Conclusions

In a cross-sectional analysis of 300 patients with IPF, select circulating proteins strongly associated with respiratory death or lung transplant, even after considering clinical factors known to influence outcomes.
We report a protein signature for predicting respiratory death or lung transplant in patients with IPF that can be evaluated in a validation cohort.
Important considerations for validation studies will include the method of protein measurement (aptamer vs ELISA) and exposure to antifibrotic drugs.