Background
respiratory disease
RESPIRATORY DISEASE, NON-CYSTIC FIBROSIS BRONCHIECTASIS
Non-cystic fibrosis bronchiectasis (NCFBE) is a chronic, progressive respiratory disease defined by permanent dilatation of the bronchi [Dipeptidyl peptidase-1 (DPP-1, EC 3.4.14.1), also known as cathepsin C, is an enzyme responsible for activating NE, PR3, and CatG zymogens in promyelocytes during neutrophil differentiation in the bone marrow [Brensocatib is an investigational, oral, selective, reversible inhibitor of DPP-1 [In a phase 2b, randomized, double-blind, placebo-controlled trial in 256 patients with NCFBE (WILLOW), 24 weeks of treatment with 10 or 25 mg brensocatib was associated with improvements in clinical outcomes including prolongation in the time to first exacerbation and reduction in the frequency of exacerbations [In this manuscript, we conducted an exploratory analysis to evaluate the effect of brensocatib on PR3 and CatG activity in sputum, and NE activity in white blood cell (WBC) extracts, which were not previously reported in the phase 2b trial in NCFBE [