The Coagulation Cascade in IPF
Research type
Research Study
Full title
Investigating the Role of the Coagulation Cascade in Idiopathic Pulmonary Fibrosis
IRAS ID
191454
Contact name
Joanna Porter
Contact email
Sponsor organisation
University College London
Duration of Study in the UK
1 years, 0 months, 0 days
Research summary
Idiopathic Pulmonary fibrosis (IPF) is a progressive lung scarring disease that affects 10,000 new patients each year in the UK. Treatment options have broadened in recent years with the development of drugs that aim to slow the rate of lung function decline, but without significantly impacting on symptoms or life expectancy.
One of the challenges to developing better treatments is incomplete understanding of what causes the disease. We aim to investigate the coagulation cascade as a candidate pathway in the initiation and progression of IPF.
We do know that IPF involves tissue injury followed by inappropriate wound healing. One of the first biological pathways to be activated following injury is the coagulation cascade, leading to the formation of plugs that prevent blood loss and further damage. Thrombin is a product of this activation and higher than normal levels have been demonstrated in IPF patients. These patients also display a pro thrombotic state i.e. a tendency towards inappropriate clot formation. As well as being involved in clotting thrombin leads to immune activation and the formation of proteins (cytokines) that are result in lung inflammation and scarring.
Previous studies using PET scans, which detect metabolically active cells, have demonstrated increased cellular activity in IPF lungs; with the level of activity having prognostic value. At present the source of this activity is not known, however, one might hypothesise that it is the result of excess coagulation stimulation with clumping of red blood cells and micro clots within the lungs. We aim to demonstrate this by manipulating the coagulation system (i.e. causing an anti-coagulant effect using a medication) and detecting a reduction in the level of activity seen on sequential PET scans.
REC name
London - Westminster Research Ethics Committee
REC reference
16/LO/1143
Date of REC Opinion
15 Aug 2016
REC opinion
Further Information Favourable Opinion