Evaluation of factors affecting mortality in patients with idiopathic pulmonary fibrosis

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Introduction and Objective: Idiopathic pulmonary fibrosis (IPF) has high mortality, with average survival of 2–3 years without treatment. This study aimed to determine the frequency and factors affecting mortality in IPF patients. Methods: The demographics, pulmonary function test, laboratory results, clinical outcomes and mortality data of the IPF patients were recorded retrospectively. Results: The study included 268 patients (22.8% women, 77.2% men) with median age of 72 years. Antifibrotic treatment was initiated in 70.2% of patients. 14.8% had an exacerbation, and 40.3% had a history of hospitalization. The overall mortality rate was 44% (n=118), with median survival of 29.5 months. The mortality group had higher age (p<0.001), and higher rates of coronary artery disease (p=0.023), pulmonary embolism (p=0.005), pulmonary hypertension (p<0.001), and malignancy (p=0.01). No significant difference was observed in treatment use and type of antifibrotic therapy. Exacerbations (p=0.001) and hospitalization frequency (p<0.001) were significantly higher in mortality group. GAP score (OR:11.68, 95%CI:2.63–51.88, p=0.001), pulmonary hypertension (OR:15.39, 95%CI:1.54–153.67, p=0.02), exacerbation (OR:56.2, 95%CI:1.22–2585.8, p=0.039), baseline FVC value (OR:1.103, 95%CI:1.014–1.2, p=0.022), mean platelet volume (MPV) (OR:0.29, 95%CI:0.11–0.74, p=0.01), and AST (OR:1.124, 95%CI:1.005–1.26, p=0.04) were the independent risk factors associated with mortality. Conclusion:Despite advancements in diagnosis and treatment, IPF remains a disease with high mortality. Preventing exacerbations, minimizing pulmonary function decline, and managing comorbidities are expected to reduce mortality.

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