Update Granulomatose mit Polyangiitis (GPA, Wegener-Granulomatose) |
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Authors: | PD Dr. J.U. Holle E. Reinhold-Keller W.L. Gross |
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Affiliation: | 1. UKSH, Campus Lübeck, Poliklinik für Rheumatologie und Immunologie, Klinikum Bad Bramstedt, Oskar-Alexander Str. 26, 24576, Bad Bramstedt, Deutschland 2. Die Rheuma-Spezialisten, Hamburg, Deutschland
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Abstract: | Granulomatosis with polyangitis (GPA, Wegener’s granulomatosis) is characterized by a granulomatous inflammation of the respiratory tract and a necrotizing ANCA-associated small to medium-size vessel vasculitis with a predilection for the lungs (pulmonary capillaritis) and kidneys (necrotizing glomerulonephritis). The disease evolves stage-wise and typically starts as inflammation of the respiratory tract followed by development of systemic vasculitis manifestations. Today, treatment is evidence-based and adapted according to activity and disease stage which has resulted in a significant improvement in long-term outcome. Early mortality during the first year of treatment poses one of the main problems and is a result of infections under immunosuppressive treatment. Furthermore, treatment of refractory disease activity which is often represented by granulomatous manifestations is still a challenge and may result in significant organ damage if not treated successfully. |
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