ANCA-assoziierte Vaskulitiden

Zusammenfassung

Die seit kurzem auch als Granulomatose mit Polyangiitis bezeichnete Wegenersche Granulomatose und die Mikroskopische Polyangiitis sind mit Autoantikörpern gegen neutrophile zytoplasmatische Antigene (ANCA) assoziiert und werden aufgrund ihrer zahlreichen klinischen Gemeinsamkeiten auch als ANCA-assoziierte Vaskulitiden (AAV) bezeichnet. Neben den definitionsgemäß der GPA zugeordneten granulomatösen Manifestationen dominieren Zeichen einer Vaskulitis überwiegend kleiner Gefäße das klinische Bild der AAV. Die AAV sind potentiell lebensbedrohliche Erkrankungen, auch wenn sich die Prognose durch bessere Früherkennung, Entwicklung standardisierter Therapiestrategien und Zentrumsbehandlung in den vergangenen 2 Jahrzehnten weiter verbessert hat. Im ersten Erkrankungsjahr stellen auch heute noch eine nicht-kontrollierte Vaskulitis aber auch Infek­tionen als Folge der immunsuppressiven Therapie die Haupttodesursachen dar. Gegenstand dieser Übersicht sind die neue Nomenklatur, Leitsymp­tome und Diagnose, aktuelle Therapieempfehlungen und die Prognose der AAV.

Abstract

Granulomatosis with polyangiitis (GPA), which was formerly named Wegener’s granulomatosis, and microsscopic polyangiitis are associated with autoantibodies against neutrophil cytoplasmatic antigens (ANCA) and are together designated as ANCA-associated vasculitis (AAV) because they also share many clinical features. While granulomatous disease manifestations are by defintion only found in GPA, a vasculitis of predominantly small vessels determines in the clinical picture of the AAV. AAVs are protentially life-threatening diseases, although the prognosis has substantially improved with the last 2 decades due to earlier diagnosis, development of standardised treatment strategies and expert-centre care. Still, in the first year of disease, uncontrolled vasculitis and infections due to immunosuppressive therapy are the major causes of death. In this review we summarise the new nomenclature, symtoms and diagnosis of the AAV and discuss the current treatment recommendations and prognosis.

Schlüsselwörter

Granulomatose mit ­Polyangiitis - Mikrokopische Polyangiitis - ANCA - ANCA-assoziierte ­Vaskulitiden (AAV)

Key words

granulomatotis with ­polyangiitis - microscopic polyangiitis - ANCA - ANCA-associated vasculitis (AAV)

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