Systemische Vaskulitiden: Revidierte Nomenklatur, neue Therapiestrategien

Zusammenfassung

Vaskulitiden sind eine heterogene Gruppe von entzündlichen Erkrankungen der Blutgefäße, die gleichermaßen durch ihre Diversität als auch durch eine Überlappung klinischer und pathologischer Befunde und Manifestationen gekennzeichnet sind. Die Ätiologie der meisten Vaskulitiden ist noch nicht geklärt. Ihre Nomenklatur und Klassifikation stellt daher eine Herausforderung für Internisten, Immunologen und Pathologen dar. Die revidierte Chapel Hill Consensus Conference (CHCC) 2012 Nomenklatur beinhaltet eine Aktualisierung der Namen und Definitionen der in der CHCC 1994 Nomenklatur genannten Vaskulitiden. Außerdem wurden 4 neue Kategorien eingeführt, um das Spektrum der Vaskulitiden umfassender abzubilden. Aufgrund des Nebenwirkungsspektrums von zytotoxisch wirksamen Immunsuppressiva besteht ein Bedarf an therapeutischen Alternativen, die eine effektivere und gezieltere Therapie bei günstigerem Nebenwirkungsprofil ermöglichen. Erste Studienergebnisse zur Therapie von Vaskulitiden mit Biologicals lassen eine zukünftige Erweiterung der therapeutischen Optionen erwarten.

Abstract

Vasculitides comprise a heterogeneous group of inflammatory diseases of the blood vessels, which are characterized both by their diversity and an overlap of clinical and pathological findings and manifestations. The etiology of most vasculitides is not yet clear. Their nomenclature and classification is therefore a challenge for internists, immunologists, and pathologists. The revised Chapel Hill Consensus Conference (CHCC) 2012 nomenclature includes an update of the names and definitions of the CHCC 1994 nomenclature. In addition, four new categories were introduced to describe the spectrum of vasculitis comprehensively. Because of the side effects of cytotoxic immunosuppressive agents there is a need for safer and more effective targeted therapies. The first results from studies using biologicals in the treatment of vasculitides are promising.

Schlüsselwörter

systemische Vaskulitis - Chapel-Hill-Klassifikation - eosinophile Granulomatose mit Polyangiitis - Granulomatose mit Polyangiitis - HCV-assoziierte kryoglobulinämische Vaskulitis - Interleukin-2 - Riesenzellarteriitis - Rituximab - Tocilizumab

Keywords

systemic vasculitis - Chapel-Hill classification - eosinophilic granulomatosis with polyangiitis - granulomatosis with polyangiitis - HCV-associated cryoglobulinemic vasculitis - interleukin-2 - giant cell arteritis - rituximab - tocilizumab

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