Sildenafil added to pirfenidone in patients with advanced idiopathic pulmonary fibrosis and risk of pulmonary hypertension: A Phase IIb, randomised, double-blind, placebo-controlled study - Rationale and study design
Behr J, Nathan SD, Harari S et al.
KEYWORDS
6-Minute walk test; Clinical trial; Echocardiogram; Hypertension; Idiopathic pulmonary fibrosis; Phosphodiesterase-5 inhibitor; Pirfenidone; Sildenafil
BACKGROUND - Pulmonary hypertension (PH) is commonly observed in patients with advanced idiopathic pulmonary fibrosis (IPF). Despite the availability of therapies for both IPF and PH, none are approved for PH treatment in the context of significant pulmonary disease. This study will investigate the use of sildenafil added to pirfenidone in patients with advanced IPF and risk of PH, who represent a group with a high unmet medical need.