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

Publication History

Article published online:
11 October 2021

• Literatur

• 1 Neudorf U, Jakob A, Lilienthal E, Hospach T. Kawasaki Syndrom (S2k Leitlinie). https//www.awmf.org/leitlinien/detail/ll/185-003.html
• 2 Jakob A, Whelan J, Kordecki M. et al Kawasaki Disease in Germany – A prospective, population-based study adjusted for underreporting. Pediatr Infect Dis J 2016; 35: 129-134
• 3 Saji BT, Newburger JW, Burns JC, Takahashi M. eds Kawasaki Disease 2017. Tokyo, Japan: Springer; 2017
• 4 Ozen S, Pistorio A, Iusan SM. et al Paediatric Rheumatology International Trials Organisation (PRINTO). EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis 2006; 65: 936-941
• 5 Jennette JC, Falk RJ, Bacon PA. et al 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65: 1-11
• 6 McCrindle BW, Rowley AH, Newburger JW. et al American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young; Council on Cardiovascular and Stroke Nursing; Council on Cardiovascular Surgery and Anesthesia; and Council on Epidemiology and Prevention. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals from the American Heart Association. Circulation 2017; 135: e927-e999
• 7 Marchesi A, Tarissi de Jacobis I, Rigante D. et al Kawasaki disease: guidelines of the Italian Society of Pediatrics, part I – definition, epidemiology, etiopathogenesis, clinical expression and management of the acute phase. Ital J Pediatr 2018; 44: 102
• 8 Marchesi A, Tarissi de Jacobis I, Rigante D. et al Kawasaki disease: guidelines of Italian Society of Pediatrics, part II – treatment of resistant forms and cardiovascular complications, follow-up, lifestyle and prevention of cardiovascular risks. Ital J Pediatr 2018; 44: 103
• 9 de Graeff N, Groot N, Ozen S, Eleftheriou D. et al European consensus-based recommendations for the diagnosis and treatment of Kawasaki disease – the SHARE initiative. Rheumatology 2019; 58: 672-682
• 10 Kobayashi T, Saji T, Otani T. et al RAISE study group investigators. Efficacy of immunoglobulin plus prednisolone for prevention of coronary artery abnormalities in severe Kawasaki disease (RAISE study): a randomised, open-label, blinded-endpoints trial. Lancet 2012; 379: 1613-1620
• 11 Smith LB, Newburger JW, Burns JC. Kawasaki syndrome and the eye. Ped Infect Dis J 1989; 08: 116-118
• 12 Jacob JL. Ocular manifestations in Kawasaki disease (mucocutaneous lymph node disease. Can J Ophthalmol 1982; 17: 199-202
• 13 Farvardin M. Sudden uniklateral blindness in a girl with Kawasaki disease. J Pediatr Ophthalmol Strabismus 2007; 44: 303-304
• 14 Smith KA, Yunker WK. Kawasaki disease is associated with sensorineural hearing loss: a systematic review. Int J Otorhinolaryngeol 2014; 78: 1216-1220
• 15 Aggarwal V Etinger V, Orjuela AF. Sensorineural hearing loss in Kawasaki disease. Ann Pediatr Cardiol 2016; 09: 87-89
• 16 Dietz SM, Tacke CE, Kuipers IM. et al Cardiovascular imaging in children and adults following Kawasaki disease. Insights Imaging 2015; 06: 697-705
• 17 van Stijn-Bringas Dimitriades D, Planken RN, Groenink M. et al Coronary artery assessment in Kawasaki disease with dual-source CT angiography to uncover vascular pathology. Eur Radiol 2019; 19: 1-10
• 18 Dallaire F. New equations and a critical appraisal of coronary artery Z scores in healthy children. J Am Soc Echocardiogr 2011; 24: 60-74