Clinical characteristics and management of antiphospholipid syndrome associated with cerebral venous thrombosis

Abstract: OBJECTIVE: To investigate the clinical characteristics, management and outcomes of cerebral venous thrombosis (CVT) patients with antiphospholipid syndrome (APS) in a Chinese cohort. METHODS: A retrospective cohort study was conducted on 121 consecutive CVT patients admitted to Peking Union Medical College Hospital from 2015 to 2023. Data on demographics, clinical manifestations, imaging findings, laboratory tests, treatment, and outcomes at discharge were analyzed. RESULTS: APS was identified in 16.5% (20/121) of CVT patients. The median age at onset was 34.5 years, with 60% (12/20) being female. 75% of patients had not experienced other thrombotic and obstetric events before CVT. In APS-CVT cases, chronic onset was observed in 50% (10/20), with a median diagnostic delay of 20 days. Headache was the most common symptom. Additional risk factors were present in 85% of APS-CVT patients. All patients received anticoagulation therapy, and 80% achieved favorable outcomes (modified Rankin Scale: < 2) at discharge. While most clinical and imaging features showed no significant differences, APS-CVT patients exhibited less frequent transverse/sigmoid sinus involvement (55% [APS-CVT] vs. 72.3% [non-APS-CVT], P = 0.045) and multisinus thrombosis (15% [APS-CVT] vs. 40.6% [non-APS-CVT], P = 0.020). Laboratory findings revealed lower hemoglobin levels (118 g/L [APS-CVT] vs. 130 g/L [non-APS-CVT], P < 0.05) and a higher prevalence of concomitant extracranial venous thrombosis (45% [APS-CVT] vs. 11.9% [non-APS-CVT], P < 0.001) in the APS-CVT group. CONCLUSION: APS represents a significant risk factor for CVT. CVT associated with APS primarily affects young individuals and women, often presents insidiously, and is frequently accompanied extracranial venous thrombosis. Given the challenges in diagnosing APS-CVT, screening for APS should be strongly considered in CVT patients with these clinical contexts. The characteristic pattern of venous sinus involvement in APS-CVT may provide insights into its unique pathogenesis. Most patients were treated with warfarin, which may be associated with a favorable outcome.