Damage measured by Damage Index for Antiphospholipid Syndrome (DIAPS) in antiphospholipid antibody-positive patients included in the APS ACTION registry
Authors: Balbi Gustavo G M , Ahmadzadeh Yasaman , Tektonidou Maria G , Pengo Vittorio , Sciascia Savino , Ugarte Amaia , Belmont H Michael , Lopez-Pedrera Chary , Fortin Paul R , Wahl Denis , Gerosa Maria , de Jesús Guilherme R , Ji Lanlan , Atsumi Tatsuya , Efthymiou Maria , Branch D Ware , Nalli Cecilia , Rodriguez Almaraz Esther , Petri Michelle , Cervera Ricard , Knight Jason S , Artim-Esen Bahar , Willis Rohan , Bertolaccini Maria Laura , Cohen Hannah , Roubey Robert , Erkan Doruk , de Andrade Danieli Castro Oliveira ,   for AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) Clinical Database and Repository (APS ACTION), 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 Michael , Levine Steven , Rand Jacob , Barbhaiya Medha , Erkan Doruk , Salmon Jane , Lockshin Michael , Duarte Garcia Ali A , Ware Branch D 
Published: June 2023

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 &lt; 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.