Background Kawasaki disease (KD) is an acute multisystem vasculitis predominantly affecting the coronary arteries of still unknown etiology. IV gammaglobulin (IVIG) is the standard treatment to reduce the occurrence of coronary artery abnormalities. Methylprednisolone (MP) added to the standard therapy has been shown to provide no additional benefit in the primary therapy of KD but has a role for IVIG-resistant KD. For developing countries, the financial burden of IVIG poses a significant barrier to treatment. The striking immune perturbances in KD is quite similar to other primary systemic vasculitis which are treated with corticosteroids, hence MP may provide some benefit as a primary therapy for KD. In our institution, parents who cannot afford IVIG consented to intravenous MP as an alternative treatment option.
Objectives To review the immediate and long term outcome of KD patients treated with IV MP as primary therapy.
Methods Retrospective chart review of patients who fulfilled the criteria for the diagnosis of KD and treated with IV pulse MP [30mg/kg/dose (maximum of 1 gm/dose) daily for 3 consecutive days] plus high dose aspirin from January 2006 to December 2010 was done.
Results A total of 22 patients were included in the study. There was clinical improvement in all patients with immediate lysis of fever after the first dose of IV MP and significant decrease in CRP levels. Repeat echocardiography done at 8 weeks, 6 months, 1 year and 2 years from the time of diagnosis by a single pediatric cardiologist revealed no coronary artery abnormalities in all patients. No adverse events reported during and after the infusion.
Conclusions Methylprednisolone is effective in controlling the systemic manifestations and in preventing coronary aneurysm and safe for patients with KD. It can be an alternative first line treatment for patients who cannot afford IVIG.
Disclosure of Interest None declared