The cardiovascular system in idiopathic pulmonary fibrosis

The incidence and prevalence of ILDs, especially IPF, is on the rise. Despite improved clinical management, IPF still remains a challenging disease, especially with regard to its cardiovascular comorbidity. This chapter provides readers with recent updates regarding cardiovascular involvement in IPF. There is a significant association between CAD and IPF, and this may be due to a combination of common shared risk factors, hypoxia and oxidative stress. The mechanism behind the development of PH in IPF is incompletely understood; comparison with other ILDs may offer clarification. Importantly, arrhythmias are present in a significant portion of the daily activities of IPF patients, as well as post-lung transplant IPF patients. Antifibrotics such as pirfenidone and nintedanib are generally safe from the cardiac point of view. The impact of cardiovascular comorbidities on the survival of IPF patients is discussed before we explore the challenges faced in managing this vulnerable patient population.