Cyclosporine experience in nephrotic syndrome

To analyze the use of Cyclosporine (CyA) in nephrotic syndrome.

Thirty five children of mean age of 5.9 years with steroid dependent (n = 26) or steroid resistant (n = 9) primary nephrotic syndrome with normal renal functions and who received CyA were studied. CyA was used at a dosage of 6-7 mg/kg/day orally in two divided doses. The mean duration of therapy was 9.6 weeks. All received a minimum of 8 weeks of CyA therapy. In a few who received longer therapy, the dose was reduced to 4 mg/kg/day. All patients were monitored serially for hepatotoxicity and nephrotoxicity. The nephrotic state was evaluated serially with biochemical tests and followed up for a mean period of 2.55 years.

Thirty one patients completed the study. The response to therapy was categorized into 5 groups-no response (4 patients), good response (4 patients), partial response (4 patients), cyclosporine dependence (16 patients), and infrequent relapsers (3 patients). Good response was defined as complete remission lasting for at least one year after cessation of therapy. Patients who showed partial response had reduction in quantitative proteinuria and needed less diuretics. Sixteen patients went into complete remission while on therapy but relapsed within 3 months of discontinuation (CyA dependence). The response to CyA correlated more with steroid-responsiveness than with the underlying histopathology. The drug was well tolerated.

In steroid-dependent or steroid-resistant nephrotic children with normal renal functions, CyA therapy may be considered as one of the possible therapeutic options. Our results suggest that a longer duration of CyA therapy may possibly be indicated in these cases.