Clinical picture and outcome of primary focal segmental glomerulosclerosis

Patients with primary focal segmental glomerulosclerosis (FSGS present with proteinuria (often the nephrotic syndrome), microscopic haematuria, hypert ension and renal insufficiency. Overall, this glomerular lesion is seen in similar to 20% of nephrotic adults and children, but is observed much more commonly in the black than the white population (prevalence as high as 80%) . Characteristically, nephrotic patients, particularly those with massive p roteinuria, have a significantly poorer prognosis than non-nephrotic patien ts, with 50% progressing to end-stage renal disease (ESRD) over 3-8 years a s compared with a 10-year survival of > 80%, respectively. III addition, th e recurrence rate of this lesion is high in transplanted patients with prim ary FSGS. When clinical and histological features at presentation have been evaluated by multivariate analysis, the significant positive predictors of progression to ESRD have consistently been the serum creatinine (> 1.3 mg/ dl), amount of proteinuria and the presence of interstitial fibrosis (great er than or equal to 20%). The only factor found to be a significant negativ e predictor of progression to ESRD has been the achievement of a remission in proteinuria. Unfortunately, spontaneous remissions are rare in FSGS, occ urring in less than or equal to 6% of patients. The factor identified as mo st associated with achieving a remission in nephrotic patients with primary FSGS has been treatment.