Budd Chiari syndrome associated with antiphospholipid syndrome: Clinical characteristics and prognosis of 17 patients from single center

Abstract

Background and Aim

In this retrospective, descriptive study, we aimed to identify clinical and laboratory characteristics, and prognoses of patients with Budd-Chiari syndrome (BCS) secondary to antiphospholipid syndrome (APS) and to compare with non-BCS vascular thrombotic APS patients.

Methods

Data of 194 patients with thrombotic APS (17 with BCS) ± systemic lupus erythematosus (SLE) in a single center between 1982 and 2023 were evaluated. Antiphospholipid serology consisting of lupus anticoagulant (LA), anticardiolipin (aCL) IgG/IgM, anti-beta2 glycoprotein I (aβ2GPI) IgG/IgM and adjusted global APS score (aGAPSS) were evaluated to determine thrombotic risk. Damage was identified for all patients by applying the damage index for APS (DIAPS). All patients with BCS were screened for hereditary or acquired prothrombotic disorders.

Results

Patients with BCS had higher aGAPSS and recurrent thrombosis (70.6% vs 40.7%) compared to those with non-BCS. BCS was the first thrombotic event in eight patients and three had recurrent thrombosis. The most common presenting manifestation of BCS was abdominal pain which was followed by abdominal distention and fever. The second prothrombotic factor was detected in six patients: three had heterozygous factor V Leiden mutation, three were in pregnancy period. Additionaly, two patients had SLE flare. The DIAPS of the patients in the BCS and non-BCS groups were similar, but those in the BCS group had higher mortality rates.

Conclusions

APS patients with BCS may have a higher risk of recurrent thrombosis and mortality. Acquired or hereditary prothrombotic disorders are not uncommon in this group and should be screened in APS patients with BCS.

Keywords

Antiphospholipid syndrome Budd-Chiari syndrome hepatic vein obstruction thrombotic risk adjusted global antiphospholipid syndrome score

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