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

3 Scopus citations


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 m 2/min (IQR - 51 to + 8) at 1 year and - 3 mL/min/1.73 m 2/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. A higher resolution version of the Graphical abstract is available as Supplementary information.

Original languageEnglish (US)
Pages (from-to)3139-3145
Number of pages7
JournalPediatric Nephrology
Issue number12
StatePublished - Dec 2022

Bibliographical note

Funding Information:
Creation of the Pediatric Glomerulonephritis with Crescents Registry was funded in part by the Department of Pediatrics at East Carolina University and the Brody School of Medicine. The lead site for the registry is now Hackensack Meridian Health. The Pediatric Nephrology Research Consortium did not provide funding for this investigator-initiated study. The authors would like to thank all study coordinators and data managers at participating consortia sites for their work to generate the Pediatric Glomerulonephritis with Crescents Registry: Nancy Darden-Saad, Alice Raad, Maurice Smith and Julia Fish at East Carolina University; Corinna Bowers, Marcia Dyas, Stephanie Webster-Cheng, Beth Burkhart, Lisa Fuerer, Amy Dutcher and Sarah Higgins at Nationwide Children’s Hospital (data coordinating center); Margaret Kamel and Eme Anderson at Emory University; Belkis Wandique-Rapalo at Joe DiMaggio Children’s Hospital; Theresa Kump at Medical College of Wisconsin; Patricia Infinger at Medical University of South Carolina; Margaret Mahaffa at University of Iowa; Barbara Smith at University of Michigan; Joann Narus and Richard Drake at University of Utah; Verena Aziz and Megan Kelton at Seattle Children’s Hospital; and Sherry Kanosky at West Virginia University. Dr. Alvaro Orjuela is now the primary site investigator at Baylor College of Medicine.

Publisher Copyright:
© 2022, The Author(s), under exclusive licence to International Pediatric Nephrology Association.


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

PubMed: MeSH publication types

  • Journal Article
  • Multicenter Study
  • Research Support, Non-U.S. Gov't


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