Clinicopathological studies of 10 cases with focal segmental glomerulosclerosis

The clinical picture, histopathological findings, therapy and prognosis of focal segmental glomerulosclerosis (FSGS) were investigated in a retrospective study involving 10 cases. The age of patient at the time of detection ranged from 3 to 19 years, 11 years on the average, being 9 years or above in 6 cases. There was noted a slight tendency toward predominance of males in this series. The disease was detected casually on the occasion of mass survey of urine at school in 6 cases (60%), while by clinical examination on visiting us with nephrotic syndrome in the other 4 (40%). Of the former 6 cases, 5 developed nephrotic syndrome while placed under medical surveillance. Nine of the 10 cases were treated mainly with corticosteroids, to which 5 (50%) were unresponsive and 4 (40%) responsive, with 1 (10%) of these 4 becoming unresponsive since a recurrence. Corticosteroids were not used in 1 case (10%). During follow-up period (which ranged from 1 to 10 years) 6 experienced an elevation of serum creatinine above 2.0 mg/dl, with 5 of them being unresponsive to corticosteroids and 3 begun on hemodialysis therapy. Histologically, cases in which the sum of the proportions of glomeruli affected with segmental sclerosis and with global sclerosis exceeded 30% and, in addition, there were severe tubulointerstitial lesions tended to have a poor prognosis, while those in which sclerosis involved less than 30% of glomeruli and no interstitial damage was discernible had a relatively favorable progonsis and were more frequently responsive to corticosteroids. These findings led us to conclude that FSGS has an ominous prognosis as reported previously and notably, the prognosis is much poorer for the non-steroid-responding type than for the responding type. The study also suggests that the degree of severity of histological changes is determinant of the prognosis of the disease.