[Clinicopathological study of 212 children with primary focal segmental glomerular sclerosis]

To evaluate the correlation between clinico-pathological features and outcome of children with primary focal segmental glomerular sclerosis (FSGS).

A total of 212 pediatric patients with D'Agati (2004) primary FSGS were included in this study between 1997 and 2008. According to FSGS histologic classification criteria, 5 pathologic variants were recognized: collapsing (COLL), cellular (CELL), glomerular tip lesion (GTL), perihilar, and not otherwise specified (NOS). Retrospective analysis of the therapeutic response, the relationship between the clinical efficacy and pathology and the outcome of the patients was made.

Of the 212 patients, 178 (83.9%) had nephritic syndrome (NS), 97 (45.8%) had simple NS, 81 (38.2%) had nephritis-type NS, GTL variants were mostly appeared to be nephritic syndrome (n = 28) and COLL variants were the fewest (n = 11). The difference between the two variants had statistical significance (P < 0.05). Fourteen cases (6.6%) had nephrotic proteinuria, 20 cases (9.4%) had proteinuria with micro-hematuria. According to histologic classification, NOS (n = 86, 40.6%) was the most common type; perihilar type was seen in 25 cases (11.8%); CELL was seen in 58 cases (27.4%), COLL in 12 cases (5.6%), GTL in 31 cases (14.6%). Chronic tubular injury was present in most cases. CEL variants were mostly found in the early infancy. GTL and NOS variants initially appeared to be responsive to steroids, but subsequently became resistant or frequently recurrent; CELL and COLL appeared to be primarily steroid resistant, GTL and COLL variants had statistically significant differences (P < 0.05). The patients were followed-up for 5 months to 10 years. A response to therapy was observed in 50%, COLL FSGS had the highest rate of ESRD; 2 years renal survival rates were 67%, 3 years were 41%.

FSGS is defined as a clinicopathologic syndrome manifesting proteinuria and focal and segmental glomerular sclerosis with foot process effacement. The location of the sclerosis within the glomeruli proved to have prognostic significance. Collapsing glomerulopathy is the most aggressive variant of FSGS. Compared with other variants, GTL variant may be the best type. Different histologic variants of FSGS have substantial differences in clinical features at the time of biopsy diagnosis and substantial differences in renal outcomes. Prolonged treatment of FSGS-NS with corticosteroids and immune suppressive agents may have some effects in achieving sustained remission and improve prognosis in children.