Zusammenfassung
Die Granulomatose mit Polyangiitis (GPA, Wegener-Granulomatose) ist durch eine granulomatöse Entzündung des Respirationstrakts und eine nekrotisierende, ANCA-assoziierte Vaskulitis kleiner bis mittelgroßer Gefäße gekennzeichnet, die eine Prädilektion für Lunge (pulmonale Kapillaritis) und Niere (nekrotisierende Kleingefäßvaskulitis) aufweist. Die Erkrankung verläuft stadienabhängig und beginnt typischerweise mit einer Entzündung des Respirationstrakts und generalisiert im Verlauf. Die Therapie erfolgt heute evidenzbasiert in Abhängigkeit von Aktivität und Krankheitsstadium, was zu einer deutlichen Verbesserung des Langzeitoutcomes geführt hat. Problematisch ist weiterhin die hohe Frühmortalität im ersten Jahr der Behandlung, die vor allem durch Infektionen unter Immunsuppression bedingt ist. Eine Herausforderung ist auch die Therapie refraktärer Organmanifestationen, die vor allem auch durch die granulomatöse Entzündung repräsentiert werden und erhebliche chronische Organschäden nach sich ziehen können.
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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Holle, J., Reinhold-Keller, E. & Gross, W. Update Granulomatose mit Polyangiitis (GPA, Wegener-Granulomatose). Z. Rheumatol. 71, 745–753 (2012). https://doi.org/10.1007/s00393-012-0982-z
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DOI: https://doi.org/10.1007/s00393-012-0982-z