Zeitschrift für Rheumatologie

, Volume 67, Issue 2, pp 137–144

Das Churg-Strauss-Syndrom

CME Weiterbildung • Zertifizierte Fortbildung

Zusammenfassung

Das Churg-Strauss-Syndrom (CSS) ist eine systemische ANCA-assoziierte Vaskulitis, die nahezu ausschließlich bei Patienten mit einer Asthmaanamnese auftritt. Häufige klinische Manifestationen sind neben einer ausgeprägten Bluteosinophilie und Asthma bronchiale eine chronische Sinusitis, Kardiomyopathie, pulmonale Infiltrate, gastrointestinale Beschwerden und eine Mononeuritis multiplex. Das morphologische Substrat ist eine nekrotisierende Vaskulitis mit prominenter Eosinophilie. Andere eosinophile Erkrankungen wie Parasitosen, Allergien und das idiopathische Hypereosinophiliesyndrom müssen ausgeschlossen werden. Die Therapie besteht aus hoch dosierten Glukokortikoiden und, bei Patienten mit schlechter Prognose, einer additiven zytotoxischen Therapie.

Schlüsselwörter

Churg-Strauss-Syndrom Eosinophilie Vaskulitis ANCA Glukokortikoide 

Churg-Strauss syndrome

Abstract

Churg-Strauss syndrome is a systemic ANCA-associated vasculitis arising almost exclusively in patients with a pre-existent asthma. Common clinical manifestations are marked blood eosinophilia, asthma, chronic sinusitis, cardiomyopathy, pulmonary infiltrates, gastrointestinal complaints and a multiplex neuropathy. The morphological substrate is an eosinophilic necrotizing vasculitis.

Other eosinophilic disorders such as parasitic diseases, allergies and idiopathic hyper-eosinophilic syndrome have to be excluded. The mainstay of therapy is high-dose corticosteroids with the addition of cytotoxic drugs in patients with poor prognosis.

Keywords

Churg-Strauss syndrome Eosinophilia Vasculitis ANCA Corticosteroids 

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Copyright information

© Springer Medizin Verlag 2008

Authors and Affiliations

  1. 1.Medizinische Klinik 3 mit Rheumatologie und Klinischer ImmunologieUniversität Erlangen-NürnbergErlangenDeutschland

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