1/89. pulmonary eosinophilia associated with montelukast.Antileukotriene drugs are new therapeutic agents that have recently been approved for the treatment of asthma. Several cases of eosinophilic conditions including churg-strauss syndrome have been reported to be associated with zafirlukast, a cysteinyl leukotriene type 1 receptor antagonist. So far no other leukotriene modifier has been associated with the syndrome. The case history is presented of a man with allergic rhinitis and asthma who had received intermittent pulse therapy with oral corticosteroids. pulmonary eosinophilia developed while he was receiving treatment with montelukast, a chemically distinct cysteinyl leukotriene type 1 receptor antagonist. After discontinuation of montelukast therapy and administration of systemic corticosteroids the patient's symptoms reversed rapidly and there was prompt resolution of the pulmonary infiltrates. We believe that cysteinyl leukotriene type 1 receptor antagonists are safe and effective drugs for most patients with asthma but caution is needed for those with more severe disease who require systemic corticosteroids, especially if they show characteristics of the atypical allergic diathesis seen in the prodromal phase of churg-strauss syndrome.- - - - - - - - - - ranking = 1keywords = rate (Clic here for more details about this article) |
2/89. Cutaneous extravascular necrotizing granuloma (Churg Strauss granuloma).Churg Strauss granuloma (cutaneous extravascular necrotizing granuloma) is a distinct entity which is associated with systemic immunoreactive or autoimmune diseases in a majority of cases. Typically, Churg Strauss granuloma presents as symmetrical papules or nodules on the extremities. There are two histological patterns: the classic pattern reveals palisading granuloma with central degenerated collagen, interspersed polymorphonuclear leukocytes, and leukocytoclastic debris; the focal basophilic necrosis pattern does not show palisading granuloma. We report two cases of Churg Strauss granuloma with different histopathological patterns.- - - - - - - - - - ranking = 1keywords = rate (Clic here for more details about this article) |
3/89. 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 = 4.6861846687159keywords = death (Clic here for more details about this article) |
4/89. 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 = 5keywords = rate (Clic here for more details about this article) |
5/89. 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 = 4keywords = rate (Clic here for more details about this article) |
6/89. 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 = 2keywords = rate (Clic here for more details about this article) |
7/89. churg-strauss syndrome in patients receiving montelukast as treatment for asthma.STUDY OBJECTIVES: We previously reported eight patients who developed churg-strauss syndrome in association with zafirlukast treatment for asthma and postulated that the syndrome resulted from unmasking of a previously existing condition due to corticosteroid withdrawal and not from a direct drug effect. The availability of montelukast, a new leukotriene receptor antagonist with a different molecular structure, permitted us to test this hypothesis. Our goals were to ascertain whether the churg-strauss syndrome developed in patients taking montelukast and other novel asthma medications, and to describe potential mechanisms for the syndrome. DESIGN: Case series. SETTING: Outpatient and hospital practices of pulmonologists in the united states and belgium. patients: Four adults (one man, three women) who received montelukast as treatment for asthma; two women who received salmeterol/fluticasone therapy, but not montelukast. RESULTS: churg-strauss syndrome developed in the four asthmatic patients who received montelukast. In each case, there was a long history of difficult-to-control asthma characterized by multiple exacerbations that had required frequent courses of oral systemic corticosteroids or high doses of inhaled corticosteroids for control. Two other asthmatics who received fluticasone and salmeterol but not montelukast therapy developed the same syndrome with tapering doses of oral or high doses of inhaled corticosteroids. CONCLUSIONS: The occurrence of churg-strauss syndrome in asthmatic patients receiving leukotriene modifiers appears to be related to unmasking of an underlying vasculitic syndrome that is initially clinically recognized as moderate to severe asthma and treated with corticosteroids. Montelukast does not appear to directly cause the syndrome in these patients.- - - - - - - - - - ranking = 1keywords = rate (Clic here for more details about this article) |
8/89. churg-strauss syndrome complicated by eosinophilic endomyocarditis.A 34-year-old woman with asthma had increasing dyspnea on exertion for 9 months and new-onset mononeuritis multiplex. An examination demonstrated sinus tachycardia, elevated jugular venous pressure, and a tender nonpulsatile liver. The leukocyte count was 15.8 x 10(9)/L, with 23% eosinophils. echocardiography revealed a laminated thrombus obliterating much of the right ventricular cavity, with encasement of the tricuspid valve. Ultrafast computed tomography showed no evidence of pulmonary emboli. biopsy specimens of skin nodules revealed extravascular palisading granulomas. The thrombus was refractory to corticosteroids, and right ventricular thrombectomy was performed. To our knowledge, this is the third reported case of churg-strauss syndrome with thrombotic complications from coexistent eosinophilic endomyocarditis. In an asthmatic patient with chronic dyspnea, eosinophilic tissue infiltration, and neuropathy, churg-strauss syndrome should be considered; evaluation for cardiac involvement may be warranted.- - - - - - - - - - ranking = 1keywords = rate (Clic here for more details about this article) |
9/89. Severe polyneuropathy in a patient with churg-strauss syndrome.We describe the clinicopathologic features of a 56-year-old woman affected with churg-strauss syndrome with major peripheral nerve involvement. The patient presented with a 1-month history of mainly distal upper-limb symmetrical paresthesias and hypostenia (bilateral "wrist drop"), palpable purpura and eosinophilia. Multiple pulmonary infiltrates and asthma had been present since the age of 52. skin biopsy demonstrated an eosinophilic necrotizing vasculitis. During the hospitalization she was submitted to cardiac, bronchopulmonary, renal, and gastrointestinal evaluation and EMG. Peripheral nerve and skeletal muscle biopsies were performed. sural nerve biopsy showed a marked degree of demyelination. A perivascular cellular infiltrate within the epineurium was immunoreactive for T lymphocytes and macrophages. Strong HLA-DR immunostaining was present in the endoneurium. IgM, IgE and fibrinogen deposition was found in some epi- and endoneurial vessels. Muscle biopsy showed neurogenic changes and 1 thrombosed vessel surrounded by mononuclear cells. Membrane attack complex (MAC) deposition was present in a few capillaries and major histocompatibility complex products I (MHCP I) was expressed at the subsarcolemmal level in a few isolated perivascular muscle fibers. After immunosuppressive therapy, the patient showed progressive improvement of both clinical symptoms and neurophysiological parameters.- - - - - - - - - - ranking = 3keywords = rate (Clic here for more details about this article) |
10/89. churg-strauss syndrome with perforating ulcers of the colon.We report a case of a 72-year-old woman with churg-strauss syndrome, who presented with intestinal perforation. She has had bronchial asthma with peripheral blood eosinophilia for 30 years. Gross findings of a resected colon showed multiple ulcers with perforation. Histologic findings demonstrated transmural inflammation infiltrated with large numbers of eosionophils, neutrophils and lymphoplasma cells, and characteristic extravascular granuloma in the subserosa. There were multifocally-distributed transmural vasculitis showing all stages of activity in medium and small-sized arteries and veins located in the submucosa, and proper muscle and subserosal layers of the colon, some of which revealed granulomatous inflammation. Histologic finding of liver showed chronic viral hepatitis B with mild inflammatory activity and macronodular cirrhosis. Immunohistochemical findings, acid fuschin orange G staining and electromicroscope found no evidence of hepatitis b virus infection contributing to the pathogenesis of this lesion.- - - - - - - - - - ranking = 2keywords = rate (Clic here for more details about this article) |
| | Next -> |