Granulomatosis with polyangiitis overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]
Overview
Granulomatosis with polyangiitis is a form of vasculitis that affects the lungs, kidneys and other organs. Due to its end-organ damage, it can be a serious disease that requires long-term immune suppression.[1] It is named after Dr. Friedrich Wegener, who described the disease in 1936.[2]
Granulomatosis with polyangiitis is part of a larger group of vasculitic syndromes, all of which feature the presence of an abnormal type of circulating antibody termed ANCAs (antineutrophil cytoplasmic antibodies) that affect small and medium-size blood vessels. Apart from Granulomatosis with polyangiitis, this category includes Churg-Strauss syndrome and microscopic polyangiitis.[1] Although Granulomatosis with polyangiitis affects small and medium-sized vessels,[3] it is formally classified as one of the small vessel vasculitides in the Chapel Hill system.[4]
References
- ↑ 1.0 1.1 Seo P, Stone JH. The antineutrophil cytoplasmic antibody-associated vasculitides. Am J Med 2004;117:39-50. PMID 15210387.
- ↑ Template:WhoNamedIt
- ↑ "Wegener's Granulomatosis: Vasculitis: Merck Manual Professional". Retrieved 2009-01-08.
- ↑ Silva, Fred; Jennette, J. Charles; Heptinstall, Robert H.; Olson, Jean T.; Schwartz, Melvin (2007). Hepinstall's pathology of the kidney. Hagerstwon, MD: Lippincott Williams & Wilkins. p. 677. ISBN 0-7817-4750-3.