Long-term cyclosporin A treatment of minimal-change nephrotic syndrome of childhood

We evaluated the efficacy of long-term cyclosporin A (CyA) treatment in the maintenance of remission in 40 children with steroid-dependent minimal-change nephrotic syndrome (MCNS). CyA was given in an initial dose of 5 mg/kg per day, adjusted to maintain a trough whole blood level of 50–150 ng/ml. All the 40 children received CyA for 1 year. In 18 patients, CyA was continued for a further period of at least a year without interruption; 9 patients had a second course of CyA therapy after an interval of at least 1 month. Of the 40 children 29 (72%) had one or more relapses during treatment with CyA, with 16 (40%) relapsing during the 1st year. During the second period of CyA, 10 (56%) of the 18 children treated continuously relapsed, whereas all the 9 children who had an interrupted course of therapy relapsed. CyA was discontinued at one time in 27 patients, all of whom subsequently relapsed, with a median time to relapse of 26 days. Long-term prednisolone in addition to CyA was required to maintain remission in 16 (40%) of the whole group. The results suggest that the long-term use of CyA is able to maintain remission of MCNS, although 40% of the patients also required low-dose alternate-day steroids; patients appeared to fare worse if the CyA course was interrupted; no patient experienced a long-term remission after CyA was stopped.