Protrahiertes febriles Myalgie-Syndrom bei Kindern mit Familiärem Mittelmeerfieber

Zusammenfassung

Das protrahierte febrile Myalgie-Syndrom (PFMS) ist eine seltene Form des Familiären Mittelmeerfiebers (FMF), gekennzeichnet durch anhaltende Muskelschmerzen, Fieber und erhöhte Entzündungsmarker. Es kann Bauchschmerzen, Durchfall und Hautausschläge verursachen, die eine IgA-Vaskulitis imitieren. PFMS kann die erste Manifestation von FMF sein, was zu einer verzögerten Diagnose führt. Ziel war eine Literaturübersicht zu klinischen Merkmalen und Behandlungsmöglichkeiten von PFMS bei Kindern sowie eine eigene Falldarstellung. 18 Artikel und 7 Lehrbücher wurden untersucht. Ergebnisse zeigen, dass PFMS oft als erste FMF-Manifestation auftritt und bei über der Hälfte der Patienten Kortikosteroide wirksam sind. MRT zeigt bei PFMS-Patienten häufig Muskelödeme. Genetische Tests und MRT sind wichtige diagnostische Hilfsmittel.

Schlüsselwörter

Protrahiertes febriles Myalgie-Syndrom - klinische Merkmale - Behandlung - diagnostische Rolle der MRT

Publikationsverlauf

Artikel online veröffentlicht:
29. August 2025

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