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

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.