Clinical picture and outcome of primary focal segmental glomerulosclerosis

Abstract

Patients with primary focal segmental glomerulosclerosis (FSGS) present with proteinuria (often the nephrotic syndrome), microscopic haematuria, hypertension and renal insufficiency. Overall, this glomerular lesion is seen in approximately 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 proteinuria, have a significantly poorer prognosis than non-nephrotic patients, with 50% progressing to end-stage renal disease (ESRD) over 3-8 years as compared with a 10-year survival of >80%, respectively. In addition, the recurrence rate of this lesion is high in transplanted patients with primary 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 (> or =20%). The only factor found to be a significant negative predictor of progression to ESRD has been the achievement of a remission in proteinuria. Unfortunately, spontaneous remissions are rare in FSGS, occurring in < or =6% of patients. The factor identified as most associated with achieving a remission in nephrotic patients with primary FSGS has been treatment.