Vascular liver disorders in patients with antiphospholipid syndrome: a national retrospective multicentre study

Abstract Objective Antiphospholipid syndrome (APS) is an acquired autoimmune prothrombotic condition. Vascular liver disorders (VLD), such as portal vein thrombosis (PVT), Budd-Chiari syndrome (BCS) and porto-sinusoidal vascular disorder (PSVD), are rare and related to an underlying hypercoagulable state in most cases. We aimed to describe the clinical and immunological features of APS patients with VLD. Methods The study is a national, multicentre, retrospective study of APS patients followed in 4 French tertiary university centres, including 3 national referral centres for rare autoimmune diseases. Clinical, serological and liver characteristics at diagnosis of APS patients with VLD were collected. New thrombotic events and major bleeding during follow-up were analysed. Results Forty-one VLD were reported in 34 APS patients (59% women; mean age 35+/-15 years; 65% of primary APS) including PVT (n = 26/41, 63%), BCS (n = 12/41, 29%) and PSVD (n = 3/41, 7%). VLD was the first thrombotic event in 79% (n = 27/34) of patients. Fifteen patients (n = 15/34, 44%) had portal hypertension, including 10 (n = 10/34, 29%) with esophageal varices at the time of VLD diagnosis. All patients were treated with oral anticoagulants including VKA in all but one case. Over a median of 9.5 (5;14) years of follow-up, 62% (n = 21/34) of patients displayed a new thrombotic event and 26% (n = 9/34) suffered major bleeding. Conclusion Although rare, VLD may be the presenting manifestation of APS. APS patients with VLD are at high risk of both recurrent thrombotic events and major bleeding.