Presenting Features and Short-Term Outcome According to Pathologic Variant in Childhood Primary Focal Segmental Glomerulosclerosis

Abstract

Background: This was a retrospective analysis of children in one center who had primary (idiopathic) FSGS. Design, setting, participants, and measurements: There were 41 patients: 34.1% female, 65.9% male, 80.5% black, and 19.5% white. At presentation, the mean age was 10.9 ؎ 0.9 yr. The mean time of follow-up was 3.9 ؎ 0.5 yr.Results: During the observation period, the systolic BP (SBP) and diastolic BP (DBP) remained stable, serum albumin rose slightly, and the GFR was stable. Among those who received corticosteroids at presentation, 21.2% were steroid sensitive. At last follow-up among all patients, 71% were in remission, 78% had stage 1 or 2 chronic kidney disease, and 4.9% had reached ESRD. At last follow-up, the GFR was significantly higher (P ‫؍‬ 0.01) in patients who were initially steroid sensitive. Ethnicity had no effect on clinical data or response to therapy. The pathologic variants were as follows: Cellular, 32%; collapsing, 24%; and not otherwise specified (NOS), 44%. The chronicity scores were as follows: Cellular, 4.3; collapsing 6.4; and NOS, 4.0 (significantly higher, P ‫؍‬ 0.02, in collapsing versus NOS). At presentation, SBP (P ‫؍‬ 0.03) and DBP (P ‫؍‬ 0.03) were significantly higher and GFR was lower (P ‫؍‬ 0.03) in patients with the collapsing compared with NOS variant. Remission after the initial course of corticosteroids was less common with the collapsing variant. At last follow-up, SBP (P ‫؍‬ 0.02) and DBP (P ‫؍‬ 0.04) were significantly higher in patients with the collapsing versus NOS variant.Conclusions: The short-term outcome in pediatric primary FSGS is generally favorable, but a more guarded prognosis exists for patients with collapsing FSGS.

Collapsing Focal Segmental Glomerulosclerosis in a Young Child

“…Collapsing FSGS has been described by many authors from different parts of the world as a cause of SRNS in children [9,12,13,14]. Our patient had high blood pressure at presentation, and this is similar to previous reports of initial high blood pressure in affected children [13].…”

Focal Segmental Glomerulosclerosis

“…The latter might be considered to include familial/genetic forms, virus-associated forms, drug-induced forms, and forms mediated by adaptive structural-functional responses (i.e., in the setting of congenital or acquired reduction of renal mass/nephron complement). Clinical response and prognosis may relate to the histologic variant, most notably the glucocorticioid responsiveness of the tip lesion and the aggressive, unrelenting nature of the collapsing variants (21,22). It is with this in mind that the variants are included in standard pathology reports.…”

Association of Histologic Variants in FSGS Clinical Trial with Presenting Features and Outcomes

“…The FSGS-CT findings support significant differences in demographic features between histologic subtypes. Tip and collapsing variants were more common in teenagers and adults than children (5,6,12,(14)(15)(16)(17)(18)(19). Only 29% of tip patients were children aged 2-12 years at onset of FSGS, whereas 43% were over 18 years.…”

“…These variants include the common generic NOS type, the aggressive collapsing type, the abruptly presenting tip type, the early-stage cellular type, and the perihilar type common in secondary adaptive FSGS (13)(14)(15)(16)(17). Several retrospective series have suggested that FSGS histologic subtypes vary in incidence and correlate with remission status and outcome.…”