Oxidative stress as a thrombophilic factor in Behçet syndrome

Abstract

Behçet syndrome (BS) is a multisystemic disease that includes a heterogeneous variety of different organ manifestations. The disease is sustained by an inflammatory state, which mediates different types of involvement, among which vascular is one. The vascular manifestations of BS usually involve the venous and, more rarely, the arterial branches, with inflammation-induced thrombosis being a common event. Notably, vascular involvement related to this syndrome is treated with immunosuppressant agents, rather than with anticoagulant treatments. To date, the mechanisms sustaining the pathophysiology of thrombo-inflammation in BS are poorly described. Recent findings suggest a possible role of modification of fibrinogen, a protein involved both in inflammatory processes and in the fibrin network formation and in platelet aggregation. Fibrinogen undergoes different posttranslational modifications and is responsible for its structural and functional changes, which are mainly mediated by reactive oxygen species (ROS). In BS, fibrinogen oxidation and thrombus formation seem to be mediated by neutrophils, which are pivotal in the pathogenetic mechanisms leading to BS-related damages. Understanding the molecular mechanisms that sustain inflammation-induced thrombosis in BS may help to identify potential targets for new therapeutic strategies.