1/21. 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 = vessel (Clic here for more details about this article) |
2/21. 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 = 2keywords = vessel (Clic here for more details about this article) |
3/21. Antigen inhalation as a triggering factor in systemic small-sized-vessel vasculitis. Four cases.In order to identify small-sized-vessel vasculitis occurring immediately after massive inhalation of particles which could be considered antigenic, we performed a retrospective review of patients who consulted our Department of internal medicine between 1980 and 1998 and were diagnosed as having small-vessel vasculitis that developed immediately after massive exposure to inhaled particles. Four patients, 27 to 55 years old, presented with small-sized-vessel vasculitis (one with Wegener's granulomatosis and three with churg-strauss syndrome) which developed after massive inhalation of particles. Clinical manifestations occurred from a few hours to 10 days after inhalation of diesel fumes, cereal dust, flour or cereal dust and/or pigeon droppings. Three patients remain disease-free 8, 9 and 18 years after discontinuing treatment. One patient remains well after 3 years on low-dose corticosteroids and cyclophosphamide therapy. Our data suggest that primary antigens can cause small-sized-vessel vasculitides. Identifying such etiological factors could improve our understanding of the pathogenesis of systemic vasculitides and prevent relapses due to similar antigenic exposure.- - - - - - - - - - ranking = 8keywords = vessel (Clic here for more details about this article) |
4/21. Pulmonary vasculitis may obscure large cell lung carcinoma. A case report.Several vasculitic syndromes are recognized as paraneoplastic syndromes of an underlying malignant disease. Most frequently small vessel vasculitis of the skin has been reported. We describe the case of a 62-year-old man with a pulmonary mass due to pulmonary vasculitis. After resection of the pulmonary mass, the patient displayed bone metastasis. Retrospectively, tumor cells were found in the pulmonary mass that had been resected 9 months before. In this case report the rare association of vasculitis and lung carcinoma is reviewed. Our report indicates that pulmonary vasculitis may obscure the histologic findings of lung carcinoma and that in patients with localized pulmonary vasculitis special attention has to be paid to the possible presence of malignant cells.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/21. asthma therapies and churg-strauss syndrome.The pulmonary vasculitides are a group of rare but serious disorders that require early recognition, accurate diagnosis, and effective therapy. churg-strauss syndrome (CSS) is classified as small vessel vasculitis. Four different definitions for the diagnosis of CSS have been developed: (1) the pathologic criteria put forth by Churg and Strauss, (2) the criteria based on clinical grounds from Lanham and colleagues, (3) the criteria based on clinical grounds from the American College of rheumatology, and (4) the criteria from the Chapel Hill consensus Conference, which closely concur with the Churg and Strauss definition. It is apparent that cessation, diminution, or even a switch from low-dose systemic to inhaled corticosteroid therapy can precipitate the appearance of CSS. The term forme fruste has been used to indicate that the signs and symptoms of CSS were (inadvertently) suppressed by cortico-steroids. The clinical risk factors for CSS are moderately severe or severe asthma, chronic sinusitis, or reductions in systemic corticosteroid therapy. Differential diagnosis, treatment, and ongoing monitoring of CSS therapeutic responses are reviewed. The introduction of leukotriene modifiers and high-potency inhaled corticosteroids have allowed control of asthma symptoms, which results in avoidance or reduction in oral corticosteroid use. The advent of these agents has been associated with reports of CSS appearing in patients with asthma. The available data regarding the association of CSS and antiasthma agents are most consistent with the unmasking of a previously contained pathologic condition (forme-fruste CSS) or disease that progresses because systemic corticosteroids were avoided. Early recognition and immunosuppressive therapy are the keystones of successful treatment of this rare disorder.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/21. Delayed relapse of churg-strauss syndrome manifesting as colon ulcers with mucosal granulomas: 3 cases.churg-strauss syndrome (CSS) is characterized by small vessel vasculitis and extravascular granulomas. The American College of rheumatology classification criteria for CSS include asthma, eosinophili, and clinical manifestation of vasculitis. Gastrointestinal (GI) manifestations occur in 30% of patients, but are inaugural in only 16%. They denote vasculitis of the stomach and small bowel wall, and consist in protean, nonspecific pain. GI involvement is of adverse prognostic significance in CSS. ulcer formation in the GI tract mucosa is a rarer manifestation, usually discovered upon laparotomy or autopsy. We describe 3 new cases of colonic ulcers in CSS. Unusual features were diagnosis of the ulcers during a delayed relapse and presence of eosinophilic granulomas within the mucosa.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/21. churg-strauss syndrome presenting as spontaneous subarachnoid haemorrhage.churg-strauss syndrome (CSS) is a systemic small-vessel vasculitis characterised by the presence of asthma and eosinophilia. central nervous system involvement (cerebral infarctions or intracerebral haemorrhage) is rare in CSS. Spontaneous subarachnoid hemorrhage (SAH) has been described in other systemic vasculitides. SAH is exceptional in CSS. We present a 47-year-old woman with CSS presenting as a spontaneous SAH with cerebral angiography findings consistent with vasculitis of the basilar artery and without aneurysms or arteriovenous malformations. She received treatment with prednisone and cyclophosphamide, and 2 months later the basilar artery was normal on magnetic resonance angiography.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/21. Atypical presentation of churg-strauss syndrome: another "forme fruste" of the disease?Vasculitis is a clinicopathologic process characterized by inflammation and damage to blood vessels. A broad and heterogenous group of syndromes may result from this process, because any type, size, and location of blood vessel may be involved. The cause of these conditions remains unclear, but an autoimmune inflammatory process, characterized by involvement of both neutrophils and endothelial cells, seems to play an important role. In 1951, Churg and Strauss described a clinical syndrome of severe asthma, hypereosinophilia with eosinophilic infiltrates, eosinophilic vasculitis, and granulomata in various organs. asthma may precede this vasculitis by many years. We report a case of anti-neutrophil cytoplasmic antibody-positive, pauci-immune, crescentic, necrotizing glomerulonephritis with peripheral and interstitial eosinophilia but without asthma. This is very unusual in churg-strauss syndrome.- - - - - - - - - - ranking = 12.748569621607keywords = blood vessel, vessel (Clic here for more details about this article) |
9/21. Familial vasculitides: churg-strauss syndrome and Wegener's granulomatosis in 2 first-degree relatives.churg-strauss syndrome (CSS) and Wegener's granulomatosis (WG) are uncommon primary vasculitides, characterized by the involvement of the small to medium size vessels and by the frequent presence of serum antineutrophil cytoplasmic antibodies (ANCA). The pathogenesis of ANCA associated vasculitides is unclear, but roles for both genetic and environmental factors have been suggested. Familial cases of WG, but not CSS, have been reported. We describe the occurrence of CSS in a man and, 5 years later, WG in his son. These patients live together in an urban area of Northern italy and share the HLA haplotype A*03; B*07; C*w07; DRB 1*0404, DQB 1*0302. To our knowledge, this is the first report of the familial clustering of CSS and WG in first-degree relatives.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/21. Fluticasone-associated cutaneous allergic granulomatous vasculitis.Allergic granulomatous vasculitis, or churg-strauss syndrome, is a small-vessel, multisystem vasculitis that can affect the skin, lungs, heart, and nervous system. Recent reports have implicated leukotriene receptor antagonists and inhaled corticosteroids in the development of this rare syndrome. We present a patient with no history of allergic asthma who acutely developed skin-limited Churg-Strauss-like granulomatous vasculitis after initiating therapy with inhaled fluticasone and salmeterol (Advair Diskus).- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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