Efficacy of cyclosporine A for steroid-resistant severe Henoch-Schönlein purpura nephritis.

Aggressive treatment is necessary for continuous high-range proteinuria in cases of pediatric Henoch-Schönlein purpura nephritis (HSPN) as the long-term prognosis is sometimes poor. Cyclosporine (CyA) has immunosuppressive effects as well as a very selective inhibitory effect on T-helper cell function. Here we report two 7-year-old boys with steroid-resistant HSPN treated with CyA. After diagnosis of HSPN, we treated both patients with methylprednisolone and urokinase pulse therapy (MUT) combined with multiple drugs; however, high-range proteinuria persisted and CyA was added to the treatment regimen. The proteinuria subsequently decreased gradually and pathological findings at the second renal biopsy were improved. Furthermore, neither patient showed any adverse effects, such as hypertension, encephalopathy, or chronic nephrotoxicity, to the CyA treatment.In conclusion, these results suggest that CyA may be a safe and effective treatment for steroid-resistant severe HSPN.