Clinical Management of Thrombotic Antiphospholipid Syndrome

Abstract

Thrombotic manifestations of antiphospholipid syndrome are often a therapeutic dilemma and challenge. Despite our increasing knowledge of this relatively new disease, many issues remain widely unknown and controversial. In this review, we summarise the latest literature and guidelines on the management of thrombotic antiphospholipid syndrome. These include the laboratory assays involved in antiphospholipid antibodies (aPL) testing, the use of direct oral anticoagulants in secondary prevention, management of recurrent thrombosis, individuals with isolated aPL, and catastrophic antiphospholipid syndrome. Treatment aims to prevent the potentially fatal and often disabling complications of APS with antithrombotic and cardiovascular risks prevention strategies. Some insights and updates on topical issues in APS are provided. We also include our current practice, which we believe is the pragmatic approach based on the currently available evidence.

Malignant Isolated Cortical Vein Thrombosis as the Initial Manifestation of Primary Antiphospholipid Syndrome: Lessons on Diagnosis and Management From a Case Report

“…APS is an immune-mediated thrombophilic disorder propelled by a heterogeneous group of persistent circulating aPLs and associated proteins ( 9 , 21 ). APS is characterized by recurrent vascular thrombosis, pregnancy morbidity, and non-criteria manifestations, such as livedo reticularis, thrombocytopenia, and valve disease ( 6 , 9 ). Well‐established antiphospholipid antibodies include aCL, aβ2GPI, and LA.…”

“…The diagnosis of APS still relies on the 2006 Sydney criteria ( 6 ). As the clinical events of thrombosis can occur in the vascular beds of any size and organ, the clinical presentation of APS varies enormously ( 9 ). APS contributes to 6%–17% of CVST patients across cohort studies ( 9 ).…”

“…As the clinical events of thrombosis can occur in the vascular beds of any size and organ, the clinical presentation of APS varies enormously ( 9 ). APS contributes to 6%–17% of CVST patients across cohort studies ( 9 ). However, CVST is a relatively rare presentation of APS, with a reported prevalence of only 0.7% ( 9 , 22 ).…”

“…APS contributes to 6%–17% of CVST patients across cohort studies ( 9 ). However, CVST is a relatively rare presentation of APS, with a reported prevalence of only 0.7% ( 9 , 22 ). A recent systematic review on APS-related CVST patients showed that CVST predominantly affects young women and involves the superior sagittal sinus and lateral venous system (60% and 69%, respectively) ( 23 ).…”

“…Common clinical features of APS include venous thromboembolism, stroke, and recurrent abortion ( 6 ). APS contributes to 6%–17% of CVST cases, yet it is rarely considered an etiology of ICoVT ( 3 , 8 , 9 ). To date, only three cases of ICoVT have been reported to be associated with APS ( 10 – 12 ).…”