Primary antiphospholipid syndrome (APS) is a disease characterized by the presence of autoantibodies reacting with proteins
bound to phospholipids, leading to thrombosis and gestation abnormalities. Prothrombotic states and impaired clot dissolution are believed
to contribute to the occurrence of chronic thromboembolic pulmonary hypertension (CTEPH) in APS. Whether preventive anticoagulation
therapy in patients with antiphospholipid autoantibodies without a history of thrombosis reduces the risk of thrombosis is currently
unclear. The diagnosis and treatment of CTEPH in APS is similar to CTEPH complicated by other predisposing conditions, with
surgical treatment being the most effective. However, not every patient with CTEPH is suitable for pulmonary thromboendartarectomy
and such individuals may benefit from pharmacotherapy of pulmonary hypertension, given the presence of pulmonary microvascular abnormalities
similar to those in idiopathic pulmonary hypertension. Anticoagulation therapy is the mainstay of management because of the
high risk of recurrent embolization and local in-situ thrombosis.
Keywords: Antiphospholipid syndrome, pulmonary hypertension, thrombosis, pulmonary embolism.
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