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Granulomatosis with polyangiitis is part of a larger group of vasculitic syndromes called systemic vasculitides or necrotizing vasculopathies, all of which feature an autoimmune attack by an abnormal type of circulating antibody termed ANCAs (antineutrophil cytoplasmic antibodies) against small and medium-size blood vessels.
Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as allergic granulomatosis, [3] [4] is an extremely rare autoimmune condition that causes inflammation of small and medium-sized blood vessels in persons with a history of airway allergic hypersensitivity . [5] It usually manifests in three stages.
Friedrich Wegener (7 April 1907, Varel – 9 July 1990, Lübeck, [veːɡɐnəɐ̯]) was a German pathologist who is notable for his description of a rare disease originally referred to Wegener disease and now referred to as granulomatosis with polyangiitis. Although this disease was known before Wegener's description, from the 1950s onwards it ...
The negative immunofluorescence pattern, however, is called "pauci-immune" and is often associated with systemic vasculitides (plural of vasculitis) including: microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (EGPA), and granulomatosis with polyangiitis (GPA).
I became sick with a rare disease called granulomatosis with polyangiitis at the age of 14, and from that point on, much of my life involved hearing what I couldn’t do. That’s because when the ...
Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis (WG), is a rare immune-mediated systemic disease with an unclear etiology. It manifests pathologically as an inflammatory response pattern in the kidneys, upper and lower respiratory tracts , and granulomatous inflammation, which includes necrosis .
Microscopic polyangiitis is the most common cause of pulmonary-renal syndrome. [ citation needed ] Other causes include systemic lupus erythematosus , eosinophilic granulomatosis with polyangiitis , microscopic polyangiitis , dermatomyositis , polymyositis , mixed connective tissue disease , poststreptococcal glomerulonephritis , rheumatoid ...
Subsequent studies found that the cells infiltrating the midline tissues in cases of lethal midline granuloma that were not clearly diagnosed as granulomatosis with polyangiitis were: a) infected by the Epstein–Barr virus [2] and b) consisted of malignant lymphocytes, usually NK cells or, rarely, cytotoxic T cells. [3]