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This group demonstrated similar mortality and disease progression as compared to patients with a definite diagnosis of IPF as per diagnostic guidelines (log-rank test P = 0.771 and P = 0.139, respectively). Such findings were confirmed on multivariate analysis (HR 1.19, 95% CI 0.49-2.89, P = 0.7 for death; HR 1.42, 95% CI 0.83-2.44, P = 0.201 for disease progression). CONCLUSION In patients receiving antifibrotics following a working diagnosis of IPF, disease progression rates were similar to patients with a confident diagnosis of