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Genetic variability associates with ancestry, age at disease onset, organ involvement and disease severity in juvenile-onset systemic lupus erythematosus

Title: Genetic variability associates with ancestry, age at disease onset, organ involvement and disease severity in juvenile-onset systemic lupus erythematosus
Authors: Natoli, V; Charras, A; Hasoon, MSR; Jorgensen, AL; Smith, EMD; Caamaño Gutiérrez, E; Beresford, MW; Hedrich, CM; Roberts, C; Al-Abadi, E; Armon, K; Bailey, K; Ciurtin, C; Gardner-Medwin, J; Haslam, K; Hawley, DP; Leahy, A; Leone, V; McErlane, F; Modgil, G; Pilkington, C; Ramanan, AV; Rangaraj, S; Riley, P; Sridhar, A
Publisher Information: Elsevier BV
Publication Year: 2025
Collection: The University of Liverpool Repository
Description: Juvenile-onset systemic lupus erythematosus (jSLE) is a complex autoimmune/inflammatory disease in which genetic factors likely contribute to pathophysiology and clinical expression. This study explored associations between general (alternate allele counts; AAC) and gene-specific (alternate allele scores; GAAS) sequence variability, age at onset, sex, ancestry, disease activity/severity, organ involvement and treatments in jSLE. 289 participants from the UK JSLE Cohort Study underwent panel sequencing of 62 genes/genomic regions. Weighted AAC and GAAS were calculated. Correlation analyses and generalized linear models assessed associations between genetic burden, ancestry, age at diagnosis and clinical variables. AAC inversely correlated with age at diagnosis (R = -0.15, p = 0.01), primarily driven by South Asians (R = -0.28, p < 0.001). African/Caribbean patients exhibited higher AAC (p < 0.001). Clinical variables, including severity of renal involvement (ACP5, ITGAM, LYN, p < 0.001; TNFAIP3, p = 0.007), associated with GAAS. Genetic variability likely contributes to early disease expression and severity in jSLE, supporting patient stratification and personalised care.
Document Type: article in journal/newspaper
Language: English
ISSN: 1521-6616
Relation: Collapse authors list. Natoli, V, Charras, A orcid:0000-0001-6760-5459 , Hasoon, MSR, Jorgensen, AL orcid:0000-0002-6977-9337 , Smith, EMD orcid:0000-0002-8371-7597 , Caamaño Gutiérrez, E, Beresford, MW orcid:0000-0002-5400-9911 , Hedrich, CM orcid:0000-0002-1295-6179 , Roberts, C, Al-Abadi, E et al (show 15 more authors) , Armon, K, Bailey, K, Ciurtin, C, Gardner-Medwin, J, Haslam, K, Hawley, DP, Leahy, A, Leone, V, McErlane, F, Modgil, G, Pilkington, C, Ramanan, AV, Rangaraj, S, Riley, P and Sridhar, A (2025) Genetic variability associates with ancestry, age at disease onset, organ involvement and disease severity in juvenile-onset systemic lupus erythematosus Clinical Immunology, 279. 110540-. ISSN 1521-6616, 1521-7035
DOI: 10.1016/j.clim.2025.110540
Availability: https://livrepository.liverpool.ac.uk/3193512/; https://doi.org/10.1016/j.clim.2025.110540
Accession Number: edsbas.789F3CC7
Database: BASE