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Paediatric systemic lupus erythematosus: prognostic impact of antiphospholipid antibodies
E Descloux1, I Durieu, P Cochat
1Service de Médecine Interne, Centre Hospitalier Lyon Sud 69 495, Pierre Bénite cedex, France. elodiedescloux@hotmail.com
Insights
Antiphospholipid antibodies (aPL) in pediatric systemic lupus erythematosus (p-SLE) are linked to a higher risk of thrombosis and overall poor prognosis. Early detection and management are crucial for these patients.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Clinical Medicine
Background:
- Pediatric-onset systemic lupus erythematosus (p-SLE) is a chronic autoimmune disease with significant morbidity.
- Antiphospholipid antibodies (aPL) are associated with thrombosis in adult SLE, but their prognostic impact in p-SLE requires further investigation.
Purpose of the Study:
- To investigate the prognostic significance of antiphospholipid antibodies (aPL) in children diagnosed with systemic lupus erythematosus (p-SLE).
Main Methods:
- Retrospective analysis of 56 p-SLE patients.
- Comparison of aPL-positive and aPL-negative groups using statistical tests (Chi2, Fisher's exact, incidence rate ratio, Kaplan-Meier curves).
- Assessment of thrombosis, organ damage (SDI), and treatment needs.
Main Results:
- 49% of patients had anti-cardiolipin antibodies, 35% had lupus anticoagulants.
- aPL presence, especially persistent aPL, significantly increased thrombosis risk (OR=6.42) and occurred earlier.
- aPL-positive patients had a threefold higher risk of organ damage (SDI ≥ 1) and a higher mortality rate.
Conclusions:
- Antiphospholipid antibodies (aPL) are a significant risk factor for thrombosis in p-SLE.
- aPL presence indicates a poorer overall prognosis in pediatric-onset systemic lupus erythematosus.
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. Chi2-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 > or = 1. The risk of damage (SDI > or = 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.
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