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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 ...
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]
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. [13 ...
The additional cells are sometimes a clue to the cause of the granuloma. For example, granulomas with numerous eosinophils may be a clue to coccidioidomycosis or allergic bronchopulmonary fungal disease, and granulomas with numerous neutrophils suggest blastomycosis, granulomatosis with polyangiitis, aspiration pneumonia, or cat-scratch disease.
Granulomatosis with polyangiitis: Crusting rhinorrhea, sinusitis, chronic otitis media, nasal obstruction, shortness of breath, and chronic cough. [15] [16] Eosinophilic granulomatosis with polyangiitis: Asthma, allergic rhinitis, sinusitis, nasal polyps, peripheral neuropathy, pulmonary infiltrates, and abdominal pain. [17] [18]
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The term polyangiitis overlap syndrome refers to a systemic vasculitis that shares features with two or more distinct vasculitis syndromes. The most common type of polyangiitis overlap syndrome is microscopic polyangiitis (MPA), which shares features with EGPA, granulomatosis with polyangiitis and panarteritis nodosa .