Utility of the 2018 revised ISN/RPS thresholds for glomerular crescents in childhood-onset lupus nephritis: a Pediatric Nephrology Research Consortium study

Pooja Patel, Marietta de Guzman, M. John Hicks, Joseph G. Maliakkal, Michelle N. Rheault, David T. Selewski, Katherine Twombley, Jason M. Misurac, Cheryl L. Tran, Alexandru R. Constantinescu, Ali M. Onder, Meredith Seamon, Wacharee Seeherunvong, Vaishali Singh, Cynthia Pan, Daryl M. Okamura, Abiodun Omoloja, Mahmoud Kallash, William E. Smoyer, Guillermo HidalgoScott E. Wenderfer

Research output: Contribution to journalArticlepeer-review

Abstract

Background: The revised 2018 ISN/RPS Classification System for lupus nephritis (LN) includes calculations for both activity index (A.I.) and chronicity index (C.I.). Unchanged were the thresholds of < 25%, 25–50%, and > 50% crescents to distinguish between mild, moderate, and severe activity/chronicity. We aimed to evaluate these thresholds for percent crescents in childhood-onset LN. Methods: Eighty-six subjects < 21 years of age were enrolled from the Pediatric Glomerulonephritis with Crescents Registry, a retrospective multi-center cohort sponsored by the Pediatric Nephrology Research Consortium. Thresholds of 10%, 25%, and 50% for both cellular/fibrocellular and fibrous crescents were interrogated for primary outcomes of kidney failure, eGFR, and eGFR slope. Results: Median age at time of initial biopsy was 14 years (range 1–21). Median follow-up time was 3 years (range 1–11). Cumulative incidence of kidney failure was 6% at 1 year and 10% at latest follow-up. Median eGFR slope was − 18 mL/1.73 m2/min (IQR − 51 to + 8) at 1 year and − 3 mL/min/1.73 m2/year (IQR − 19 to + 6) at latest follow-up. We found no difference in kidney failure at the proposed < 25% and 25–50% cellular crescents thresholds, and thus added a new provisional threshold of 10% that better predicted outcomes in children. Moreover, use of 10% and 25% thresholds for fibrous crescents showed a fourfold and sevenfold increase in risk of kidney failure. Conclusions: In children with crescentic LN, use of 10% and 25% thresholds for cellular crescents better reflects disease activity, while these thresholds for fibrous crescents better discriminates kidney disease outcomes. Graphical abstract: A higher resolution version of the Graphical abstract is available as Supplementary information [Figure not available: see fulltext.]

Original languageEnglish (US)
JournalPediatric Nephrology
DOIs
StateAccepted/In press - 2022
Externally publishedYes

Keywords

  • Biopsy
  • Crescentic glomerulonephritis
  • Lupus
  • Nephritis
  • Outcomes
  • Pediatric

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Nephrology

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