Churg-Strauss Syndrome

Churg Strauss syndrome (CSS) was first defined in 1951 as an allergic granulomatosis affecting small and medium-sized vessels The American College of Rheumatology established six criteria for its classification in 1990: asthma, peripheral eosinophilia, sinus involvement, pulmonary infiltrates, mononeuritis multiplex, and histology compatible with vasculitis The presence of 4 or more criteria yielded a sensitivity of 85% and a specificity of 99.7%. Before that, three criteria—asthma, peripheral eosinophilia, and systemic vasculitis—were needed Histological lesions include small-vessel angiitis and extravascular necrotizing granulomas, usually containing eosinophilic infiltrates Vasculitis may be granulomatous or nongranulomatous and typically involves both arteries and veins in pulmonary and systemic vessels. The granulomas have a center formed by necrotic eosinophils surrounded by palisading histiocytes and multinucleated giant cells. They are not present in all patients and are seen in other diseases. There are three partially-overlapping phases in CSS: 1) a prodromal phase with allergic rhinitis, nasal polyps, and/or sinusitis, followed by asthma; 2) an eosinophilic phase with peripheral blood eosinophilia and eosinophilic infiltration of organs, and 3) a vasculitic phase. GI and hepatic manifestations of CSS using the 75 articles in the English literature from 1960 to 2011 found using a Medline search, Google scholar, and the references in the articles obtained. The emphasis is on recent literature, particularly studies fulfilling the 1990 criteria for diagnosis, although the description of cases is not always adequate to make this determination.

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Last date updated on April, 2024