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Cited by 39Systemic Lupus Erythematosus ResearchBlood Coagulation and Thrombosis MechanismsVasculitis and related conditions

The Differences Between Childhood and Adult Onset Antiphospholipid Syndrome

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Wincup, Chris, Ioannou, Yiannis·2018·Frontiers in Pediatrics
DOI 10.3389/fped.2018.00362PMC PMC6277799
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Abstract

Antiphospholipid syndrome (APS) is a rare autoimmune disease of unknown etiology that represents a leading cause of acquired thromboembolism and recurrent miscarriage. It is characterized by the persistent elevated presence of pathogenic antiphospholipid auto-antibodies directed against cardiolipin, ß2-glycoprotein-I, and/or a positive lupus anticoagulant test. As with many autoimmune disorders, the pathogenesis of APS is believed to be the result of a complex interaction between environmental triggers and genetic predisposition. Although more common in adults, APS occasionally manifests in the neonatal period and throughout childhood. Adut-onset APS classification criteria are poorly validated to the pediatric population (in which pregnancy related complications are seldom seen) and as a result, assessment of the prevalence of the disease in childhood is difficult. Thromboembolic events seen in children include deep venous thrombosis in addition to stroke and pulmonary embolism, which can lead to significant long-term disability. The disease can be classified as either primary (when occurring in isolation) or secondary, in which the disease is diagnosed in the context of another underlying disease, most commonly systemic lupus erythematosus. A variety of laboratory and clinical difference are seen between pediatric and adult-onset APS. The marked female predominance seen in adult-onset disease is less evident in childhood where the gender split is more evenly spread. In addition, children with APS are at a higher risk of recurrent thromboembolism than adults. The treatment of childhood-onset APS is challenging due to a lack of large-scale prospective studies in the pediatric population. Therapeutic options are often based upon treatment guidelines that have been based upon literature from the adult-onset form of the disease. In the majority of cases, treatment is focused on the prevention of further thrombosis through treatment with long-term anti-coagulation therapy. The evidence for the use of antiplatelet agents (such as aspirin) and hydroxychloroquine is inconclusive. It is important to remember that anti-coagulation can have significant lifestyle implications for the child with APS and it is essential to consider potential implications relating to school and recreational activities, with contact sports often discouraged due to the increased risk of bleeding.

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  • Children with upper respiratory tract infection can have LA as a consequence of an isolated prolonged aPTT observed in pre-surgery investigations for (adeno)tonsillectomy.

    “A paradigmatic setting characterized by these findings is represented by children suffering from upper respiratory tract infection, diseases that are common in the youngest ages [108,109,110].”

    From Lupus Anticoagulant Detection under the Magnifying Glass (2023) · cites this paper as [110]

    The Introduction says infections can cause a positive antiphospholipid-antibody test, and Diagnosis > Serological abnormalities in APS reports transient positivity in children likely related to prior infection. That section also discusses lupus anticoagulant in pediatric APS, but the paper does not identify upper respiratory tract infection, isolated prolonged aPTT, or pre-surgery testing for (adeno)tonsillectomy as the claimed setting. Page numbers are unavailable in our copy. (LLM, conf=0.83)

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Cited by

Sorted by citations

Authors
Wincup, C., & Ioannou, Y.
Affiliations
Arthritis Research UK (GB)
Corresponding author
Wincup, Chris
ORCID
Venue
Frontiers in Pediatrics
Publisher
Frontiers Media SA
Published
2018-11-27
Volume
6
Pages
362-362
ISSN
2296-2360 (ISSN-L)
DOI
10.3389/fped.2018.00362
PMC
PMC6277799
OpenAlex
W2903197412
Cited by
39
References
3
Citations by year
2026: 3 · 2025: 3 · 2024: 10 · 2023: 4 · 2022: 8 · 2021: 6 · 2020: 3 · 2019: 2
FWCI
0.46
Type
journal article
Language
en
Open access
Licence
Locations
  • Frontiers in Pediatrics (publishedVersion, cc-by)
  • Europe PMC (PubMed Central) (publishedVersion, cc-by)
  • PubMed Central (publishedVersion, cc-by)
  • PubMed (publishedVersion)
  • DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion)
  • UCL Discovery (University College London) (submittedVersion)
Topics
  • Health Sciences › Medicine › Rheumatology › Systemic Lupus Erythematosus Research
  • Health Sciences › Medicine › Hematology › Blood Coagulation and Thrombosis Mechanisms
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Vasculitis and related conditions
Keywords
pediatric antiphospholipid syndrome, antiphospholipid antibodies, thromboembolism, deep vein thrombosis, recurrent thrombosis, systemic lupus erythematosus, anticoagulation, antiplatelet therapy, hydroxychloroquine, lupus anticoagulant, beta2-glycoprotein I, anticardiolipin antibodies, disease classification criteria, bleeding risk

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