Management of acute and refractory Kawasaki disease

Abstract

Acute Kawasaki disease (KD) is treated with high-dose intravenous immunoglobulin (IVIG), which is proven to decrease the incidence of coronary artery aneurysms from 25% to less than 5%. Aspirin is also given, although the evidence base is less secure. There is increasing evidence for steroid therapy as adjunctive primary therapy with IVIG, especially in Asian children. Approximately 10–30% of patients fail to respond to the initial IVIG and are at increased risk of coronary artery aneurysms. The optimal treatment for IVIG-nonresponsive KD remains controversial. Management options include further dose(s) of IVIG, corticosteroids, TNF-α blockade, cyclosporin A, anti-IL-1 and anti-CD20 therapy. In this article, the authors review the current evidence for treatment of acute KD and discuss options for IVIG nonresponders.

Keywords::

• anti-CD20
• anti-TNF-α
• aspirin
• IL-1
• IVIG
• Kawasaki disease
• nonresponder
• review
• treatment

Financial & competing interests disclosure

CE Tacke was supported by the Stinafo Foundation (Stichting Nationaal Fonds ‘Het Gehandicapte Kind’, The Hague, The Netherlands), and partly by the Rare Disease Foundation (Stichting Zeldzame Ziekten Fonds, The Hague, The Netherlands). DP Burgner is supported by a Career Development Fellowship from the National Health and Medical Research Council, Australia. The work was supported in part by the Victorian Government’s Operational Infrastructure Support Program. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.