Forty cases of focal sclerosing glomerulonephropathy with
nephrotic syndrome or
proteinuria were studied retrospectively in regard to clinical presentation, response to
steroid therapy and
clinical course, and histopathology of the lesion. Morphologically there was a focal segmental and global
sclerosis with subendothelial hyaline deposits, collapse of the capillary loops, intracapillary hyaline material or foam cells, filling and widening of the mesangium with mesangial matrix, focal tubular
atrophy, and focal interstitial
fibrosis. Thirty-four patients had been treated with
prednisone; initial complete remission of the
nephrotic syndrome occurred in only 4 patients and partial remission in 10. Nine of these 14 patients had nephrotic relapse or became resistant to
steroids. Thirty-three percent of the patients progressed to
end-stage renal failure and an additional 25 percent had impairment of renal function after a mean of 8 years from onset. Three patients received kidney allografts, and in two the disease recurred in the transplanted kidney. Focal sclerosing glomerulonephropathy associated with
nephrotic syndrome or
proteinuria appears to be a clinicopathologic entity characterized by resistance to
steroid treatment, frequent progression to
end-stage renal disease, and recurrence in the transplanted kidney.