Clinical course of 110 children and adolescents with primary focal segmental glomerulosclerosis

Abstract

The purpose of this retrospective cohort study was to report the clinical course of children and adolescents with primary focal segmental glomerulosclerosis (FSGS). The records of 110 patients with biopsy-proven FSGS admitted between 1972 and 2004 were retrospectively reviewed. Demographic, clinical and laboratory data were recorded and histopathological data were reanalyzed by one pathologist who had no information about the outcome of the patients. Renal survival analysis was performed using the Kaplan-Meier method. Differences between subgroups (response to corticosteroids) were assessed by the two-sided log rank test. The median age at admission was 5 years (range: 1-15 years). Forty-two patients (38.2%) presented with hematuria at admission, and 55 (50%) presented blood pressure levels above the 95th percentile. Mean follow-up time was 10 years (SD 5.5). Twenty-four patients (21.8%) presented chronic kidney disease (CKD). It was estimated that the probability of CKD was 8% at 5 years, 17% at 10 years, and 32% at 15 years after diagnosis of nephrotic syndrome. In conclusion, on the basis of the clinical and histological characteristics observed, apparently our cohort of idiopathic FSGS is comparable with other published series. However, the long-term overall renal survival seems to be better in our cohort.

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

“…In a subgroup in which the mean follow-up period was almost 7 yr, the clinical features remained generally favorable during the entire observation period. These results are consistent with those of Abrantes et al (13) and Paik et al (17), who showed higher rates of renal survival in children with FSGS compared with previous studies in children and adults. The improved short-term outcome among the three recent pediatric studies is Figure 1.…”

“…Indeed, approximately 30 to 40% of adult patients who develop FSGS will experience significant renal insufficiency within the first decade after diagnosis (7), although there is evidence that the prognosis may be less ominous in childhood primary FSGS. For example, Abrantes et al (13) showed that the probability of severe renal impairment in childhood FSGS is 8% at 5 yr, 17% at 10 yr, and 32% at 15 yr, which confirmed the data in another study of childhood FSGS (21). However, other studies in pediatric FSGS suggest a grimmer prognosis.…”

“…First, approximately 16% of their patients did not have the classical segmental scar that many consider to be essential for the diagnosis of FSGS. Second, the age of onset of disease (5 yr) in their study population was significantly younger than that in other studies of pediatric FSGS (13). Another recent pediatric study showed that the majority (72%) of children with FSGS had the NOS variant, and there were no clinical indicators that differentiated the variants (17).…”

“…To this end, the Columbia classification of primary FSGS was recently defined and prioritized into a hierarchy of five distinct pathologic variants: (1) Collapsing, (2) tip lesion, (3) cellular, (4) hilar, and (5) not otherwise specified (NOS) (9,10). Clinical heterogeneity of FSGS may be explained, in part, by the presence of these pathologic variants (1,2,(11)(12)(13).…”

Tip variant of focal segmental glomerulosclerosis: is it truly a benign variant?

“…5,6 Various histological variants of FSGS have been described in the past and attempts have been made to co-relate the longterm outcomes of patients with the histopathological variant. 1,[7][8][9][10] The Columbia classification of primary FSGS has defined and classified the variants into five subtypes including (1) collapsing, (2) tip lesion, (3) cellular, (4) hilar and (5) not otherwise specified (NOS). 1,7 Various studies have proved that the histological variants do have a significant impact on the long-term outcome of the patient.…”