Abstract
Objectives: Portal vein thrombosis (PVT) is a rare and severe clinical phenotype of antiphospholipid syndrome (APS) with a poor prognosis. Anticoagulation therapy is efficient but is associated with potentially severe bleeding episodes, especially for those patients with thrombocytopenia. We conducted this case-control study to explore the clinical features and associated factors of PVT in APS patients, the re-canalization rate of the PVT after anticoagulation and investigate the beneficial effects of early initiation of anticoagulation in patients with APS associated PVT.Methods: We enrolled patients with APS associated PVT as the case group, and age-, and entry-time-matched APS patients without PVT (1:2) as the control group. We explored the associated factors of PVT in APS patients using multivariate logistic regression analysis. The re-canalization rate of the PVT after anticoagulation was analyzed using the survival analysis.Results: A total of 34 patients (8 males and 26 females) with APS-PVT were enrolled, with a median follow-up time of 3 years (1.5, 7 years). Multivariate logistic regression analysis showed that thrombocytopenia (OR 6.4, 95%CI 1.561–26.218, P = 0.01), hypersensitive c-reactive protein >3 mg/L (OR 4.57, 95%CI 1.426–14.666, P = 0.011), anti β2GPI positive (OR 5, 95%CI 1.816–13.772, P = 0.002) and aPL double-positive (OR 4.08, 95%CI 1.312–12.429, P = 0.013) were independent associated factors for PVT in APS. Survival analysis revealed that effective anticoagulation could increase re-canalization rate significantly (log-rank p = 0.001), with better prognosis (lower mortality rate, log-rank p = 0.045).Conclusions: PVT could be the first presentation of APS with insidious onset and atypical clinical symptoms and easily be misdiagnosed. For patients with APS, double aPLs positive, thrombocytopenia, and inflammation could be the associated factors of PVT. Early diagnosis and anticoagulation treatment can bring thrombus re-canalization thereby significantly improving the prognosis.
Highlights
Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by recurrent arterial venous thrombosis, habitual abortion, and/or thrombocytopenia and persistent antiphospholipid antibodies positive in the blood
Multivariate logistic regression analysis showed that thrombocytopenia, hypersensitive c-reactive protein >3 mg/L, anti β2GPI positive and aPL double-positive were independent associated factors for Portal venous system thrombosis (PVT) in APS
PVT could be the first presentation of APS with insidious onset and atypical clinical symptoms and be misdiagnosed
Summary
Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by recurrent arterial venous thrombosis, habitual abortion, and/or thrombocytopenia and persistent antiphospholipid antibodies (aPLs) positive in the blood. PVT is a rare, serious, and highly heterogeneous phenotype of APS [1]. Portal venous system thrombosis (PVT) includes the thrombus in the portal vein, the superior mesenteric vein/splenic vein, and the inferior mesenteric vein. According to the course of thrombosis, portal vein thrombosis is divided into acute PVT, chronic PVT, and portal vein degeneration. PVT has been related to liver dysfunction, neoplasm, genetic factors [2, 3], hemodynamic factors, and hypercoagulability states [4]. Antiphospholipid antibodies have been implicated as one of the causes of PVT in a few previous studies [5, 6]
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