Targeting coagulation factor receptors – protease‐activated receptors in idiopathic pulmonary fibrosis. (16th February 2017)

• Record Type: Journal Article
• Authors: Lin, C.; Borensztajn, K.; Spek, C. A.
• Is Part Of: Journal of thrombosis and haemostasis. Volume 15:Number 4(2017)
• Page Start: 597
• Page End: 607
• Publication Date: 2017-02-16
• Subjects: anticoagulants -- fibrosis -- idiopathic pulmonary fibrosis -- receptor, PAR‐1 -- receptor, PAR‐2 -- receptors, proteinase‐activated; Thrombosis -- Periodicals; Hemostasis -- Periodicals; Blood coagulation disorders -- Periodicals; 616.1
• DOI: 10.1111/jth.13623
• Languages: English
• ISSNs: 1538-7933

Abstract

Abstract : Summary: Idiopathic pulmonary fibrosis (IPF) is a lethal lung disease with a 5‐year mortality rate of > 50% and unknown etiology. Treatment options remain limited and, currently, only two drugs are available, i.e. nintedanib and pirfenidone. However, both of these antifibrotic agents only slow down the progression of the disease, and do not remarkably prolong the survival of IPF patients. Hence, the discovery of new therapeutic targets for IPF is crucial. Studies exploring the mechanisms that are involved in IPF have identified several possible targets for therapeutic interventions. Among these, blood coagulation factor receptors, i.e. protease‐activated receptors (PARs), are key candidates, as these receptors mediate the cellular effects of coagulation factors and play central roles in influencing inflammatory and fibrotic responses. In this review, we will focus on the controversial role of the coagulation cascade in the pathogenesis of IPF. In the light of novel data, we will attempt to reconciliate the apparently conflicting data and discuss the possibility of pharmacologic targeting of PARs for the treatment of fibroproliferative diseases.