{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T07:12:52Z","timestamp":1780557172295,"version":"3.54.1"},"reference-count":25,"publisher":"SAGE Publications","issue":"14","license":[{"start":{"date-parts":[[2022,10,7]],"date-time":"2022-10-07T00:00:00Z","timestamp":1665100800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Lupus"],"published-print":{"date-parts":[[2022,12]]},"abstract":"<jats:sec>\n                    <jats:title>Background\/Purpose<\/jats:title>\n                    <jats:p>APS ACTION Registry was created to study the outcomes of patients with persistently positive antiphospholipid antibodies (aPL) with or without other systemic autoimmune disease (SAIDx). Given that immunosuppression (IS) is used for certain aPL manifestations, for example, thrombocytopenia (TP), our primary objective was to describe the indications for IS in aPL-positive patients without other SAIDx. Secondly, we report the type of IS used in patients with selected microvascular or non-thrombotic aPL manifestations.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>An online database is used to collect clinical data. The inclusion criteria are positive aPL based on the laboratory section of the APS Classification Criteria, tested at least twice within one year prior to enrollment. Patients are followed every 12 \u00b1 3\u00a0months. For this descriptive retrospective and prospective analysis, we included aPL-positive patients without other SAIDx and excluded those with new SAIDx classification during follow-up. For each patient, we retrieved clinical data at baseline and follow-up including selected aPL manifestations (diffuse alveolar hemorrhage [DAH], antiphospholipid-nephropathy [aPL-N], livedoid vasculopathy [LV]-related skin ulcers, TP, autoimmune hemolytic anemia [AIHA], cardiac valve disease [VD]), and IS medications.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Of 899 patients enrolled, 537 were included in this analysis (mean age 45 \u00b1 13\u00a0years, female 377 [70%], APS Classification in 438 [82%], and at least one selected microvascular or non-thrombotic aPL manifestation in 141 (26%)). Of 537 patients, 76 (14%) were reported to use IS (ever), and 41\/76 (54%) received IS primarily for selected aPL manifestation. In six of 8 (75%) DAH patients, 6\/19 (32%) aPL-N, 4\/28 (14%) LV, 25\/88 (28%) TP, 6\/11 (55%) AIHA, and 1\/43 (2%) VD, the IS (excluding corticosteroids\/hydroxychloroquine) indication was specific for selected aPL manifestation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>In our international cohort, 14% of aPL-positive patients without other SAIDx were reported to receive IS; the indication was at least one of the selected microvascular and\/or non-thrombotic aPL-related manifestations in half. Thrombocytopenia was the most frequent among those selected aPL-related manifestations; however, approximately one-third received IS specifically for that indication. Diffuse alveolar hemorrhage was frequently treated with IS followed by AIHA and aPL-N. Systematic controlled studies are urgently needed to better define the role of IS in APS.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/09612033221128742","type":"journal-article","created":{"date-parts":[[2022,10,7]],"date-time":"2022-10-07T14:26:42Z","timestamp":1665152802000},"page":"1770-1776","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":9,"title":["Immunosuppression use in primary antiphospholipid antibody-positive patients: Descriptive analysis of the AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) Clinical Database and Repository (\u201cRegistry\u201d)"],"prefix":"10.1177","volume":"31","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9099-8649","authenticated-orcid":false,"given":"Zeynep B","family":"Erton","sequence":"first","affiliation":[{"name":"Rheumatology, Hospital for Special Surgery, New York, NY, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rebecca","family":"K Leaf","sequence":"additional","affiliation":[{"name":"Hematology, Massachusetts General Hospital, Boston, MA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0381-1808","authenticated-orcid":false,"given":"Danieli","family":"de Andrade","sequence":"additional","affiliation":[{"name":"Rheumatology, University of S\u00e3o Paulo, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ann E","family":"Clarke","sequence":"additional","affiliation":[{"name":"Clinical Epidemiology, University of Calgary, Calgary, AB, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2238-0975","authenticated-orcid":false,"given":"Maria G","family":"Tektonidou","sequence":"additional","affiliation":[{"name":"Pathophysiology, National and Kapodistrian University of Athens, Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2064-6071","authenticated-orcid":false,"given":"Vittorio","family":"Pengo","sequence":"additional","affiliation":[{"name":"Cardiothoracic and Vascular Sciences, Padova University Hospital, Padova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1266-9441","authenticated-orcid":false,"given":"Savino","family":"Sciascia","sequence":"additional","affiliation":[{"name":"Centro Multidisciplinare di Ricerche di Immunopatologia e Documentazione su Malattie Rare, Struttura Complessa a Direzione Universitaria di Immunologia Clinica, University of Turin, Turin, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amaia","family":"Ugarte","sequence":"additional","affiliation":[{"name":"Rheumatology, BioCruces Bizkaia Health Research Institute, Barakaldo, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H. Michael","family":"Belmont","sequence":"additional","affiliation":[{"name":"Rheumatology, New York University Langone Medical Center, New York, NY, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Gerosa","sequence":"additional","affiliation":[{"name":"Clinical Sciences and Community Health, University of Milan, Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul R","family":"Fortin","sequence":"additional","affiliation":[{"name":"Rheumatology, CHU de Qu\u00e9bec- Universit\u00e9 Laval, Quebec, QC, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chary","family":"Lopez-Pedrera","sequence":"additional","affiliation":[{"name":"Rheumatology, Maimonides Institute for Biomedical Research of C\u00f3rdoba, C\u00f3rdoba, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tatsuya","family":"Atsumi","sequence":"additional","affiliation":[{"name":"Medicine II, Hokkaido University Hospital, Sapporo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhouli","family":"Zhang","sequence":"additional","affiliation":[{"name":"Rheumatology, Peking University First Hospital, Beijing, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2032-390X","authenticated-orcid":false,"given":"Hannah","family":"Cohen","sequence":"additional","affiliation":[{"name":"Haematology, University College London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6715-0180","authenticated-orcid":false,"given":"Guilherme","family":"Ramires de Jes\u00fas","sequence":"additional","affiliation":[{"name":"Obstetrics, Universidade Do Estado Do Rio de Janeiro, RJ, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1718-5094","authenticated-orcid":false,"given":"David W","family":"Branch","sequence":"additional","affiliation":[{"name":"Obstetrics and Gynecology, University of Utah and Intermountain Healthcare, Salt Lake City, UT, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Denis","family":"Wahl","sequence":"additional","affiliation":[{"name":"Rheumatology, Universit\u00e9 de Lorraine, Inserm DCAC, and CHRU-Nancy, Nancy, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Andreoli","sequence":"additional","affiliation":[{"name":"Rheumatology, University of Brescia, Brescia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0839-5644","authenticated-orcid":false,"given":"Esther","family":"Rodriguez-Almaraz","sequence":"additional","affiliation":[{"name":"Rheumatology, Hospital Universitario 12 de Octubre, Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1441-5373","authenticated-orcid":false,"given":"Michelle","family":"Petri","sequence":"additional","affiliation":[{"name":"Rheumatology, Johns Hopkins University, Baltimore, MD, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppe","family":"Barilaro","sequence":"additional","affiliation":[{"name":"Rheumatology, Hospital Clinic Barcelona, Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6721-7755","authenticated-orcid":false,"given":"Yu","family":"Zuo","sequence":"additional","affiliation":[{"name":"Rheumatology, University of Michigan, Ann Arbor, MI, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5659-3955","authenticated-orcid":false,"given":"Bahar","family":"Artim-Esen","sequence":"additional","affiliation":[{"name":"Internal Medicine, 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Calgary, Calgary, AB, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8623-139X","authenticated-orcid":false,"given":"Maria L","family":"Bertolaccini","sequence":"additional","affiliation":[{"name":"Vascular Surgery, King\u2019s College London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Roubey","sequence":"additional","affiliation":[{"name":"Rheumatology, Allergy & Immunolog, University of North Carolina, Chapel Hill, NC, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Doruk","family":"Erkan","sequence":"additional","affiliation":[{"name":"Barbara Volcker Center for Women and Rheumatic Disease, Hospital for Special Surgery Weill Cornell Medicine, New York, NY, 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