Thrombocytopenia and autoimmune hemolytic anemia in antiphospholipid antibody-positive patients: Descriptive analysis of the AntiPhospholipid syndrome alliance for clinical trials and InternatiOnal networking (APS ACTION) clinical database and reposi

Background/PurposeAPS ACTION Registry was created to study the natural course of antiphospholipid syndrome (APS) over 10 years in persistently antiphospholipid antibody (aPL) positive patients with or without systemic autoimmune rheumatic diseases (SARDs). Our primary objective was to compare the characteristics of aPL-positive patients with or without thrombocytopenia (TP) and/or autoimmune hemolytic anemia (AIHA).MethodsThe registry inclusion criteria are positive aPL based on the Revised Sapporo APS Classification Criteria, tested at least twice within 1 year prior to enrollment. For the primary comparison of demographic, clinical, and serologic characteristics in this retrospective study, we divided patients into two groups: TP/AIHA ever and never. Thrombocytopenia was defined as a platelet count of <100,000 x 10<sup>9</sup>/L tested twice at least 12 weeks apart, and AIHA was defined as anemia with hemolysis and a positive direct antiglobulin test (DAT). For the secondary analysis, we compared patients with TP versus AIHA, and the immunosuppressive use stratified by systemic lupus erythematosus (SLE) classification.ResultsAs of April 2022, of 1,039 patients (primary aPL/APS: 618 [59%]; SLE classification: 334 [31%]) included in the registry, 228 (22%) had baseline (historical or current) TP and/or AIHA (TP only: 176 [17%]; AIHA only: 35 [3%], and both: 17 [2%]). Thrombocytopenia and/or AIHA was significantly associated with Asian race, SLE classification, cardiac valve disease, catastrophic/microvascular APS, triple aPL (lupus anticoagulant, anticardiolipin antibody, and anti-β<sub>2</sub>-glycoprotein-I antibody) positivity, and SLE-related serologic and inflammatory markers. When 101/618 (16%) primary aPL/APS patients and 101/334 (34%) SLE patients with TP and/or AIHA were compared, azathioprine and mycophenolate mofetil were more commonly reported in lupus patients, however corticosteroid, intravenous immunoglobulin, and rituximab use were similar between groups.ConclusionIn our large multi-center international cohort of persistently aPL-positive patients, approximately one-fifth had active or historical TP and/or AIHA at registry entry; half of these patients had additional SLE. Cardiac valve disease, catastrophic/microvascular APS, and triple aPL-positivity were aPL-related clinical and laboratory manifestations associated with TP and/or AIHA, suggesting a more severe APS clinical phenotype in aPL-patients with TP and/or AIHA.

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Lupus
Published
2025-04-03
DOI
https://doi.org/10.1177/09612033251332258
Primary Topic
Systemic Lupus Erythematosus Research
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article
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article

Thrombocytopenia and autoimmune hemolytic anemia in antiphospholipid antibody-positive patients: Descriptive analysis of the AntiPhospholipid syndrome alliance for clinical trials and InternatiOnal networking (APS ACTION) clinical database and reposi

Esther Rodríguez-Almaraz, Guilherme Ramires de Jesús, Vittorio Pengo, Maria Efthymiou et al.
Lupus
Systemic Lupus Erythematosus Research
article

Thrombocytopenia and autoimmune hemolytic anemia in antiphospholipid antibody-positive patients: Descriptive analysis of the AntiPhospholipid syndrome alliance for clinical trials and InternatiOnal networking (APS ACTION) clinical database and reposi

Esther Rodríguez-Almaraz, Guilherme Ramires de Jesús, Vittorio Pengo, Maria Efthymiou, Nina Kello, Savino Sciascia, Ann E. Clarke, Alí Duarte‐García, María Laura Bertolaccini, Pier Luigi Meroni, Hui Shi, Rebecca Karp Leaf, D. Paredes, Leslie Skeith, Bahar Artım-Esen, Ricard Cervera, José Pardos‐Gea, Yu Zuo, Massimo Radin, Giulia Pazzola, H. Michael Belmont, Rosana Quintana, Rohan Willis, Laura Andréoli, C. López-Pedrera, D. Ware Branch, Hannah Cohen, Maria G. Tektonidou, Paul R. Fortin, Zhouli Zhang, Tatsuya Atsumi, Zeynep Belce Erton, Michelle Petri, Danieli Andrade, Doruk Erkan, Megan R W Barber, Robert Roubey
article en

Abstract

Background/PurposeAPS ACTION Registry was created to study the natural course of antiphospholipid syndrome (APS) over 10 years in persistently antiphospholipid antibody (aPL) positive patients with or without systemic autoimmune rheumatic diseases (SARDs). Our primary objective was to compare the characteristics of aPL-positive patients with or without thrombocytopenia (TP) and/or autoimmune hemolytic anemia (AIHA).MethodsThe registry inclusion criteria are positive aPL based on the Revised Sapporo APS Classification Criteria, tested at least twice within 1 year prior to enrollment. For the primary comparison of demographic, clinical, and serologic characteristics in this retrospective study, we divided patients into two groups: TP/AIHA ever and never. Thrombocytopenia was defined as a platelet count of <100,000 x 10<sup>9</sup>/L tested twice at least 12 weeks apart, and AIHA was defined as anemia with hemolysis and a positive direct antiglobulin test (DAT). For the secondary analysis, we compared patients with TP versus AIHA, and the immunosuppressive use stratified by systemic lupus erythematosus (SLE) classification.ResultsAs of April 2022, of 1,039 patients (primary aPL/APS: 618 [59%]; SLE classification: 334 [31%]) included in the registry, 228 (22%) had baseline (historical or current) TP and/or AIHA (TP only: 176 [17%]; AIHA only: 35 [3%], and both: 17 [2%]). Thrombocytopenia and/or AIHA was significantly associated with Asian race, SLE classification, cardiac valve disease, catastrophic/microvascular APS, triple aPL (lupus anticoagulant, anticardiolipin antibody, and anti-β<sub>2</sub>-glycoprotein-I antibody) positivity, and SLE-related serologic and inflammatory markers. When 101/618 (16%) primary aPL/APS patients and 101/334 (34%) SLE patients with TP and/or AIHA were compared, azathioprine and mycophenolate mofetil were more commonly reported in lupus patients, however corticosteroid, intravenous immunoglobulin, and rituximab use were similar between groups.ConclusionIn our large multi-center international cohort of persistently aPL-positive patients, approximately one-fifth had active or historical TP and/or AIHA at registry entry; half of these patients had additional SLE. Cardiac valve disease, catastrophic/microvascular APS, and triple aPL-positivity were aPL-related clinical and laboratory manifestations associated with TP and/or AIHA, suggesting a more severe APS clinical phenotype in aPL-patients with TP and/or AIHA.

LupusVol. 34(6)
Intermountain Healthcare (US), University of North Carolina at Chapel Hill (US), Hospital for Special Surgery (US), Northwell Health (US), University of Padua (IT), Johns Hopkins University (US), University of Calgary (CA), Universidade de São Paulo (BR), SUNY Downstate Health Sciences University (US), Shanghai Jiao Tong University (CN), King's College London (GB), University of Milan (IT), National and Kapodistrian University of Athens (GR), Peking University (CN), Cornell University (US), University of Utah (US), University of Michigan (US), Johns Hopkins Medicine (US), Ruijin Hospital (CN), WinnMed (US), Brescia University (US), Universidade do Estado do Rio de Janeiro (BR), NYU Langone Health (US), Massachusetts General Hospital (US), BioCruces Health research Institute (ES), Azienda Sanitaria Unità Locale di Reggio Emilia (IT), Instituto Maimónides de Investigación Biomédica de Córdoba (ES), Hospital Universitario 12 De Octubre (ES), Hospital Provincial de Rosario (AR), Hospital Clínic de Barcelona (ES), Hospital Universitario Reina Sofía (ES), Mayo Clinic in Arizona (US), Torino e-district (IT), Association of British Orchestras (GB), Peking University First Hospital (CN), IRCCS Istituto Auxologico Italiano (IT), Hokkaido University Hospital (JP), Istituti di Ricovero e Cura a Carattere Scientifico (IT), Université Laval (CA), University College London (GB), University of Córdoba (ES), University of Turin (IT), The University of Texas Medical Branch at Galveston (US), Cordoba University (US), Istanbul University (TR), University of Brescia (IT)
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Openalex Percentile: Top 90%
Systemic Lupus Erythematosus Research
DOISourceOpenAlex
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