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Rheumatology Advance Access published online on May 4, 2009
Rheumatology, doi:10.1093/rheumatology/kep067
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© The Author 2009. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Influence of age at disease onset in the outcome of paediatric systemic lupus erythematosus

Elodie Descloux1, Isabelle Durieu1,2, Pierre Cochat2,3, Denis Vital-Durand1,2, Jacques Ninet2,4, Nicole Fabien5 and Rolando Cimaz2,3

1Service de médecine interne, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon,2Université Claude Bernard Lyon 1,3Service de pédiatrie, Hôpital Femme-Mère-Enfant,4Service de médecine interne, Hôpital Edouard Herriot and 5Laboratoire d'immunologie, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Lyon, France.

Correspondence to: Elodie Descloux, Service de Médecine Interne, Centre Hospitalier Lyon Sud, 69 495 Pierre Bénite cedex, France. E-mail: elodiedescloux{at}hotmail.com


  Abstract

Objectives. 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 >=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.

KEY WORDS: Systemic lupus erythematosus, Paediatric, Age, Damage, Treatment, Prognosis, Outcome

Submitted 21 August 2008; Accepted 5 March 2009


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