title: Features of 847 Childhood-Onset Systemic Lupus Erythematosus Patients in Three Age Groups at Diagnosis: A Brazilian Multicenter Study.
abstract: 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).
  
  
  
  This was a Brazilian multicenter cohort retrospective study in 10 pediatric rheumatology centers, including 847 childhood-onset SLE patients.
  
  
  
  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.
  
  
  
  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.
authors.affiliation: Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, Brazil.
authors.forename: Roberta C
authors.initials: RC
authors.lastname: Gomes
authors.affiliation: Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, Brazil.
authors.forename: Marco F
authors.initials: MF
authors.lastname: Silva
authors.affiliation: Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, Brazil.
authors.forename: Katia
authors.initials: K
authors.lastname: Kozu
authors.affiliation: Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, Brazil.
authors.forename: Eloisa
authors.initials: E
authors.lastname: Bonfá
authors.affiliation: Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, Brazil.
authors.forename: Rosa M
authors.initials: RM
authors.lastname: Pereira
authors.affiliation: Universidade Federal de Sao Paulo, Sao Paulo, Brazil.
authors.forename: Maria T
authors.initials: MT
authors.lastname: Terreri
authors.affiliation: Sao Paulo State University (UNESP), Sao Paulo, Brazil.
authors.forename: Claudia S
authors.initials: CS
authors.lastname: Magalhães
authors.affiliation: Irmandade da Santa Casa de Misericórdia de Sao Paulo, Sao Paulo, Brazil.
authors.forename: Silvana B
authors.initials: SB
authors.lastname: Sacchetti
authors.affiliation: State University of Campinas (UNICAMP), Sao Paulo, Brazil.
authors.forename: Roberto
authors.initials: R
authors.lastname: Marini
authors.affiliation: Universidade Federal de Sao Paulo, Sao Paulo, Brazil.
authors.forename: Melissa
authors.initials: M
authors.lastname: Fraga
authors.affiliation: University of Sao Paulo, Sao Paulo, Brazil.
authors.forename: Luciana M
authors.initials: LM
authors.lastname: Carvalho
authors.affiliation: Hospital Infantil Darcy Vargas, Sao Paulo, Brazil.
authors.forename: Cássia M
authors.initials: CM
authors.lastname: Barbosa
authors.affiliation: Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, Brazil.
authors.forename: Magda
authors.initials: M
authors.lastname: Carneiro-Sampaio
authors.affiliation: Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, Brazil. clovisaasilva@gmail.com.
authors.forename: Clovis A
authors.initials: CA
authors.lastname: Silva
mesh_terms: D000293:Adolescent; D000367:Age Factors; D017668:Age of Onset; D001323:Autoantibodies / Q000097:blood; D001938:Brazil; D002648:Child; D015922:Complement C1q / Q000032:analysis / Q000172:deficiency; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000473:pathology*; D020945:Lupus Vasculitis, Central Nervous System / Q000209:etiology; D008297:Male; D009393:Nephritis / Q000209:etiology; D012189:Retrospective Studies; D012720:Severity of Illness Index; D012737:Sex Factors
chemical_list: D001323:Autoantibodies; D015922:Complement C1q