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Granulomatosis with polyangiitis (GPA), formerly referred to as Wegener's granulomatosis (WG), is a systemic disorder that involves both granulomatosis and polyangiitis. It is a form of vasculitis (inflammation of blood vessels) that affects small- and medium-size vessels in many organs. Damage to the lungs and kidneys can be fatal. It requires long-term immunosuppression. The condition was originally named for Friedrich Wegener, who described the disease in 1936. As a response to Wegener's association with the German Nazi party, professional bodies and journals have replaced his name with a descriptive name.〔 However, the older name is still often seen. 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. Apart from GPA, this category includes Churg–Strauss syndrome and microscopic polyangiitis.〔 Although GPA affects small- and medium-size vessels, it is formally classified as one of the small vessel vasculitides in the Chapel Hill system.〔Ottoman BAE: Strawberry gingivitis of Wegener’s granulomatosis: A clinicopathological and immunohistochemical study and review of literature.Contemporary Journal immunology: Vol. 2 No. 1 pp. 59-67)〕 ==Signs and symptoms== Initial signs are extremely variable, and diagnosis can be severely delayed due to the nonspecific nature of the symptoms. In general, rhinitis is the first sign in most people.〔〔 * Kidney: rapidly progressive glomerulonephritis (75%), leading to chronic kidney failure * Upper airway, eye and ear disease: * * Nose: pain, stuffiness, nosebleeds, rhinitis, crusting, ''saddle-nose'' deformity due to a perforated septum * * Ears: conductive hearing loss due to auditory tube dysfunction, sensorineural hearing loss (unclear mechanism) * *Oral cavity: (strawberry gingivitis ), underlying bone destruction with loosening of teeth, non-specific ulcerations throughout oral mucosa * * Eyes: pseudotumours, scleritis, conjunctivitis, uveitis, episcleritis * Trachea: subglottal stenosis * Lungs: pulmonary nodules (referred to as "coin lesions"), infiltrates (often interpreted as pneumonia), cavitary lesions, pulmonary haemorrhage causing haemoptysis, and rarely bronchial stenosis. * Arthritis: Pain or swelling (60%), often initially diagnosed as rheumatoid arthritis * Skin: nodules on the elbow, purpura, various others (see ''cutaneous vasculitis'') * Nervous system: occasionally sensory neuropathy (10%) and rarely mononeuritis multiplex * Heart, gastrointestinal tract, brain, other organs: rarely affected. 抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』 ■ウィキペディアで「granulomatosis with polyangiitis」の詳細全文を読む スポンサード リンク
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