Disease Mechanisms and Therapeutic Advances in Idiopathic and Progressive Pulmonary Fibrosis: From Approved Drugs to Emerging Strategies.

In the IPF subgroup ( n = 92), inhaled treprostinil produced a placebo-corrected mean improvement in forced vital capacity (FVC) of 168.5 mL at week 16 (95% CI 40.1–297.0; p = 0.011), although the difference at week 8 did not reach statistical significance (84.5 mL; 95% CI −20.4 to 189.5; p = 0.11).

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