Familial autoimmunity in the childhood arthritis and rheumatology research alliance registry

• Type of Material: Article
• Authors: Sampath Prahalad, Emory University; Courtney E. McCracken, Emory University; Lori A. Ponder, Children’s Healthcare of Atlanta; Sheila Angeles-Han, Emory University; Kelly Rouster Stevens, Emory University; Larry Vogler, Emory University; Carl D. Langefeld, Wake Forest University; Susan D. Thompson, Cincinnati Children’s Hospital Medical Center
• Language: English
• Date: 2016-03-10
• Publisher: BioMed Central
• Publication Version: Final Published Version
• Copyright Statement: © 2016 Prahalad et al. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
• License: Creative Commons Attribution 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1186/s12969-016-0075-7
• Title of Journal or Parent Work: Pediatric Rheumatology
• ISSN: 1546-0096
• Volume: 14
• Issue: 1
• Start Page: 14
• End Page: 14
• Grant/Funding Information: CARRA Registry is supported by grants from NIAMS (RC2-AR058934), Friends of CARRA, the Arthritis Foundation, and the Duke Clinical Research Institute.; Dr. Prahalad is supported by grants from The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) (01-AR060893), Arthritis Foundation and The Marcus Foundation Inc.; Dr. Thompson is supported by grants from NIAMS (P01-AR048929 and P30-AR0470363), The Arthritis Foundation and Cincinnati Children’s Hospital Research Foundation.
• Keywords: SYSTEMIC-LUPUS-ERYTHEMATOSUS; AGGREGATION; Pediatrics; INDIVIDUALS; CARRA REGISTRY; GENETIC RISK-FACTORS; GENOME-WIDE ASSOCIATION; Rheumatology; DISEASES; Science & Technology; Life Sciences & Biomedicine; LOCI; JUVENILE IDIOPATHIC ARTHRITIS; CROSS-SECTIONAL ANALYSIS
• Research Categories: Health Sciences, Immunology; Health Sciences, Medicine and Surgery

Abstract

Background Clinically distinct autoimmune phenotypes share genetic susceptibility factors. We investigated the prevalence of familial autoimmunity among subjects with juvenile idiopathic arthritis (JIA), childhood systemic lupus erythematosus (cSLE) and juvenile dermatomyositis (JDM) in the CARRA Registry, the largest multicenter observational Registry for pediatric rheumatic disease. Methods Children with JIA, cSLE and JDM enrolled in the CARRA Registry between May 2010 and May 2012 were investigated for differences in proportion of subjects who had first-degree relatives (FDR) with autoimmunity. If a significant difference was detected, pairwise comparisons, adjusted for multiple comparisons, were made. Results There were 4677 JIA, 639 cSLE and 440 JDM subjects. The proportion of subjects having FDR with any autoimmune disease in the JDM group (20.5%) was less compared to subjects with JIA (31.8%, p < 0.001) or SLE (31.9%; p < 0.001). Significantly greater proportion of JIA cases had FDR with inflammatory arthritis (13%) compared to cSLE (9.2%, p = 0.007) or JDM (4.3%, p <0.001). Significantly greater proportion of cSLE cases had FDR with SLE (11.1% vs. 1.7% for JIA and 1.1% for JDM p < 0.001) or type-I diabetes (7.4% for cSLE vs. 3.1% for JIA and 3.0% for JDM p < 0.001). Conclusion Higher proportions of subjects with JIA and cSLE have FDR with autoimmunity compared to those of JDM. Relatives of cSLE cases had an increased prevalence of SLE, and relatives of JIA cases were enriched for inflammatory arthropathies demonstrating distinct patterns of familial autoimmunity among these phenotypes.