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Arthritis und Rheuma 2025; 45(06): 415-423
DOI: 10.1055/a-2676-2075
Schwerpunkt

Makrophagenaktivierungssyndrom: Notfall mit therapeutischen Herausforderungen

Autoren

  • Tatjana Welzel

    1   Pädiatrische Rheumatologie, Universitäts-Kinderspital beider Basel (UKBB), Universität Basel, Basel, Schweiz
    2   Pädiatrisches Forschungszentrum, Universitäts-Kinderspital beider Basel (UKBB), Universität Basel, Basel, Schweiz
    3   Deutsches Rheumaforschungszentrum Berlin, Berlin, Deutschland

  • Claas Hinze 

    4   Klinik für Pädiatrische Rheumatologie und Immunologie, Universitätsklinikum Münster, Münster, Deutschland
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Zusammenfassung

Das Makrophagenaktivierungssyndrom (MAS) ist eine gefürchtete potenziell lebensbedrohliche Komplikation rheumatologisch-entzündlicher Erkrankungen. Durch massive Aktivierung und Proliferation von T-Lymphozyten und Makrophagen kommt es beim MAS zu Hyperinflammation und Zytokinsturm unter Beteiligung proinflammatorischer Zytokine. Diese umfassen vor allem IL-1, -6, -18 (IL: Interleukin) und IFN-γ (IFN: Interferon). Labormedizinische Warnzeichen für ein MAS bei systemischer Inflammation sind eine (Thrombo-)Zytopenie, Transaminasenerhöhung und Hyperferritinämie. Auch ein Abfall der Blutsenkungsgeschwindigkeit (BSG) bei hohem C-reaktivem Protein (CRP) und eine Koagulopathie sollten alarmieren. Dabei ist der Trend im Labor mitunter entscheidender als die absoluten Werte. Die Diagnosestellung eines MAS ist herausfordernd; klinische Scores, Klassifikationskriterien und Biomarker (z. B. IL-18, CXCL9 [CXC-Ligand 9] und sIL-2R [löslicher Interleukin-2-Rezeptor]) können bei der Differenzialdiagnose unterstützen. Eine schnelle Diagnosestellung mit effektiver Therapie ist maßgeblich für das Outcome. Therapeutisch sind Hochdosis-Glukokortikoide sowie IL-1- und IFN-γ-Inhibitoren vielversprechend, wobei die Evidenzlage bei nicht mit einem Still-Syndrom assoziierten MAS limitiert bleibt.

Schlüsselwörter

Hyperinflammation - Zytokinsturm-Syndrome - systemische juvenile idiopathische Arthritis/Still-Erkrankung - autoinflammatorische Erkrankungen


Publikationsverlauf

Artikel online veröffentlicht:
01. Dezember 2025

© 2025. Thieme. All rights reserved.

Georg Thieme Verlag KG
Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany

 

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