Cited by 620Systemic Lupus Erythematosus ResearchReproductive System and PregnancyBlood Coagulation and Thrombosis Mechanisms

Pathogenesis of antiphospholipid syndrome: understanding the antibodies

Meroni, Pier Luigi, Borghi, M. Orietta, Raschi, Elena, Tedesco, Francesco·2011·Nature Reviews Rheumatology

Abstract

Antiphospholipid antibodies (aPL) are both diagnostic markers for, and pathogenic drivers of, antiphospholipid syndrome (APS). Although the presence of aPL is a necessary pre-condition, APS-associated clotting is seemingly triggered by an additional 'second hit', frequently related to innate inflammatory immune responses. β(2) glycoprotein I (β(2)GPI)-dependent aPL, the most important subset of these antibodies, mediate several--not necessarily alternative--thrombogenic mechanisms, mainly on the basis of their reactivity with β(2)GPI expressed on the membrane of cells that participate in the coagulation cascade. Recurrent pregnancy complications associated with aPL cannot be explained solely by thrombosis, and alternative pathogenic mechanisms have been reported. Although one in vivo model of fetal loss suggests a mechanism of aPL-mediated acute placental inflammation, other models and the histopathological examination of APS placentae do not support a widespread inflammatory signature. β(2)GPI-dependent aPL are thought to recognize their antigen on placental tissues, inhibit the growth and differentiation of trophoblasts, and eventually cause defective placentation. Why antibodies with similar antigen specificity produce different clinical manifestations is not clear. Characterization of the molecular basis of the pathogenic mechanisms involved, including the putative second hits and the role of complement activation, might offer an answer to this question.

Integrity signals

Registry

Crossref · 10.1038/nrrheum.2011.52

Cites

89 of 89 references verified0 citation errors · 0 not checkable

Statistics

Recomputing p-values needs the paper’s full text.

Claims the paper makes

This paper’s full text isn’t openly available, so its claims and self-check weren’t run. The registry and citation checks still apply.

Claims other papers make about this one

Each is checked against this paper’s own text: does it say what the citing paper says it does?

Cites

Flagged first, then by citations

Cited by

Sorted by citations

Authors
Meroni, P. L., Borghi, M. O., Raschi, E., & Tedesco, F.
Affiliations
  • University of Milan (IT)
  • IRCCS Istituto Auxologico Italiano (IT)
  • University of Trieste (IT)
Corresponding author
Meroni, Pier Luigi
Venue
Nature Reviews Rheumatology
Published
2011-05-10
Volume
7
Issue
6
Pages
330-339
ISSN
  • 1759-4790 (ISSN-L)
  • 1759-4804
OpenAlex
W1999555834
Cited by
620
References
89
Citations by year
2026: 38 · 2025: 34 · 2024: 35 · 2023: 34 · 2022: 39 · 2021: 47 · 2020: 34 · 2019: 41 · 2018: 43 · 2017: 43 · 2016: 54 · 2015: 33 · 2014: 63 · 2013: 37 · 2012: 36
FWCI
6.72
Type
journal article
Language
en
Open access
closed
Locations
  • Nature Reviews Rheumatology (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Rheumatology › Systemic Lupus Erythematosus Research
  • Life Sciences › Immunology and Microbiology › Immunology › Reproductive System and Pregnancy
  • Health Sciences › Medicine › Hematology › Blood Coagulation and Thrombosis Mechanisms
Keywords
antiphospholipid syndrome, antiphospholipid antibodies, beta2-glycoprotein I, thrombosis, recurrent pregnancy loss, placental inflammation, trophoblast differentiation, complement activation, innate immune response, autoantibody pathogenesis
MeSH terms
Female, Humans, Pregnancy, Pregnancy Complications, Antiphospholipid Syndrome, Antibodies, Antiphospholipid

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

Are you an author, or do you see a problem with this page? Request a review