Paediatric systemic lupus erythematosus: prognostic impact of antiphospholipid antibodies

• Published in: Rheumatology (Oxford, England) Vol. 47; no. 2; pp. 183 - 187
• Main Authors: Descloux, E., Durieu, I., Cochat, P., Vital Durand, D., Ninet, J., Fabien, N., Cimaz, R.
• Format: Journal Article
• Language: English
• Published: Oxford Oxford University Press 01.02.2008; Oxford Publishing Limited (England)
• Subjects: Adolescent; Adult; Anti-phospholipid antibodies; Antibodies, Antiphospholipid - therapeutic use; Biological and medical sciences; Child; Child, Preschool; Female; Follow-Up Studies; Hematologic and hematopoietic diseases; Humans; Immunoenzyme Techniques; Immunoglobulin M - immunology; Infant; Lupus Erythematosus, Systemic - drug therapy; Lupus Erythematosus, Systemic - immunology; Lupus Erythematosus, Systemic - mortality; Male; Medical sciences; Paediatric; Patient Selection; Platelet diseases and coagulopathies; Prognosis; Retrospective Studies; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Survival Analysis; Systemic lupus erythematosus; Time Factors; Treatment Outcome; Paediatric; Systemic lupus erythematosus; Prognosis; Anti-phospholipid antibodies; Human; Immunopathology; Connective tissue disease; Skin disease; Antiphospholipid antibody syndrome; Antibody; Phospholipid; Autoimmune disease; Cardiovascular disease; Hemopathy; Vascular disease; Platelet; Systemic disease; Child
• ISSN: 1462-0324; 1462-0332
• DOI: 10.1093/rheumatology/kem335

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. χ2-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.