infected individuals. There is great geographical etherogeneity in the prevalence of HCV-related disorders. This epidemiological observation suggests a multifactorial and multistep process in the pathogenesis of these conditions, involving other unknown genetic and/or environmental factors. HCV lymphotropism may explain the mono-oligoclonal B-lymphocyte expansion observed in HCV-infected individuals, particularly in MC patients. The 'benign' lymphoproliferative disorder, classified as monotypic lymphoproliferative disorders of undetermined significance (MLDUS), may be responsible for the wide production of CIC, including cryoglobulins, rheumatoid factor and different organ and non-organ specific autoantibodies. The consequence is the appearance of various HCV-related autoimmune diseases, including MC syndrome. This latter may be complicated by B-NHL in 10% of the cases; moreover, HCV infection has been confirmed in a significant percentage of 'idiopathic B-NHL. For a correct therapeutic approach to cryoglobulinaemic vasculitis, as well as to other HCV-related disorders, we should deal with concomitant, conflicting conditions: HCV infection, autoimmune and lymphoproliferative alterations. In this scenario, we can treat the diseases at three different levels by means of etiologic, pathogenetic and/or symptomatic therapies. The eradication of HCV by combined interferon and ribavirin therapy can be achieved in only a minority of cases. On the contrary, severe complications such as glomerulonephritis, sensory-motor neuropathy or diffuse vasculitis can be effectively treated by a combination of corticosteroids, plasma exchange and cyclophosphamide. More recently, a pathogenetic treatment with rituximab, a monoclonal chimeric antibody that binds to the B-cell surface antigen CD20 with selective B-cell blockade, was proposed in patients with HCV-related MC syndrome. PMID:15285001

  8. Takayasu's Arteritis with Systemic Lupus Erythematosus: A Rare Association

    PubMed Central

    Bandyopadhyay, Dhrubajyoti; Ganesan, Vijayan; Bhar, Debarati; Bhowmick, Diptak; Sasmal, Sibnarayan; Choudhury, Cankatika; Mukhopadhyay, Sabyasachi; Hajra, Adrija; Layek, Manas; Karmakar, Partha Sarathi

    2015-01-01

    We report the case of a 24-year-old nondiabetic, nonhypertensive lady with history of fatigue, dyspnoea and limb claudication. She has been diagnosed with Takayasu's arteritis. Subsequently she developed rash, alopecia, joint pain, and various other laboratory abnormalities which led to a diagnosis of SLE. Takayasu's arteritis (TA) rarely coexists with systemic lupus erythematosus (SLE). The absence of specific SLE markers in patients with TA who subsequently develop SLE suggests that the coexistence of these conditions may be coincidental. The antiphospholipid syndrome in patients with SLE may mimic the occlusive vasculitis of TA.

  9. Wegener Granulomatosis: Otologic Manifestation as First Symptom

    PubMed Central

    Costa, Carla Fabiane da; Polanski, Jose Fernando

    2014-01-01

    Introduction?Wegener granulomatosis is a systemic vasculitis affecting small and medium-sized vessels of the upper and lower respiratory tract and kidneys. Objective?To describe a case of Wegener disease with atypical manifestation. Resumed Report?We describe the case of a 50-year-old woman with chronic otitis media and sensorineural hearing loss as the primary symptoms, without other manifestations. Conclusion?In cases of acute ear manifestations with or without hearing loss and with poor response to usual treatments, Wegener granulomatosis should be included among the possible etiologies. After adequate diagnoses and treatment of this rare disease, there was favorable evolution.

  10. The trigeminal trophic syndrome: an unusual cause of nasal ulceration.

    PubMed

    Monrad, Seetha U; Terrell, Jeffrey E; Aronoff, David M

    2004-06-01

    Trigeminal trophic syndrome (TTS) is an unusual complication after peripheral or central damage to the trigeminal nerve, characterized by anesthesia, paresthesias, and ala nasi ulceration. We describe a patient with classic TTS after trigeminal rhizotomy who underwent several extensive evaluations for nasal ulceration and received prolonged immunosuppressive therapy for a presumed autoimmune disorder before the correct diagnosis was made. An understanding of the predisposing factors and clinical presentation of TTS is important to ensure a timely diagnosis of this difficult-to-treat illness. Differentiation of TTS from malignancy, infection, or vasculitis is possible on the basis of clinical history, tissue biopsy, and serologic evaluation. PMID:15153901

  11. Wegener Granulomatosis: Otologic Manifestation as First Symptom.

    PubMed

    Costa, Carla Fabiane da; Polanski, Jose Fernando

    2015-07-01

    Introduction?Wegener granulomatosis is a systemic vasculitis affecting small and medium-sized vessels of the upper and lower respiratory tract and kidneys. Objective?To describe a case of Wegener disease with atypical manifestation. Resumed Report?We describe the case of a 50-year-old woman with chronic otitis media and sensorineural hearing loss as the primary symptoms, without other manifestations. Conclusion?In cases of acute ear manifestations with or without hearing loss and with poor response to usual treatments, Wegener granulomatosis should be included among the possible etiologies. After adequate diagnoses and treatment of this rare disease, there was favorable evolution. PMID:26157503

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

    PubMed

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

    2015-08-01

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

  13. Oesophageal lesions as first manifestation of necrotising sarcoid granulomatosis.

    PubMed

    Attou, Rachid; Duttmann, Ruth; Devriendt, Arnaud; De Bels, David

    2015-01-01

    Necrotising sarcoid granulomatosis is a rare disease associating sarcoid-like granulomas, vasculitis and necrosis in the lungs and seldom in extrapulmonary locations. We report the case of a 23-year-old woman with an initial symptom consisting of epigastric pain. Oesophagoscopy demonstrated oesophagitis. Evolution was marked by fever, dyspnoea and pulmonary lesions. The first presumptive diagnosis was Wegener disease but biopsies (of the lungs and the oesophagus) demonstrated the diagnosis of necrotising sarcoid granulomatosis. Evolution was favourable under corticoid therapy. Necrotising sarcoid granulomatosis may present as an extrapulmonary disease and is an important differential diagnosis of other disease such as Wegener disease. PMID:26150629

  14. Ocular Manifestations of Systemic Lupus Erythematosus: A Review of the Literature

    PubMed Central

    Palejwala, Neal V.; Walia, Harpreet S.; Yeh, Steven

    2012-01-01

    About one-third of patients suffering from systemic lupus erythematosus have ocular manifestations. The most common manifestation is keratoconjunctivitis sicca. The most vision threatening are retinal vasculitis and optic neuritis/neuropathy. Prompt diagnosis and treatment of eye disease is paramount as they are often associated with high levels of systemic inflammation and end-organ damage. Initial management with high-dose oral or IV corticosteroids is often necessary. Multiple “steroid-sparing” treatment options exist with the most recently studied being biologic agents. PMID:22811887

  15. Blocking von Willebrand factor for treatment of cutaneous inflammation.

    PubMed

    Hillgruber, Carina; Steingräber, Annika K; Pöppelmann, Birgit; Denis, Cécile V; Ware, Jerry; Vestweber, Dietmar; Nieswandt, Bernhard; Schneider, Stefan W; Goerge, Tobias

    2014-01-01

    Von Willebrand factor (VWF), a key player in hemostasis, is increasingly recognized as a proinflammatory protein. Here, we found a massive accumulation of VWF in skin biopsies of patients suffering from immune complex (IC)-mediated vasculitis (ICV). To clarify the impact of VWF on cutaneous inflammation, we induced experimental ICV either in mice treated with VWF-blocking antibodies or in VWF(-/-) mice. Interference with VWF led to a significant inhibition of the cutaneous inflammatory response. We confirmed the major findings in irritative contact dermatitis, a second model of cutaneous inflammation. In vivo imaging of cutaneous inflammation in the dorsal skinfold chamber revealed unaffected leukocyte rolling on anti-VWF treatment. However, we identified that reduced leukocyte recruitment is accompanied by reduced vascular permeability. Although VWF-mediated neutrophil recruitment to the peritoneum was described to require the VWF receptor on platelets (glycoprotein Ib? (GPIb?)), the VWF/GPIb? axis was dispensable for cutaneous inflammation. As assessed in tail bleeding assays, we could exclude interference of VWF blockade with hemostasis. Of particular importance, anti-VWF treatment was effective both in prophylactic and therapeutic administration. Thus, VWF represents a promising target for the treatment of cutaneous inflammation, e.g., leukocytoclastic vasculitis. PMID:23812299

  16. The utility of tumour necrosis factor blockade in orphan diseases

    PubMed Central

    Keystone, E

    2004-01-01

    A variety of rheumatic disorders have been successfully treated with tumour necrosis factor (TNF) blockers. However, TNF blockade may be useful in a number of rare diseases. Preliminary data suggest that several forms of vasculitis appear responsive to TNF antagonists—Behçet's disease, Churg–Strauss vasculitis, polyarteritis nodosa, and giant cell arteritis, among others. Wegener's granulomatosis and sarcoidosis have been shown to improve with infliximab but not with etanercept. These results lend further support for the concept of differential mechanism(s) of action of the two antagonists with infliximab being more effective for the treatment of granulomatous diseases. Polymyositis/dermatomyositis may also be responsive to TNF blockade. TNF likely plays little role in Sjögren's syndrome as evidenced by the lack of efficacy of both TNF antagonists. Etanercept has been shown to be useful in the treatment of hepatitis C both in reducing the viral load and improving liver function. A number of other more rare disorders also may be responsive to TNF blockade. Further studies with larger numbers of well characterised patients and treatment regimens are necessary to provide more definitive evidence of the utility of the TNF antagonists in these serious and often life threatening diseases. PMID:15479879

  17. Gastrointestinal manifestations of Behçet's disease.

    PubMed

    Ebert, Ellen C

    2009-02-01

    Behçet's disease is a rare vasculitis diagnosed by the presence of recurrent oral ulcers and two of the following: genital ulcers, typical eye lesions, typical skin lesions, and positive pathergy test. It is most commonly seen in countries along the ancient silk road from Eastern Asia to the Mediterranean Basin. Young adults between the second and fourth decades of life are mainly affected, with abdominal pain being the most common symptom. The ileocecal region is most commonly affected, with ulcerations that may penetrate or perforate. Rarely, the esophagus and stomach may have ulcerations. Bowel wall thickening is the most common finding on computed tomography (CT) scan. Pathology shows a vasculitis mainly involving the small veins or, alternatively, nonspecific inflammation. Corticosteroids, with or without other immunosuppressive drugs, are used for severe eye disease. Their use in intestinal disease is largely empirical. Surgery may be required for perforation. Behçet's disease runs a chronic, unpredictable course with exacerbations and remissions which decrease in frequency and severity over time. Death is mainly due to major vessel disease and neurological involvement. PMID:18594975

  18. Therapy of Sjögren's syndrome. New aspects and future directions.

    PubMed

    Vlachoyiannopoulos, P G

    1998-02-01

    Therapy of Sjögren's syndrome remains empirical and symptomatic. The main goals are to treat the disease related features, especially sicca manifestations, since the immunosuppressive therapy has not given promising results. For the treatment of keratoconjunctivitis sicca: local stimulators of tear secretion, protective bicarbonate buffered solutions, replacement therapy or supportive operative procedures should be tried. For oral manifestations: stimulators of salivary secretion such as pilocarpine, or agents changing the consistency of saliva such as bromhexine orally should be tried. Saliva substitutes have a transient effect. Frequent ingestion of sugar free liquids may help. Oral hygiene is important to avoid oral candidiasis and dental caries. Treatment of parotid gland swelling is not necessary. Pulmonary manifestations include pulmonary infiltrates in perialveolar areas, nodular or cavitary lesions which may represent lymphoma. Hilar adenopathy, solid or cavitary lesions should be biopsied. In case of vasculitis prednisolone 1 mg/kg/day with progressive tapering should be tried. Renal involvement is manifested mainly as interstitial disease. Administration of NaHCO3 or sodium citrate is important to prevent acidosis and nephrocalcinosis. Vasculitis, when it is of the leukoclasic form, does not need therapy; when it is manifested with severe major organ involvement corticosteroids and/or cytotoxic therapy should be tried. Lymphoma is treated as in the patients without Sjögren's in close collaboration with the oncology department. PMID:11490519

  19. Levamisole-adulterated cocaine induced skin necrosis of nose, ears, and extremities: Case report

    PubMed Central

    Jiron, Jose L.; Lin, Ho-Sheng; Folbe, Adam J.

    2014-01-01

    Levamisole is an immunomodulatory and antihelminthic drug, previously removed from the United States market, and now estimated to be present in the vast majority of cocaine distributed in the United States. Levamisole-adulterated cocaine (LAC) exposure can result in neutropenia, thrombocytopenia, and vasculitis with a predilection for subsites of the face. The objective of this review is to increase awareness among otolaryngologists of the manifestations of LAC exposure. We present the case of a 33-year-old woman with a history of cocaine use, consulted for purpuric, necrotic lesions of the nose, cheeks, and ears, with accompanying leukopenia, thrombocytopenia, and positive antineutrophil cytoplasmic antibodies (ANCA). The effects of levamisole are immune mediated, with antibodies directed against neutrophils causing neutropenia, and vasculitis caused by antibody deposition or secondary to induction of antiphospholipid antibodies causing thrombosis. LAC exposure can be differentiated from other similar appearing pathologies by evaluating serology for specific ANCA. The most important treatment is cessation of cocaine use, which most often results in complete resolution of symptoms. Awareness of the presentation, complications, and treatment of LAC exposure may be especially important for otolaryngologists, who may be one of the firsts to evaluate an affected patient. PMID:25565048

  20. Erythema elevatum diutinum: clinical, histopathologic, and immunohistochemical characteristics of six patients.

    PubMed

    Wahl, Carter E; Bouldin, Molly B; Gibson, Lawrence E

    2005-10-01

    Erythema elevatum diutinum (EED) is a chronic cutaneous vasculitis occurring in association with a variety of conditions including autoimmunity, infectious disease, and hematological abnormalities. The role of associated medical problems is controversial, and the exact pathogenesis of EED is unknown. A series of six cases is reported. The typical clinical presentation was that of erythematous papules and plaques involving the extensor surfaces of the extremities. Histologically, a spectrum from leukocytoclastic vasculitis to vessel occlusion and dermal fibrosis was seen. The lesions of EED have many mimics clinically and histologically. Establishing the diagnosis of EED is important so appropriate screening for associated conditions can ensue. The vascular endothelium of EED stains positive for CD31, CD34, VEGF, and factor VIIIa and negative for factor XIIIa, TGFB, and LANA, a reaction pattern that does not distinguish it from similar appearing lesions. Thus, the chronic and recurrent nature of EED is the primary means of distinguishing it from entities that are clinically and histologically similar. PMID:16148408