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Cited by 5Systemic Lupus Erythematosus ResearchVasculitis and related conditionsSystemic Sclerosis and Related Diseases

Non-Criteria Antiphospholipid Antibodies and Pediatric Rheumatic Disease: A Case Series

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Mahmud, Shawn A., Bullock, Danielle R., Correll, Colleen K., Hobday, Patricia M., Riskalla, Mona M., Vehe, Richard K., Binstadt, Bryce A.·2022·Pediatric Rheumatology
DOI 10.1186/s12969-022-00732-4PMC PMC9392300
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Abstract

Background: Non-criteria antiphospholipid antibodies (NC-aPL) are a relatively undefined subgroup of antiphospholipid antibodies (aPL). Knowledge about NC-aPL in adults is limited and even less is known in pediatric patients. Routine tests for antiphospholipid syndrome (APS)—a clinical state marked by the presence of aPL in association with vascular thrombosis—usually include lupus anticoagulant (LAC), anti-cardiolipin (aCL) and -beta-2 glycoprotein I (aβ2GPI). LAC is a functional screen for prothrombotic aPL, while the latter tests identify specific autoantibodies. Specific targets of NC-aPL include, but are not limited to, phosphatidylethanolamine, phosphatidylserine, and prothrombin. Presentation of cases: We present single-center data from eight pediatric patients with NC-aPL identified during a three-year period. All patients had presenting features raising suspicion for APS. Most patients were female with a primary rheumatic disease. One patient had a stroke. Another patient had alveolar hemorrhage and pulmonary hypertension. Raynaud’s phenomenon, rashes involving distal extremities, and headaches were common. Most patients had a positive LAC, yet their routine aPL tests were negative, prompting testing for NC-aPL. Conclusions: Our findings suggest NC-aPL are associated with typical signs and symptoms of APS in pediatric patients. Pediatricians and pediatric subspecialists should consider NC-aPL when clinical suspicion is high and routine aPL tests are negative, particularly when LAC is positive. While guidelines for NC-aPL do not yet exist for children or adults, these autoantibodies have pathogenic potential. Actionable items could include evaluation for the presence of other (primary) rheumatic diseases, and consultation with hematologists and/or obstetricians regarding anticoagulation/platelet inhibition and thrombosis education. Future guidelines regarding NC-aPL will only be generated by gathering more data, ideally prospectively.

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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 [109]

    The Presentation of Cases says infections can cause transient antiphospholipid-antibody positivity, and reports that most patients had a positive lupus anticoagulant and abnormal coagulation tests. It does not identify upper respiratory infections or describe an isolated prolonged aPTT found during preoperative testing for (adeno)tonsillectomy; no infectious cause was identified in its case of transient antibody positivity. (LLM, conf=0.82)

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Cited by

Sorted by citations

Authors
Mahmud, S. A., Bullock, D. R., Correll, C. K., Hobday, P. M., Riskalla, M. M., Vehe, R. K., & Binstadt, B. A.
Affiliations
University of Minnesota (US)
Corresponding author
Binstadt, Bryce A.
ORCID
Venue
Pediatric Rheumatology
Publisher
Springer Science and Business Media LLC
Published
2022-08-20
Volume
20
Issue
1
Pages
70-70
ISSN
1546-0096 (ISSN-L)
DOI
10.1186/s12969-022-00732-4
PMC
PMC9392300
OpenAlex
W4294094027
Cited by
5
References
0
Citations by year
2026: 1 · 2024: 2 · 2023: 1 · 2022: 1
FWCI
0.73
Type
journal article
Language
en
Open access
Licence
Locations
  • Pediatric Rheumatology (publishedVersion, cc-by)
  • PubMed Central (publishedVersion, cc-by)
  • PubMed (publishedVersion)
  • DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
Funders
University of Minnesota
Topics
  • Health Sciences › Medicine › Rheumatology › Systemic Lupus Erythematosus Research
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Vasculitis and related conditions
  • Health Sciences › Medicine › Pathology and Forensic Medicine › Systemic Sclerosis and Related Diseases
Keywords
pediatric antiphospholipid syndrome, lupus anticoagulant, pediatric rheumatic diseases, thrombosis, alveolar hemorrhage, pulmonary hypertension, stroke, autoantibody detection, anticoagulation
MeSH terms
Adult, Autoantibodies, Child, Female, Humans, Male, Rheumatic Diseases, Thrombosis, Lupus Coagulation Inhibitor, Antiphospholipid Syndrome, Antibodies, Antiphospholipid

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