title: Assessment of damage in juvenile-onset systemic lupus erythematosus: a multicenter cohort study.
abstract: OBJECTIVE
  To investigate the prevalence of cumulative organ damage in patients with juvenile-onset systemic lupus erythematosus (SLE) and its association with demographic and clinical variables, medication use, and quality of life.
  
  
  METHODS
  The occurrence of organ system damage, as measured by the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI), was determined for 387 patients consecutively enrolled in pediatric rheumatology centers from Europe, the US, Mexico, and Japan. Risk factors for damage included demographic variables; clinical manifestations at diagnosis; previous corticosteroid, immunosuppressive, and antimalarial therapies; disease activity; and quality of life.
  
  
  RESULTS
  Overall, 195 (50.5%) patients had damage within a mean of 5.7 years after disease onset. Renal (21.8%) and neuropsychiatric (15.8%) system involvement were observed most frequently, followed by musculoskeletal (11.7%), ocular (10.9%) and skin (9.6%) system involvement, with a mean SDI score of 1.1. In multivariate models, the occurrence of neuropsychiatric manifestations at diagnosis, a longer disease duration, and a greater number of intravenous cyclophosphamide pulses showed the strongest association with the presence of damage.
  
  
  CONCLUSIONS
  We found evidence of cumulative organ damage, as measured by the SDI, in half of the patients with juvenile-onset SLE. Damage was significantly more likely in patients who had experienced neuropsychiatric manifestations at diagnosis, had a longer disease duration, and had received more intravenous pulses of cyclophosphamide.
authors.affiliation: Istituto di Ricovero e Cura a Carattere Scientifico G. Gaslini, Genoa, Italy.
authors.forename: Angelo
authors.initials: A
authors.lastname: Ravelli
authors.forename: Carolina
authors.initials: C
authors.lastname: Duarte-Salazar
authors.forename: Silvia
authors.initials: S
authors.lastname: Buratti
authors.forename: Andreas
authors.initials: A
authors.lastname: Reiff
authors.forename: Bram
authors.initials: B
authors.lastname: Bernstein
authors.forename: Maria Rocio
authors.initials: MR
authors.lastname: Maldonado-Velazquez
authors.forename: Rosalia
authors.initials: R
authors.lastname: Beristain-Manterola
authors.forename: Nobuaki
authors.initials: N
authors.lastname: Maeno
authors.forename: Syuji
authors.initials: S
authors.lastname: Takei
authors.forename: Valeria
authors.initials: V
authors.lastname: Gerloni
authors.forename: Charles H
authors.initials: CH
authors.lastname: Spencer
authors.forename: Polyxeni
authors.initials: P
authors.lastname: Pratsidou-Gertsi
authors.forename: Nicolino
authors.initials: N
authors.lastname: Ruperto
authors.forename: Angela
authors.initials: A
authors.lastname: Pistorio
authors.forename: Alberto
authors.initials: A
authors.lastname: Martini
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006115:Greece / Q000453:epidemiology; D006801:Humans; D007223:Infant; D007558:Italy / Q000453:epidemiology; D007564:Japan / Q000453:epidemiology; D008180:Lupus Erythematosus, Systemic / Q000188:drug therapy / Q000453:epidemiology*; D008297:Male; D008800:Mexico / Q000453:epidemiology; D012720:Severity of Illness Index; D014481:United States / Q000453:epidemiology