Presence of a prothrombotic state in people with idiopathic pulmonary fibrosis: a population-based case-control study

Navaratnam, Vidya; Fogarty, Andrew W.; McKeever, Tricia M.; Thompson, Norma; Jenkins, Gisli; Johnson, Simon R.; Dolan, Gerard; Kumaran, Maruti; Pointon, Kate; Hubbard, Richard B.

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.

Citation

Navaratnam, V., Fogarty, A. W., McKeever, T. M., Thompson, N., Jenkins, G., Johnson, S. R., Dolan, G., Kumaran, M., Pointon, K., & Hubbard, R. B. (2014). Presence of a prothrombotic state in people with idiopathic pulmonary fibrosis: a population-based case-control study. Thorax, 69(3), 207-215. https://doi.org/10.1136/thoraxjnl-2013-203740

• Journal Article Type: Article
• Acceptance Date: Jul 29, 2013
• Online Publication Date: Sep 3, 2013
• Publication Date: 2014-03
• Deposit Date: May 30, 2017
• Publicly Available Date: May 30, 2017
• Journal: Thorax
• Print ISSN: 0040-6376
• Electronic ISSN: 1468-3296
• Publisher: BMJ Publishing Group
• Peer Reviewed: Peer Reviewed
• Volume: 69
• Issue: 3
• Pages: 207-215
• DOI: https://doi.org/10.1136/thoraxjnl-2013-203740
• Public URL: https://nottingham-repository.worktribe.com/output/996637
• Publisher URL: http://thorax.bmj.com/content/69/3/207
• Contract Date: May 30, 2017