Nephrology Dialysis Transplantation, Vol 14, Issue 90003 68-73, Copyright © 1999 by Oxford University Press
S Korbet
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 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 (⩾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 ⩽6% of patients. The
factor identified as most associated with achieving a remission in
nephrotic patients with primary FSGS has been treatment.
ORIGINAL ARTICLES
Clinical picture and outcome of primary focal segmental glomerulosclerosis
Section of Nephrology, Department of Medicine, Rush Presbyterian St Lukes Medical Centre, 1653 W Congress Parkway, Chicago, IL 60612, USA
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