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Granulomatosis with polyangiitis; Other names: Wegener's granulomatosis (WG) (formerly) Micrograph showing features characteristic of granulomatosis with polyangiitis – a vasculitis and granulomas with multi-nucleated giant cells. H&E stain. Specialty: Immunology, rheumatology Causes: Autoimmune disease
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 ...
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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]
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 .
Wegener's granulomatosis (now known as granulomatosis with polyangiitis) Wegmann–Jones–Smith syndrome; Weil syndrome; Weinstein–Kliman–Scully syndrome; Weismann–Netter–Stuhl syndrome; Weissenbacher–Zweymuller syndrome; Welander distal myopathy, Swedish type; Weleber–Hecht–Bigley syndrome; Wellesley–Carmen–French syndrome
Eosinophilic granulomatosis with polyangiitis consists of three stages, but not all patients develop all three stages or progress from one stage to the next in the same order; [7] whereas some patients may develop severe or life-threatening complications such as gastrointestinal involvement and heart disease, some patients are only mildly affected, e.g. with skin lesions and nasal polyps. [8]
An example is Wegener's Granulomatosis; it was renamed granulomatosis with polyangiitis when Dr. Wegener was found to have had Nazi ties. Its referent varies by country (e.g., sideropenic dysphagia is Plummer–Vinson syndrome in the US and Australia, Patterson–Kelly syndrome in the UK, and Waldenstrom–Kjellberg syndrome in Scandinavia).