Management of IVIG-Resistant Kawasaki Disease with Cardiac Involvement

Introduction to IVIG-Resistant Kawasaki Disease

Approximately 10% of Kawasaki disease patients are IVIG-resistant, and the presence of left atrial dilation indicates significant cardiac involvement and ongoing inflammation requiring aggressive treatment 1, 2

The American Heart Association guidelines recommend that steroid treatment be restricted to children in whom 2 infusions of IVIG have been ineffective in alleviating fever and acute inflammation, but cardiac involvement warrants escalation to steroids even after a single IVIG failure 1, 2, 3

Second dose of IVIG (2 g/kg) is a commonly recommended first-line retreatment for IVIG-resistant Kawasaki disease 1, 2, 4
IV methylprednisolone (20-30 mg/kg for 3 days) is an acceptable alternative to second IVIG for patients with cardiac involvement 5, 6, 7
Infliximab (5 mg/kg) can be considered as an alternative to second IVIG or steroids for patients with severe disease 5, 6, 7

High-dose aspirin (80-100 mg/kg/day) should be continued until the patient is afebrile for 48 hours, then transitioned to low-dose aspirin (3-5 mg/kg/day) for 6-8 weeks if no coronary abnormalities develop 4
Frequent echocardiography is necessary to assess progression of cardiac involvement and monitor for development of coronary artery aneurysms, which peak in risk during the first 3 months 4, 7

If pulse steroid therapy fails, a longer tapering course of prednisolone (2 mg/kg/day tapered over 2-3 weeks) may be considered, as Japanese studies show longer steroid courses may suppress persistent vascular inflammation more effectively 5, 6, 7
Infliximab (5 mg/kg) can be considered as next-line therapy if steroids fail, and cyclosporine (4-6 mg/kg/day orally) may be used for highly refractory cases 5, 6, 7
Plasma exchange is reserved for patients failing all medical therapies 1, 7