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Background: 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.
Objectives: We aimed to describe the clinical and immunological features of APS patients with VLD.
Methods: The study is a national, multicentric, retrospective study involving APS patients followed in 4 French tertiary university centers including 3 national referral centers for rare autoimmune diseases. Clinical and serological data of APS patients with VLD were analyzed and compared with APS patients included in the EuroPhospholipid cohort.
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. 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. As compared with EuroPhospholipid cohort (82% women; mean age 34±13 years; 53% of primary APS), APS patients with VLD have a higher frequency of both thrombotic recurrence (OR [95%CI]: 8.9 [4.8-16.5]; p<0.001) and major bleeding (OR [95%CI]: 5.5 [2.7-11.4]; p<0.001).
Conclusion: Although rare, VLD may be the presenting manifestation of APS. APS patients with VLD have a more severe phenotype and a higher risk of major bleeding.
REFERENCES: NIL.
Acknowledgements: NIL.
Disclosure of Interests: None declared.
© The Authors 2025. This abstract is an open access article published in Annals of Rheumatic Diseases under the CC BY-NC-ND license (