Outcomes of 847 childhood-onset systemic lupus erythematosus patients in three age groups

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dc.contributor.authorLopes, S. R.M.
dc.contributor.authorGormezano, N. W.S.
dc.contributor.authorGomes, R. C.
dc.contributor.authorAikawa, N. E.
dc.contributor.authorPereira, R. M.R.
dc.contributor.authorTerreri, M. T.
dc.contributor.authorMagalhães, C. S. [UNESP]
dc.contributor.authorFerreira, J. C.
dc.contributor.authorOkuda, E. M.
dc.contributor.authorSakamoto, A. P.
dc.contributor.authorSallum, A. M.E.
dc.contributor.authorAppenzeller, S.
dc.contributor.authorFerriani, V. P.L.
dc.contributor.authorBarbosa, C. M.
dc.contributor.authorLotufo, S.
dc.contributor.authorJesus, A. A.
dc.contributor.authorAndrade, L. E.C.
dc.contributor.authorCampos, L. M.A.
dc.contributor.authorBonfá, E.
dc.contributor.authorSilva, C. A.
dc.contributor.institutionUniversidade de São Paulo (USP)
dc.contributor.institutionUniversidade Federal de São Paulo (UNIFESP)
dc.contributor.institutionUniversidade Estadual Paulista (Unesp)
dc.contributor.institutionIrmandade da Santa Casa de Misericórdia de São Paulo
dc.contributor.institutionUniversidade Estadual de Campinas (UNICAMP)
dc.contributor.institutionHospital Infantil Darcy Vargas
dc.contributor.institutionHospital Menino Jesus
dc.date.accessioned2018-12-11T17:13:19Z
dc.date.available2018-12-11T17:13:19Z
dc.date.issued2017-08-01
dc.description.abstractObjective The objective of this study was to assess outcomes of childhood systemic lupus erythematosus (cSLE) in three different age groups evaluated at last visit: group A early-onset disease (<6 years), group B school age (≥6 and <12 years) and group C adolescent (≥12 and <18 years). Methods An observational cohort study was performed in ten pediatric rheumatology centers, including 847 cSLE patients. Results Group A had 39 (4%), B 395 (47%) and C 413 (49%). Median disease duration was significantly higher in group A compared to groups B and C (8.3 (0.1-23.4) vs 6.2 (0-17) vs 3.3 (0-14.6) years, p < 0.0001). The median Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR-DI) (0 (0-9) vs 0 (0-6) vs 0 (0-7), p = 0.065) was comparable in the three groups. Further analysis of organ/system damage revealed that frequencies of neuropsychiatric (21% vs 10% vs 7%, p = 0.007), skin (10% vs 1% vs 3%, p = 0.002) and peripheral vascular involvements (5% vs 3% vs 0.3%, p = 0.008) were more often observed in group A compared to groups B and C. Frequencies of severe cumulative lupus manifestations such as nephritis, thrombocytopenia, and autoimmune hemolytic anemia were similar in all groups (p > 0.05). Mortality rate was significantly higher in group A compared to groups B and C (15% vs 10% vs 6%, p = 0.028). Out of 69 deaths, 33/69 (48%) occurred within the first two years after diagnosis. Infections accounted for 54/69 (78%) of the deaths and 38/54 (70%) had concomitant disease activity. Conclusions This large multicenter study provided evidence that early-onset cSLE group had distinct outcomes. This group was characterized by higher mortality rate and neuropsychiatric/vascular/skin organ damage in spite of comparable frequencies of severe cumulative lupus manifestations. We also identified that overall death in cSLE patients was an early event mainly attributed to infection associated with disease activity.en
dc.description.affiliationDivision of Rheumatology Faculdade de Medicina Universidade de São Paulo, Av. Dr Eneas Carvalho Aguiar, 647-Cerqueira Cesar
dc.description.affiliationPediatric Rheumatology Unit Children's Institute Faculdade de Medicina Universidade de São Paulo
dc.description.affiliationPediatric Rheumatology Unit Universidade Federal de São Paulo
dc.description.affiliationPediatric Rheumatology Division São Paulo State University (UNESP) Faculdade de Medicina de Botucatu
dc.description.affiliationPediatric Rheumatology Unit Irmandade da Santa Casa de Misericórdia de São Paulo
dc.description.affiliationPediatric Rheumatology Unit State University of Campinas (UNICAMP)
dc.description.affiliationPediatric Rheumatology Unit Ribeirão Preto Medical School University of São Paulo
dc.description.affiliationPediatric Rheumatology Unit Hospital Infantil Darcy Vargas
dc.description.affiliationPediatric Rheumatology Unit Hospital Menino Jesus
dc.description.affiliationUnespPediatric Rheumatology Division São Paulo State University (UNESP) Faculdade de Medicina de Botucatu
dc.format.extent996-1001
dc.identifierhttp://dx.doi.org/10.1177/0961203317690616
dc.identifier.citationLupus, v. 26, n. 9, p. 996-1001, 2017.
dc.identifier.doi10.1177/0961203317690616
dc.identifier.file2-s2.0-85023167108.pdf
dc.identifier.issn1477-0962
dc.identifier.issn0961-2033
dc.identifier.lattes7098310008371632
dc.identifier.orcid0000-0002-7631-7093
dc.identifier.scopus2-s2.0-85023167108
dc.identifier.urihttp://hdl.handle.net/11449/174890
dc.language.isoeng
dc.relation.ispartofLupus
dc.relation.ispartofsjr1,410
dc.relation.ispartofsjr1,410
dc.rights.accessRightsAcesso aberto
dc.sourceScopus
dc.subjectChildhood-onset systemic lupus erythematosus
dc.subjectdeath
dc.subjectmortality and cumulative damage
dc.subjectnephritis
dc.subjectoutcome
dc.titleOutcomes of 847 childhood-onset systemic lupus erythematosus patients in three age groupsen
dc.typeArtigo
unesp.author.lattes7098310008371632[7]
unesp.author.orcid0000-0002-7631-7093[7]

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