1/47. 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 = 1keywords = vasculitis (Clic here for more details about this article) |
2/47. 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 = 0.5keywords = vasculitis (Clic here for more details about this article) |
3/47. 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 = 1keywords = vasculitis (Clic here for more details about this article) |
4/47. 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 = 1keywords = vasculitis (Clic here for more details about this article) |
5/47. churg-strauss syndrome associated with montelukast therapy.churg-strauss syndrome is a rare form of eosinophilic vasculitis associated with asthma. There have been several recent case reports of the condition in association with leukotriene antagonists and it has been speculated that the churg-strauss syndrome was unmasked when oral corticosteroids were withdrawn. We report a case of churg-strauss syndrome associated with montelukast therapy in an asthmatic patient in whom there had been no recent oral corticosteroid use. We believe that this is the first such reported case and would suggest that clinicians need to be vigilant in all patients who develop systemic symptoms when starting treatment with leukotriene antagonists.- - - - - - - - - - ranking = 0.5keywords = vasculitis (Clic here for more details about this article) |
6/47. A variant form of churg-strauss syndrome: initial temporal non-giant cell arteritis followed by asthma--is this a distinct clinicopathologic entity?The clinical manifestations of the classical vasculitis syndromes are extraordinarily heterogenous with considerable overlap among them. Recently, several cases of unusual presentation of the vasculitis syndromes have been reported. We describe a patient who initially manifested with temporal arteritis and Raynaud's phenomenon and subsequently developed bronchial asthma, ie, a case of an atypical form of churg-strauss syndrome (allergic angiitis and granulomatosis) and discuss whether this case is a distinct clinicopathological entity.- - - - - - - - - - ranking = 19.025718875092keywords = angiitis, vasculitis (Clic here for more details about this article) |
7/47. Allergic granulomatous angiitis (churg-strauss syndrome) associated with allergic bronchopulmonary candidiasis.We describe a case of churg-strauss syndrome (CSS) associated with allergic bronchopulmonary candidiasis (ABPC). A 61-year-old man who had been given a diagnosis of ABPC based on serologic and radiographic findings experienced pain and purpuric rash on the left leg accompanied with motor weakness. The diagnosis of CSS was made from skin, nerve and muscle biopsies. Although immunosuppressant and prednisolone were administered and resulted in transient improvement, candidal pneumonia was suspected to have developed 60 days after the administration and the patient finally died of respiratory failure. To our knowledge, this is the first case of CSS associated with ABPC.- - - - - - - - - - ranking = 72.102875500367keywords = angiitis (Clic here for more details about this article) |
8/47. Acral purpuric plaques in a woman with asthma: a case of allergic granulomatosis angiitis.Allergic granulomatosis angiitis (AGA) is a rare systemic vasculitis of unknown etiology. Most patients are adults in their third to fourth decade of life. The combination of asthma, eosinophilia, and necrotizing vasculitis is almost invariably present. Cutaneous lesions are found in up to 70% of the patients and include nodules, hemorrhagic lesions, and erythema multiformelike lesions. We provide a case report of a 30-year-old woman with asthma who presented with acral purpuric plaques and was diagnosed with AGA.- - - - - - - - - - ranking = 91.128594375459keywords = angiitis, vasculitis (Clic here for more details about this article) |
9/47. Intravenous drug abuse mimicking vasculitis."Mimickers" of vasculitis are well-documented in the literature. We report a case of intravenous drug abuse manifesting with signs and symptoms suggestive of vasculitis. This case highlights the need for diagnostic precision in the evaluation of suspected vasculitis.- - - - - - - - - - ranking = 3.5keywords = vasculitis (Clic here for more details about this article) |
10/47. churg-strauss syndrome after reduction of inhaled corticosteroid in a patient treated with pranlukast for asthma.Recently, various forms of churg-strauss syndrome (CSS) have been reported in association with the use of leukotriene receptor antagonists. A 53-year-old woman with a 5-year history of asthma associated with chronic sinusitis presented mononeuropathy, hypereosinophilia, and positive P-ANCA in October 1999. She had been treated with pranlukast (450 mg/day) and beclomethasone dipropionate (BDP) at a dose of 1,200 microg/day which had gradually been tapered to 800 microg/day over the previous 17 months. She was found to have CSS, and 60 mg/day of prednisolone was administered instead of pranlukast, resulting in an improvement of her symptoms and eosinophilia. Later, we confirmed that serum P-ANCA had been positive before the pranlukast treatment, but CSS vasculitis had not appeared at that time. We speculated that an underlying incomplete form of CSS was being masked in this case and that the reduction of inhaled corticosteroid might have been responsible for the unmasking of CSS.- - - - - - - - - - ranking = 0.5keywords = vasculitis (Clic here for more details about this article) |
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