Long-term Follow-up of Adult-onset Minimal Change Nephrotic Syndrome

Chang, Kuk Jin; Park, Sung Bae; Kim, Hyun Chul

Abstract

BACKGROUND
Adult-onset minimal change nephrotic syndrome has been associated with a good response to corticosteroids and a benign prognosis. However, there are few longterm outcome data reported. METHODS: A series of 355 patients with adult-onset minimal change nephrotic syndrome (MCNS) who were admitted to Keimyung University Dongsan Medical Center from December 1978 to May 2002, was retrospectively analyzed to evaluate the initial response to corticosteroids, subsequent response to cytotoxic agents, the stability of remission, and long-term follow-up outcome. RESULTS: Of the 145 patients who followed up greater than two years, 69 patients (47.6%) showed early relapse, late relapser in 54 patients (37.2%), and no relapse in 22 patients (15.2%). Early relapsers showed younger age at onset, more frequent relapse, and longer duration of total corticosteroid treatment and shorter duration of corticosteroid used duration at relapse. Alkylating agents or cyclosporine were administered to 45 patients (19 steroid dependent, 13 frequent relapses, 8 steroid side effect, 2 infrequent relapses, 3 steroid resistant), 36 patients achieved complete remission, five in partial remission, and four in no response at all. Among 29 patients who were treated with cyclosporine, 23 patients showed complete remission, 4 achieved partial remission, and 2 showed no response. Final outcome of 145 patients who were followed for at least more than 2 years, were cyclosporine A dependent in 19 (13.2%) patients. steroid dependent in 16 (11.0%), persistent remission in 57 (39.3%), infrequent relapse in 49 (33.8%), death 2 (1.3%), renal failure 1 (0.7%), and no response to any therapy in 1 (0.7%). CONCLUSION: Adult-onset minimal change nephrotic syndrome shows favorable response to corticosteroid therapy with good long-term outcome and rarely progress to end-stage renal failure. However, new treatment regimens to minimize drug-related side effects and to maintain longer remission period should be evaluated prospectively.