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Published on: January 29, 2014
Clinical and laboratory characteristics of children positive for antiphospholipid antibodies
Paola Giordano1, Riccardina Tesse, Giuseppe Lassandro
1Department of Biomedicine of Developmental Age, University of Bari, Bari, Italy. giordano@bioetaev.uniba.it
Insights
Children with antiphospholipid antibodies (aPL) show varied clinical features. Persistent aPL positivity is linked to higher thrombotic event rates, suggesting distinct criteria for pediatric antiphospholipid syndrome (APS).
Area of Science:
- Pediatric Rheumatology
- Immunology
- Hematology
Background:
- Standardized criteria for pediatric antiphospholipid syndrome (APS) are lacking, making prevalence estimation challenging.
- Antiphospholipid antibodies (aPL) in children present heterogeneous clinical and laboratory features.
- This study investigates clinical and laboratory characteristics of children with aPL.
Purpose of the Study:
- To assess clinical and laboratory characteristics of children with antiphospholipid antibodies (aPL).
- To contribute to understanding heterogeneous aPL-related features in childhood.
- To inform the development of classification criteria for pediatric APS.
Main Methods:
- Forty-four pediatric patients with prolonged activated partial thromboplastin time were enrolled.
- Patients were categorized into transiently positive (Group I) or persistently positive (Group II) for aPL based on antibody detection over at least 12 weeks.
- Standard procedures were used to detect lupus anticoagulant (LA), anticardiolipin (aCL), and anti-β2-glycoprotein I (anti-β2GPI) antibodies. Clinical history and symptoms were recorded.
Main Results:
- Group II (persistently positive) had a higher rate of thrombotic events (36%) compared to Group I (transiently positive).
- Persistently positive patients showed a significantly higher prevalence of anticardiolipin (aCL) antibodies, specifically aCL IgG and anti-β2GPI IgM subtypes.
- While LA positivity rates were similar, specific aPL subtypes differed significantly between transient and persistent groups.
Conclusions:
- Pediatric patients with antiphospholipid antibodies exhibit diverse clinical and laboratory profiles.
- Persistent aPL positivity is associated with an increased risk of thrombotic events and specific antibody subtypes.
- Findings underscore the need for distinct classification criteria for pediatric antiphospholipid syndrome (APS).
Background:
It is difficult to estimate the actual prevalence of antiphospholipid syndrome (APS) in the paediatric population since there are no standardised criteria. We aimed to assess clinical and laboratory characteristics of a cohort of children positive for antiphospholipid antibodies (aPL) to contribute to the understanding of the heterogeneous aPL-related features in childhood.
Materials And Methods:
Forty-four patients with prolonged activated partial thromboplastin time were enrolled and assigned to group I ("transiently positive") or group II ("persistently positive"), based on the detection of elevated aPL plasma levels [lupus anticoagulant (LA), anticardiolipin (aCL), and anti-β2-glycoprotein I (anti-β2GPI) antibodies] on, respectively, one or more occasions, at least 12 weeks apart, by standard procedures. The clinical history and symptoms of all patients were recorded.
Results:
Thirty-three (75%) patients were assigned to group I, while the other 11 (25%) formed group II. Major associated diseases in group I were urticarial vasculitis (21%), acute infections (18%) and thalassaemia (12%). Five subjects (15%) were asymptomatic. Four out of the 11 subjects (36%) in group II had thrombotic events; they were all persistently aPL-positive and two of them had concomitant systemic lupus erythematosus. The rate of detection of LA-positivity was not significantly different between the two groups (76% vs 91%, p>0.05), whereas the percentage of patients positive for overall aCL was higher in group II than in group I (54% vs 42%, respectively; p<0.05). Specifically, aCL IgG and anti-β2GPI IgM subtypes were significantly more represented in group II than in group I (100% vs 62% and 75% vs 33%, respectively; p<0.05).
Discussion:
Our study shows that aPL-positive children have different features that should be taken into account in the classification of criteria for paediatric APS.
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