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The New Histopathologic Classification of ANCA-Associated GN and Its Association with Renal Outcomes in Childhood
Authors:Damien G. Noone  Marinka Twilt  Wesley N. Hayes  Paul S. Thorner  Susanne Benseler  Ronald M Laxer  Rulan S. Parekh  Diane Hebert
Affiliation:*Divisions of Nephrology.;Rheumatology, and;Pathology.;§The Research Institute, and ;**Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, Ontario, Canada, and ;Department of Pathobiology and Laboratory Medicine and;Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada
Abstract:

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 a median of 2.4 years. The biopsy specimens were 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 compared with the focal category. The probability of having an eGFR>60 ml/min per 1.73 m2 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.
Keywords:ANCA   glomerulonephritis   pediatric nephrology   renal biopsy   vasculitis
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