Aktuelle Diagnostik und Therapie des adulten Still-Syndroms

Zusammenfassung

Beim Still-Syndrom handelt es sich um eine seltene, polygenetische autoinflammatorische Erkrankung, die durch die klinische Trias aus intermittierendem Fieber, Arthralgien/Arthritis und flüchtigem Exanthem gekennzeichnet ist [1], [2]. Man versteht das Still-Syndrom mit Beginn im Erwachsenenalter (adultes Still-Syndrom, AOSD) und das Still-Syndrom im Kindesalter (systemische juvenile idiopathische Arthritis oder M. Still) zunehmend als Krankheitskontinuum [3]. Unbehandelt kann die Erkrankung zu lebensbedrohlichen Komplikationen wie dem Makrophagenaktivierungssyndrom führen [2]. Die Diagnostik beruht auf dem Erkennen von charakteristischen klinischen Symptomen und Laborveränderungen sowie dem Ausschluss alternativer rheumatischer, infektiologischer und neoplastischer Erkrankungen [4], [5]. Therapeutisch setzt man Glukokortikoide, steroidsparende konventionelle Medikamente (v. a. Methotrexat und Ciclosporin) und Inhibitoren des IL1- oder IL6-Signalweges ein [4], [5]. Die kürzlich erschienene europäische Leitlinie zum Still-Syndrom [5] weicht von den aktuellen deutschen AOSD-Leitlinien [4] dahingehend ab, dass sie primär den Einsatz der gezielten Zytokinblockade empfiehlt.

Schlüsselwörter

M. Still - systemische juvenile idiopathische Arthritis - Autoinflammation

Publication History

Article published online:
29 August 2025

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