Assessment of damage in juvenile-onset systemic lupus erythematosus: a multicenter cohort study.
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.
Ravelli
Angelo
A
Istituto di Ricovero e Cura a Carattere Scientifico G. Gaslini, Genoa, Italy.
Duarte-Salazar
Carolina
C
Buratti
Silvia
S
Reiff
Andreas
A
Bernstein
Bram
B
Maldonado-Velazquez
Maria Rocio
MR
Beristain-Manterola
Rosalia
R
Maeno
Nobuaki
N
Takei
Syuji
S
Gerloni
Valeria
V
Spencer
Charles H
CH
Pratsidou-Gertsi
Polyxeni
P
Ruperto
Nicolino
N
Pistorio
Angela
A
Martini
Alberto
A
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