Nintedanib and Sildenafil in Patients with Idiopathic Pulmonary Fibrosis and Right Heart Dysfunction. A Prespecified Subgroup Analysis of a Double-Blind Randomized Clinical Trial (INSTAGE)

• DOI: 10.1164/rccm.201903-0488oc
• Publication Date: 2019-07-31T21:55:06Z
• Authors: Jürgen Behr; Martin Kolb; Jin Woo Song; Fabrizio Luppi; Birgit Schinzel; Susanne Stowasser; Manuel Quaresma; Fernando J. Martinez
• Keywords: Nintedanib; Subgroup analysis

Rationale: In the INSTAGE trial in patients with idiopathic pulmonary fibrosis (IPF) and severely impaired gas exchange, nintedanib plus sildenafil was associated numerical benefits on St. George's Respiratory Questionnaire (SGRQ) total score, brain natriuretic peptide (BNP), FVC decline versus alone. Exploratory analyses of STEP-IPF (Sildenafil Trial Exercise Performance IPF) suggested that may have a greater effect SGRQ score IPF who right heart dysfunction (RHD).Objectives: Assess whether RHD influenced effects alone trial.Methods: Subgroup (n = 117) those without 156) echocardiographic signs at baseline.Measurements Main Results: There no heterogeneity between subgroups by presence change Week 12 (P 0.74) or 24 0.90), 0.58) 0.55). both subgroups, had numerically reducing Between-group differences BNP were -119.9 ng/L (95% confidence interval -171.3 to -68.5) -3.6 -47.2 40.0) baseline, respectively < 0.01).Conclusions: trial, there significant changes baseline. The benefit combination therapy stabilizing more pronounced baseline.Clinical registered www.clinicaltrials.gov (NCT02802345).