Infliximab for the treatment of intravenous immunoglobulin resistant Kawasaki disease complicated by coronary artery aneurysms: a case report

Kawasaki disease (KD) is associated with the development of systemic vasculitis complicated by coronary and peripheral arterial aneurysms, and myocardial infarction in some patients [1]. KD has an incidence of 8/100 000 children in the UK, and has superseded rheumatic fever in that KD is now the commonest cause of acquired heart disease in children in the United Kingdom and the USA [2]. Treatment with intravenous immunoglobulin (IVIG) and aspirin reduces the incidence of coronary artery lesions (CAL) from approximately 20–40% to <5% [1,2], although recently in the UK we and others have reported an alarmingly higher incidence of CAL despite IVIG therapy, probably relating to delayed diagnosis of KD in the UK [3,4]. It is generally accepted that an as yet undefined infectious trigger in a genetically predisposed individual results in the disease [5].Despite intensive research into the illness the cause remains unknown, and although there have been significant improvements in diagnosis and treatment of children with the disease there are still a number of important unanswered questions regarding aetiopathogenesis [6], treatment of IVIG resistant cases, and long-term outlook [2]. This case report describes an 8 year old male with severe coronary artery aneurysms (CAA) and ongoing inflammation despite therapy with IVIG, aspirin and corticosteroids, treated successfully with infliximab.The patient was an eight year old boy presenting to his local hospital with a three day history of pyrexia, irritability, erythematous rash in the groin, bilateral knee arthritis and generalised arthralgia, and a one day history of bilateral non-purulent conjunctivitis. There was no prior past medical or family history of note and he was fully immunised. Examination revealed red, cracked lips but there was no lymphadenopathy or clinically overt focus of infection. Laboratory investigations at that stage revealed haemoglobin (Hb) 12.6 g/dL (normal reference range 11.5–15.5 g/dL), white cell