Treatment of immune globulin-resistant Kawasaki disease with pulsed doses of corticosteroids☆,☆☆,★

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Abstract

We describe four children with Kawasaki disease resistant to treatment with intravenously administered immune globulin who were treated with high doses of methylprednisolone. All four patients apparently responded with normalization of symptoms, and none had significant progression of coronary artery abnormalities or adverse events. We recommend pulse methylprednisolone therapy (30 mg/kg per day) during a 1- to 3-day period for patients with Kawasaki disease who do not respond to intravenous immune globulin therapy or who have recrudescent disease after adequate therapy. (J PEDIATR 1996;128:146-9)

Section snippets

Subjects

The subjects were two boys and two girls, aged 3 months to 27 months, treated for KD at the Children's Hospital, Boston, between June 1990 and October 1994. All children met the Centers for Disease Control and Prevention criteria for the diagnosis of KD.4 All patients were treated initially within the first 10 days of onset of fever and were given aspirin, 80 to 100 mg/kg per day, and IVIG (IVEEGAM, Immuno AG, Vienna, Austria), 2 gm/kg in a single infusion during an 8- to 10-hour period.

Corticosteroid therapy

RESULTS

Children who were treated with methylprednisolone had a median age of 10 months (range, 3 months to 27 months); three were less than 1 year of age and two were boys (Table). In each child who was so treated, the fever was attributable to active KD and there were no clinical indications of disease other than KD at the time of treatment (e.g., arthritis, intercurrent infection). All children were receiving high doses of aspirin or ibuprofen at the time of retreatment.

The four patients with

DISCUSSION

A subgroup of patients with KD is resistant to IVIG therapy3; these patients are at greatest risk of development of coronary artery aneurysms and long-term sequelae of the disease. No effective treatment for these patients with refractory disease has been established, but the use of corticosteroids is thought to be contraindicated. Prednisone and related medications are the treatment of choice in other forms of vasculitis, but they are considered to be unsafe in KD.6 This conclusion is based

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☆

From the Department of Medicine, Division of Immunology (Rheumatology), and the Department of Cardiology, Children's Hospital, and the Department of Pediatrics, Harvard Medical School, Boston, Massachusetts

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Reprint requests: Dowain A. Wright, MD, PhD, Division of Immunology, Children's Hospital, 300 Longwood, Ave., Enders 8, Boston, MA 02115.

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