Cited by 24Systemic Lupus Erythematosus ResearchBlood Coagulation and Thrombosis MechanismsVasculitis and related conditions

Pathogenesis of the antiphospholipid syndrome revisited: time to challenge the dogma

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Lackner, K.J., Müller‐Calleja, N.·2016·Journal of Thrombosis and Haemostasis

Abstract

For more than a decade the antiphospholipid syndrome (APS) has been reported to be caused mainly by antiphospholipid antibodies (aPL), which are not directed against phospholipids but against a complex of phospholipids and phospholipid binding proteins, so called cofactors (e.g. β2-glycoprotein I [β2GPI]). In fact, many researchers propose that the only relevant antigens in the APS are the cofactors themselves, with β2GPI being the most important. Antibodies that bind to phospholipids in a cofactor-independent manner are considered insignificant for the pathogenesis of the APS. We review the evidence for this current pathophysiologic concept and argue that it has never been proven and is now clearly no longer tenable. First, there is undisputable evidence that cofactor-independent aPL are pathogenic and present in the blood of APS patients. Second, available epidemiologic and clinical studies do not support a dominant pathogenic role for anti-β2GPI.

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Crossref · 10.1111/jth.13320

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  • These results add to the growing body of evidence that shows inconsistent associations between aPL-associated autoantibodies and anamnestic thrombotic risk.

    “These results confirm and validate several previous reports that have implicated these antibodies in the pathogenesis of APS [9, 10, 12, 13, 29, 30], but they also add to the growing body of evidence that shows inconsistent associations between aPL-associated autoantibodies and anamnestic thrombotic risk [3, 16].”

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Authors
Lackner, K. J., & Müller‐Calleja, N.
Affiliations
University Medical Center of the Johannes Gutenberg University Mainz (DE)
Corresponding author
Lackner, K.J.
Venue
Journal of Thrombosis and Haemostasis
Publisher
Elsevier BV
Published
2016-06
Volume
14
Issue
6
Pages
1117-1120
ISSN
1538-7836 (ISSN-L)
OpenAlex
W2305409522
Cited by
24
References
31
Citations by year
2025: 1 · 2024: 2 · 2021: 2 · 2020: 1 · 2019: 3 · 2018: 4 · 2017: 7 · 2016: 4
FWCI
6.41
Type
journal article
Language
en
Open access
Locations
  • Journal of Thrombosis and Haemostasis (publishedVersion)
  • PubMed (publishedVersion)
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
antiphospholipid syndrome, pathogenesis, antiphospholipid antibodies, beta2-glycoprotein I, phospholipid-binding proteins, autoantibody pathogenicity
MeSH terms
Humans, Thromboembolism, Antiphospholipid Syndrome, Antibodies, Antiphospholipid, beta 2-Glycoprotein I

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