Cardiovascular Disease in Antiphospholipid Syndrome

Abstract

: Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by venous, arterial or microvascular thrombosis or obstetrical events in the presence of persistently positive antiphospholipid antibodies and constitutes a major cause of cardiovascular events in young people. Τhis review aims to highlight the pathophysiology of cardiovascular complications in patients with APS and possible treatment options. Patients with APS have endothelial dysfunction, accelerated proliferation and hyperplasia, atherogenesis, platelet activation and aggregation, inflammatory products secretion and coagulation-fibrinolytic dysregulation. Cardiovascular complications constitute accelerated atherosclerosis, acute coronary syndrome, Libman- Sachs endocarditis, cardiomyopathy or intracardiac thombi. Moreover, pulmonary hypertension and peripheral microvascular dysfunction are common findings for these patients. Management of these patients is not well documented. The role of primary thrombosis prevention remains controversial in individuals with positive antiphospholipid antibodies. Treatment of traditional cardiovascular risk factors according to current guidelines for prevention of cardiovascular disease in the general population is recommended for primary prevention of APS. Anticoagulation therapy with unfractionated or low-molecular-weight heparin overlapped with a vitamin K antagonist remains the mainstay of treatment for APS patients with venous thrombosis, whereas direct oral anticoagulants are not yet recommended. Data are scarce regarding secondary arterial thrombosis prevention and it is not clear whether dual or triple antithrombotic therapy is necessary. To date, it is recommended to follow current guidelines for the management of acute coronary syndrome in the general population. New treatment targets are promising options for patients with catastrophic APS.

Case report: Mitral valve replacement for Libman-Sacks endocarditis and cerebral embolism of primary antiphospholipid syndrome

“…Antiphospholipid syndrome is a non-inflammatory systemic autoimmune disease with an incidence of five new cases/100,000 persons per year and a prevalence of 40–50 cases/100,000 persons ( 3 ) and is more common in young women ( 4 ). “Definite” APS must have recurrent arteriovenous thrombosis and/or morbid pregnancy (habitual abortion, or stillbirth in the middle and late stages) as its main clinical manifestations ( 5 ).…”

Acute Lower Limb Ischemia in Antiphospholipid Syndrome: A Retrospective Cohort Study

“…Therapy was performed in accordance with each patient's symptoms, classification of limb ischemia, and imaging detection. This retrospective analysis of prospectively collected data considered ALI patients with definite APS and positive diagnoses in aPL antibody tests (LA, aCL and anti-β2GPIA) [14]. Patients were included in the cohort if they tested positive for both LA and anti-β2GPIA, and aCL antibodies exceeded 40 IgG phospholipid and/or 40 IgM phospholipid units [15].…”

NEDD9, a Hypoxia-upregulated Mediator for Pathogenic Platelet–Endothelial Cell Interaction in Pulmonary Hypertension

“…Emerging studies have reported the contribution of platelet activation in the formation of pulmonary vascular thrombosis and the development of pulmonary vascular remodeling. Upon activation, the platelets aggregate to the damaged pulmonary vasculature and release vasoactive mediators, angiogenic agents, growth factors, chemokines and cytokines, many of which are implicated in the pathological pulmonary vascular remodeling and microvascular thrombosis in PH and PAH (6). However, the detailed molecular mechanisms underlying the platelet and pulmonary arterial endothelial cell (PAEC) adhesion under conditions of hypoxia and PH remain largely unknown.…”