Damage measured by Damage Index for Antiphospholipid Syndrome (DIAPS) in antiphospholipid antibody-positive patients included in the APS ACTION registry

Author:

Balbi Gustavo G M12ORCID,Ahmadzadeh Yasaman3,Tektonidou Maria G4ORCID,Pengo Vittorio5ORCID,Sciascia Savino6ORCID,Ugarte Amaia7,Belmont H Michael8ORCID,Lopez-Pedrera Chary9,Fortin Paul R10,Wahl Denis1112ORCID,Gerosa Maria13ORCID,de Jesús Guilherme R14,Ji Lanlan15,Atsumi Tatsuya16,Efthymiou Maria17,Branch D Ware18,Nalli Cecilia19,Rodriguez Almaraz Esther20ORCID,Petri Michelle21,Cervera Ricard22ORCID,Knight Jason S23,Artim-Esen Bahar24,Willis Rohan25,Bertolaccini Maria Laura26,Cohen Hannah17,Roubey Robert27,Erkan Doruk3,de Andrade Danieli Castro Oliveira1ORCID,Vega JoAnn,Pons-Estel Guillermo,Giannakopoulos Bill,Krilis Steve,de Jesus Guilherme,Levy Roger,Signorelli Flavio,Andrade Danieli,Balbi Gustavo,Clarke Ann E,Skeith Leslie,Fortin Paul R,Ji Lanlan,Zhang Zhouli,Yang Chengde,Shi Hui,Zuily Stephane,Wahl Denis,Tektonidou Maria G,Nalli Cecilia,Andreoli Laura,Tincani Angela,Chighizola Cecilia B,Gerosa Maria,Meroni Pierluigi,Pengo Vittorio,Cheng Chunyan,Pazzola Giulia,Sciascia Savino,Foddai Silvia,Radin Massimo,Davis Stacy,Amengual Olga,Atsumi Tatsuya,Uthman Imad,Limper Maarten,de Groot Philip,Ruiz - Irastorza Guillermo,Ugarte Amaia,Rodriguez-Pinto Ignasi,Cervera Ricard,Pardos-Gea Jose,Rodriguez Almaraz Esther,Zamorano Maria Angeles Aguirre,Lopez-Pedrera Chary,Artim-Esen Bahar,Bertolaccini Maria Laura,Cohen Hannah,Efthymiou Maria,Mackie Ian,Sanna Giovanni,Knight Jason,Zuo Yu,Petri Michelle,Leaf Rebecca K,Roubey Robert,Ortel Thomas,Willis Rohan,Kello Nina,Belmont MichaelORCID,Levine Steven,Rand Jacob,Barbhaiya Medha,Erkan Doruk,Salmon Jane,Lockshin Michael,Duarte Garcia Ali A,Ware Branch D,

Affiliation:

1. Universidade de São Paulo , São Paulo, São Paulo, Brazil

2. Universidade Federal de Juiz de Fora , Juiz de Fora, Minas Gerais, Brazil

3. Barbara Volcker Center for Women and Rheumatic Disease, Hospital for Special Surgery, Weill Cornell Medicine , New York, NY, USA

4. National and Kapodistrian University of Athens , Athens, Greece

5. University Hospital Padova , Padova, Italy

6. Center of Research of Immunopathology and Rare Diseases, University of Turin , Turin, Italy

7. Hospital Universitario Cruces , País Vasco, Barakaldo, Spain

8. Hospital for Joint Diseases, New York University , New York, NY, USA

9. Rheumatology Service, IMIBIC/Reina Sofia Hospital, University of Cordoba , Cordoba, Spain

10. CHU de Québec-Université Laval , Québec, Québec, Canada

11. Université de Lorraine, INSERM, DCAC , Nancy, France

12. Vascular Medicine Division and Regional Competence Center for Rare Vascular and Systemic Autoimmune Diseases, CHRU-Nancy , Nancy, France

13. Clinical Immunology & Rheumatology Unit, IRCCS Istituto Auxologico Italiano , Milan, Italy

14. Universidade do Estado do Rio de Janeiro , Rio de Janeiro, Brazil

15. Rheumatology and Immunology Department, Peking University First Hospital , Beijing, China

16. Hokkaido University Hospital , Sapporo, Japan

17. Haemostasis Research Unit, Department of Haematology, University College London , London, UK

18. University of Utah and Intermountain Healthcare , Salt Lake City, UT, USA

19. Rheumatology and Immunology Unit, ASST Spedali Civili of Brescia , Brescia, Italy

20. Hospital Universitario, 12 de Octubre , Madrid, Spain

21. Johns Hopkins University School of Medicine , Baltimore, MD, USA

22. Department of Autoimmune Diseases, Hospital Clínic , Barcelona, Catalonia, Spain

23. Division of Rheumatology, University of Michigan , Ann Arbor, MI, USA

24. Istanbul University School of Medicine , İstanbul, Turkey

25. Antiphospholipid Standardization Laboratory, University of Texas Medical Branch , Galveston, TX, USA

26. Academic Department of Vascular Surgery, King’s College London British Heart Foundation Centre of Excellence, School of Cardiovascular Medicine & Sciences , London, UK

27. University of North Carolina , Chapel Hill, NC, USA

Abstract

Abstract Objectives Our primary objective was to quantify damage burden measured by Damage Index for Antiphospholipid Syndrome (DIAPS) in aPL-positive patients with or without a history of thrombosis in an international cohort (the APS ACTION cohort). Secondly, we aimed to identify clinical and laboratory characteristics associated with damage in aPL-positive patients. Methods In this cross-sectional study, we analysed the baseline damage in aPL-positive patients with or without APS classification. We excluded patients with other autoimmune diseases. We analysed the demographic, clinical and laboratory characteristics based on two subgroups: (i) thrombotic APS patients with high vs low damage; and (ii) non-thrombotic aPL-positive patients with vs without damage. Results Of the 826 aPL-positive patients included in the registry as of April 2020, 586 with no other systemic autoimmune diseases were included in the analysis (412 thrombotic and 174 non-thrombotic). In the thrombotic group, hyperlipidaemia (odds ratio [OR] 1.82; 95% CI 1.05, 3.15; adjusted P = 0.032), obesity (OR 2.14; 95% CI 1.23, 3.71; adjusted P = 0.007), aβ2GPI high titres (OR 2.33; 95% CI 1.36, 4.02; adjusted P = 0.002) and corticosteroid use (ever) (OR 3.73; 95% CI 1.80, 7.75; adjusted P < 0.001) were independently associated with high damage at baseline. In the non-thrombotic group, hypertension (OR 4.55; 95% CI 1.82, 11.35; adjusted P = 0.001) and hyperlipidaemia (OR 4.32; 95% CI 1.37, 13.65; adjusted P = 0.013) were independent predictors of damage at baseline; conversely, single aPL positivity was inversely correlated with damage (OR 0.24; 95% CI 0.075, 0.77; adjusted P = 0.016). Conclusions DIAPS indicates substantial damage in aPL-positive patients in the APS ACTION cohort. Selected traditional cardiovascular risk factors, steroids use and specific aPL profiles may help to identify patients more prone to present with a higher damage burden.

Publisher

Oxford University Press (OUP)

Subject

Pharmacology (medical),Rheumatology

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