Damage measured by Damage Index for Antiphospholipid Syndrome (DIAPS) in antiphospholipid antibody-positive patients included in the APS ACTION registry
Gustavo Guimarães Moreira Balbi, Yasaman Ahmadzadeh, Maria G. Tektonidou, Vittorio Pengo, Savino Sciascia, Amaia Ugarte, H. Michael Belmont, C. López-Pedrera, Paul R. Fortin, Denis Wahl, Maria Gerosa, Guilherme Ramires de Jesús, Lanlan Ji, Tatsuya Atsumi, Maria Efthymiou, D. Ware Branch, Cecilia Nalli, Esther Rodríguez-Almaraz, Michelle Petri, Ricard Cervera, Jason S. Knight, Bahar Artım-Esen, Rohan Willis, María Laura Bertolaccini, Hannah Cohen, Robert Roubey, Doruk Erkan, Danieli Andrade, JoAnn Vega, Guillermo Pons‐Estel, Bill Giannakopoulos, Steve Krilis, Guilherme Ramires de Jesús, Roger A. Levy, Flávio Signorelli, Danieli Andrade, Gustavo Guimarães Moreira Balbi, Ann E. Clarke, Leslie Skeith, Paul R. Fortin, Lanlan Ji, Zhouli Zhang, Chengde Yang, Hui Shi, Stéphane Zuily, Denis Wahl, Maria G. Tektonidou, Cecilia Nalli, Laura Andréoli, Anǵela Tincani, Cecilia Beatrice Chighizola, Maria Gerosa, Pier Luigi Meroni, Vittorio Pengo, Chunyan Cheng, Giulia Pazzola, Savino Sciascia, Silvia Grazietta Foddai, Massimo Radin, Stacy Davis, Olga Amengual, Tatsuya Atsumi, Imad Uthman, Maarten Limper, Philip de Groot, Guillermo Ruiz‐Irastorza, Amaia Ugarte, Ignasi Rodríguez‐Pintó, Ricard Cervera, José Pardos‐Gea, Esther Rodríguez-Almaraz, M. Á. Aguirre, C. López-Pedrera, Bahar Artım-Esen, María Laura Bertolaccini, Hannah Cohen, Maria Efthymiou, Ian Mackie, Giovanni Sanna, Jason S. Knight, Yu Zuo, Michelle Petri, Rebecca Karp Leaf, Robert Roubey, Thomas L. Ortel, Rohan Willis, Nina Kello, H. Michael Belmont, Steven R. Levine, Jacob H. Rand, Medha Barbhaiya, Doruk Erkan, Jane E. Salmon, Michael D. Lockshin, Ali A. Duarte Garcia, D. Ware Branch
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.