Changes in lung function and mortality risk in patients with idiopathic pulmonary fibrosis (IPF)

<bold>Introduction:</bold> IPF is a progressive fibrosing interstitial lung disease associated with lung function decline and death. <bold>Aim:</bold> Examine the relationship between changes in lung function and mortality risk in a real-world cohort of patients with IPF. <bold>Methods:</bold> Patients were enrolled into the IPF-PRO Registry at 46 sites in the US. A joint model was used to estimate FVC and DLco % predicted for each day of follow-up. Cox regression with a time-dependent binary indicator was used to assess associations between the first occurrence of a relative decline in FVC or DLco % predicted exceeding 2%, 5%, or 10%, or a relative decline in DLco % predicted exceeding 15%, and subsequent death or lung transplant. The model was adjusted for age, sex, smoking status, body mass index, oxygen use and lung function at enrollment. Median follow-up time was 38.4 months. <bold>Results:</bold> Median (Q1, Q3) relative changes in FVC and DLco % predicted over 12 months were −3.3% (−6.3, 0.3) and −11.2% (−15.8, −7.2), respectively (n=838). For each threshold of decline examined, there was a significant increase in the risk of death or lung transplant (Table). <bold>Conclusions:</bold> Real-world data suggest that in patients with IPF, even small relative declines in lung function independently inform prognostication. <fig><object-id>erj;64/suppl_68/PA674/F1</object-id><object-id>F1</object-id><object-id>F1</object-id><graphic></graphic></fig>