Abstract
Background/purposePediatric antiphospholipid syndrome (APS) is a thromboinflammatory disease characterized by the presence of circulating antiphospholipid antibodies and either thrombotic events or pregnancy morbidity. The objective of this study was to review a large institution’s experience to better understand the characteristics of children with APS.MethodsWe conducted a retrospective review of pediatric APS at a tertiary referral center. The electronic medical record system was queried from 2000 through 2019, and 21 cases were included based on meeting the revised Sapporo Classification criteria by age 18 or younger. Comparisons between primary and secondary APS patients were made with two-tailed t-tests.ResultsTwenty-one patients were included with a median age at diagnosis of 16 years and median follow-up of 5.8 years. Secondary APS was slightly more common than primary APS (11 vs. 10 cases) and was primarily diagnosed in the context of systemic lupus erythematosus. Two thirds of patients (67%) also had “non-criteria” manifestations of APS including thrombocytopenia, autoimmune hemolytic anemia, and livedo reticularis/racemosa. Almost half of patients (43%) had recurrent thrombosis, typically when patients were subtherapeutic or non-adherent with anticoagulation. Damage Index in Patients with Thrombotic APS (DIAPS) scores indicated a chronic burden of disease in both primary and secondary APS patients.ConclusionThis case series of pediatric APS provides important context regarding disease phenotypes displayed by children with APS. High prevalence of non-criteria clinical manifestations highlights the need to consider these characteristics when developing pediatric-specific classification criteria and when considering this relatively rare diagnosis in pediatric practice.
Highlights
Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by thrombotic events and/or pregnancy morbidity in the setting of persistently positive antiphospholipid antibodies
The 15th International Congress on Antiphospholipid Antibodies Task Force on Pediatric Antiphospholipid Syndrome published a report that highlighted the different characteristics seen in pediatric antiphospholipid syndrome (APS) and underlined the importance of large, age-stratified studies to better identify risk for thrombotic events and to test the hypothesis that adult criteria may not be as useful in children [22]
Recurrent thrombosis is common in nearly half of patients, and among those, subtherapeutic anticoagulation or anticoagulant medication non-adherence is almost universally identified
Summary
Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by thrombotic events and/or pregnancy morbidity in the setting of persistently positive antiphospholipid antibodies (aPL). The Sapporo criteria (first developed in 1999 and revised in 2006) are used to formally classify APS for research purposes. These criteria require the presence of at least one clinical event Madison et al Pediatric Rheumatology (2022) 20:17 vessel thrombosis, or pregnancy-related morbidity) and the durable presence over at least 12 weeks of at least one laboratory feature: positive lupus anticoagulant (a functional assay that screens for aPL), anticardiolipin IgG or IgM in medium or high titer (> 40 GPL/MPL or titer > 99th percentile), or anti-beta-2 glycoprotein I (β2GPI) IgG or IgM (titer > 99th percentile) [1]. There is an ongoing effort to develop new APS classification criteria, including criteria specific for pediatric patients with APS [4]
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