Severe, Complicated Kawasaki Disease Treated With Corticosteroids

Abstract

Source: Dale RC, Saleem MA, Daw S, Dillon MJ. Treatment of severe complicated Kawasaki disease with oral prednisolone and aspirin. J Pediatr. 2000;137:723–726.Dale et al from the Great Ormond Street Hospital for Children in London report on 7 patients with severe Kawasaki Disease (KD) complicated by prolonged fever, cardiac failure, or progression of coronary lesions who were treated with oral prednisolone. Six of these patients had failed to respond to treatment with intravenous immunoglobulin (IVIG) as judged by continued fever and progressive cardiovascular disease. An eighth patient, a Jehovah’s Witness with religious objections to IVIG, was treated with oral prednisolone and aspirin as primary therapy. Oral prednisolone was administered as 2mg/kg/day for 2 weeks and then was weaned over a 6-week period. All patients received aspirin 30mg/kg/d in the acute phase and 5mg/kg/d in the convalescent phase. Four of the 6 patients who failed to respond to initial treatment with IVIG had received 2 doses of IVIG (2gm/kg) prior to prednisolone, while the other 2 patients received their second IVIG dose at the time prednisolone was begun. An additional patient, a 3-month-old child, developed peripheral gangrene with autoamputation of 3 digits in association with a giant right coronary aneurysm prior to any treatment and was given both IVIG and oral prednisolone as delayed primary therapy. Seven of the 8 patients became afebrile within 24–72 hours of initiation of prednisolone and had no further progression of coronary dilation. None of the 8 patients had further acute vasculitic episodes. Five of 7 patients developed coronary artery abnormalities that persisted as long as 5 years; 3 of the 5 had persistent giant coronary abnormalities. The authors conclude that for selected patients at the severe end of the KD spectrum oral steroids may be beneficial, and that re-appraisal of the role of steroids in KD is appropriate.Since the landmark study by Kato et al in 1979 that appeared to show a detrimental effect of steroids in KD,1 these agents have generally been considered contraindicated in KD by most authorities. Over the past several years, however, a few reports of the use of pulsed intravenous methylprednisolone (30mg/kg/d x 1–3 doses) as second-line therapy in patients failing IVIG treatment have appeared.2 The report of Dale and colleagues is among the first to suggest the use of a relatively long course of oral steroids for complicated or refractory cases of KD. Those who support the use of steroids in KD point out that these agents are the mainstays of therapy for other vasculitides. Although clearly a vasculitis, KD differs from other forms of vasculitis by its acute, self-limited nature and by the epidemiologic and immunopathologic features that strongly suggest an infectious etiology.3 That steroids could be detrimental in such circumstances is potentially worrisome.It is reassuring that, in the relatively small number of reported KD patients treated with steroids to date, untoward effects apparently have been few. Nevertheless, IVIG and aspirin should remain standard primary therapy for KD and treatment failures should be retreated at least once with 2gm/kg IVIG prior to serious consideration of oral or intravenous steroids. Until results of additional randomized treatment trials are available, the latter should be reserved for the rare patient with severe disease associated with continued or recurrent active inflammation as manifested by persistent fever and other clinical signs.