Emerging therapies for idiopathic pulmonary fibrosis, a progressive age-related disease
Author:
Publisher
Springer Science and Business Media LLC
Subject
Drug Discovery,Pharmacology,General Medicine
Link
http://www.nature.com/articles/nrd.2017.170.pdf
Reference227 articles.
1. Wynn, T. A. & Ramalingam, T. R. Mechanisms of fibrosis: therapeutic translation for fibrotic disease. Nat. Med. 18, 1028–1040 (2012).
2. Wynn, T. A. Common and unique mechanisms regulate fibrosis in various fibroproliferative diseases. J. Clin. Invest. 117, 524–529 (2007).
3. Idiopathic Pulmonary Fibrosis Clinical Research Network et al. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N. Engl. J. Med. 366, 1968–1977 (2012). This study clearly demonstrated that patients with IPF treated with a combination of prednisone, azathioprine and NAC have an increased risk of death and hospitalization, supporting the notion that IPF is not an inflammatory-driven disease.
4. King, T. E. Jr. et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N. Engl. J. Med. 370, 2083–2092 (2014). This phase III study confirms and extends the findings that pirfenidone reduces disease progression in IPF patients with mild-to-moderate physiological impairment with an acceptable side-effect profile.
5. Richeldi, L. et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N. Engl. J. Med. 370, 2071–2082 (2014). In both INPULSIS trials, it was shown that nintedanib, a potent kinase inhibitor blocking the effects of growth factors implicated in the pathogenesis of IPF, reduced the decline of FEV in patients with mild-to-moderate impairment of pulmonary function, which is consistent with a slowing of disease progression, and in general with tolerable adverse events.
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