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Published on: June 16, 2020
Changes in Lung Function and Mortality Risk in Patients With Idiopathic Pulmonary Fibrosis
Justin M Oldham1, Megan L Neely2, Daniel M Wojdyla3
1Division of Pulmonary and Critical Care Medicine, University of Michigan, Ann Arbor, MI.
Background:
Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial lung disease associated with lung function decline and high mortality.
Research Question:
What are the associations between thresholds of lung function decline and the risk of mortality in patients with IPF?
Study Design And Methods:
The Idiopathic Pulmonary Fibrosis Prospective Outcomes Registry enrolled patients with IPF that was diagnosed or confirmed at the enrolling center within the prior 6 months. Associations between time to first decline in FVC or diffusing capacity of the lungs for carbon monoxide (Dlco) of ≥ 2% predicted, ≥ 5% predicted, and ≥ 10% predicted (and ≥ 15% predicted for Dlco) and risk of subsequent death or lung transplantation was assessed using Cox proportional hazards models with a time-dependent covariate. Models were unadjusted or adjusted for FVC and Dlco % predicted, age, sex, smoking status, BMI, antifibrotic treatment (yes or no), and oxygen use at enrollment.
Results:
Among 1,001 patients, median follow-up time was 38.4 months. Significant associations were observed between all thresholds of decline in FVC and Dlco % predicted and the risk of death or lung transplantation in unadjusted and adjusted analyses. In adjusted analyses, absolute declines in FVC of ≥ 2% predicted, ≥ 5% predicted, and ≥ 10% predicted were associated with 1.8-fold, 2.3-fold, and 2.7-fold increases in the risk of subsequent death or lung transplantation, whereas absolute declines in Dlco of ≥ 2% predicted, ≥ 5% predicted, ≥ 10% predicted, and ≥ 15% predicted were associated with 2.0-fold, 1.4-fold, 1.5-fold, and 1.9-fold increases in the risk of subsequent death or lung transplantation, respectively. For Dlco, but not FVC, the increase in risk generally was greater for patients meeting a threshold based on a relative rather than an absolute decline.
Interpretation:
Our results show that even small declines in FVC and Dlco % predicted inform prognosis in patients with IPF.
Clinical Trial Registry:
ClinicalTrials.gov; No.: NCT01915511; URL: www.
Clinicaltrials:
gov.
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