Mm. Schwartz et al., PRIMARY FOCAL SEGMENTAL GLOMERULAR SCLEROSIS IN ADULTS - PROGNOSTIC VALUE OF HISTOLOGIC VARIANTS, American journal of kidney diseases, 25(6), 1995, pp. 845-852
Primary focal segmental glomerular sclerosis (FSGS) is a clinicopathol
ogic syndrome in which variable amounts of proteinuria are associated
with the renal biopsy finding of segmental glomerular scarring in some
, but not all, of the glomeruli. Additional histologic features have b
een described in FSGS, including the position of the scar relative to
the vascular and tubular pole of the glomerulus, foam cells, hyalinosi
s, mesangial deposits of immunoglobulin M, diffuse mesangial hypercell
ularity, glomerular visceral epithelial cell hyperplasia and hypertrop
hy, and the extent of associated interstitial fibrosis and tubular atr
ophy. We performed a retrospective study on 81 patients with biopsy-pr
oven, primary FSGS to determine whether any of the histologic features
of FSGS correlated with renal function at the time of biopsy and the
incidence of end-stage renal disease at follow-up. Sixty patients were
nephrotic and 21 had nonnephrotic proteinuria. Only the degree of int
erstitial fibrosis correlated with the initial serum creatinine (r = 0
.536) and none of the histologic features predicted the presence of ne
phrotic-range proteinuria at the time of biopsy. Segmental scars invol
ved 21% +/- 14% of the glomeruli per biopsy specimen, but their positi
on within the glomerulus was uniform in only 13% of the cases. Diffuse
mesangial hypercellularity was present in 17% of the biopsy specimens
, and glomerular epithelial cell lesions were present in 57% of the bi
opsy specimens. Multivariate analysis showed that only the degree of i
nterstitial fibrosis predicted end-stage renal disease in all 81 patie
nts and in the 60 patients with nephrotic-range proteinuria. The curre
nt data do not support different therapeutic approaches in primary FSG
S based on histologic subtypes. (C) 1995 by the National Kidney Founda
tion, Inc.