Churg-Strauss syndrome overview: Difference between revisions
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==Classification== | ==Classification== | ||
Revised International Chapel Hill Consensus Conference 2012 on nomenclature of vasculitides, defines [[eosinophilic granulomatosis with polyangiitis]] (formerly known as Churg - Strauss syndrome) as an [[eosinophilic]], [[Inflammation|granulomatous inflammatory disease]] affecting most commonly the [[Respiratory tract|conducting pulmonary airways]], and leading to a [[necrosis]] of the small and/or medium sized vessels. [[Eosinophilic granulomatosis with polyangiitis]] is often synonymous with adult-onset [[asthma]]. According to revised CHCC 2012, [[eosinophilic granulomatosis with polyangiitis]] is considered as a variant of the [[Anti-neutrophil cytoplasmic antibody|ANCA]] - associated vasculitis. | |||
==Pathophysiology== | ==Pathophysiology== | ||
Revision as of 16:04, 2 April 2018
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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
Revised International Chapel Hill Consensus Conference 2012 on nomenclature of vasculitides, defines eosinophilic granulomatosis with polyangiitis (formerly known as Churg - Strauss syndrome) as an eosinophilic, granulomatous inflammatory disease affecting most commonly the conducting pulmonary airways, and leading to a necrosis of the small and/or medium sized vessels. Eosinophilic granulomatosis with polyangiitis is often synonymous with adult-onset asthma. According to revised CHCC 2012, eosinophilic granulomatosis with polyangiitis is considered as a variant of the ANCA - associated vasculitis.
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.