Renal Survival in Children with Glomerulonephritis with Crescents: A Pediatric Nephrology Research Consortium Cohort Study

• Type of Material: Article
• Authors: Joseph G. Maliakkal, Baylor College of Medicine; M. John Hicks, Texas Childrens Hospital; Mini Michael, Baylor College of Medicine; David T. Selewski, Medical University of South Carolina; Katherine Twombley, Medical University of South Carolina; Michelle N. Rheault, University of Minnesota; Meredith Seamon, University of Utah; Jason M. Misurac, University of Iowa; Cheryl L. Tran, Mayo Clinic; Loretta Reyes, Emory University; Joseph T. Flynn, University of Washington; Ali M. Onder, University of West Virginia; Alexandru R. Constantinescu, Joe DiMaggio Childrens Hospital; Vaishali Singh, Medical College of Wisconsin; Cynthia Pan, Medical College of Wisconsin; Abiodun Omoloja, Wright State University; Qiang Wu, East Carolina University; William E. Smoyer, Ohio State University; Guillermo Hidalgo, East Carolina University; Scott E. Wenderfer, Baylor College of Medicine
• Language: English
• Date: 2020-08-01
• Publisher: MDPI
• Publication Version: Final Published Version
• Copyright Statement: © 2020 by the authors. Licensee MDPI, Basel, Switzerland.
• License: Creative Commons Attribution 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.3390/jcm9082385
• Title of Journal or Parent Work: Journal of Clinical Medicine
• Volume: 9
• Issue: 8
• Start Page: 1
• End Page: 13
• Grant/Funding Information: This research was funded in part by the Department of Pediatrics at East Carolina University and the Brody School of Medicine.; The PNRC sponsored Pediatric Glomerulonephritis with Crescents Registry is an investigator-initiated registry.
• Keywords: fibrous crescents; Multicenter; Science & Technology; Nephropathy; General & Internal Medicine; glomerulonephritis; Spectrum; Medicine, General & Internal; Outcomes; Prediction; targeted biologic therapies; Life Sciences & Biomedicine; Single center; Etiology; cellular crescents; Lupus nephritis; immunosuppression; Rituximab
• Research Categories: Health Sciences, Medicine and Surgery; Health Sciences, Pathology; Health Sciences, Public Health

Abstract

There is no evidence-based definition for diagnosing crescentic glomerulonephritis. The prognostic implications of crescentic lesions on kidney biopsy have not been quantified. Our objective was to determine risk factors for end-stage kidney disease (ESKD) in patients with glomerulonephritis and crescents on kidney biopsy. A query of the Pediatric Nephrology Research Consortium’s Pediatric Glomerulonephritis with Crescents registry identified 305 patients from 15 centers. A retrospective cohort study was performed with ESKD as the primary outcome. Median age at biopsy was 11 years (range 1–21). The percentage of crescents was 3–100% (median 20%). Etiologies included IgA nephropathy (23%), lupus (21%), IgA vasculitis (19%) and ANCA-associated GN (13%), post-infectious GN (5%), and anti-glomerular basement membrane disease (3%). The prevalence of ESKD was 12% at one year and 16% at last follow-up (median = 3 years, range1–11). MediantimetoESKDwas100days. RiskfactorsforESKDincluded%crescents,presence of fibrous crescents, estimated GFR, and hypertension at biopsy. For each 1% increase in %crescents, therewasa3%decreaseinlogoddsof1-yearrenalsurvival(p = 0.003)anda2%decreaseinlogodds of renal survivalat last follow-up(p < 0.001). Thesefindings providean evidencebase for enrollment criteria for crescentic glomerulonephritis in future clinical trials.