System Maintenance Notice

Please note that maintenance work is scheduled between 4 AM and 5 AM - CET. During this time, you may experience reduced performance. Thanks for your patience.

Publications

Journal Article Ppublish

PubMed: 42690699 DOI: 10.1177/09612033261479899

Clinical characteristics of patients with primary antiphospholipid syndrome and "single" antiphospholipid antibody: Retrospective results from the APS ACTION clinical database and repository ("registry").

Lupus 35(13):1426-1432

Authors: Silvia Grazietta Foddai, Irene Cecchi, Massimo Radin, Laura Bertolaccini, Danieli de Andrade, Maria Tektonidou, Vittorio Pengo, Chary Lopez-Pedrera, Diana Paredes-Ruiz, Howard Michael Belmont, Paul R Fortin, Denis Wahl, Ware Branch, Maria Gerosa, Guilherme de Jesus, Zhouli Zhang, Tatsuya Atsumi, Maria Efthymiou, Laura Andreoli, Esther Rodriguez, Michelle Petri, Ricard Cervera, Bahar Artim-Esen, Rosana Quintana, Jason S Knight, Rohan Willis, Reyhan Kose Cobanoglu, Hannah Cohen, Doruk Erkan, Savino Sciascia

Affiliations

  • University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-net, ERN-ReCONNET and RITA-ERN Member), Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley, San Giovanni Bosco Hub Hospital, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.
  • School of Cardiovascular and Metabolic Medicine & Sciences, Faculty of Life Sciences & Medicine, King's College London, London, UK.
  • Rheumatology Department, University of São Paulo, São Paulo, São Paulo, Brazil.
  • Rheumatology Unit, First Department of Propaedeutic Internal Medicine, Joint Academic Rheumatology Program, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece.
  • Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Padua, Italy.
  • Rheumatology Service, Maimonides Institute for Biomedical Research of Córdoba (IMIBIC), Reina Sofia University Hospital, University of Córdoba, Córdoba, Spain.
  • Biobizkaia Health Research Institute, Barakaldo, Bizkaia, Spain.
  • Division of Rheumatology, Department of Medicine, NYU Grossman School of Medicine, New York, NY, USA.
  • Centre ARThrite, CHU de Québec-Université Laval, Department of Medicine, Université Laval, Québec City, Québec, Canada.
  • Department of Vascular Medicine and Angiology, CHRU Nancy, Faculty of Medicine, Université de Lorraine, Nancy, France.
  • University of Utah Health and Intermountain Healthcare, Salt Lake City, UT, USA.
  • Department of Clinical Sciences and Community Health, University of Milan; Lupus Clinic, Clinical Rheumatology Unit, ASST Gaetano Pini-CTO, Milan, Italy.
  • Department of Obstetrics, Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Rio de Janeiro, Brazil.
  • Peking University First Hospital, Peking University, Beijing, China.
  • Hokkaido University Hospital, Hokkaido University, Sapporo, Hokkaido, Japan.
  • University College London, London, UK.
  • Rheumatology and Clinical Immunology Unit, Department of Clinical and Experimental Sciences, University of Brescia and ASST Spedali Civili di Brescia, Brescia, Italy.
  • Hospital Universitario 12 de Octubre, Madrid, Spain.
  • Division of Rheumatology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
  • Department of Autoimmune Diseases, Hospital Clínic de Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Catalonia, Spain.
  • Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Türkiye.
  • Centro Regional de Enfermedades Autoinmunes y Reumáticas, Grupo Oroño (GO-CREAR), Rosario, Santa Fe, Argentina.
  • Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA.
  • University of Texas Medical Branch, Galveston, TX, USA.
  • Division of Rheumatology, Department of Internal Medicine, School of Medicine, Aydin Adnan Menderes University, Aydin, Türkiye.
  • Barbara Volcker Center for Women and Rheumatic Disease, Hospital for Special Surgery, Weill Cornell Medicine, New York, NY, USA.

Abstract

ObjectiveWhile triple antiphospholipid antibody (aPL) positivity is associated with a higher risk of thrombosis, the clinical significance of single aPL positivity remains unclear. This study aimed to assess the prevalence, clinical characteristics, and cardiovascular disease (CVD) risk profile of persistently single aPL-positive primary antiphospholipid syndrome (PAPS) patients.MethodsWe conducted a retrospective analysis for APS patients from the APS ACTION registry with confirmed persistent single aPL positivity (after testing for lupus anticoagulant [LA], anticardiolipin [aCL] IgG/IgM, or anti-β 2-glycoprotein I [aβ 2GPI] IgG/IgM), defined by the Revised Sapporo APS Classification Criteria. Triple aPL-tested single aPL-positive patients with no APS classification were used as a control group. Demographics, clinical characteristics, aPL profiles, and adjusted Global APS Score (aGAPSS) were compared between two groups.ResultsAmong 233 PAPS patients tested, 84 (36%) had single aPL positivity: 67 (79%) LA positive, eight (10%) aCL IgG or IgM positive, and 9 (11%) aβ 2GPI IgG or IgM positive. Among 97 aPL-positive patients with no APS classification, 22 (22.7%) had single aPL-positivity: nine LA positive, eight aCL IgG or IgM positive, and 5 aβ 2GPI IgG or IgM positive. Single LA positivity was significantly more common in PAPS patients, whereas aCL IgM or aβ 2GPI IgM positivity were significantly more common in those without APS classification. Historical thrombosis recurrence was observed in 26% of single aPL-positive PAPS patients, predominantly among those with LA positivity (86%).ConclusionsIn this international cohort of persistently aPL-positive individuals, 36% had single aPL positivity, mostly positive LA test. Although "single" aPL-positivity is traditionally considered as a lower-risk profile, findings highlight the importance of differentiating between single LA-positivity and single aCL/aβ 2GPI positivity, and the need for individualized risk stratification.

MeSH

Registries, Antibodies, Antiphospholipid, Classification, Lupus Erythematosus, Systemic, Antiphospholipid Syndrome, Lupus Coagulation Inhibitor, Thrombosis