A child with resistant Kawasaki disease successfully treated with anakinra: a case report

Abstract

BackgroundKawasaki disease (KD) is an acute self-limited systemic vasculitis of unknown etiology. Intravenous immunoglobulin (IVIG) is an effective treatment and decreases the risk of cardiac complications to less than 5%. In spite of its effectiveness, some children do not respond to this therapy and still develop coronary aneurysms (CAA). The optimal treatment for IVIG non-responsive patients remains controversial although corticoids have been suggested to be an effective treatment in some patients. For those patients still resistant to IVIG and corticoids, interleukin-1 receptor antagonists (IL-1RA) such anakinra could be an alternative.Case presentationWe present a 3 year-old Caucasian patient with KD without cardiac complications but with important resistance to treatment. After becoming resistant to IVIG and corticoids, anakinra proved to be an effective treatment.ConclusionsTo our knowledge, this is the first report of the utility of IL-1RA in refractory KD without coronary impairment. The patient fulfilled the classical criteria for KD and, after becoming resistant to first and second line treatments, anakinra proved to be an effective treatment. Further studies are required to determine if this is an effective treatment option for other cases of resistant Kawasaki disease.

Usefulness and safety of anakinra in refractory Kawasaki disease complicated by coronary artery aneurysm

“…Three clinical cases have been reported in the literature. [8][9][10] Currently, there is no recommendation for its use in Kawasaki disease. Studies are in progress to assess efficacy and tolerance of interleukin-1 receptor antagonist in Kawasaki disease.…”

Complete Regression of Giant Aneurysms in an Infant with Delayed Diagnosis and Refractory Kawasaki Disease via Combination Anticytokine Therapy: Case Report and Review of Similar Cases

“…e third case report is of a 3-year-old without CAA from KD but who was resistant to treatment with IVIG. Two weeks after admission, he was started on anakinra and within hours his CRP, ESR, platelets, and hemoglobin normalized [10]. e fourth case reviews an episode of KD in a 16-year-old male patient with aneurysms of between 0.6 and 0.9 cm in all three arteries (approx.…”

High-dose anakinra as treatment for macrophage activation syndrome caused by refractory Kawasaki disease in an infant

“…In this case anakinra was chosen as the best possible treatment, based on multiple case reports revealing the effect of anakinra in refractory KD16–20 and the recommendation from The American Heart Association in refractory KD 1. In addition, the successful use of anakinra as treatment for both KD-MAS,20 21 and sJIA-MAS,8 is reported, suggesting that IL-1 most likely plays a key role in the inflammatory response.…”