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

dc.citation.issue5
dc.citation.volume55
dc.contributor.authorPereira, Kaline Medeiros Costa [UNIFESP]
dc.contributor.authorFaria, Atila Granados Afonso de [UNIFESP]
dc.contributor.authorLiphaus, Bernadete L.
dc.contributor.authorJesus, Adriana A.
dc.contributor.authorSilva, Clovis A.
dc.contributor.authorCarneiro-Sampaio, Magda
dc.contributor.authorAndrade, Luiz Eduardo Coelho [UNIFESP]
dc.coverageOxford
dc.date.accessioned2020-07-22T13:22:58Z
dc.date.available2020-07-22T13:22:58Z
dc.date.issued2016
dc.description.abstractObjective. 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 = 1.7en
dc.description.abstract95% CI: 1.5, 1.9) and C4B (mean = 1.5en
dc.description.abstract95% CI: 1.3, 1.6) compared with healthy individuals (mean C4A = 2.3en
dc.description.abstract95% CI: 2.2, 2.5, P< 0.001en
dc.description.abstractC4B = 2.0en
dc.description.abstract95% CI: 1.8, 2.1en
dc.description.abstractP< 0.001) or with aSLE patients (mean C4A = 1.9en
dc.description.abstract95% CI: 1.8, 2.1, P = 0.006en
dc.description.abstractmean C4B = 1.8en
dc.description.abstract95% 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%en
dc.description.abstractP< 0.001). The same was observed for low C4A (<= 1 copy) (52% vs 18%en
dc.description.abstractP< 0.001) and for low C4B (60% vs 31%en
dc.description.abstractP< 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 = 3.26en
dc.description.abstract95% CI: 1.95, 5.47) than aSLE (C4 OR= 2.03en
dc.description.abstract95% CI: 1.32, 3.13en
dc.description.abstractC4A OR= 2.36en
dc.description.abstract95% CI: 1.46, 3.81en
dc.description.abstractC4B OR= 1.13en
dc.description.abstract95% CI: 0.73, 1.74). In addition, pericarditis in JSLE patients was associated with low C4 (OR= 4.13en
dc.description.abstract95% CI: 1.02, 16.68en
dc.description.abstractP = 0.047) and low C4A (OR = 5.54en
dc.description.abstract95% CI: 1.37, 22.32en
dc.description.abstractP = 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.en
dc.description.affiliationUniv Fed Sao Paulo, Escola Paulista Med, Div Rheumatol, Rua Botucatu 740, BR-04023062 Sao Paulo, SP, Brazil
dc.description.affiliationUniv Sao Paulo, Childrens Hosp, Sao Paulo, Brazil
dc.description.affiliationUniv Sao Paulo, Fac Med, Dept Pediat, Disciplina Reumatol, Sao Paulo, Brazil
dc.description.affiliationUnifespUniv Fed Sao Paulo, Escola Paulista Med, Div Rheumatol, Rua Botucatu 740, BR-04023062 Sao Paulo, SP, Brazil
dc.description.sourceWeb of Science
dc.description.sponsorshipFAPESP (Fundo de Amparo a Pesquisa do Estado de Sao Paulo)
dc.description.sponsorshipIDFAPESP: 08/57316-1
dc.description.sponsorshipIDFAPESP: 08/58238-4
dc.format.extent869-873
dc.identifierhttp://dx.doi.org/10.1093/rheumatology/kev436
dc.identifier.citationRheumatology. Oxford, v. 55, n. 5, p. 869-873, 2016.
dc.identifier.doi10.1093/rheumatology/kev436
dc.identifier.issn1462-0324
dc.identifier.urihttps://repositorio.unifesp.br/handle/11600/55966
dc.identifier.wosWOS:000376107800014
dc.language.isoeng
dc.publisherOxford Univ Press
dc.relation.ispartofRheumatology
dc.rightsinfo:eu-repo/semantics/openAccess
dc.subjectsystemic lupus erythematosusen
dc.subjectjuvenile SLEen
dc.subjectComplement systemen
dc.subjectpediatric rheumatologyen
dc.subjectimmunogeneticsen
dc.subjectgene copy numberen
dc.titleLow C4, C4A and C4B gene copy numbers are stronger risk factors for juvenile-onset than for adult-onset systemic lupus erythematosusen
dc.typeinfo:eu-repo/semantics/article
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