Abstract

Idiopathic pulmonary fibrosis (IPF) is a disease with limited therapeutic options. Corticosteroids and immunouppressive agents have been the mainstays of treatment, primarily due to their anti-inflammatory properties. However, they have demonstrated minimal efficacy and significant toxicity. Recent efforts have sought to target other aspects of the pathophysiology of this condition. Several investigators have hypothesized that imbalances of coagulation and fibrinolysis play a role in the pathophysiology of IPF. Thrombin has been identified as a possible trigger of fibroblast proliferation in IPF, suggesting a role for thrombin inhibition in modifying the course of disease. Furthermore, anticoagulation may have a role in preventing thrombotic complications in IPF patients with secondary pulmonary hypertension. This review will discuss the potential role for anticoagulation in the treatment of IPF. One randomised control trial has demonstrated a significant survival benefit associated with anticoagulation of IPF patients. We will review the evidence supporting this treatment strategy, as well as its possible mechanisms of action.

Keywords: Idiopathic pulmonary fibrosis, anticoagulation, thrombin