Pooja Patel1,2,3, Marietta de Guzman1,2, M John Hicks2,4, Joseph G Maliakkal5, Michelle N Rheault6, David T Selewski7, Katherine Twombley7, Jason M Misurac8, Cheryl L Tran9, Alexandru R Constantinescu10, Ali M Onder11, Meredith Seamon12, Wacharee Seeherunvong13, Vaishali Singh14, Cynthia Pan14, Daryl M Okamura15, Abiodun Omoloja16, Mahmoud Kallash17, William E Smoyer17, Guillermo Hidalgo18, Scott E Wenderfer19,20. 1. Pediatric Rheumatology, Texas Children's Hospital, TX, Houston, USA. 2. Baylor College of Medicine, Houston, TX, USA. 3. Pediatric Rheumatology, Northwestern University, Chicago, IL, USA. 4. Pathology, Texas Children's Hospital, Houston, TX, USA. 5. Pediatric Nephrology, Saint Louis University, St. Louis, MO, USA. 6. Pediatric Nephrology, University of Minnesota, Minneapolis, MN, USA. 7. Pediatric Nephrology, Medical University of South Carolina, Charleston, SC, USA. 8. Pediatric Nephrology, University of Iowa, Iowa City, IA, USA. 9. Pediatric Nephrology, Mayo Clinic, Rochester, MN, USA. 10. Pediatric Nephrology, Joe DiMaggio Children's Hospital, Hollywood, FL, USA. 11. Pediatric Nephrology, University of Mississippi, Jackson, MS, USA. 12. Pediatric Nephrology, University of Utah, Salt Lake City, UT, USA. 13. Pediatric Nephrology, University of Miami, Miami, FL, USA. 14. Pediatric Nephrology, Medical College of Wisconsin, Milwaukee, WI, USA. 15. Pediatric Nephrology, University of Washington, Seattle Children's Hospital, Seattle, WA, USA. 16. Pediatric Nephrology, Wright State University, Dayton, OH, USA. 17. Pediatric Nephrology, Nationwide Children's Hospital, Columbus, OH, USA. 18. Pediatric Nephrology, Hackensack Meridian, Neptune, NJ, USA. 19. Baylor College of Medicine, Houston, TX, USA. scott.wenderfer@cw.bc.ca. 20. Pediatric Nephrology, Texas Children's Hospital, Houston, TX, USA. scott.wenderfer@cw.bc.ca.
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. A higher resolution version of the Graphical abstract is available as Supplementary information.
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. A higher resolution version of the Graphical abstract is available as Supplementary information.
Authors: Linda T Hiraki; Candace H Feldman; Jun Liu; Graciela S Alarcón; Michael A Fischer; Wolfgang C Winkelmayer; Karen H Costenbader Journal: Arthritis Rheum Date: 2012-08
Authors: Ingeborg M Bajema; Suzanne Wilhelmus; Charles E Alpers; Jan A Bruijn; Robert B Colvin; H Terence Cook; Vivette D D'Agati; Franco Ferrario; Mark Haas; J Charles Jennette; Kensuke Joh; Cynthia C Nast; Laure-Hélène Noël; Emilie C Rijnink; Ian S D Roberts; Surya V Seshan; Sanjeev Sethi; Agnes B Fogo Journal: Kidney Int Date: 2018-02-16 Impact factor: 10.612
Authors: Jan J Weening; Vivette D D'Agati; Melvin M Schwartz; Surya V Seshan; Charles E Alpers; Gerald B Appel; James E Balow; Jan A Bruijn; Terence Cook; Franco Ferrario; Agnes B Fogo; Ellen M Ginzler; Lee Hebert; Gary Hill; Prue Hill; J Charles Jennette; Norella C Kong; Philippe Lesavre; Michael Lockshin; Lai-Meng Looi; Hirofumi Makino; Luiz A Moura; Michio Nagata Journal: J Am Soc Nephrol Date: 2004-02 Impact factor: 10.121