Recent clinical trials have shown a beneficial effect of
mizoribine (Miz), an immunosuppressive
drug, in the treatment of new-onset pediatric
IgA nephropathy (IgAN). In this study, we evaluated the efficacy of Miz treatment in three children with established
steroid-resistant IgAN. The patients had IgAN featuring persistent
proteinuria and diffuse mesangial proliferation and had failed to respond to 2 years of treatment with
prednisolone. Based upon the second biopsy results, patients were given
methylprednisolone (mPSL) pulse
therapy that induced a transient reduction in
proteinuria, which was reversed when the mPSL dose was tapered. Miz
therapy was then instigated in place of pulse mPSL. All three patients showed a substantial reduction in
proteinuria and resolution of
hematuria within 5 months. A follow-up biopsy in two of the patients showed a substantial reduction in the severity of glomerular lesions and a decrease in the number of activated macrophages. In conclusion, Miz
therapy was found to be a safe and effective
therapy in three cases of
steroid-resistant pediatric IgAN. The ability of Miz to reduce the number of activated macrophages may be an important mechanism by which this
drug ameliorated renal disease in these patients.