Kawasaki disease: diagnosis, management and cardiac sequelae

Kawasaki disease: diagnosis, management and cardiac sequelae

May 2007, Vol. 5, No. 3, Pages 553-561
(doi:10.1586/14779072.5.3.553)

Louise Wood and Robert Tulloh

† Author for correspondence

Kawasaki disease (KD) is an acute self-limiting systemic vasculitis of unknown etiology and the most common cause of acquired coronary disease in children aged 6 months to 5 years. The inflammatory process results in coronary arteritis, aneurysmal lesions, arterial thrombotic occlusion or even sudden death. The diagnostic tests are unknown but treatment with immunoglobulin and aspirin is effective at reducing cardiac complications from 25 to 4.7% in the UK. Myocardial, endocardial or pericardial inflammation may occur acutely or many years later and abnormalities of myocardial blood flow may require ongoing medication, interventional catheterization or even cardiac surgery. There are several new drugs that may have important roles to play in managing KD in children and young adults.

Future Drugs

Randomized trial of pulsed corticosteroid therapy for primary treatment of Kawasaki disease.

Randomized trial of pulsed corticosteroid therapy for primary treatment of Kawasaki disease.

N Engl J Med. 2007 Feb 15

Newburger JW,
Sleeper LA,
McCrindle BW,
Minich LL,
Gersony W,
Vetter VL,
Atz AM,
Li JS,
Takahashi M,
Baker AL,
Colan SD,
Mitchell PD,
Klein GL,
Sundel RP;
Pediatric Heart Network Investigators.
Department of Cardiology, Children's Hospital and Harvard Medical School, Boston, MA 02115, USA. jane.newburger@cardio.chboston.org

BACKGROUND: Treatment of acute Kawasaki disease with intravenous immune globulin and aspirin reduces the risk of coronary-artery abnormalities and systemic inflammation, but despite intravenous immune globulin therapy, coronary-artery abnormalities develop in some children. Studies have suggested that primary corticosteroid therapy might be beneficial and that adverse events are infrequent with short-term use.

METHODS: We conducted a multicenter, randomized, double-blind, placebo-controlled trial to determine whether the addition of intravenous methylprednisolone to conventional primary therapy for Kawasaki disease reduces the risk of coronary-artery abnormalities. Patients with 10 or fewer days of fever were randomly assigned to receive intravenous methylprednisolone, 30 mg per kilogram of body weight (101 patients), or placebo (98 patients). All patients then received conventional therapy with intravenous immune globulin, 2 g per kilogram, as well as aspirin, 80 to 100 mg per kilogram per day until they were afebrile for 48 hours and 3 to 5 mg per kilogram per day thereafter.

RESULTS: At week 1 and week 5 after randomization, patients in the two study groups had similar coronary dimensions, expressed as z scores adjusted for body-surface area, absolute dimensions, and changes in dimensions. As compared with patients receiving placebo, patients receiving intravenous methylprednisolone had a somewhat shorter initial period of hospitalization (P=0.05) and, at week 1, a lower erythrocyte sedimentation rate (P=0.02) and a tendency toward a lower C-reactive protein level (P=0.07). However, the two groups had similar numbers of days spent in the hospital, numbers of days of fever, rates of retreatment with intravenous immune globulin, and numbers of adverse events.

CONCLUSIONS: Our data do not provide support for the addition of a single pulsed dose of intravenous methylprednisolone to conventional intravenous immune globulin therapy for the routine primary treatment of children with Kawasaki disease. (ClinicalTrials.gov number, NCT00132080 [ClinicalTrials.gov].)

Copyright 2007 Massachusetts Medical Society.