Low C4, C4A and C4B gene copy numbers are stronger risk factors for juvenile-onset than for adult-onset systemic lupus erythematosus

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art_PEREIRA_Low_C4_C4A_and_C4B_gene_copy_numbers_2016.PDF (73.14 KB)
Citações na Scopus
33
Tipo de produção
article
Data de publicação
2016
DOI
Scopus
Web of Science
PubMed
Título da Revista
ISSN da Revista
Título do Volume
Editora
OXFORD UNIV PRESS
Autores
PEREIRA, Kaline M. C.
FARIA, Atila G. A.
JESUS, Adriana A.
ANDRADE, Luis E. C.
Citação
RHEUMATOLOGY, v.55, n.5, p.869-873, 2016
Projetos de Pesquisa
Unidades Organizacionais
Fascículo
Resumo
Objective. Complete deficiency of Complement C4 component is a strong genetic risk factor for SLE. C4 is encoded by two different genes, C4A and C4B, which show considerable gene copy number (GCN) variation. This study investigates the association of total C4, C4A and C4B GCN with JSLE. Methods. Ninety JSLE patients, 170 adult-onset SLE (aSLE) patients and 200 healthy individuals were evaluated for C4A and C4B GCN by quantitative real-time PCR. Results. JSLE patients had lower GCN for C4A (mean = 1.7; 95% CI: 1.5, 1.9) and C4B (mean = 1.5; 95% CI: 1.3, 1.6) compared with healthy individuals (mean C4A = 2.3; 95% CI: 2.2, 2.5, P< 0.001; C4B = 2.0; 95% CI: 1.8, 2.1; P< 0.001) or with aSLE patients (mean C4A = 1.9; 95% CI: 1.8, 2.1, P = 0.006; mean C4B = 1.8; 95% CI: 1.7, 1.9, P< 0.001). Low total C4 GCN (< 4 copies) was more frequent in JSLE than in healthy individuals (59% vs 28%; P< 0.001). The same was observed for low C4A (<= 1 copy) (52% vs 18%; P< 0.001) and for low C4B (60% vs 31%; P< 0.001). JSLE had a stronger association with low total C4 (OR = 3.68, 95% CI: 2.19, 6.20), C4A (OR = 4.98, 95% CI: 2.88, 8.62) and C4B (OR = 3.26; 95% CI: 1.95, 5.47) than aSLE (C4 OR= 2.03; 95% CI: 1.32, 3.13; C4A OR= 2.36; 95% CI: 1.46, 3.81; C4B OR= 1.13; 95% CI: 0.73, 1.74). In addition, pericarditis in JSLE patients was associated with low C4 (OR= 4.13; 95% CI: 1.02, 16.68; P = 0.047) and low C4A (OR = 5.54; 95% CI: 1.37, 22.32; P = 0.016). Conclusion. Low total C4, C4A and C4B GCN were associated with a stronger risk for developing JSLE than aSLE. Additionally, low total C4 and C4A GCN are risk factors for pericarditis in JSLE.
Palavras-chave
systemic lupus erythematosus, juvenile SLE, Complement system, pediatric rheumatology, immunogenetics, gene copy number
URI
https://observatorio.fm.usp.br/handle/OPI/14339
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Coleções
Artigos e Materiais de Revistas Científicas - FM/MPE
Artigos e Materiais de Revistas Científicas - HC/ICr
Artigos e Materiais de Revistas Científicas - LIM/36