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

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

Methods: 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.

Conclusions: 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.