Coronary artery calcium score is a prognostic factor for mortality in idiopathic pulmonary fibrosis

BACKGROUND: Cardiovascular diseases are frequent in idiopathic pulmonary fibrosis (IPF) and impact on survival. We investigated the association of coronary artery calcium (CAC) score at IPF diagnosis and during mid-term follow-up, with adverse cardiovascular events and all-cause mortality. METHODS: Consecutive patients with IPF were retrospectively analyzed. Demographic data, smoking history, comorbidities and pulmonary function tests (PFTs) were recorded. All patients had at least two chest high resolution computed tomography (HRCT) performed 2 years apart. The total CAC score and visual fibrotic score were calculated, and all clinically significant cardiovascular events and deaths were reported. RESULTS: The population consisted of 79 patients (57 males, mean age: 74.4 +/- 7.6 years); 67% of patients had a history of smoking, 48% of hypertension, 37% of dyslipidemia and 22.8% of diabetes. The visual score was 21.28 +/- 7.99% at T0 and 26.54 +/- 9.34% at T1, respectively (T1-T0 5.26 +/- 6.13%, P<0.001). CAC score at T0 and at T1 was 537.93 +/- 839.94 and 759.98 +/- 1027.6, respectively (T1-T0 224.66 +/- 406.87, P<0.001). Mean follow-up time was 2.47 +/- 1.1 years. On multivariate analysis, male sex (HR=3.58, 95% CI: 1.14-11.2) and CAC score at T0 (HR=1.04, 95% CI: 1.01-1.07) correlated with mortality and cardiovascular events. CAC score at T0 >= 405 showed 82% sensitivity and 100% specificity for predicting mortality and adverse cardiovascular events. CONCLUSIONS: IPF patients with a CAC score at diagnosis >= 405 have a poor prognosis over a mid-term follow-up. A higher CAC score is associated with mortality and cardiovascular events.