1/152. Marked improvement of Churg-Strauss vasculitis with intravenous gammaglobulins.Churg-Strauss vasculitis (CSV) is a systemic vasculitis usually treated with steroids and cytotoxic drugs. Herein, we describe the beneficial effect of intravenous immune globulin (IVIG) in a 50-year-old man with CSV. The IVIG therapy brought a marked clinical improvement, with impressive decline in the titers of antineutrophil cytoplasmic autoantibody.- - - - - - - - - - ranking = 1keywords = vasculitis (Clic here for more details about this article) |
2/152. Childhood churg-strauss syndrome.churg-strauss syndrome or allergic granulomatosis and angiitis is a vasculitis that is found in adults, but is extremely rare in children. We describe a 14-year-old boy who presented with prolonged fever, weight loss, sinusitis, myalgia and arthralgia, testicular pain, pulmonary infiltrations, pericardial effusion, peripheral neuropathy, and eosinophilia. Muscle biopsy showed necrotizing arteritis with eosinophil infiltration. His clinical course was complicated by several seizures secondary to cerebral vasculitis and severe asthma, resulting in death. The clinical features and outcomes of childhood churg-strauss syndrome are reviewed.- - - - - - - - - - ranking = 9.8561564187221keywords = angiitis, vasculitis (Clic here for more details about this article) |
3/152. IgA glomerulonephritis associated with microscopic polyangiitis or Churg-Strauss syndrome.When renal insufficiency occurs in classical antineutrophil cytoplasm antibody- (ANCA) associated vasculitides, histological examination usually finds pauci-immune focal segmental glomerulonephritis. We report on 2 cases of histologically proven necrotizing vasculitis associated with IgA nephropathy. Concomitant vasculitis and IgA nephropathy has only rarely been reported but this joint occurrence may not be coincidental as its pathophysiology is not known. Among vasculitides, IgA nephropathy has more frequently been associated with Henoch-Schoenlein purpura: one microscopic polyangiitis unusual because the patient simultaneously presented ANCA and microaneurysms, and the other churg-strauss syndrome associated with mild renal insufficiency. This uncommon association might represent a possible overlap syndrome between these ANCA-associated vasculitides and IgA nephropathy or simply a new type of glomerulonephritis that must be taken into account in these vasculitides.- - - - - - - - - - ranking = 47.947448760277keywords = angiitis, vasculitis (Clic here for more details about this article) |
4/152. Leukotriene modifiers and churg-strauss syndrome: adverse effect or response to corticosteroid withdrawal?Zafirlukast, montelukast and pranlukast are all cysteinyl leukotriene receptor antagonists that have recently been approved for the treatment of asthma. Within 6 months of zafirlukast being made available on the market, 8 patients who received the agent for moderate to severe asthma developed eosinophilia, pulmonary infiltrates, cardiomyopathy and other signs of vasculitis; the syndrome that these patients developed was characteristic of the churg-strauss syndrome. All of the patients had discontinued systemic corticosteroid use within 3 months of presentation and all developed the syndrome within 4 months of zafirlukast initiation. The syndrome dramatically improved in each patient upon reinitiation of corticosteroid therapy. Since the initial report, there have been multiple similar cases reported to the relevant pharmaceutical companies and to federal drug regulatory agencies in association with zafirlukast as well as with pranlukast, montelukast, and with use of high doses of inhaled corticosteroids, thus leading to an increased incidence rate of the churg-strauss syndrome. Many potential mechanisms for the association between these drugs and the churg-strauss syndrome have been postulated including: increased syndrome reporting due to bias; potential for allergic drug reaction; and leukotriene imbalance resulting from leukotriene receptor blockade. However, careful analysis of all reported cases suggests that the churg-strauss syndrome develops primarily in those patients taking these asthma medications who had an underlying eosinophilic disorder that was being masked by corticosteroid treatment and unmasked by novel asthma medication-mediated corticosteroid withdrawal, similar to the forme fruste of the churg-strauss syndrome. It remains unclear what the exact mechanism for this syndrome is and whether this represents an absolute increase in cases of vasculitis, but it appears that none of the asthma medications implicated in leading to the development of churg-strauss syndrome was directly causative of the syndrome. These agents remain well tolerated and effective medications for the treatment of asthma, although physicians must be wary for the signs and symptoms of the churg-strauss syndrome, particularly in patients with moderate to severe asthma in whom corticosteroids are tapered.- - - - - - - - - - ranking = 0.33333333333333keywords = vasculitis (Clic here for more details about this article) |
5/152. Anti-adhesion molecule therapy as an interventional strategy for autoimmune inflammation.Functional inactivation of leukocyte adhesion molecules has been used to intervene in the development of tissue injury in experimental models of postperfusion infarction as well as autoimmune inflammation. We investigated the use of humanized monoclonal antibodies (mAb) against CD18 in the treatment of five patients with vasculitic tissue injury sufficient to threaten infarction or gangrene. The treatment was monitored in three ways: (i) whole-body gamma camera scintiscanning of autologous indium-labeled PMN, (ii) an index of the therapeutic inhibition of adhesion derived from comparison pre, during, and post mAb treatment of the ability of patients' PMN to be aggregated after activation by fMLP, and (iii) flow cytometric analysis of PMN CD18 expression. Four of five patients given anti-CD18 at 20 mg/day for up to 3 weeks showed prompt clinical improvement, with healing of the ulceration and restoration of limb function within 4 weeks, which was sustained. The fifth patient, who was not doing well clinically, decided to withdraw from all active treatment: at autopsy there was no evidence of the underlying vasculitis evident pretreatment. Our findings suggest that anti-adhesion molecule treatment might be an effective immediate treatment in severe vasculitis especially when tissue viability is threatened by progressive infarction and/or development of gangrene.- - - - - - - - - - ranking = 0.33333333333333keywords = vasculitis (Clic here for more details about this article) |
6/152. Seven cases of complete and incomplete forms of churg-strauss syndrome not related to leukotriene receptor antagonists.BACKGROUND: Various forms of churg-strauss syndrome have been reported in association with the use of leukotriene receptor antagonists in asthmatic patients. OBJECTIVE: Our purpose was to increase awareness that different forms of the churg-strauss syndrome occur in patients not receiving leukotriene modifiers. methods: We searched for all the cases of churg-strauss syndrome that were seen in the University of Rochester Medical Center, new york, in the past 4 years. RESULTS: We identified 7 patients, 6 of whom fulfilled the American College of rheumatology criteria for the classification of churg-strauss syndrome. None of them used leukotriene receptor antagonists. All had asthma and sinus disease. The duration and severity of their asthma varied considerably. In the majority of the patients the features of churg-strauss syndrome became obvious as the systemic corticosteroid dose was being tapered or discontinued, although 3 patients had not been receiving maintenance oral corticosteroids at disease onset. Three patients had positive antineutrophil cytoplasmic antibodies test result (perinuclear pattern). There was histologic documentation of vasculitis in 4 patients. Five of 7 patients responded to high-dose corticosteroid treatment. CONCLUSION: Our 7 cases are similar to the various forms of churg-strauss syndrome that have been reported in association with the leukotriene receptor antagonists. Complete or incomplete forms of this syndrome can become apparent in asthmatic patients as systemic corticosteroids are being tapered but can also occur in patients with mild asthma of short duration who use only inhaled corticosteroids.- - - - - - - - - - ranking = 0.16666666666667keywords = vasculitis (Clic here for more details about this article) |
7/152. Cardiac involvement and left ventricular failure in a patient with the churg-strauss syndrome.The churg-strauss syndrome is characterised by a history of asthma and paranasal sinus disease, eosinophilia of more than 10 per cent, non-fixed pulmonary infiltrates on chest radiography and vasculitis which may affect multiple organ systems. The condition usually manifests in the 4th decade. We present a 21-year old female with a history of asthma since one year of age who developed symptoms and signs of pneumonia, a pulmonary infiltrate on chest radiography and eosinophilia. This was followed a few weeks later by vasculitis which affected the skin and myocardium and associated with a peripheral eosinophilia of more than 80%. physical examination revealed palpable purpura and signs of left ventricular failure. echocardiography confirmed significant diminution of left ventricular contractility. A rapid improvement was observed after steroid therapy. echocardiography after two months showed normal left ventricular function. In this presentation we review the cardiac manifestations of the churg-strauss syndrome and its management.- - - - - - - - - - ranking = 0.33333333333333keywords = vasculitis (Clic here for more details about this article) |
8/152. hepatitis c virus infection with and without cryoglobulinemia as a case of churg-strauss syndrome.Chronic hepatitis c virus (HCV) infection may be associated with numerous immune disorders, with vasculitis including polyarteritis nodosa, or with both. cryoglobulinemia, which is often present, can also be expressed by vasculitis. We describe 2 cases of churg-strauss syndrome (CSS) in patients with HCV infection. We found no previous case of CSS accompanying HCV infection in the literature. The current patients were women aged 40 and 66 years. In both cases, a clinical and laboratory pattern suggesting CSS was found before the HCV infection was discovered. One patient had cryoglobulinemia. One patient was successfully treated with interferon (IFN). The other was treated for 18 months with IFN and corticosteroids. Second-line therapy consisting of IFN with ribavirin was successful. The emergence of HCV infection may have led to an induced form of CSS. The relationship among HCV, cryoglobulinemia, and CSS is not clear, but may be similar to that existing between polyarteritis nodosa and hepatitis b virus. These observations suggest that IFN-alpha therapy may be effective against CSS in HCV infected patients with or without cryoglobulinemia.- - - - - - - - - - ranking = 0.33333333333333keywords = vasculitis (Clic here for more details about this article) |
9/152. Marginal infiltrative ulcerative keratitis secondary to churg-strauss syndrome: a case report.PURPOSE: To describe a patient with keratitis associated with churg-strauss syndrome. methods: A 44-year-old lady with known churg-strauss syndrome was examined with marginal infiltrative ulcerative keratitis. RESULTS: Autoimmune disorders with systemic vasculitis are a major cause of marginal keratitis, and in this patient, the diagnosis of Churg-Strauss was confirmed clinically and pathologically. CONCLUSIONS: churg-strauss syndrome should be considered in the differential diagnosis of marginal keratitis.- - - - - - - - - - ranking = 0.16666666666667keywords = vasculitis (Clic here for more details about this article) |
10/152. Inhaled corticosteroids and churg-strauss syndrome: a report of five cases.churg-strauss syndrome is an eosinophil-associated, small vessel granulomatous vasculitis, characterized by late onset asthma, upper airways disease, eosinophilia, and clinical manifestations of systemic vasculitis. Several cases of churg-strauss syndrome have been recognized in patients treated with cysteinyl leukotriene-receptor antagonists and weaned off systemic corticosteroids. These cases have led to a general warning on the possible development of Churg-Strauss syndrome after taking cysteinyl leukotriene-receptor antagonists. The authors report five cases of churg-strauss syndrome in severe steroid dependent asthmatics in whom inhaled corticosteroids allowed systemic corticosteroid withdrawal. It is concluded that physicians should monitor patients carefully when severe asthma is controlled with any substance allowing withdrawal from (or even avoidance) of systemic corticosteroids. case-control studies should identify more precisely the risk factors of churg-strauss syndrome.- - - - - - - - - - ranking = 0.33333333333333keywords = vasculitis (Clic here for more details about this article) |
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