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Idiopathic Pulmonary Fibrosis: 8 Years On After Nintedanib and Pirfenidone Approval—What Is on the Horizon?

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Abstract

Purpose of Review

The approval of nintedanib and pirfenidone has changed the treatment landscape of idiopathic pulmonary fibrosis (IPF); however, both drugs only slow disease progression and are burdened by tolerability issues. We summarize the most advanced developmental drugs in IPF, but also mention selected compounds in earlier phases.

Recent Findings

Several compounds are currently being tested in IPF; the number of trials has increased exponentially in the last 3 years. Four compounds have reached phase 3: BI101550, an oral PDE4B preferential inhibitor; Pamrevlumab, an anticonnective tissue growth factor intravenous monoclonal antibody; Pentraxin-2, a recombinant human form of serum amyloid protein; Treprostinil, a synthetic prostanoid, with an inhaled formulation, currently used for pulmonary hypertension.

Summary

New drugs are likely to reach the clinic in the near future. This will provide more opportunities for treatment of IPF but will also pose unprecedented challenges regarding drug selection and administration(i.e., sequential vs. combination).

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  1. Respiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova and Padova University Hospital, Padova, Italy

    Gioele Castelli, Elisabetta Cocconcelli, Nicol Bernardinello, Elisabetta Balestro & Paolo Spagnolo

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PS reports institutional grants, personal fees, and non-financial support from PPM Services and Boehringer Ingelheim; institutional grants from Roche; personal fees from Chiesi, Novartis, Galapagos, Lupin, Pieris, Behring, AstraZeneca, Glycocore Pharma, and Menarini outside the submitted work; wife employee of AstraZeneca. EB reports personal fees from Boehringer Ingelheim and Roche outside the submitted work. EC reports personal fees from Boehringer Ingelheim outside the submitted work. GC and NB do not have any conflicts of interest to declare.

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Castelli, G., Cocconcelli, E., Bernardinello, N. et al. Idiopathic Pulmonary Fibrosis: 8 Years On After Nintedanib and Pirfenidone Approval—What Is on the Horizon?. Curr Pulmonol Rep 12, 113–124 (2023). https://doi.org/10.1007/s13665-023-00315-y

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