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Rheumatology Advance Access published online on December 26, 2007
Rheumatology, doi:10.1093/rheumatology/kem335
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© The Author 2007. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Paediatric systemic lupus erythematosus: prognostic impact of antiphospholipid antibodies

E. Descloux1, I. Durieu1, P. Cochat2, D. Vital Durand1, J. Ninet3, N. Fabien4 and R. Cimaz2

1Service de Médecine Interne, Centre Hospitalier Lyon Sud, 2Département de Pédiatrie, 3Service de Médecine Interne, Hôpital Edouard Herriot and 4Laboratoire d’Immunologie, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Université Claude Bernard Lyon 1, Lyon, France.

Correspondence to: E. 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 our study was to investigate the prognostic impact of aPL in paediatric onset systemic lupus erythematosus (p-SLE).

Methods. This retrospective study included 56 patients with p-SLE. {chi}2-test, Fisher's exact test, incidence rate ratio and Kaplan–Meier survival curves were used to compare aPL-positive and aPL-negative patients considering the value of SDI (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index for SLE) at the end of follow-up, the occurrence of thromboses, organ system involvements and need for immunosuppressive treatment in addition to corticosteroids.

Results. Anti-cardiolipin antibodies and lupus anticoagulants were detected in 27 (49%) and 19 (35%) patients, respectively. These aPL were frequently transient or intermittent (10 and 15 cases, respectively), and only rarely persistent over time (five cases). The risk of thrombosis was significantly higher (odds ratio = 6.42) and occurred earlier in the presence of aPL, especially if aPL were persistent (P < 0.05). The association between aPL and neurological, renal, haematological manifestations or need for immunosuppressive treatment was not statistically significant. After a mean follow-up of 7.2 yrs, 30 patients (54.5%) had an SDI score ≥1. The risk of damage (SDI ≥ 1) in aPL-positive patients was three times higher than in aPL-negative patients (P < 0.05). Four of the six fatal cases occurred in the aPL-positive group.

Conclusions. The presence of aPL in p-SLE could represent not only a risk factor for thrombosis but also a poor prognostic factor overall.

KEY WORDS: Systemic lupus erythematosus, Paediatric, Anti-phospholipid antibodies, Prognosis

Submitted 5 June 2007; revised version accepted 15 November 2007.
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