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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-sized 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. Friedrich Wegener (7 April 1907, Varel – 9 July 1990, Lübeck, [veːɡɐnəɐ̯]) was a German pathologist who is notable for being a high-ranking Nazi physician and for his description of a rare disease originally referred to Wegener disease and now referred to as granulomatosis with polyangiitis.
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).
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.
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]
Granulomatosis with polyangiitis, formerly known as Wegener's granulomatosis, 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 .
Saddle nose is a condition associated with nasal trauma, congenital syphilis, relapsing polychondritis, granulomatosis with polyangiitis, cocaine abuse, and leprosy, among other conditions. [1] The most common cause is nasal trauma. It is characterized by a loss of height of the nose, because of the collapse of the nasal bridge. The depressed ...