FOCAL SEGMENTAL GLOMERULAR SCLEROSIS IN ADULTS - PRESENTATION, COURSE, AND RESPONSE TO TREATMENT

The authors performed a retrospective clinicopathologic study in 81 pa tients with primary focal segmental glomerular sclerosis (FSGS) to det ermine whether they could identify clinical or histologic features at presentation that could be predictive of outcome and response to thera py. Males constituted 58% of patients, and 53% were black. At biopsy t he patients were 40 +/- 17 years old; 74% were nephrotic, and renal in sufficiency was present in 62%. The average time from presentation to biopsy was 16 months, and the average total follow-up was 62 months. N ephrotic patients had a significantly poorer prognosis as compared wit h nonnephrotic patients (5- and 10-year survivals of 76% and 57% v 92% and 92%; P < 0.05). A multivariate analysis was done on histologic an d clinical features at biopsy, assessing for risk factors leading to e nd-stage renal disease, showing only the serum creatinine and the degr ee of interstitial fibrosis to have a significant correlation. Thirty nephrotic patients received prednisone, with a treatment time of 5.5 /- 4 months and a total dose of 5.9 +/- 2.9 g per course of treatment. Fifteen of these patients (50%) achieved a remission by 3.7 +/- 2 mon ths (10 complete remission and 5 partial remissions), with all patient s responding within 9 months. Only two patients had spontaneous remiss ions (both partial). The 5- and 10-year survival for patients in remis sion were both 100% as compared with 66% and 41% (P < 0.01), respectiv ely, for nephrotic patients not in remission. No clinical feature at p resentation or biopsy was predictive of response to therapy when a mul tivariate analysis was performed. Nephrotic patients responding to the rapy have a significantly better prognosis than nonresponsive patients . Response to therapy requires at least 3 to 4 months of treatment wit h prednisone. Unfortunately, no clinical feature at presentation or bi opsy predicts which patients are more likely to respond to treatment. This experience suggests that nephrotic patients with primary FSGS may benefit from a more prolonged course of therapy with prednisone. Furt hermore, these findings underscore the need for a controlled trial in nephrotic patients with FSGS to confirm the response to and establish guidelines for therapy. (C) 1995 by the National Kidney Foundation, In c.