Clinical Characteristics and Treatment Patterns of Children and Adults With IgA Nephropathy or IgA Vasculitis: Findings From the CureGN Study

• Type of Material: Article
• Authors: David T. Selewski, University of Michigan; Josephine M. Ambruzs, Arkana Laboratories; Gerald B. Appel, Columbia University; Andrew S. Bomback, Columbia University; Raed Bou Matar, Cleveland Clinic Foundation; Yi Cai, Helen DeVos Children's Hospital; Daniel C. Cattran, University of Toronto; Aftab S. Chishti, University of Kentucky; Vivette D. D'Agati, Columbia University; Cynthia J. D'Alessandri-Silva, Connecticut Children's Medical Center; Rasheed A. Gbadegesin, Duke University; Jonathan J. Hogan, University of Pennsylvania; Sandra Iragorri, Oregon Health and Science University; J. Charles Jennette, University of North Carolina; Larry Greenbaum, Emory University
• Language: English
• Date: 2018-11-01
• Publisher: Elsevier
• Publication Version: Final Published Version
• Copyright Statement: © 2018 International Society of Nephrology
• License: Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1016/j.ekir.2018.07.021
• Title of Journal or Parent Work: Kidney International Reports
• ISSN: 2468-0249
• Volume: 3
• Issue: 6
• Start Page: 1373
• End Page: 1384
• Grant/Funding Information: Funding for the CureGN consortium is provided by UM1DK100845, UM1DK100846, UM1DK100876, UM1DK100866, and UM1DK100867 from the NIDDK. Patient recruitment is supported by NephCure Kidney International. Jennifer McCready-Maynes, an employee of Arbor Research Collaborative for Health, provided editorial assistance.
• Keywords: Henoch-Schönlein purpura (HSP); glomerulonephritis; IgA nephropathy (IgAN); IgA vasculitis (IgAV)
• Research Categories: Health Sciences, General

Abstract

Introduction: The Cure Glomerulonephropathy Network (CureGN) is a 66-center longitudinal observational study of patients with biopsy-confirmed minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, or IgA nephropathy (IgAN), including IgA vasculitis (IgAV). This study describes the clinical characteristics and treatment patterns in the IgA cohort, including comparisons between IgAN versus IgAV and adult versus pediatric patients. Methods: Patients with a diagnostic kidney biopsy within 5 years of screening were eligible to join CureGN. This is a descriptive analysis of clinical and treatment data collected at the time of enrollment. Results: A total of 667 patients (506 IgAN, 161 IgAV) constitute the IgAN/IgAV cohort (382 adults, 285 children). At biopsy, those with IgAV were younger (13.0 years vs. 29.6 years, P < 0.001), more frequently white (89.7% vs. 78.9%, P = 0.003), had a higher estimated glomerular filtration rate (103.5 vs. 70.6 ml/min per 1.73 m2, P < 0.001), and lower serum albumin (3.4 vs. 3.8 g/dl, P < 0.001) than those with IgAN. Adult and pediatric individuals with IgAV were more likely than those with IgAN to have been treated with immunosuppressive therapy at or prior to enrollment (79.5% vs. 54.0%, P < 0.001). Conclusion: This report highlights clinical differences between IgAV and IgAN and between children and adults with these diagnoses. We identified differences in treatment with immunosuppressive therapies by disease type. This description of baseline characteristics will serve as a foundation for future CureGN studies.