Factors associated with mortality in cases of idiopathic pulmonary fibrosis with mild to moderate functional impairment

Abstract

: BACKGROUND AND AIM: Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive lung disease with a poor prognosis, characterized by advanced fibrosis. The course of the disease varies from patient to patient. The factors that determine the course of the disease are yet to be clarified. Here, we aimed to assess patient characteristics, overall mortality, and mortality-associ-ated factors in our IPF patient cohort. METHODS: Our multicenter, retrospective cohort study reviewed the records of 169 patients diagnosed with IPF who had mild-to-moderate functional impairment and were followed up for at least one year from diagnosis until death between 2009 and 2019. RESULTS: The mean age of the 169 IPF patients was 69.7±8.8 years, and 73.4% were male. The diagnosis was established clinically and radiologically in 152 (89.9%) patients and histopathologically in 17 (10%) patients. A smoking history was found in 72.2% of the patients, with an average smoking quantity of 35.6±14.7 pack-years. Among the patients, 28 (16.6%) did not receive treatment, 87 (51.5%) received pirfenidone, and 54 (31.9%) nintedanib treatment. The median Gender, Age, and Physiology (GAP) score of the patients was 3. The mean forced vital capacity (FVC) was 79.6%±19.7%, the mean diffusing capacity of the lungs for carbon monoxide (DLCO) was 52.8%