16th International Congress on Antiphospholipid Antibodies Task Force Report on Antiphospholipid Syndrome Treatment Trends

Abstract

Antiphospholipid syndrome (APS), an acquired autoimmune thrombophilia, is characterised by thrombosis and/or pregnancy morbidity in association with persistent antiphospholipid antibodies. The 16th International Congress on Antiphospholipid Antibodies Task Force on APS Treatment Trends reviewed the current status with regard to existing and novel treatment trends for APS, which is the focus of this Task Force report. The report addresses current treatments and developments since the last report, on the use of direct oral anticoagulants in patients with APS, antiplatelet agents, adjunctive therapies (hydroxychloroquine, statins and vitamin D), targeted treatment including rituximab, belimumab, and anti-TNF agents, complement inhibition and drugs based on peptides of beta-2-glycoprotein I. In addition, the report summarises potential new players, including coenzyme Q10, adenosine receptor agonists and adenosine potentiation. In each case, the report provides recommendations for clinicians, based on the current state of the art, and suggests a clinical research agenda. The initiation and development of appropriate clinical studies requires a focus on devising suitable outcome measures, including a disease activity index, an optimal damage index, and a specific quality of life index.

Monitoring of anticoagulation in thrombotic antiphospholipid syndrome

“…13,14, Should recurrent thrombosis occur while on standardintensity VKA, other therapeutic options may include an increased target INR range, treatment dose LMWH, or the addition of antiplatelet therapy. [12][13][14] Warfarin should be used with caution in patients with aPLrelated thrombocytopenia, in view of its long half-life of 26 to 48 hours; 44 split-dose LMWH, half-life approximately 4 hours after subcutaneous injection, 45 should be considered instead. The prevalence of thrombocytopenia (platelets < 100 x 10 9 /L) was 26.9% in 1000 APS patients in the Euro-Phospholipid project.…”

“…The European Medicines Agency (EMA), following a risk assessment triggered by the TRAPS (Rivaroxaban in Thrombotic APS) RCT, 62 stated that DOACs are not recommended for thrombotic APS patients, especially those who are triple aPL‐positive 63 . The ISTH 12 and 16th International Congress on Antiphospholipid Antibodies Task Force Report on APS Treatment Trends 13 concur in recommending that in single‐ or double aPL‐positive APS patients following a first VTE, DOACs initiated as standard care may be continued, with consideration of the perceived risks and uncertainties and discussion with the patient, for shared decision‐making. The doses of DOACs used in APS patients reported in the literature have been shown to be as effective as warfarin at a target INR range of 2.0 to 3.0 in general‐population patients following a first VTE.…”

“…The RISAPS (Rivaroxaban for Stroke Patients with Antiphospholipid Syndrome) phase 2/3 RCT aims to assess the efficacy of high‐intensity rivaroxaban 15mg twice daily versus high‐intensity warfarin in APS patients with stroke or other brain ischaemic injury (ClinicalTrials.gov Identifier: NCT03684564). All cases of DOAC use in APS patients should be reported to the ISTH‐supported international registry (ClinicalTrials.gov Identifier: NCT04262492) 12,13 …”

“…9 Systematic reviews suggest that ~ 14% of patients with ischemic strokes and 17% of those under the age of 50 years have aPL. 7,10 These figures, as well as the estimated prevalence of APS, 50 per 100 000 of the population, with approximately 80% of patients having DVT or pulmonary embolism, and 45% stroke or TIA, 11 12,13 Parenteral anticoagulant options in APS include low molecular weight heparin (LMWH), unfractionated heparin (UFH), and fondaparinux. LMWH, in combination with low dose aspirin (LDA), is standard treatment for thrombotic APS during pregnancy.…”

“…The standard anticoagulant treatment for thrombotic APS is life‐long warfarin or an alternative vitamin K antagonist (VKA). The role of direct oral anticoagulants (DOACs) for thrombotic APS has not been established due to the lack of definitive evidence, and has been addressed in International Society on Thrombosis and Haemostasis (ISTH) and 16th International Congress on Antiphospholipid Antibodies Task Force Report on APS Treatment Trends guidance 12,13 . Parenteral anticoagulant options in APS include low molecular weight heparin (LMWH), unfractionated heparin (UFH), and fondaparinux.…”