The approvals of nintedanib and pirfenidone changed the treatment paradigm in idiopathic pulmonary fibrosis (IPF), and increased our understanding of the underlying disease mechanisms. Nonetheless, many challenges and unmet needs remain in the management of patients with IPF and other progressive fibrosing interstitial lung diseases.This review describes how the nintedanib clinical programme has helped to address some of these challenges. Data from this programme have informed changes to the IPF diagnostic guidelines, the timing of treatment initiation, and the assessment of disease progression. The use of nintedanib to treat patients with advanced lung function impairment, concomitant emphysema, patients awaiting lung transplantation and patients with IPF and lung cancer is discussed. The long-term use of nintedanib and an up-to-date summary of nintedanib in clinical practice are discussed. Directions for future research, namely emerging therapeutic options, precision medicine and other progressive fibrosing interstitial lung diseases, are described.Further developments in these areas should continue to improve patient outcomes.
- Valenzuela, C.
- Torrisi, S. E.
- Kahn, N.
- Quaresma, M.
- Stowasser, S.
- Kreuter, M.
Keywords
- Disease Progression
- Humans
- Idiopathic Pulmonary Fibrosis/diagnosis/*drug therapy
- Indoles/*therapeutic use
- Protein Kinase Inhibitors/therapeutic use
- Tomography, X-Ray Computed
- challenges
- idiopathic pulmonary fibrosis
- literature review
- nintedanib
- Ingelheim and Galapagos, outside the submitted work
- SET has received honoraria for
- lectures from Boehringer Ingelheim and F. Hoffmann-La Roche Ltd
- NK declares that he
- has no competing interests
- MQ and SS are employees of Boehringer Ingelheim
- International GmbH
- MK or his institution have received reimbursement for
- counselling and grants from Boehringer Ingelheim and F. Hoffmann-La Roche.
