, et al
Efficacy of immunoglobulin plus prednisolone for prevention of coronary artery abnormalities in severe Kawasaki disease (RAISE study): a randomised, open-label, blinded-endpoints trial
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Investigators from 74 Japanese hospitals studied children with severe Kawasaki disease (KD) between 2008 and 2010 to determine if adding prednisolone to intravenous immunoglobulin (IVIG) therapy reduced the incidence of coronary artery abnormalities. Eligible patients included those with KD based on Japanese diagnostic guidelines and risk score ≥5 for initial treatment failure. The risk score assigns 2 points each for serum sodium ≤133 mmol/L, ≤4 days of illness at time of diagnosis, aspartate amino transferase ≥100 U/L, and white blood differential count of ≥80% neutrophils; and 1 point each for platelet count ≤30,000/μL, C-reactive protein ≥100 mg/L, and age ≤12 months.

The control group received IVIG 2 g/kg over 24 hours and aspirin 30 mg/kg per day until afebrile, then aspirin 3 to 5 mg/kg per day for at least 28 days. The intervention group received IVIG plus prednisolone 2 mg/kg per day in 3 divided doses for 5 days; when the fever resolved, prednisolone was given orally, and when the C-reactive protein normalized (≤5 mg/L), prednisolone was tapered over 15 days. Two-dimensional echocardiographs (ECHO) obtained at baseline and at weeks 1, 2, and 4 were read by cardiologists blinded to the patient’s identity and treatment arm. The primary endpoint was coronary abnormalities during the study period with a secondary endpoint of coronary abnormalities within 4 weeks after enrollment. Additional secondary outcomes included time to resolution of fever and inflammatory markers.

There were 248 patients enrolled, of whom 125 were randomized to the intervention group and 123 to the control group. The incidence of coronary artery abnormalities was significantly lower in the intervention group compared to the control group during the study period (3% vs 23%, respectively; P < .0001) and at 4 weeks (3% vs 13%, respectively; P = .014). The intervention group also had significantly faster resolution of fever and inflammatory markers. Numbers of serious adverse events were similar in both groups.

The authors conclude that treatment with IVIG plus corticosteroids for Japanese patients with severe KD decreased coronary artery abnormalities compared to standard treatment.

Dr Fremgen has disclosed no financial relationship relevant to this commentary. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.

KD, an acute multisystem inflammatory illness, is the leading cause of acquired coronary artery aneurysms (CAA) in children in developed nations. Current North American diagnostic criteria include a fever of 5 days or more and 4 of the following: bilateral nonsuppurative conjunctival injection, cervical lymphadenopathy, polymorphous exanthem, extremity changes, and mucous membrane changes.

CAA develop in 5% of patients receiving standard IVIG therapy and in 15% to 25% of those untreated. ECHO is recommended at diagnosis and at 2 weeks and 6 to 8 weeks...

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