The preclinical and phase 1 development of the novel oral cathepsin C inhibitor BI 1291583

Bronchiectasis is a heterogeneous lung disease characterised by chronic inflammation, infection, impaired mucociliary clearance and progressive structural lung damage [1, 2]. These features interact in a "vicious vortex", leading to exacerbations and declining pulmonary function with associated morbidity and mortality [1, 2]. Footnotes This manuscript has recently been accepted for publication in the ERJ Open Research . It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJOR online. Please open or download the PDF to view this article. Conflict of interest : J.D. Chalmers reports grant support from AstraZeneca, Boehringer Ingelheim, Gilead Sciences, GlaxoSmithKline, Grifols, Insmed, Novartis and Zambon and consulting fees from AstraZeneca, Boehringer Ingelheim, GlaxoSmithKline, Insmed, Pfizer and Zambon. Conflict of interest : P. Badorrek has nothing to disclose. Conflict of interest : C. Diefenbach, H. Kögler, W. Sauter and S. Kreideweiss are employees of Boehringer Ingelheim. Conflict of interest : J. M. Hohlfeld reports grant support paid to his institution from AltamiraPharma GmbH, Astellas Pharma GmbH, AstraZeneca AB, Bayer AG, Boehringer Ingelheim Pharma GmbH & Co. KG, CSL Behring GmbH, Desitin Arzneimittel GmbH, EpiEndo, F. Hoffmann-La Roche AG, Genentech, Inc., M&P Pharma AG, Novartis AG, OM Pharma SA and Sanofi-Aventis Deutschland GmbH, personal fees for consultancy from Boehringer Ingelheim Pharma GmbH & Co. KG, Cureteq and Roche, personal fees for lectures from Novartis AG, and personal fees for board service from CSL Behring GmbH and Nocion.