Phenylbutazone-induced systemic vasculitis with crescentic glomerulonephritis

Two patients had phenylbutazone-induced systemic vasculitis syndrome. Both presented with acute oliguric renal failure, and renal biopsies showed severe crescentic glomerulonephritis with marked interstitial inflammatory cell infiltration. Withdrawal of phenylbutazone and treatment with immunosuppressives and plasma exchange led to recovery of renal function in one case.     

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

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

Temporal arteritis associated with systemic necrotizing vasculitis

OBJECTIVE: To evaluate the clinical and laboratory characteristics of patients with systemic vasculitis associated with temporal artery involvement. METHODS: From a cohort of 120 patients fulfilling American College of Rheumatology criteria for temporal arteritis, we retrospectively identified 7 patients with systemic necrotizing vasculitis associated with histological temporal arteritis. RESULTS: Among the 7 patients, 2 had classic polyarteritis nodosa, one had unclassified systemic vasculitis, one had Wegener's granulomatosis (WG), and 3 had microscopic polyangiitis. The mean age of the patients was 70.2 years, and cranial symptoms revealed the disease in all but one patient. Temporal arteritis was generally associated with extracephalic manifestations suggestive of systemic vasculitis. Antineutrophilic cytoplasmic antibodies were positive in 3 of the 4 patients with small vessel vasculitis. Pathologically, the main temporal artery was involved in all but one patient, with inflammatory infiltrate of vasa vasorum and adventitia associated in 5 with small tributary involvement. Fibrinoid necrosis was rare, observed in 2 specimens; 2 patients with unclassified systemic vasculitis and WG had a classic giant cell arteritis (GCA) histologic pattern. Only one patient had exclusive involvement of small vessels, surrounding the spared main temporal artery. Muscle biopsies showed histopathological evidence of vasculitis in 2 patients, skin biopsy in one, and vein biopsy in the other. CONCLUSION: Temporal artery involvement in systemic necrotizing vasculitis was generally associated with extracranial clinical features suggestive of systemic vasculitis. Temporal artery biopsy is a simple tool for diagnosis of vasculitis, but the histopathological findings do not always discriminate between necrotizing vasculitis and classic GCA.     

Autoantibodies in systemic vasculitis

Abstract We have studied 495 sera that were referred to us from patients suspected on clinical and/or histological grounds to have a small vessel vasculitis. These sera were tested for antibodies against neutrophil cytoplasm antigens (anti-neutrophil cytoplasm antibodies, ANCA) using assays based on neutrophil acid extract, myeloperoxidase and elastase. Such antibodies are commonly found in Wegener's granulomatosis (WG) and microscopic polyarteritis (MPA), and sometimes in other small vessel vasculitides. One hundred and twenty-six of these sera (25%) were positive in the acid extract ELISA, 68 (14%) in the assay for anti-myeloperoxidase antibodies and 35 (16%) in the assay for anti-elastase antibodies. A total of 166 sera (34%) were positive for antibodies against neutrophil cytoplasm constituents. No ANCA, anti-myeloperoxidase or anti-elastase antibodies were detected in 26 convalescent sera from patients either with WG or MPA, or who had previously been positive. The mean time between positive and negative sera was eight weeks (range three weeks to six months) and three out of three who relapsed again developed ANCA of the same specificity as the original sera. Of the 228 sera also tested for anti-GBM antibodies, 13 (5.7%) were positive. All these contained antibodies against neutrophil cytoplasm constituents (three against the acid extract, eight against myeloperoxidase and two against elastase). Forty-nine of the 74 sera (66%) tested for ANA were positive. Twenty-nine (39%) had a speckled and 20 (27%) had a homogeneous pattern. Twenty of these were positive for ANCA or anti-myeloperoxidase or anti-elastase antibodies and 29 were negative. ANCA, anti-myeloperoxidase and anti-elastase antibodies are common in patients in whom a vasculitis is suspected on clinical and/or histological grounds. The presence of these antibodies correlates with disease activity. Anti-GBM antibodies and ANA are not uncommon in this group. A single ELISA for ANCA is insufficient to detect all antibodies directed against neutrophil cytoplasm constituents. (Aust NZ J Med 1991; 21: 433–437.)     

Sarcoidosis and systemic vasculitis

BACKGROUND: Systemic vasculitis is an unusual complication of sarcoidosis. Over a 10-year period, the authors have provided care for six patients who had features of both sarcoidosis and vasculitis. Vasculitis could not be attributed to other causes. OBJECTIVES: To report six patients (five children) who had sarcoidosis and systemic vasculitis and compare our experience with previous literature. To better delineate the clinical spectrum of sarcoid vasculitis and its response to therapy. METHODS: Retrospective analysis and a Medline literature review of sarcoid and concurrent vasculitis from 1966. RESULTS: Our six patients had systemic illnesses that included fever, peripheral adenopathy, hilar adenopathy, rash, pulmonary parenchymal disease, musculoskeletal symptoms, and scleritis or iridocyclitis. Biopsies revealed features compatible with the diagnosis of sarcoidosis or necrotizing sarcoid granulomata in either skin, lymph node, lung, synovium, bone, bone marrow, liver, trachea, or sclera. Arteriography showed features of large vessel vasculitis in three patients, all of whom were African American, whereas patients with small vessel vasculitis were white. Prior reports of sarcoid and vasculitis included 14 adults, of whom half had predominantly small vessel disease, and half had medium- or large-sized vessel disease. Eight previously reported children included seven with primarily large vessel sarcoid vasculitis. Racial background was noted in 15 reported cases and included whites (6), African Americans (5), and Asians (4). Among the authors' six patients, four improved when treated with prednisone alone. However, relapses occurred when the drug was tapered or withdrawn. CONCLUSIONS: Sarcoidosis may be complicated by systemic vasculitis that can affect small- to large-caliber vessels. Sarcoid vasculitis can mimic hypersensitivity vasculitis, polyarteritis nodosa, microscopic polyangiitis, or Takayasu's arteritis. African American and Asian patients are disproportionately represented among cases with large vessel involvement. Corticosteroid and cytotoxic therapy is palliative for all forms of sarcoid vasculitis. However, relapses and morbidity from disease and treatment is common.     

系统性血管炎的分类

系统性血管炎是一组以血管炎症为特征的全身性自身免疫性疾病,其临床表现和病理特征复杂多变,取决于受 累血管的部位和类型。根据主要受累血管的类型,2012年Chapel Hill共识会议（CHCC）对多种血管炎进行了命名和 定义,是目前使用最为广泛的血管炎命名系统。文章详细阐述了系统性血管炎的分类,并对系统性血管炎的定义、病 因、流行病学和诊断进行了概述。     

Necrotizing systemic vasculitis with features of both Wegener's granulomatosis and Churg-Strauss vasculitis

We describe a patient who had nasal biopsy-demonstrated eosinophilic vasculitis and renal biopsy-demonstrated necrotizing glomerulonephritis with tissue eosinophilia. Despite corticosteroid therapy, the patient's renal function deteriorated, and nodular pulmonary infiltrates developed. Both conditions responded dramatically when cyclophosphamide was added to the treatment regimen. The renal disease activity was monitored with the aid of cytodiagnostic urinalysis, a technique of limited, albeit well-established, validity in monitoring renal allograft patients for signs of tissue rejection. This technique provided an improved, semi-quantitative method for examining urine sediment and, in this patient, was helpful as a measure of renal disease activity.     

Epidemiology of systemic vasculitis

The systemic vasculitides are heterogeneous conditions of unknown etiology characterized by inflammation and necrosis of different sized blood vessels. Wegener’s Granulomatosis, microscopic polyangiitis, and Churg Strauss syndrome are associated with anti-neutrophil cytoplasmic antibodies and affect small and medium blood vessels. They are very rare in childhood and peak in the 65 to 70 year old age group. Wegener’s Granulomatosis appears to be more common in the North of Europe compared with the South. All are more common in whites compared with other populations. Genetic and environmental factors, including infection, drugs, and silica, are important in etiology. Giant cell arteritis is predominantly a disease of whites over the age of 50. It appears more common in individuals with Nordic descent. Incidence may be increasing over time and cyclical variation in disease may reflect an infectious etiology. Takayasu arteritis is a disease of the aorta and its branches, however pulmonary and cardiac arteries may be involved. Patients are usually under 40-years of age at presentation and there are no apparent differences in incidence or clinical characteristics/aortic involvement across the globe. Kawasaki disease (KD) and Henoch-Schonlein purpura are diseases of children and rarely affect adults. Both have been reported to be more common in Asians than whites. The incidence of KD is higher in Japan and China compared with other regions. No definite trigger factors have been found, but KD has been linked to infection, house dust mite and chemicals, and Henoch-Schonlein purpura to a pesticide and drugs.     

Nephrogenic systemic fibrosis in a patient with a p-ANCA systemic vasculitis

Nephrogenic systemic fibrosis (NSF) is a fibrosing skin condition of unknown origin. Most cases have been described in patients with acute or chronic renal failure. The cutaneous changes include firm and thickened, indurate skin plaques and papules on the extremities and trunk. Histopathology typically shows an increase in dermal fibroblast-like cells associated with mucin deposition. Previous exposition to gadolinium-based contrast agents was closely associated with its onset. We described a patient with the clinical and pathologic picture of NSF presented after an acute renal failure in the course of a perinuclear antineutrophil cytoplasmic antibodies associated systemic vasculitis.     

Treatment of primary systemic vasculitis with TNF alpha-antagonists

After the introduction of the TNF alpha blocking drugs Etanercept and Infliximab for the standard therapy of rheumatoid arthritis these effective substances have also been used successfully in many patients with primary systemic vasculitides, who were unresponsive to standard therapy. From pathophysiologic findings their use is justified by the prominent role of TNF alpha in the inflammation of small and large vessels. So far only open studies with a maximum of 20 patients and case reports are published. In summary there are reports of the successful treatment of 7 patients with rheumatoid vasculitis, 39 patients with Wegener's granulomatosis, 3 patients each with microscopic Polyangiitis, and 5 patients each with temporal Arteritis or Takayasu disease with Etanercept as well as with Infliximab. In addition, Infliximab was also used with a good response in severe Polymyalgia rheumatica in 4 cases and in one case of a cryoglobulinemic vasculitis. More than 60 patients with Panuveitis and other manifestations of Beh?et's disease were treated with Infliximab or Etanercept according to preliminary reports. Because of the overall positive reports with TNF alpha collected in this review controlled investigations for their use in primary systemic vasculitis are necessary.     

Infliximab in a child with therapy-resistant systemic vasculitis

Treatment of systemic vasculitides is usually based on the use of corticosteroids and other immunosuppressive drugs. We describe a 10-year-old girl with systemic vasculitis resistant to immunosuppressive treatment who had a rapid and impressive response to treatment with infliximab.