Four-Year Maintenance Therapy with Cyclosporin A for Patients with Steroid-Refractory Idiopathic Minimal Change Disease Allergic to Rituximab

Abstract

Background: Patients with Steroid-Refractory Idiopathic Minimal change disease (SRIM) have high morbidity and mortality and inherent side effects of immunosuppressive therapy. Except for its cost, R has been shown to be the most practical, safe and effective maintenance therapy for patients with multiple glomerulopathy, including those with SRIM. Being a monoclonal antibody, hypersensitivity reactions are common, and its use is limited in 10% of treated patients. Cyclosporin A (Cy) was a useful alternative, yet it had the potential for chronic interstitial fibrosis in the long term. Hence, a safe management protocol, for its use, was sought. Patients and methods: Over the past 10 years a total of 35 patients were treated with Cyc for SRIM, 11 of whom were children (<14 years). Initially all patients were treated with 100 mg twice daily aiming at an initial trough level 100 - 150 ng/ml. Three months later, the dose was reduced to 50 mg twice daily. Two years later the dose was reduced to 25 mg twice daily for the remaining 2 years. Results: Complete remission of disease (proteinuria <150 mg/day) was achieved 1 month after Cyclo-therapy that was maintained till the end of the study despite dose decrement. Within 1 year, subsequent to Cy-therapy, 4 patients relapsed, yet responded to the reinstitution of Cy. Side effects were tolerable and did not require drug discontinuation, and all patients had normal creatinine clearance at follow-up. In conclusion, 4-year treatment with Cy offers a safe and effective alternative to R allergic patients with SRIM.