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 (“Registry”)

Zeynep Belce Erton¹, Rebecca Karp- Leaf², Danieli Andrade³, Maria Tektonidou⁴, Vittorio Pengo⁵, Savino Sciascia⁶, Amaia Ugarte⁷, H. Michael Belmont⁸, Maria Gerosa⁹, Paul R Fortin¹⁰, Chary lopez-pedrera¹¹, Lanlan Ji¹², Tatsuya Atsumi¹³, Hannah Cohen¹⁴, Guilherme Ramires de Jesus¹⁵, D. Ware Branch¹⁶, Cecilia Nalli¹⁷, Nina Kello¹⁸, Michelle Petri¹⁹, Esther Rodriguez-Almaraz²⁰, Ricard Cervera²¹, Jason Knight²², Bahar Artim-Esen²³, Rohan Willis²⁴, Maria Laura Bertolaccini²⁵, Robert Roubey²⁶, Doruk Erkan¹ and on Behalf of APS ACTION¹, ¹Hospital for Special Surgery, New York, NY, ²Massachusetts General Hospital, Boston, MA, ³Hospital das Clinicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil, ⁴FORZAFORTE HELLAS LTD, Athens, Greece, ⁵Padova University Hospital, Padova, Italy, ⁶University of Turin, Turin, Italy, ⁷Hospital Universitario Cruces, Barakaldo, Spain, ⁸NYU School of Medicine, New York, NY, ⁹University of Milan, Milan, Italy, ¹⁰CHU de Quebec - Universite Laval, Québec City, QC, Canada, ¹¹IMIBIC/Reina Sofia Hospital/University of Córdoba, Córdoba, Spain, ¹²Peking University First Hospital, Beijing, China (People's Republic), ¹³Hokkaido University, Sapporo, Japan, ¹⁴Department of Haematology, University College London Hospitals NHS Foundation Trust, London, United Kingdom, ¹⁵Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil, ¹⁶University of Utah, Salt Lake City, UT, ¹⁷ASST SPEDALI CIVILI DI BRESCIA, Brescia, Italy, ¹⁸Zucker School of Medicine at Hofstra/Northwell Health, Manhasset, NY, ¹⁹Johns Hopkins University School of Medicine, Baltimore, MD, ²⁰Hospital Universitario 12 de Octubre, Madrid, Spain, ²¹Hospital Clinic Barcelona, Barcelona, Spain, ²²University of Michigan, Ann Arbor, MI, ²³Istanbul University School of Medicine, İstanbul, Turkey, ²⁴University of Texas Medical Branch, Galveston, TX, ²⁵King's College London, London, United Kingdom, ²⁶University of North Carolina, Chapel Hill, NC

Meeting: ACR Convergence 2021

Keywords: antiphospholipid syndrome, immunosuppression

Session Information

Date: Saturday, November 6, 2021

Title: Antiphospholipid Syndrome Poster (0069–0083)

Session Type: Poster Session A

Session Time: 8:30AM-10:30AM

Background/Purpose: The APS 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 diseases (SAIDx). Given data to support the role of immunosuppression (IS) in the management of APS patients with certain clinical phenotypes, e.g, diffuse alveolar hemorrhage (DAH), our primary objective was to characterize IS use in aPL-positive patients without other SAIDx.

Methods: A central online database was used to collect detailed clinical data. The inclusion criteria were positive aPL based on the laboratory section of the current APS Classification Criteria, tested at least twice within one year prior to enrollment. For this descriptive analysis, we only included aPL-positive patients without other SAIDx and excluded those with catastrophic APS (CAPS). We retrieved data on demographics, aPL/APS-related history including selected non-criteria manifestations (DAH, antiphospholipid-nephropathy [aPL-N], livedoid vasculopathy-related skin ulcers [LV], thrombocytopenia [TP], hemolytic anemia [HA], and cardiac valve disease [VD]); and IS use (ever) (Table).

Results: As of 1/2021, 866 patients were included in the registry; 325 (38%) were excluded due to another SAIDx and an additional five due to CAPS. Of the remaining 536 patients (mean age at entry: 45±13y; 70% female; 70% white; 432 [81%] meeting the APS Classification; and 143 [27%] with at least one selected non-criteria manifestations), 70 (13%) used IS (ever) excluding corticosteroids (CS) and hydroxychloroquine (HCQ). Of 70 IS users (non-CS/HCQ), 50 (71%) had at least one of the selected non-criteria manifestations. Of 143 patients with at least one of the selected non-criteria manifestations, 38 (27%) had no history of thrombosis; 19/38 (50%) received anticoagulation with/without antiplatelets, 15/38 (39%) antiplatelets alone, and 4/38 (11%) no antithrombotic agents. Four of 5 (80%) DAH patients, 6/15 (40%) aPL-N, 10/26 (39%) LV, 32/85 (38%) TP, 7/12 (58%) HA, and 9/35 (26%) VD patients were reported to receive non-CS/HCQ immunosuppression (Table).

Conclusion: In our multi-center international cohort, 13% of aPL-positive patients without other systemic autoimmune diseases, mostly those with selected non-criteria manifestations, were reported to use immunosuppressives other than corticosteroids and hydroxychloroquine. Given the inconsistent reporting of immunosuppression use in aPL-positive patients with non-criteria manifestations, systematic studies are urgently needed to better define the role of immunosuppression for different aPL-related non-criteria manifestations.

Disclosures: Z. Erton, None; R. Karp- Leaf, None; D. Andrade, None; M. Tektonidou, None; V. Pengo, None; S. Sciascia, None; A. Ugarte, None; H. Belmont, Alexion, 6; M. Gerosa, None; P. Fortin, Lilly, 1, AbbVie, 1, AstraZeneca, 1; C. lopez-pedrera, None; L. Ji, None; T. Atsumi, Takeda Pharmaceutical CO., Ltd., 6, Astellas Pharma Inc., 5, 6, Mitsubishi Tanabe Pharma Co., 5, 6, Chugai Pharmaceutical Co., Ltd., 5, 6, Daichii Sankyo Co. Ltd., 5, 6, Pfizer Inc., 2, 5, 6, Alexion Inc., 6, TEIJIN PHARMA LIMITED., 5, 6, Novartis Pharma K.K., 2, 5, 6, Eli Lilly Japan K.K., 5, 6, Kyowa Kirin Co., Ltd., 5, 6, AbbVie Inc., 2, 5, 6, NIPPON SHINYAKU CO.,LTD., 5, TAIHO PHARMACEUTICAL CO.,LTD., 5, Nippon Boehringer Ingelheim Co.,Ltd., 5, 6, Amgen Inc., 5, 6, UCB Japan Co. Ltd., 5, 6, Astra Zeneca plc, 2, 6, ONO Pharmaceutical Co., Ltd., 2, 5, Byaer Yakuhin, Ltd., 5; H. Cohen, Bayer Healthcare, 5, 6, 12, Support to attend scientific meetings; Honoraria for lectures at symposia paid to University College London Hospitals Charity, UCB Biopharma, 2, 12, Consultancy fees paid to University College London Hospitals Charity; G. de Jesus, None; D. Branch, UCB Pharmaceuticals, 1, 5; C. Nalli, None; N. Kello, None; M. Petri, Alexion, 1, Amgen, 1, Astrazeneca, 1, 5, Aurinia, 5, 6, Eli Lilly, 5, Emergent Biosolutions, 1, Exagen, 5, Gilead Biosciences, 2, GSK, 1, 5, IQVIA, 1, Idorsia Pharmaceuticals, 2, Janssen, 1, 5, Merck EMD Serono, 1, Momenta Pharmaceuticals, 2, PPD Development, 1, Sanofi, 2, Thermofisher, 5, UCB Pharmaceuticals, 2; E. Rodriguez-Almaraz, None; R. Cervera, None; J. Knight, None; B. Artim-Esen, None; R. Willis, Louisville APL Diagnostic Inc, 2; M. Bertolaccini, None; R. Roubey, None; D. Erkan, ACR/EULAR, 5, LCTC, 5, NIH/NIAID, 5, GSK, 5, 6, Exagen, 5, Alexion, 2, UCB, 2, UpToDate, 9, APS ACTION, 4; o. APS ACTION, None.

To cite this abstract in AMA style:

Erton Z, Karp- Leaf R, Andrade D, Tektonidou M, Pengo V, Sciascia S, Ugarte A, Belmont H, Gerosa M, Fortin P, lopez-pedrera C, Ji L, Atsumi T, Cohen H, de Jesus G, Branch D, Nalli C, Kello N, Petri M, Rodriguez-Almaraz E, Cervera R, Knight J, Artim-Esen B, Willis R, Bertolaccini M, Roubey R, Erkan D, APS ACTION o. 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 (“Registry”) [abstract]. Arthritis Rheumatol. 2021; 73 (suppl 9). https://acrabstracts.org/abstract/immunosuppression-use-in-primary-antiphospholipid-antibody-positive-patients-descriptive-analysis-of-the-antiphospholipid-syndrome-alliance-for-clinical-trials-and-international-networking-aps-actio/. Accessed .

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