PRSS8 and idiopathic pulmonary fibrosis: The adjusted HR for subsequent respiratory death per 1 sd difference in change in prostasin over 6 months was 1.33 (95% CI 1.01-1.74, p=0.041, n=290).<h4>Conclusions</h4>Circulating prostasin is an independent marker of mortality risk in patients with IPF.