title: Outcomes of 847 childhood-onset systemic lupus erythematosus patients in three age groups.
abstract: Objective 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.
authors.affiliation: 1 Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, Brazil.
authors.forename: S R M
authors.initials: SRM
authors.lastname: Lopes
authors.affiliation: 1 Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, Brazil.
authors.forename: N W S
authors.initials: NWS
authors.lastname: Gormezano
authors.affiliation: 2 Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo, Brazil.
authors.forename: R C
authors.initials: RC
authors.lastname: Gomes
authors.affiliation: 1 Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, Brazil.|2 Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo, Brazil.
authors.forename: N E
authors.initials: NE
authors.lastname: Aikawa
authors.affiliation: 1 Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, Brazil.
authors.forename: R M R
authors.initials: RMR
authors.lastname: Pereira
authors.affiliation: 3 Pediatric Rheumatology Unit, Universidade Federal de São Paulo, Brazil.
authors.forename: M T
authors.initials: MT
authors.lastname: Terreri
authors.affiliation: 4 Pediatric Rheumatology Division, São Paulo State University (UNESP) - Faculdade de Medicina de Botucatu, Brazil.
authors.forename: C S
authors.initials: CS
authors.lastname: Magalhães
authors.affiliation: 2 Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo, Brazil.
authors.forename: J C
authors.initials: JC
authors.lastname: Ferreira
authors.affiliation: 5 Pediatric Rheumatology Unit, Irmandade da Santa Casa de Misericórdia de São Paulo, Brazil.
authors.forename: E M
authors.initials: EM
authors.lastname: Okuda
authors.affiliation: 3 Pediatric Rheumatology Unit, Universidade Federal de São Paulo, Brazil.
authors.forename: A P
authors.initials: AP
authors.lastname: Sakamoto
authors.affiliation: 2 Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo, Brazil.
authors.forename: A M E
authors.initials: AME
authors.lastname: Sallum
authors.affiliation: 6 Pediatric Rheumatology Unit, State University of Campinas (UNICAMP), Brazil.
authors.forename: S
authors.initials: S
authors.lastname: Appenzeller
authors.affiliation: 7 Pediatric Rheumatology Unit, Ribeirão Preto Medical School - University of São Paulo, Brazil.
authors.forename: V P L
authors.initials: VPL
authors.lastname: Ferriani
authors.affiliation: 8 Pediatric Rheumatology Unit, Hospital Infantil Darcy Vargas, Brazil.
authors.forename: C M
authors.initials: CM
authors.lastname: Barbosa
authors.affiliation: 9 Pediatric Rheumatology Unit, Hospital Menino Jesus, Brazil.
authors.forename: S
authors.initials: S
authors.lastname: Lotufo
authors.affiliation: 2 Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo, Brazil.
authors.forename: A A
authors.initials: AA
authors.lastname: Jesus
authors.affiliation: 3 Pediatric Rheumatology Unit, Universidade Federal de São Paulo, Brazil.
authors.forename: L E C
authors.initials: LEC
authors.lastname: Andrade
authors.affiliation: 2 Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo, Brazil.
authors.forename: L M A
authors.initials: LMA
authors.lastname: Campos
authors.affiliation: 1 Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, Brazil.
authors.forename: E
authors.initials: E
authors.lastname: Bonfá
authors.affiliation: 1 Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, Brazil.|2 Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo, Brazil.
authors.forename: C A
authors.initials: CA
authors.lastname: Silva
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D001938:Brazil / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000188:drug therapy / Q000453:epidemiology / Q000401:mortality; D009026:Mortality; D009393:Nephritis / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology / Q000401:mortality; D011247:Pregnancy; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013921:Thrombocytopenia / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents
keywords: Childhood-onset systemic lupus erythematosus; death; mortality and cumulative damage; nephritis; outcome