Early versus late-onset idiopathic: focal segmental glomerulosclerosis

Glomerular diseases in children, although similar in histological appearanc e to those in adults, may have a better prognosis. There is much controvers y regarding the prognostic factors in idiopathic focal segmental glomerulos clerosis (FSGS), especially the comparative prognosis of children and adult s. A comparative analysis was carried out of 36 consecutive biopsy-proven c ases of idiopathic FSGS presenting early in life ['early onset' as seen in children less than or equal to 12 years (group I)] and 36 cases presenting later ['late-onset' as seen in older children >12 years and adults (group I I)]. Patients were compared for clinical, biochemical, and histopathologica l features, as weil as disease outcome. A significantly higher prevalence o f hypertension (P=0.002) and microscopic hematuria was seen in group II (P= 0.02). There were no differences between the two groups in glomerular filtr ation rates corrected for body surface area at initial presentation (92+/-1 1 ml/min/1.73 m(2) vs. 94+/-14 ml/min/1.73 m(2)). Patients with 'late-onset ' FSGS had a significantly higher number of glomeruli with segmental sclero sis (P=0.007), more mesangial matrix expansion (P=0.009), greater mesangial cellularity (P=0.003), and significantly higher blood vessel involvement ( P=0.03) than those with 'early onset' FSGS. There was a significantly highe r response to steroids in,group I (82.3%) than group II (36.4%) (P<0.02). A t the end of the study period, 2 patients in group I and 11 in group II had developed persistent renal failure (P=0.01). Thus 'early onset' FSGS is mo re common in males, has significantly lower prevalence of hypertension and microscopic hematuria, with less-severe histopathological involvement, is m ore often steroid responsive, and has a better prognosis than 'late-onset' FSGS.