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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 ...
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 .
LMG was thought to be a manifestation of three [1] or four [2] different diseases: the well-characterized disease of granulomatosis with polyangiitis, the ill-defined disorders of polymorphic reticulosis or mid-line malignant reticulosis, and an incompletely defined form of non-Hodgkin lymphoma.
The signs and symptoms of microscopic polyangiitis may resemble those of granulomatosis with polyangiitis (GPA) (another form of small-vessel vasculitis) but typically lacks the significant upper respiratory tract involvement (e.g., sinusitis) frequently seen in people affected by GPA. [citation needed]
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]
Life-threatening bleeding. Bleeding results in nearly one-third of deaths from traumatic injuries, which represent the top cause of death for people younger than 44 years in the U.S. A program ...
Pulmonary-renal syndromes are most commonly caused by an underlying autoimmune disease. PRS is most commonly due to ANCA-associated vasculitides (e.g., granulomatosis with polyangiitis) or due to anti-basement membrane diseases (e.g., Goodpasture's syndrome).