Kawasaki-Syndrom

ZUSAMMENFASSUNG

Das Kawasaki-Syndrom ist eine inflammatorische Systemerkrankung unklarer Ätiologie und wird den Vaskulitiden zugeordnet. Es betrifft überwiegend Kinder unter 5 Jahren. Die Diagnose wird klinisch gestellt. Die seit 2004 existierende und zuletzt 2014 überarbeitete S1-Leitlinie der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR) sollte mit Beteiligung der Deutschen Gesellschaft für pädiatrische Kardiologie und angeborene Herzfehler (DGPK) und Einschluss der Deutsche Gesellschaft für Kinder- und Jugendmedizin (DGKJ) überarbeitet werden. Dies ist in den Jahren 2018 und 2019 erfolgt [1]. Die wesentlichen Neuerungen betreffen die Definition von Risikogruppen, die Einordnung der Therapieformen (Steroide, Biologika) und die Spezifizierung der Anforderungen an die Echokardiografie. Die Empfehlungen zum Langzeit-Follow-up wurden konkretisiert. Im laufenden Prozess der Feinabstimmung der Leitlinie wurde das Interesse an der Kawasaki-Erkrankung durch die Berichte von Kawasaki-ähnlichen Krankheiten im Rahmen von COVID-19-Infektionen dramatisch hoch. Damit hat sich eine neue Krankheit – Pediatric Inflammatory Multisystemic Syndrome (PIMS) oder Multisystem Inflammatory Syndrome in Children (MIS-C) – zur Differenzialdiagnose entwickelt.

SUMMARY

Kawasaki syndrome is known as a systemic vasculitis of unknown origin in children mostly younger than 5 years of age. The diagnosis is based on defining clinical symptoms. Recommendations were first published in 2004 by the German society of pediatric rheumatology (GKJR) and updated in 2014. Recently, a revision was published as a result of collaboration of the GKJR with the pediatric cardiologists (DGPK) and general pediatricians (DGKJ) between 2018 and 2019. Major changes were the definition of risk groups, the role of steroids and biologicals in therapy and echocardiography. Recommendations for the cardiac follow-up evaluations have been refined. In the meanwhile, pediatric inflammatory multisystemic syndrome in children (PIMS) or multi systemic inflammatory syndrome associated with COVID 19 (MIS-C) became known as a new disease and needs to be differentiated from Kawasaki syndrome.

Schlüsselwörter

Kawasaki-Syndrom - Leitlinie - Risikofaktoren

Keywords

Kawasaki syndrome - guideline - risk factor

• Literatur

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