The Association between Idiopathic Pulmonary Fibrosis and Vascular Disease: A Population-based Study

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Previous studies have suggested that people with idiopathic pulmonary fibrosis (IPF) may be at increased risk of vascular disease.


To quantify the risk of cardiovascular disease before and after a diagnosis of IPF.


We used computerized primary care data from the Health Improvement Network to quantify the relative risk of having a cardiovascular event (acute coronary syndrome, angina, atrial fibrillation, deep-vein thrombosis, and cerebrovascular accident) either before or after having a diagnosis of IPF in comparison to age, sex, and community-matched general population control subjects.

Measurements and Main Results:

Our study included 920 incident case subjects of IPF (mean age at diagnosis, 71 yr; 62% male) and 3,593 matched control subjects. There was an increased risk of acute coronary syndrome (odds ratio [OR], 1.53; 95% confidence interval [CI], 1.15–2.03), angina (OR, 1.84; 95% CI, 1.48–2.29) and deep-vein thrombosis (OR, 1.98; 95% CI, 1.13–3.48) in the period before the diagnosis of IPF. During the follow-up period, there was a marked increased risk of acute coronary syndrome (rate ratio [RR], 3.14; 95%CI, 2.02–4.87) and deep-vein thrombosis (RR, 3.39; 95% CI, 1.57–7.28). None of these estimates were confounded by smoking habit or modified by age or sex.


People with IPF have an increased risk of vascular disease in comparison with the general population. This effect is most marked for acute coronary syndrome and deep-vein thrombosis after the diagnosis of IPF has been made.

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