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A child with severe relapsing Kawasaki disease rescued by IL-1 receptor blockade and extracorporeal membrane oxygenation

2012; BMJ; Volume: 71; Issue: 12 Linguagem: Inglês

10.1136/annrheumdis-2012-201658

1468-2060

Sophie Cohen, Carline E. Tacke, Bart Straver, Natasja Meijer, Irene M. Kuipers, Taco W. Kuijpers,

Mechanical Circulatory Support Devices

Kawasaki disease (KD) is an acute inflammatory vasculitis that predominantly occurs in children under 5 years of age.1 It is associated with the development of coronary artery aneurysms (CAA) in 15–25% of untreated cases.2 Standard treatment consists of high-dose intravenous immunoglobulins (IVIG) along with aspirin.3 About 15% of patients do not respond to a single dose of IVIG and need retreatment.4 When ongoing signs of active disease are present, methylprednisolone pulses are often administered.5 If there is a lack of response, alternative anti-inflammatory medication such as infliximab or plasmapheresis have been suggested in individual case series.6 ,7 We report, for the first time, the beneficial use of an interleukin-1 receptor antagonist (IL-1RA) in relapsing KD. A 2-year-old boy was presented with persistent fever, coughing and swollen cervical lymph nodes. The boy's condition had not improved with empirical antibiotic treatment. He developed a rash, conjunctivitis and swollen extremities. Upon admission the …