Influence of age at disease onset in the outcome of paediatric systemic lupus erythematosus.
OBJECTIVE
The aim of this study was to investigate the influence of age at disease onset in the outcome of paediatric SLE (pSLE).


METHODS
Fifty-six patients with pSLE, divided into three groups (pre-pubertal, peripubertal and post-pubertal onset), were studied. The SDI (SLICC/ACR Damage Index for SLE), patients' characteristics, disease manifestations and treatments were compared using Fisher's exact test and Kruskal-Wallis test. Kaplan-Meier curves were constructed to compare the risk of damage occurrence.


RESULTS
The risk of damage (SDI >or=1) significantly decreased when age at disease onset increased (89% in pre-pubertal pSLE, 57% in peripubertal pSLE and 38% in post-pubertal pSLE). This excess of risk was found in all disease duration intervals studied (1-3, 3-5, 5-8, 8-10, >10 years) and at the end of follow-up. Kaplan-Meier curves indicated a higher and earlier risk of damage in younger patients. Young children showed higher frequency of autoimmune family history. The frequency of neuropsychiatric disorders and damages decreased with age at disease onset (P < 0.05). Cumulative duration of high-dose prednisone (> 0.5 mg/kg/day) and number of immunosuppressive drugs used that seem to contribute to damage significantly increased when age at disease onset decreased.


CONCLUSIONS
The risk of damage is inversely correlated with age at disease onset in pSLE. The poorer outcome observed in younger children may be explained by a more severe disease expression, may be a higher infectious susceptibility, and a more aggressive therapy, particularly within the first 6 months of disease course.
Descloux
Elodie
E
Service de Médecine Interne, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Lyon, France. elodiedescloux@hotmail.com
Durieu
Isabelle
I
Cochat
Pierre
P
Vital-Durand
Denis
D
Ninet
Jacques
J
Fabien
Nicole
N
Cimaz
Rolando
R
D000293:Adolescent; D017668:Age of Onset; D002423:Cause of Death; D002648:Child; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use; D053208:Kaplan-Meier Estimate; D008180:Lupus Erythematosus, Systemic / Q000188:drug therapy / Q000453:epidemiology* / Q000401:mortality; D020945:Lupus Vasculitis, Central Nervous System / Q000188:drug therapy / Q000453:epidemiology / Q000401:mortality; D008297:Male; D011241:Prednisone / Q000009:adverse effects / Q000627:therapeutic use; D011379:Prognosis; D011627:Puberty; D018570:Risk Assessment / Q000379:methods; D018709:Statistics, Nonparametric; D013997:Time Factors; D016896:Treatment Outcome
D007166:Immunosuppressive Agents; D011241:Prednisone