Associations between patient-reported outcomes and death or lung transplant in idiopathic pulmonary fibrosis data from the idiopathic pulmonary fibrosis prospective outcomes registry

Rationale: Progression of idiopathic pulmonary fibrosis (IPF) is accompanied by worsening of symptoms, exercise capacity, and health-related quality of life. However, the utility of patient-reported outcomes as predictors of mortality remains uncertain. Objectives: To assess whether patient-reported outcomes are independently associated with mortality beyond clinical risk factors in patients with IPF. Methods: Data from the observational IPF Prospective Outcomes Registry were used to examine associations between patient-reported outcomes at enrollment and the composite outcome of death or lung transplant in the following year.

vital capacity % predicted, and diffusing capacity of the lungs for carbon monoxide % predicted at enrollment). Results: Among 662 patients, 45 died and 12 underwent lung transplant over 1 year. In the model adjusted for age and clinical variables that were associated with death or lung transplant, worse scores on the St. George’s Respiratory Questionnaire (SGRQ) total score (hazard ratio [HR], 1.22 [95% confidence interval (CI), 1.01–1.48] per 10-point increase), SGRQ activity score (HR, 1.25 [95% CI, 1.02–1.54] per 10-point increase) and SGRQ symptoms score (HR, 1.17 [95% CI, 1.01–1.36] per 10-point increase) were associated with death or lung transplant over 1 year. Conclusions: Patient-reported outcomes that assess symptoms and physical activity are independently associated with mortality in patients with IPF.