The course of 6 patients with childhood systemic lupus erythematosus was analysed with emphasis on the effect and limitation of intravenous methylprednisolone pulse therapy in the long-term follow-up. The present findings suggest that using pulse therapy within 2 months in the course of disease flares, probably before irreversible organ damage has occurred, may be more beneficial in suppressing the disease activity and maintaining long-term remission. The demonstration of low serum complement activity (CH 50), and in some cases, proteinuria, was useful to decide the time of pulse therapy. Complications of pulse methylprednisolone plus long-term oral prednisolone therapy included cushingoid appearance and low body height in addition to cataract and glaucoma, all of which were attributable not to methylprednisolone pulse therapy alone but to long-term accumulation of corticosteroids. The combination therapy including several immunosuppressants needs to be established in order to avoid these steroidal complications.