Presence of a prothrombotic state in people with idiopathic pulmonary fibrosis: a population-based case–control study. Issue 3 (3rd September 2013)

• Record Type: Journal Article
• Authors: Navaratnam, Vidya; Fogarty, Andrew W; McKeever, Tricia; Thompson, Norma; Jenkins, Gisli; Johnson, Simon R; Dolan, Gerard; Kumaran, Maruti; Pointon, Kate; Hubbard, Richard B
• Is Part Of: Thorax. Volume 69:Issue 3(2014)
• Page Start: 207
• Page End: 215
• Publication Date: 2013-09-03
• Subjects: Idiopathic pulmonary fibrosis -- Interstitial Fibrosis; Chest -- Diseases -- Periodicals; Thorax; Chest -- Diseases; Periodicals; Periodicals; 617.54
• DOI: 10.1136/thoraxjnl-2013-203740
• Languages: English
• ISSNs: 0040-6376

Abstract

Abstract : Background: Laboratory studies suggest that the clotting cascade is activated in fibrotic lungs. Since humans vary in their tendency to clot due to a variety of inherited or acquired defects, we investigated whether a prothrombotic state increases the chance of developing idiopathic pulmonary fibrosis (IPF) and/or worsens the prognosis of IPF. Methods: We recruited 211 incident cases of IPF and 256 age- and sex-matched general population controls and collected data on medical history, medication, smoking habit, blood samples as well as lung function and high-resolution CT scans done as part of routine clinical care. A prothrombotic state was defined as the presence of at least one inherited or acquired clotting defect or marker of fibrinolytic dysfunction. We used logistic regression to quantify the association between a prothrombotic state and IPF adjusted for age, sex, smoking habit and highly sensitive C reactive protein. Cox regression was used to determine the influence of a prothrombotic state on survival. Results: Cases were more than four times more likely than controls to have a prothrombotic state (OR 4.78, 95% CI 2.93 to 7.80; p<0.0001). Cases with a prothrombotic state were also likely to have more severe disease (forced vital capacity <70% predicted) at presentation (OR 10.79, 95% CI 2.43 to 47.91) and had a threefold increased risk of death (HR 3.26, 95% CI 1.09 to 9.75). Conclusions: People with IPF are more likely to have a prothrombotic state than general population controls and the presence of a prothrombotic state has an adverse impact on survival.