Vaskulitiden – Systematik, Diagnostik und Therapie

Die Vaskulitiden stellen eine große Gruppe der rheumatologischen Systemerkrankungen dar. Eine systematische Einteilung bietet die revidierte Definition der Chapel Hill Consensus Conference. Die häufigsten Vaskulitiden in Mitteleuropa stellen die Riesenzellarteriitis und die Gruppe der ANCA-assoziierten Vaskulitiden dar. Die Riesenzellarteriitis betrifft insbesondere die Temporalarterie bzw. die Aorta und die daraus abgehenden Gefäße. Therapeutisch werden hier Glukokortikoide und Methotrexat eingesetzt. Bei den ANCA-assoziierten Vaskulitiden besteht ein stadienabhängiger Verlauf, fast jedes Organ kann betroffen sein. Die immunsuppressive Therapie besteht neben Glukokortikoiden je nach Krankheitsstadium und Schwere aus Cyclophosphamid oder Rituximab bei lebensbedrohlichen Manifestationen sowie aus Methotrexat oder Azathioprin für nicht-organbedrohende Manifestationen und als Remissionserhaltung.

Vasculitides constitute a large group of rheumatological systemic diseases. The revised definition of the Chapel Hill Consensus Conference provides a systematic classification. The most frequent vasculitides in central Europe are giant cell arteritis and the group of ANCA-associated vasculitides. Giant cell arteritis affects especially the temporal arteries or, respectively, the aorta and vessels branching from it. Glucocorticoids and methotrexate are employed as therapy. The ANCA-associated vasculitides are characterized by a stage-dependent course; practically every organ can be affected. Besides glucocorticoids and depending on the stage and severity the immune suppressive therapy consists of cyclophosphamide or rituximab (especially for the induction of remission) for life-threatening manifestations as well as methotrexate or azathioprine for non-organ-threatening manifestations and to maintain remission.

Key words

Systemic vasculitides - giant cell arteritis - ANCA-associated vasculitides - granulomatosis with polyangiitis - microscopic polyangiitis - immunosuppressive therapy

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