Primary focal segmental glomerular sclerosis in children: clinical course and prognosis

Pediatr Nephrol. 2007 Mar;22(3):389-95. doi: 10.1007/s00467-006-0301-5. Epub 2006 Oct 21.


To review the clinical course and identify prognostic factors, we retrospectively analyzed 92 children with steroid-resistant primary focal segmental glomerulosclerosis (FSGS). The mean age of onset was 80.4+/-42.4 months. The mean follow-up duration was 98.2+/-63.3 months. Eighty-five patients presented with nephrotic syndrome and seven presented with asymptomatic proteinuria. Thirty-three patients were initial responders to steroid treatment (late non-responders) and 59 were initial nonresponders. At last follow-up, 36 patients (39.1%) were in complete remission, and 29 (31.5%) progressed to chronic renal failure (CRF). Renal survival rates at 5, 10, and 15 years were 84, 64, and 53%, respectively. By morphological classification, there were tip variants (6.1%), collapsing variants (10.6%), cellular variants (1.5%), perihilar variants (9.1%), and NOS (not otherwise specified, 72.7%). Among the variants, there were no significant differences in age of onset, degree of proteinuria, response to treatment, or progression to CRF. Poor prognostic factors for CRF included: asymptomatic proteinuria at presentation, initial renal insufficiency, higher segmental sclerosis (%), severe tubulointerstitial change, initial nonresponse, and absence of remission. In the multivariate analysis, an increase in the initial serum creatinine and resistance to treatment were independent risk factors for CRF. A more prolonged use of corticosteroid therapy and early introduction of cyclosporin A (CsA) may improve the prognosis for primary FSGS in patients with initial steroid nonresponsiveness.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Disease Progression
  • Drug Resistance
  • Female
  • Glomerulosclerosis, Focal Segmental / complications
  • Glomerulosclerosis, Focal Segmental / drug therapy
  • Glomerulosclerosis, Focal Segmental / pathology*
  • Glucocorticoids / therapeutic use
  • Humans
  • Infant
  • Infant, Newborn
  • Kidney Failure, Chronic / etiology
  • Male
  • Nephrotic Syndrome / etiology
  • Prednisolone / therapeutic use
  • Prognosis
  • Proteinuria / etiology
  • Remission Induction
  • Risk Factors


  • Glucocorticoids
  • Prednisolone