The new histopathologic classification of ANCA-associated gn and its association with renal outcomes in childhood

Damien G. Noone, Marinka Twilt, Wesley N. Hayes, Paul S. Thorner, Susanne Benseler, Ronald M. Laxer, Rulan S. Parekh, Diane Hebert

Research output: Contribution to journalArticlepeer-review

33 Scopus citations


Background and objectives A proposed histopathologic classification for ANCA-associated GN is predictive of long-term renal outcome in adult populations. This study sought to validate this system in a pediatric cohort. Design, setting, participants, & measurements This was a retrospective, single-center, cohort study of 40 children diagnosed and followed until their transition to adult care at one institution between 1987 and 2012. Renal biopsy specimens were reviewed by a pathologist blinded to patient outcome and were classified using the new histopathologic classification system of focal, crescentic, mixed, and sclerotic groups. Time to the composite outcome of CKD stages 3 and 4 (determined by eGFR with repeated creatinine measures using the Schwartz equation) or ESRD (defined as dialysis dependence or transplantation) were ascertained. Results The study population consisted of 40 children (70%female), followed for amedian of 2.4 years. The biopsy specimenswere categorized as focal in 13 patients (32.5%), crescentic in 20 (50%),mixed in two (5%), and sclerotic in five (12.5%). Mixed and crescentic were combined for analyses. Survival analysis of time to the composite renal endpoint of at least 3 months of eGFR,60 ml/min per 1.73 m2 or ESRD differed significantly among the three biopsy groups log-rank P,0.001), with an adjusted hazard ratio of 3.14 (95% confidence interval, 0.68 to 14.4) in the crescentic/mixed group and 23.6 (95% confidence interval, 3.9 to 144.2) in the sclerotic category comparedwith the focal category. The probability of having an eGFR.60 ml/min per 1.73m2 at 2 years was 100% for the focal, 56.5% for the crescentic/mixed, and 0% for the sclerotic biopsy categories. Conclusions This study showed the clinical utility of this histopathologic classification system and its ability to discriminate renal outcomes among children with ANCA GN.

Original languageEnglish (US)
Pages (from-to)1684-1691
Number of pages8
JournalClinical Journal of the American Society of Nephrology
Issue number10
StatePublished - 2014
Externally publishedYes

ASJC Scopus subject areas

  • Nephrology
  • Transplantation
  • Epidemiology
  • Critical Care and Intensive Care Medicine
  • General Medicine


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