Features of 847 Childhood-Onset Systemic Lupus Erythematosus Patients in Three Age Groups at Diagnosis: A Brazilian Multicenter Study

dc.contributor.authorGomes, Roberta C.
dc.contributor.authorSilva, Marco F.
dc.contributor.authorKozu, Katia
dc.contributor.authorBonfá, Eloisa
dc.contributor.authorPereira, Rosa M.
dc.contributor.authorTerreri, Maria T.
dc.contributor.authorMagalhães, Claudia S. [UNESP]
dc.contributor.authorSacchetti, Silvana B.
dc.contributor.authorMarini, Roberto
dc.contributor.authorFraga, Melissa
dc.contributor.authorCarvalho, Luciana M.
dc.contributor.authorBarbosa, Cássia M.
dc.contributor.authorCarneiro-Sampaio, Magda
dc.contributor.authorSilva, Clovis 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 Sao Paulo
dc.contributor.institutionUniversidade Estadual de Campinas (UNICAMP)
dc.contributor.institutionHospital Infantil Darcy Vargas
dc.description.abstractObjective: To evaluate demographic data and clinical and laboratory features at disease diagnosis in 3 different age groups of childhood-onset systemic lupus erythematosus (SLE): group A, early-onset (<6 years); group B, school age (≥6 to <12 years); and group C, adolescent (≥12 to <18 years). Methods: This was a Brazilian multicenter cohort retrospective study in 10 pediatric rheumatology centers, including 847 childhood-onset SLE patients. Results: Patients were divided into 3 groups: group A with 39 patients (4%), group B with 395 patients (47%), and group C with 413 patients (49%). Of 39 childhood-onset SLE patients in group A, 3 (8%) were ages <2 years, 4 (10%) were ≥2 to <3 years, and 32 (82%) were ≥3 and <6 years. A total of 74 childhood-onset SLE patients were analyzed for C1q levels, and complete C1q deficiency was observed in 3 of 74 patients (4%), all in group A. Groups were similar regarding high frequencies of female sex, nephritis, neuropsychiatric involvement, Systemic Lupus Erythematosus Disease Activity Index 2000 score ≥8, autoantibody profile, elevated acute phase proteins, and low complement levels (P > 0.05). However, the frequency of fever (78% versus 61% versus 47%; P < 0.0001), hepatomegaly (42% versus 29% versus 14%; P < 0.0001), splenomegaly (28% versus 12% versus 4%; P < 0.0001), and discoid lupus (13% versus 4% versus 4%; P = 0.020) was significantly higher in group A compared to groups B and C. The frequency of weight loss >2 kg (19% versus 28% versus 36%; P = 0.017), photosensitivity (34% versus 41% versus 51%; P = 0.006), leukopenia <4,000/mm3 (14% versus 25% versus 30%; P = 0.048), and lymphopenia <1,500/mm3 (22% versus 41% versus 47%; P = 0.011) was significantly lower in group A. Conclusion: Our large multicenter study identified the finding that the initial appearance of childhood-onset SLE is characterized by comparable high frequency of internal organ involvement and some distinct clinical and laboratory features in early-onset and adolescent groups.en
dc.description.affiliationFaculdade de Medicina da Universidade de Sao Paulo
dc.description.affiliationUniversidade Federal de Sao Paulo
dc.description.affiliationSao Paulo State University (UNESP)
dc.description.affiliationIrmandade da Santa Casa de Misericórdia de Sao Paulo
dc.description.affiliationState University of Campinas (UNICAMP)
dc.description.affiliationUniversity of Sao Paulo
dc.description.affiliationHospital Infantil Darcy Vargas
dc.description.affiliationUnespSao Paulo State University (UNESP)
dc.identifier.citationArthritis Care and Research, v. 68, n. 11, p. 1736-1741, 2016.
dc.relation.ispartofArthritis Care and Research
dc.rights.accessRightsAcesso restrito
dc.titleFeatures of 847 Childhood-Onset Systemic Lupus Erythematosus Patients in Three Age Groups at Diagnosis: A Brazilian Multicenter Studyen