Patients with idiopathic pulmonary fibrosis (IPF) often exhibit cardiovascular manifestations that affect patients’ morbidity and mortality according to the study”Cardiac manifestations of idiopathic pulmonary fibrosis,” published in Intractable and Rare Diseases Research.
The authors reviewed evidence that links cardiovascular diseases to IPF and how the co-morbidities impact the patient clinical outcomes.
IPF is a chronic, progressive disease characterized by shortness of breath that can lead to acute respiratory failure. The disease has long been associated with a variety of co-morbidities, including emphysema, lung cancer, obstructive sleep apnea (OSA), and many cardiovascular diseases.
One of the diseases, pulmonary hypertension (PH), is shown to significantly contribute to the morbidity and mortality of IPF. Most of the symptoms of PH overlap with IPF, which makes it difficult to diagnose PH-IPF unless high clinical suspicion exists. Analysis of a large cohort group from the ARTEMIS-IPF trial showed about 10% of patients with PH.
Patients with IPF often experience atrial arrhythmias. Those who underwent lung transplant showed the highest incidence of atrial fibrillation, a type of atrial arrhythmia, in 55.9% of cases. The odds of experiencing atrial flutter, another type of atrial arrhytmia, was shown to be 2.6 times higher in patients with IPF. The patients were likely prone to arrhythmias due to the hypoxic environment, elevation of pulmonary pressures, and presence of chronic inflammation.
Coronary artery disease (CAD) has been associated with IPF through many studies. Patients with IPF have an increased incidence of CAD compared to those with non-fibrotic lung diseases. It is hypothesized that the pulmonary fibrosis in IPF promotes atherosclerosis, leading to CAD. In a large population based study, 920 IPF patients were compared with 3,593 matched controls, revealing increased risk of acute coronary syndrome and angina.
Although no medication can currently cure IPF, new drugs pirfenidone and nintedanib, have shown to slow disease progression in clinical trials.
One trial in animals studying the effects of pirfenidone demonstrated that the drug not only did not cause cardiac side effects, it may have helped reduce myocardial fibrosis and maintain systolic function. Clinical trials for nintedanib reported cardiac adverse events that led to a small percentage of deaths, which was comparable to placebo (0.3% to 0.2%).
Some studies have reported that the presence of associated cardiovascular co-morbidities can significantly reduce the survival and outcomes in the IPF patients who undergo lung transplantation.
Research has also demonstrated that IPF patients are less likely to be prescribed statins and beta-blockers, suggesting that the diseases are not being properly managed.
The study concluded: “It is also possible that the presence of a severe lung disease might lead the attention away from routine cardiovascular care with decreased primary and secondary prevention. It is prudent to monitor these patients for cardiac manifestations and cardiac events to reduce the overall morbidity and mortality.”