Generic placeholder image

Current Pharmaceutical Design

Editor-in-Chief

ISSN (Print): 1381-6128
ISSN (Online): 1873-4286

Primary Antiphospholipid Syndrome and Pulmonary Hypertension

Author(s): Aibek E. Mirrakhimov and Nicholas S. Hill

Volume 20, Issue 4, 2014

Page: [545 - 551] Pages: 7

DOI: 10.2174/138161282004140213125951

Price: $65

Abstract

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.


Rights & Permissions Print Cite
© 2024 Bentham Science Publishers | Privacy Policy