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Related Experiment Videos

Idiopathic collapsing focal segmental glomerulosclerosis: a clinicopathologic study

A Valeri1, L Barisoni, G B Appel

  • 1Department of Medicine, Columbia University, College of Physicians and Surgeons, New York, New York, USA.

Kidney International
|November 1, 1996
PubMed
Summary

Idiopathic collapsing FSGS, a kidney disease variant, is increasing and affects Black individuals. It progresses rapidly to kidney failure and shows resistance to steroid treatment.

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Area of Science:

  • Nephrology
  • Pathology

Background:

  • Idiopathic focal segmental glomerulosclerosis (FSGS) is a significant cause of kidney disease.
  • A specific variant, collapsing FSGS, has shown an increasing incidence.
  • This variant lacks association with HIV-1 infection or intravenous drug use.

Purpose of the Study:

  • To characterize the clinicopathologic features and clinical course of idiopathic collapsing FSGS.
  • To compare idiopathic collapsing FSGS with typical idiopathic FSGS.
  • To identify predictors of progression to end-stage renal disease (ESRD).

Main Methods:

  • Retrospective review of native kidney biopsies from 1974 to 1993.
  • Identification and comparison of 43 cases of idiopathic collapsing FSGS with 50 age-matched controls with typical idiopathic FSGS.

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  • Analysis of clinical data, including serum creatinine, proteinuria, and time to ESRD.

    • Main Results:

    • Idiopathic collapsing FSGS showed a predilection for Black patients and presented with higher serum creatinine and more severe nephrotic syndrome.
    • Morphologic analysis revealed increased visceral epithelial cell changes, tubular microcysts, and interstitial edema in collapsing FSGS.
    • The median time to ESRD was significantly shorter in collapsing FSGS (13.0 months) compared to controls (62.5 months).
    • Higher initial serum creatinine and lack of proteinuria remission were associated with progression to ESRD.
    • Steroid treatment was largely ineffective, with no significant response observed in 26 treated patients.

    Conclusions:

    • Idiopathic collapsing FSGS is an emerging variant of FSGS with distinct, aggressive clinicopathologic features.
    • It is characterized by black racial predominance, rapid progression to ESRD, and resistance to steroid therapy.
    • Understanding these features is crucial for patient management and prognosis.