@article{3034428,
    title = "Characteristics of Patients With Antiphospholipid Antibody Positivity in
the APS ACTION International Clinical Database and Repository",
    author = "Sevim, Ecem and Zisa, Diane and Andrade, Danieli and Sciascia, Savino and and Pengo, Vittorio and Tektonidou, Maria G. and Ugarte, Amaia and and Gerosa, Maria and Belmont, H. Michael and Aguirre Zamorano, Maria and Angeles and Fortin, Paul R. and Ji, Lanlan and Efthymiou, Maria and and Cohen, Hannah and Branch, D. Ware and Jesus, Guilherme Ramires and and Andreoli, Laura and Petri, Michelle and Rodriguez, Esther and Cervera, and Ricard and Knight, Jason S. and Atsumi, Tatsuya and Willis, Rohan and and Roubey, Robert and Bertolaccini, Maria Laura and Erkan, Doruk and and Barbhaiya, Medha and APS ACTION Investigators",
    journal = "Arthritis Care and Research",
    year = "2022",
    volume = "74",
    number = "2",
    pages = "324-335",
    publisher = "Wiley",
    issn = "2151-464X, 1529-0123",
    doi = "10.1002/acr.24468",
    abstract = "Objective To describe the baseline characteristics of patients with
positivity for antiphospholipid antibodies (aPLs) who were enrolled in
an international registry, the Antiphospholipid Syndrome (APS) Alliance
for Clinical Trials and International Networking (APS ACTION) clinical
database and repository, overall and by clinical and laboratory
subtypes. Methods The APS ACTION registry includes adults who
persistently had positivity for aPLs. We evaluated baseline
sociodemographic and aPL-related (APS classification criteria and
“non-criteria”) characteristics of patients overall and in subgroups
(aPL-positive without APS, APS overall, thrombotic APS only, obstetric
APS only, and both thrombotic APS/obstetric APS). We assessed baseline
characteristics of patients tested for the presence of three aPLs (lupus
anticoagulant [LAC] test, anticardiolipin antibody [aCL], and
anti-beta(2)-glycoprotein I [anti-beta(2)GPI]) antibodies by aPL
profiles (LAC only, single, double, and triple aPL positivity). Results
The 804 aPL-positive patients assessed in the present study had a mean
age of 45 +/- 13 years, were 74% female, and 68% White; additionally,
36% had other systemic autoimmune diseases. Of these 804 aPL-positive
patients, 80% were classified as having APS (with 55% having
thrombotic APS, 9% obstetric APS, and 15% thrombotic APS/obstetric
APS). In the overall cohort, 71% had vascular thrombosis, 50% with a
history of pregnancy had obstetric morbidity, and 56% had experienced
at least one non-criteria manifestation. Among those with three aPLs
tested (n = 660), 42% were triple aPL-positive. While single-, double-,
and triple aPL-positive subgroups had similar frequencies of vascular,
obstetric, and non-criteria events, these events were lowest in the
single aPL subgroup, which consisted of aCLs or anti-beta(2)GPI only.
Conclusion Our study demonstrates the heterogeneity of aPL-related
clinical manifestations and laboratory profiles in a multicenter
international cohort. Within single aPL positivity, LAC may be a major
contributor to clinical events. Future prospective analyses, using
standardized core laboratory aPL tests, will help clarify aPL risk
profiles and improve risk stratification."
}