Mortality Trends of Idiopathic Pulmonary Fibrosis in the United States From 2004 Through 2017

Background: The burden of idiopathic pulmonary fibrosis (IPF)-related mortality in the United States in recent years is not well characterized. Research Question: What are the trends in IPF-related mortality rates in the United States from 2004 through 2017? Study Design and Methods: We used the Multiple Cause of Death Database available through the Centers for Disease Control and Prevention website, which contains data from all deceased US residents. IPF-related deaths were identified using International Classification of Diseases, 10th revision, codes. We examined annual trends in age-adjusted mortality rates stratified by age, sex, race, and state of residence. We also evaluated trends in place of death and underlying cause of death. Results: From 2004 through 2017, the age-adjusted mortality decreased by 4.1% in men (from 75.5 deaths/1,000,000 in 2004 to 72.4 deaths/1,000,000 in 2017) and by 13.4% in women (from 46.3 deaths/1,000,000 in 2004 to 40.1 deaths/1,000,000 in 2017). This overall decrease was driven mainly by a decline in IPF-related mortality in patients younger than 85 years. The decreasing trend also was noted in all races except White men, in whom the rate remained stable. The most common cause of death was pulmonary fibrosis. The percentage of deaths occurring in the inpatient setting and nursing homes decreased, whereas the percentage of deaths occurring at home and hospice increased. Interpretation: From 2004 through 2017, the IPF age-adjusted mortality rates decreased. This may be explained partly by a decline in smoking in the United States, but further research is needed to evaluate other environmental and genetic contributors.