Idiopathic pulmonary fibrosis (IPF) is a rare lung disease characterised by progressive loss of lung function, dyspnoea and cough. IPF has a variable clinical course but a poor prognosis. Nintedanib, a tyrosine kinase inhibitor, is one of two drugs approved for the treatment of IPF. In clinical trials, nintedanib slowed disease progression by reducing the rate of decline in forced vital capacity (FVC) in patients with IPF and mild or moderate lung function impairment. The effect of nintedanib was consistent across patient subgroups defined by baseline characteristics including FVC % predicted, diffusion capacity of the lung for carbon monoxide % predicted and the presence of emphysema. Recently, it has been shown that the rate of decline in FVC and the treatment effect of nintedanib are the same in patients with preserved lung volume (FVC >90% predicted) as in patients with greater impairment in FVC, supporting the value of early treatment of IPF. The adverse events most commonly associated with nintedanib, both in clinical trials and real-world clinical practice, are mild gastrointestinal events, particularly diarrhoea. Side effects are manageable in a majority of patients through symptomatic treatment, dose reductions and treatment interruptions, enabling most patients to stay on treatment in the long term.
- Interstitial Fibrosis
- Rare lung diseases
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Contributors The authors meet criteria for authorship as recommended by the International Committee of Medical Journal Editors (ICMJE).
Funding The authors received no direct compensation related to the development of this article. Page processing and open access charges for this article have been paid by Boehringer Ingelheim Pharmaceuticals, Inc.
Competing interests Amy Case reports personal fees from Boehringer Ingelheim outside the submitted work. Peace Johnson reports no competing interests.
Provenance and peer review Not commissioned; externally peer reviewed.
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