References
- Joint Statement of the American Thoracic Society and European Respiratory Society. American Thoracic Society/European Respiratory Society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Am. J. Respir. Crit. Care Med.165, 277–304 (2002).
- Bjoraker JA, Ryu JH, Edwin MK et al. Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am. J. Respir. Crit. Care Med.157, 199–203 (1998).
- Turner-Warwick M, Burrows B, Johnson A. Cryptogenic fibrosing alveolitis: response to corticosteroid treatment and its effect on survival. Thorax35, 593–599 (1980).
- Collard HR, Ryu JH, Douglas WW et al. Combined corticosteroid and cyclophosphamide therapy does not alter survival in idiopathic pulmonary fibrosis. Chest125, 2169–2174 (2004).
- Selman M, King TE, Pardo A. Idiopathic pulmonary fibrosis: prevailing and evolving hypotheses about its pathogenesis and implications for therapy. Ann. Intern. Med.134, 136–151 (2001).
- Demedts M, Behr J, Buhl R et al. High-dose acetylcysteine in idiopathic pulmonary fibrosis. N. Engl. J. Med.353, 2229–2242 (2005).
- Shi-Wen X, Chen Y, Denton CP et al. Endothelin-1 promotes myofibroblast induction through the ETA receptor via a rac/phosphoinositide 3-kinase/Akt-dependent pathway and is essential for the enhanced contractile phenotype of fibrotic fibroblasts. Mol. Biol. Cell15, 2707–2719 (2004).
- Teder P, Noble PW. A cytokine reborn? Endothelin-1 in pulmonary inflammation and fibrosis. Am. J. Respir. Cell Mol. Biol.23, 7–10 (2000).
- Rodriguez-Pascual F, Redondo-Horcajo M, Lamas S. Functional cooperation between Smad proteins and activator protein-1 regulates transforming growth factor-β-mediated induction of endothelin-1 expression. Circ. Res.92, 1288–1295 (2003).
- Coultas D, Zumwalt R, Black W et al. The epidemiology of interstitial lung disease. Am. J. Respir. Crit. Care Med.150, 967–972 (2004).
- Olson AL, Swigris JJ, Lezotte D et al. Mortality rates from pulmonary fibrosis (PF) steadily increased over the period from 1992 to 2003. Am. J. Respir. Crit. Care Med.175, A142 (2007).
- Raghu G, Weycker D, Edelsberg J et al. Incidence and prevalence of idiopathic pulmonary fibrosis. Am. J. Respir. Crit. Care Med.174, 810–816 (2006).
- Giaid A, Michel RP, Stewart DJ et al. Expression of endothelin-1 in lungs of patients with cryptogenic fibrosing alveolitis. Lancet341, 1550–1554 (1993).
- Actelion Pharmaceuticals US I. Bosentan full prescribing information (2005).
- Weber C, Schmitt R, Birnboeck H et al. Multiple-dose pharmacokinetics, safety, and tolerability of bosentan, an endothelin receptor antagonist, in healthy male volunteers. J. Clin. Pharmacol.39, 703–714 (1999).
- Weber C, Schmitt R, Birnboeck H et al. Pharmacokinetics and pharmacodynamics of the endothelin-receptor antagonist bosentan in healthy human subjects. Clin. Pharmacol. Ther.60, 124–137 (1996).
- Dingemanse J, Bodin F, Weidekamm E et al. Influence of food intake and formulation on the pharmacokinetics and metabolism of bosentan, a dual endothelin receptor antagonist. J. Clin. Pharmacol.42, 283–289 (2002).
- Weber C, Gasser R, Hopfgartner G. Absorption, excretion, and metabolism of the endothelin receptor antagonist bosentan in healthy male subjects. Drug Metab. Dispos.27, 810–815 (1999).
- Prakash A, Perry CM. Bosentan. Am. J. Cardiovasc. Drugs2, 335–343 (2002).
- Selman M, Pardo A. The epithelial/fibroblastic pathway in the pathogenesis of idiopathic pulmonary fibrosis. Am. J. Respir. Cell Mol. Biol.29, S93–S97 (2003).
- Hocher B, Schwarz A, Fagan KA et al. Pulmonary fibrosis and chronic lung inflammation in ET-1 transgenic mice. Am. J. Respir. Cell Mol. Biol.23, 19–26 (2006).
- Park SH, Saleh D, Giaid A et al. Increased endothelin-1 in bleomycin-induced pulmonary fibrosis and the effect of an endothelin receptor antagonist. Am. J. Respir. Crit. Care Med.156, 600–608 (1997).
- Mutsaers SE, Marshall RP, Goldsack NR et al. Effect of endothelin receptor antagonists (BQ-485, Ro 47-0203) on collagen deposition during the development of bleomycin-induced pulmonary fibrosis in rats. Pulm. Pharmacol. Ther.11, 221–225 (1998).
- Shahar I, Fireman E, Topilsky M et al. Effect of endothelin-1 on α-smooth muscle actin expression and on alveolar fibroblasts proliferation in interstitial lung diseases. Int. J. Immunopharmacol.21, 759–775 (1999).
- Jain R, Shaul PW, Borok Z et al. Endothelin-1 induces alveolar epithelial–mesenchymal transition through endothelin type A receptor-mediated production of TGF-β1. Am. J. Respir. Cell Mol. Biol.37, 38–47 (2007).
- Clozel M, Salloukh H. Role of endothelin in fibrosis and anti-fibrotic potential of bosentan. Ann. Med.37, 2–12 (2005).
- Filep JG, Bodolay E, Sipka S et al. Plasma endothelin correlates with antiendothelial antibodies in patients with mixed connective tissue disease. Circulation92, 2969–2974 (1995).
- Vancheeswaran R, Azam A, Black C et al. Localization of endothelin-1 and its binding sites in scleroderma skin. J. Rheumatol.21, 1268–1276 (1994).
- Abraham DJ, Vancheeswaran R, Dashwood MR et al. Increased levels of endothelin-1 and differential endothelin type A and B receptor expression in scleroderma-associated fibrotic lung disease. Am. J. Pathol.151, 831–841 (1997).
- Cambrey AD, Harrison NK, Dawes KE et al. Increased levels of endothelin-1 in bronchoalveolar lavage fluid from patients with systemic sclerosis contribute to fibroblast mitogenic activity in vitro. Am. J. Respir. Cell Mol. Biol.11, 439–445 (1994).
- Carpagnano GE, Kharitonov SA, Wells AU et al. Increased vitronectin and endothelin-1 in the breath condensate of patients with fibrosing lung disease. Respiration70, 154–160 (2003).
- Crestani B, Odoux C, Rolland C et al. Hypoxia reduces endothelin production by rat alveolar type II cells in primary culture. Eur. Respir. J.11, 392–399 (1998).
- Giaid A, Polak JM, Gaitonde V et al. Distribution of endothelin-like immunoreactivity and mRNA in the developing and adult human lung. Am. J. Respir. Cell Mol. Biol.4, 50–58 (1991).
- Saleh D, Furukawa K, Tsao MS et al. Elevated expression of endothelin-1 and endothelin-converting enzyme-1 in idiopathic pulmonary fibrosis: possible involvement of proinflammatory cytokines. Am. J. Respir. Cell Mol. Biol.16, 187–193 (1997).
- Uguccioni M, Pulsatelli L, Grigolo B et al. Endothelin-1 in idiopathic pulmonary fibrosis. J. Clin. Pathol.48, 330–334 (1995).
- Seibold J, Black C, Denton C et al. Bosentan versus placebo in interstitial lung disease secondary to systemic sclerosis (SSc): the BUILD-2 study. Proc. Am. Thor. Soc.3, A243 (2006).
- King TE Jr, Behr J, Brown KK et al. BUILD-1: a randomized placebo-controlled trial of bosentan in idiopathic pulmonary fibrosis. Am. J. Respir. Crit. Care Med.177, 75–81 (2008).
- Bucala R, Spiegel L, Chesney J et al. Circulating fibrocytes define a new leukocyte subpopulation that mediates tissue repair. Mol. Med.1, 71–81 (1994).
- Phillips RJ, Burdick MD, Hong K et al. Circulating fibrocytes traffic to the lungs in response to CXCL12 and mediate fibrosis. J. Clin. Invest.114, 438–446 (2004).
- Hashimoto N, Jin H, Liu T et al. Bone marrow-derived progenitor cells in pulmonary fibrosis. J. Clin. Invest.113, 243–252 (2004).
- Willis BC, Liebler JM, Luby-Phelps K et al. Induction of epithelial-mesenchymal transition in alveolar epithelial cells by transforming growth factor-β1: potential role in idiopathic pulmonary fibrosis. Am. J. Pathol.166, 1321–1332 (2005).
- Armanios MY, Chen JJ, Cogan JD et al. Telomerase mutations in families with idiopathic pulmonary fibrosis. N. Engl. J. Med.356, 1317–1326 (2007).
- Tsakiri KD, Cronkhite JT, Kuan PJ et al. Adult-onset pulmonary fibrosis caused by mutations in telomerase. Proc. Natl Acad. Sci. USA104, 7552–7557 (2007).