Kawasaki Disease Treatment & Management - Medscape Reference

In addition to their use in treatment of IVIG-resistant KD, corticosteroids have been proposed as part of primary therapy. This indication is controversial, however. Research results have been inconsistent, as follows:

• In a multicenter prospective randomized trial in Japan, the combination of corticosteroids and IVIG significantly decreased coronary artery abnormalities, duration of fever, CRP levels, and initial treatment failure. [83] The RAISE trial, conducted in Japan, also had similar findings. [84]

• A randomized trial in the United States in which a single pulsed dose of methylprednisolone (30 mg/kg) was added to conventional therapy saw a reduction in the ESR at 1 week, but no difference in coronary artery abnormalities [85]

• In a meta-analysis of 4 studies and 447 patients, Athappan et al concluded that the addition of steroids to standard therapy with IVIG and aspirin decreased the rate of re-treatment but did not decrease the incidence of coronary aneurysms or adverse events. [86]

• Chen et al reviewed 16 studies that included 2746 patients and found that the rate of coronary artery abnormalities was significantly lower in the corticosteroids plus IVIG group than in the IVIG therapy alone group. The benefit was only found in the group using corticosteroids plus IVIG for the prevention of coronary artery abnormality, and not in the group using the treatment as rescue therapy. The greatest benefit was also seen in patients predicted to be at high risk of IVIG resistance. [87]

Guidelines from the ACR and the Vasculitis Foundation conditionally recommend the use of adjunctive glucocorticoids with IVIG as initial therapy for patients with acute KD who are at high risk for IVIG resistance or the development of coronary artery aneurysms. [3]

Statins are another class of medications that have been used variably in the setting of KD, due to their cholesterol-lowering and immunomodulatory properties. Further research is necessary to determine the impact of statins in the early phase of KD as well as the potential to lower the risk for atherosclerosis in the long term.

The roles of other adjunctive therapies, including pentoxifylline and abciximab, have not yet been definitively determined. Pentoxifylline acts as an anti-inflammatory agent by inhibiting tumor necrosis factor-alpha and may reduce the incidence of aneurysms. Abciximab is a platelet glycoprotein IIb/IIIa receptor inhibitor and has been used in conjunction with standard therapies in patients with KD and giant aneurysms.

Clinical Trials

A selection of ongoing, recruiting, and completed clinical trials is as follows:

• Infliximab Plus Intravenous Immunoglobulin for the Primary Treatment of Kawasaki Disease

• A Study to Evaluate the Use and Safety of CARDIOLITE® in Pediatric Patients With Kawasaki Disease

• Etanercept in Kawasaki Disease

• Etiology Study of Kawasaki Disease

• Infliximab (Remicade) for Patients With Acute Kawasaki Disease