References
- Ley B, Collard HR, King TE. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;183:431–440.
- Raghu G, Weycker D, Edelsberg J, et al. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2006;174:810–816.
- Raghu G, Remy-Jardin M, Richeldi L, et al. Idiopathic pulmonary fibrosis (an Update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT. Clinical Practice Guideline. 2022;205:E18–E47.
- Sgalla G, Iovene B, Calvello M, et al. Idiopathic pulmonary fibrosis: pathogenesis and management. Respir Res. 2018;19(32).
- Kotsianidis I, Nakou E, Bouchliou I, et al. Global impairment of CD4+CD25+FOXP3+ regulatory T cells in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2009;179:1121–1130.
- Richeldi L, du Bois RM, Raghu G, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N. Engl. J. Med. 2014;370:2071–2082.
- Noble PW, Albera C, Bradford WZ, et al. Pirfenidone for idiopathic pulmonary fibrosis: analysis of pooled data from three multinational phase 3 trials. Eur Respir J. 2016;47:243.
- Richeldi L, Azuma A, Cottin V, et al. Trial of a preferential phosphodiesterase 4B inhibitor for idiopathic pulmonary fibrosis. N Engl J Med. 2022;386:2178–2187.
- Raghu G, Rochwerg B, Zhang Y, et al. An official ATS/ERS/JRS/ALAT clinical practice guideline: treatment of idiopathic pulmonary fibrosis: an update of the 2011 clinical practice guideline. Am J Respir Crit Care Med. 2015;192:e3–e19.
- Borie R, Justet A, Beltramo G, et al. Pharmacological management of IPF. Respirology. 2016;21:615–625.
- Taniguchi H, Ebina M, Kondoh Y, et al. Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J. 2010;35:821–829.
- Noble PW, Albera C, Bradford WZ, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011;377:1760–1769.
- King TE, Bradford WZ, Castro-Bernardini S, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370:2083–2092.
- Laurenson S, Sidhu R, Goodall M, et al. Nice Guidance on nintedanib for treating idiopathic pulmonary fibrosis. Respir Res. 2016;4(3):176–177.
- Richeldi L. du Bois RM, Raghu G, et al. Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis. N. Engl. J. Med 2014;370:2071–2082.
- Harari S, Pesci A, Albera C, et al. Nintedanib in IPF: post hoc analysis of the Italian FIBRONET Observational Study. Respiration. 2022;101:577–584.
- Vancheri C, Sebastiani A, Tomassetti S, et al. Pirfenidone in real life: a retrospective observational multicentre study in Italian patients with idiopathic pulmonary fibrosis. Respir Med. 2019;156:78–84.
- Richeldi L, Fernández Pérez ER, Costabel U, et al. Pamrevlumab, an anti-connective tissue growth factor therapy, for idiopathic pulmonary fibrosis (PRAISE): a phase 2, randomised, double-blind, placebo-controlled trial. Lancet Respir Med. 2019;8:25–33.
- Raghu G, Van Den Blink B, Hamblin MJ, et al. Effect of recombinant human pentraxin 2 vs placebo on change in forced vital capacity in patients with idiopathic pulmonary fibrosis: a randomized clinical trial. JAMA. 2018;319:2299–2307.
- Di Martino E, Provenzani A, Vitulo P, et al. Systematic review and meta-analysis of pirfenidone, nintedanib, and pamrevlumab for the treatment of idiopathic pulmonary fibrosis. Ann Pharmacother. 2021;55:723–731.
- Herrmann FE, Hesslinger C, Wollin L, et al. BI 1015550 is a PDE4B Inhibitor and a Clinical Drug Candidate for the Oral Treatment of Idiopathic Pulmonary Fibrosis. Front Pharmacol. 2022;13:838449.
- Draheim R, Egerland U, Anti-Inflammatory RC. Potential of the Selective Phosphodiesterase 4 Inhibitor N-(3,5-Dichloro-pyrid-4-yl)-[1-(4-fluorobenzyl)-5-hydroxy-indole-3-yl] -glyoxylic Acid Amide (AWD 12-281), in Human Cell Preparations. J Pharmacol Exp Ther. 2004;308:555–563.
- Bardin P, Kanniess F, Gauvreau G, et al. Roflumilast for asthma: efficacy findings in mechanism of action studies. Pulm Pharmacol Ther. 2015;35(Suppl):S4–S10.
- Maher TM, Schlecker C, Luedtke D, et al. Phase I studies of BI 1015550, a preferential phosphodiesterase 4B inhibitor, in healthy males and patients with idiopathic pulmonary fibrosis. ERJ Open Res. 2022;8(4):00240–2022.
- Kohyama T, Liu X, Wen FQ, et al. PDE4 inhibitors attenuate fibroblast chemotaxis and contraction of native collagen gels. Am J Respir Cell Mol Biol. 2002;26:694–701.
- Pan JB, Hou YH, Zhang GJ. Rolipram attenuates bleomycin A5-induced pulmonary fibrosis in rats. Respirology. 2009;14:975–982.
- Cortijo J, Iranzo A, Milara X, et al. Roflumilast, a phosphodiesterase 4 inhibitor, alleviates bleomycin-induced lung injury. Br J Pharmacol. 2009;156:534–544.
- Udalov S, Dumitrascu R, Pullamsetti SS, et al. Effects of phosphodiesterase 4 inhibition on bleomycin-induced pulmonary fibrosis in mice. BMC Pulm Med. 2010;10:26.
- Swigris JJ, Andrae DA, Churney T, et al. Development and initial validation analyses of the living with idiopathic pulmonary fibrosis questionnaire. Am J Respir Crit Care Med. 2020;202(12):1689–1697.
- Richeldi L, Azuma A, Cottin V, et al. Late breaking abstract - additive effect of BI 1015550 and nintedanib in patients with IPF. Eur Respir J. 2022;60(66):4606.
- Khan FA, Stewart I, Moss S, et al. Three-month FVC change: a trial endpoint for idiopathic pulmonary fibrosis based on individual participant data meta-analysis. Am J Respir Crit Care Med. 2022;205:936–948.
- Giembycz MA. 4D or not 4D - the emetogenic basis of PDE4 inhibitors uncovered? Trends Pharmacol. Sci. 2002;23:548.
- Dietsch G, DiPalma C, Eyre R, et al. Characterization of the inflammatory response to a highly selective PDE4 inhibitor in the rat and the identification of biomarkers that correlate with toxicity. Toxicol Pathol. 2006;34:39–51.
- Raghu G, van den Blink B, Hamblin MJ, et al. Long-term treatment with recombinant human pentraxin 2 protein in patients with idiopathic pulmonary fibrosis: an open-label extension study. Lancet Respir Med. 2019;7(8):657–664.