Churg-Strauss syndrome overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Churg-Strauss syndrome is a medium and small vessel autoimmune vasculitis, leading to necrosis. It involves mainly the blood vessels of the lungs (it begins as a severe type of asthma), gastrointestinal system, and peripheral nerves, but also affects the heart, skin and kidneys. It is a rare disease that is non-heritable, non-transmissable and often mis-diagnosed.
Historical Perspective
Classification
Pathophysiology
Eosinophilic granulomatosis with polyangiitis is a medium and small vessel vasculitis, leading to necrosis. The pathogenesis of Eosinophilic granulomatosis with polyangiitis is not fully understood. Eosinophilic granulomatosis with polyangiitis occurs as a result of a complex interaction involving genetic and environmental factors that lead to an inflammatory response involving eosinophils, lymphocytes. Autoimmunity has an evident role in the presence of ANCA, hypergammaglobulinemia, elevated levels of immunoglobulin E, and rheumatic factor in the pathogenesis. HLA-DRB4 is correlated with increased risk of development of vascular manifestations of the churg-strauss syndrome. On microscopic pathology, eosinophilic infiltration, necrotizing granulomas vasculitis and necrosis of small and medium-sized arteries can be seen.