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Kawasaki-Syndrom

Kawasaki syndrome

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Zusammenfassung

Das Kawasaki-Syndrom ist eine akute Vaskulitis der mittelgroßen, extraparenchymalen Arterien. Betroffen sind Kinder <15 Jahren, 76% der Patienten sind <5 Jahre. Die jährliche Inzidenz variiert zwischen 17 (West-Europa) und 112 (Japan) pro 100.000 Kinder unter 5 Jahren. Die Genese ist unklar; epidemiologische Daten lassen eine Interaktion aus genetischer Prädisposition und Umweltfaktoren vermuten. Das Initialstadium ist gekennzeichnet durch hohes Fieber >39°C über mehr als 5 Tage trotz antipyretischer Therapie, Exantheme mit Palmar- und Plantarerythem, beidseitige konjunktivale Injektion, entzündliche Veränderungen des Mund- und Rachenraumes und Lymphknotenschwellungen im Halsbereich. Ab der 2. Woche löst sich das Exanthem in großen Schuppen ab. Die Therapie in der akuten Phase besteht aus Acetylsalicylsäure in Hochdosis (100 mg/kg KG i.v.) sowie Immunglobulinen. Letztere reduzieren die Inzidenz von Aneurysmata der Koronararterien. Ohne Therapie treten diese in der subakuten und chronischen Phase bei bis zu 25% der Patienten auf. Die Langzeittherapie hängt von Ausmaß und Dynamik der Koronarveränderungen ab. Bei Vorliegen von Koronaraneurysmata erfolgt v. a. eine effektive orale Gerinnungshemmung. Bei sekundär auftretenden Koronarstenosen oder thrombotischen Aneurysmaverschlüssen kommen interventionelle und chirurgische Verfahren zum Einsatz. Alle Kawasaki-Patienten müssen lebenslang kardiologisch betreut werden.

Abstract

Kawasaki syndrome is an acute vasculitis of the medium-sized extraparenchymal arteries. It affects children <15 years; 76% of the patients are under 5 years. The yearly incidence ranges from 17 in Western European countries to 112 in Japan per 100,000 children of less than 5 years. The exact etiology is unknown, but epidemiological data suppose an interaction of genetic predisposition and environmental factors. In the acute state, Kawasaki disease is characterized by high fever >39°C lasting more than 5 days despite antipyretic therapy, skin exanthema with palmar and plantar erythema, bilateral conjunctivitis, and inflammation of the oropharyngeal tract with cervical lymphadenopathy. After 2 weeks, the exanthema resolves with extensive peeling. Medical treatment in the acute state consists of high-dose aspirin (100 mg/kg body weight i.v.) and gamma globulins. The latter reduce the incidence of coronary aneurysms. Without therapy, up to 25% of patients develop coronary aneurysms in the subacute and chronic phases. Long-term therapy depends on the extent and dynamic changes of the coronary manifestations. Coronary aneurysms require primarily oral anticoagulation. In the case of secondary coronary stenosis or thrombotic occlusions, catheter interventions and surgical management are indicated. All patients with Kawasaki syndrome must undergo lifelong cardiological follow-up.

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Authors and Affiliations

  1. Franz-Volhard-Klinik für molekulare und klinische Kardiologie, Charité Universitätsmedizin Berlin, HELIOS Klinikum Berlin-Buch, Schwanebecker Chaussee 50, 13125, Berlin, Deutschland

    F. von Knobelsdorff-Brenkenhoff, S. Bohl &  M.W. Bergmann

  2. Universitätsklinik für Kinder- und Jugendmedizin Tübingen, Tübingen, Deutschland

    M. Hofbeck

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  1. F. von Knobelsdorff-Brenkenhoff
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  2. M. Hofbeck
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  3. S. Bohl
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  4. M.W. Bergmann
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Correspondence to M.W. Bergmann.

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von Knobelsdorff-Brenkenhoff, F., Hofbeck, M., Bohl, S. et al. Kawasaki-Syndrom. Kardiologe 2, 151–166 (2008). https://doi.org/10.1007/s12181-008-0049-1

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