New targets in idiopathic pulmonary fibrosis: from inflammation and immunity to remodeling and repair

References

• King TE Jr, Pardo A, Selman M. Idiopathic pulmonary fibrosis. Lancet. 2011;378:1949–1961.
   PubMed Web of Science ®Google Scholar
• Travis WD, Costabel U, Hansell DM, et al. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188:733–748.
   PubMed Web of Science ®Google Scholar
• Souza CA, Muller NL, Flint J, et al. Idiopathic pulmonary fibrosis: spectrum of high-resolution CT findings. AJR Am J Roentgenol. 2005;185:1531–1539.
   PubMed Web of Science ®Google Scholar
• de Cremoux H, Bernaudin JF, Laurent P, et al. Interactions between cigarette smoking and the natural history of idiopathic pulmonary fibrosis. Chest. 1990;98:71–76.
   PubMed Web of Science ®Google Scholar
• Baumgartner KB, Samet JM, Stidley CA, et al. Cigarette smoking: a risk factor for idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1997;155:242–248.
   PubMed Web of Science ®Google Scholar
• Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183:788–824.
   PubMed Web of Science ®Google Scholar
• Lee SH, Kim DS, Kim YW, et al. Association between occupational dust exposure and prognosis of idiopathic pulmonary fibrosis: a Korean national survey. Chest. 2015;147:465–474.
   PubMed Web of Science ®Google Scholar
• Raghu G, Freudenberger TD, Yang S, et al. High prevalence of abnormal acid gastro-oesophageal reflux in idiopathic pulmonary fibrosis. Eur Respir J. 2006;27:136–142.
   PubMed Web of Science ®Google Scholar
• Stewart JP, Egan JJ, Ross AJ, et al. The detection of Epstein-Barr virus DNA in lung tissue from patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1999;159:1336–1341.
   PubMed Web of Science ®Google Scholar
• Lasithiotaki I, Antoniou KM, Vlahava V.M, et al. Detection of herpes simplex virus type-1 in patients with fibrotic lung diseases. PLoS One. 2011;6:e27800.
   PubMed Web of Science ®Google Scholar
• Sgalla G, Biffi A, Richeldi L. Idiopathic pulmonary fibrosis: diagnosis, epidemiology and natural history. Respirology. 2016;21(3):427–437.
   PubMed Web of Science ®Google Scholar
• Santangelo S, Scarlata S, Zito A, et al. Genetic background of idiopathic pulmonary fibrosis. Expert Rev Mol Diagn. 2013;13:389–406.
   PubMed Web of Science ®Google Scholar
• Kannengiesser C, Borie R, Menard C, et al. Heterozygous RTEL1 mutations are associated with familial pulmonary fibrosis. Eur Respir J. 2015;46:474–485.
   PubMed Web of Science ®Google Scholar
• Stuart BD, Choi J, Zaidi S, et al. Exome sequencing links mutations in PARN and RTEL1 with familial pulmonary fibrosis and telomere shortening. Nat Genet. 2015;47:512–517.
   PubMed Web of Science ®Google Scholar
• Nathan SD, Shlobin OA, Weir N, et al. Long-term course and prognosis of idiopathic pulmonary fibrosis in the new millennium. Chest. 2011;140:221–229.
   PubMed Web of Science ®Google Scholar
• Selman M, Carrillo G, Estrada A, et al. Accelerated variant of idiopathic pulmonary fibrosis: clinical behavior and gene expression pattern. PLoS One. 2007;2:e482.
   PubMed Web of Science ®Google Scholar
• Maher TM, Whyte MKB, Hoyles RK, et al. Development of a consensus statement for the definition, diagnosis, and treatment of acute exacerbations of idiopathic pulmonary fibrosis using the Delphi technique. Adv Ther. 2015;32(10):929–943.
   PubMed Web of Science ®Google Scholar
• Nadrous HF, Pellikka PA, Krowka MJ, et al. Pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Chest. 2005;128:2393–2399.
   PubMed Web of Science ®Google Scholar
• George TJ, Arnaoutakis GJ, Shah AS. Lung transplant in idiopathic pulmonary fibrosis. Arch Surg. 2011;146:1204–1209.
   PubMedGoogle Scholar
• Demedts M, Behr J, Buhl R, et al. High-dose acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med. 2005;353:2229–2242.
   PubMed Web of Science ®Google Scholar
• Idiopathic Pulmonary Fibrosis Clinical Research, Raghu G, Anstrom KJ, et al. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med. 2012;366:1968–1977.
   PubMed Web of Science ®Google Scholar
• Idiopathic Pulmonary Fibrosis Clinical Research, Martinez FJ, de Andrade JA, et al. Randomized trial of acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370:2093–2101.
   PubMed Web of Science ®Google Scholar
• King TE Jr, 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.
   PubMed Web of Science ®Google Scholar
• Noble PW, Albera C, Bradford WZ, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011;377:1760–1769.
   PubMed Web of Science ®Google Scholar
• Aravena C, Labarca G, Venegas C, et al. Pirfenidone for idiopathic pulmonary fibrosis: a systematic review and meta-analysis. PLoS One. 2015;10:e0136160.
   PubMed Web of Science ®Google Scholar
• 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;47:243–253.
   PubMed Web of Science ®Google Scholar
• Wollin L, Wex E, Pautsch A, et al. Mode of action of nintedanib in the treatment of idiopathic pulmonary fibrosis. Eur Respir J. 2015;45:1434–1445.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• Lee T, Park JY, Lee HY, et al. Lung cancer in patients with idiopathic pulmonary fibrosis: clinical characteristics and impact on survival. Respir Med. 2014;108:1549–1555.
   PubMed Web of Science ®Google Scholar
• Nathan N, Giraud V, Picard C, et al. Germline SFTPA1 mutation in familial idiopathic interstitial pneumonia and lung cancer. Hum Mol Genet. 2016;25(8):1457–1467.
   PubMed Web of Science ®Google Scholar
• Lenna S, Trojanowska M. The role of endoplasmic reticulum stress and the unfolded protein response in fibrosis. Curr Opin Rheumatol. 2012;24:663–668.
   PubMed Web of Science ®Google Scholar
• Chilosi M, Poletti V, Zamo A, et al. Aberrant Wnt/beta-catenin pathway activation in idiopathic pulmonary fibrosis. Am J Pathol. 2003;162:1495–1502.
   PubMed Web of Science ®Google Scholar
• Plantier L, Crestani B, Wert SE, et al. Ectopic respiratory epithelial cell differentiation in bronchiolised distal airspaces in idiopathic pulmonary fibrosis. Thorax. 2011;66:651–657.
   PubMed Web of Science ®Google Scholar
• Seibold MA, Wise AL, Speer MC, et al. A common MUC5B promoter polymorphism and pulmonary fibrosis. N Engl J Med. 2011;364:1503–1512.
   PubMed Web of Science ®Google Scholar
• Yang IV, Coldren CD, Leach SM, et al. Expression of cilium-associated genes defines novel molecular subtypes of idiopathic pulmonary fibrosis. Thorax. 2013;68:1114–1121.
   PubMed Web of Science ®Google Scholar
• DePianto DJ, Chandriani S, Abbas AR, et al. Heterogeneous gene expression signatures correspond to distinct lung pathologies and biomarkers of disease severity in idiopathic pulmonary fibrosis. Thorax. 2015;70:48–56.
   PubMed Web of Science ®Google Scholar
• Datta A, Alexander R, Sulikowski MG, et al. Evidence for a functional thymic stromal lymphopoietin signaling axis in fibrotic lung disease. J Immunol. 2013;191:4867–4879.
   PubMed Web of Science ®Google Scholar
• Froidure A, Shen C, Gras D, et al. Myeloid dendritic cells are primed in allergic asthma for thymic stromal lymphopoietin-mediated induction of Th2 and Th9 responses. Allergy. 2014;69(8):1068–1076.
   PubMed Web of Science ®Google Scholar
• Chilosi M, Poletti V, Murer B, et al. Abnormal re-epithelialization and lung remodeling in idiopathic pulmonary fibrosis: the role of deltaN-p63. Lab Invest. 2002;82:1335–1345.
   PubMed Web of Science ®Google Scholar
• Honda T, Uehara T, Sano K. Heterogeneous proliferation of type II pneumocytes in usual interstitial pneumonia. Pathology. 2006;38:433–436.
   PubMed Web of Science ®Google Scholar
• Melboucy-Belkhir S, Pradere P, Tadbiri S, et al. Forkhead Box F1 represses cell growth and inhibits COL1 and ARPC2 expression in lung fibroblasts in vitro. Am J Physiol Lung Cell Mol Physiol. 2014;307:L838–L847.
   PubMed Web of Science ®Google Scholar
• Joannes A, Brayer S, Besnard V, et al. FGF9 and FGF18 in idiopathic pulmonary fibrosis promote survival and migration and inhibit myofibroblast differentiation of human lung fibroblasts in vitro. Am J Physiol Lung Cell Mol Physiol. 2016;310:L615–L629.
   PubMed Web of Science ®Google Scholar
• Fernandez IE, Eickelberg O. New cellular and molecular mechanisms of lung injury and fibrosis in idiopathic pulmonary fibrosis. Lancet. 2012;380:680–688.
   PubMed Web of Science ®Google Scholar
• Cigna N, Farrokhi Moshai E, Brayer S, et al. The hedgehog system machinery controls transforming growth factor-beta-dependent myofibroblastic differentiation in humans: involvement in idiopathic pulmonary fibrosis. Am J Pathol. 2012;181:2126–2137.
   PubMed Web of Science ®Google Scholar
• Seibold MA, Smith RW, Urbanek C, et al. The idiopathic pulmonary fibrosis honeycomb cyst contains a mucocilary pseudostratified epithelium. PLoS One. 2013;8:e58658.
   PubMed Web of Science ®Google Scholar
• Marchal-Somme J, Uzunhan Y, Marchand-Adam S, et al. Dendritic cells accumulate in human fibrotic interstitial lung disease. Am J Respir Crit Care Med. 2007;176:1007–1014.
   PubMed Web of Science ®Google Scholar
• Ziesche R, Hofbauer E, Wittmann K, et al. A preliminary study of long-term treatment with interferon gamma-1b and low-dose prednisolone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 1999;341:1264–1269.
   PubMed Web of Science ®Google Scholar
• Antoniou KM, Nicholson AG, Dimadi M, et al. Long-term clinical effects of interferon gamma-1b and colchicine in idiopathic pulmonary fibrosis. Eur Respir J. 2006;28:496–504.
   PubMed Web of Science ®Google Scholar
• Raghu G, Brown KK, Bradford WZ, et al. A placebo-controlled trial of interferon gamma-1b in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2004;350:125–133.
   PubMed Web of Science ®Google Scholar
• Molyneaux PL, Cox MJ, Willis-Owen SA, et al. The role of bacteria in the pathogenesis and progression of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2014;190:906–913.
   PubMed Web of Science ®Google Scholar
• Han MK, Zhou Y, Murray S, et al. Lung microbiome and disease progression in idiopathic pulmonary fibrosis: an analysis of the COMET study. Lancet Respir Med. 2014;2:548–556.
   PubMed Web of Science ®Google Scholar
• Habiel DM, Hogaboam C. Heterogeneity in fibroblast proliferation and survival in idiopathic pulmonary fibrosis. Front Pharmacol. 2014;5:2.
   PubMed Web of Science ®Google Scholar
• Iyer SN, Gurujeyalakshmi G, Giri SN. Effects of pirfenidone on transforming growth factor-beta gene expression at the transcriptional level in bleomycin hamster model of lung fibrosis. J Pharmacol Exp Ther. 1999;291:367–373.
   PubMed Web of Science ®Google Scholar
• Lin X, Yu M, Wu K, et al. Effects of pirfenidone on proliferation, migration, and collagen contraction of human Tenon’s fibroblasts in vitro. Invest Ophthalmol Vis Sci. 2009;50:3763–3770.
   PubMed Web of Science ®Google Scholar
• Hisatomi K, Mukae H, Sakamoto N, et al. Pirfenidone inhibits TGF-beta1-induced over-expression of collagen type I and heat shock protein 47 in A549 cells. BMC Pulm Med. 2012;12:24.
   PubMed Web of Science ®Google Scholar
• Nakazato H, Oku H, Yamane S, et al. A novel anti-fibrotic agent pirfenidone suppresses tumor necrosis factor-alpha at the translational level. Eur J Pharmacol. 2002;446:177–185.
   PubMed Web of Science ®Google Scholar
• Giri SN, Leonard S, Shi X, et al. Effects of pirfenidone on the generation of reactive oxygen species in vitro. J Environ Pathol Toxicol Oncol. 1999;18:169–177.
   PubMedGoogle Scholar
• Yamagami K, Oka T, Wang Q, et al. Pirfenidone exhibits cardioprotective effects by regulating myocardial fibrosis and vascular permeability in pressure-overloaded hearts. Am J Physiol Heart Circ Physiol. 2015;309:H512–H522.
   PubMed Web of Science ®Google Scholar
• Rangarajan S, Kurundkar A, Kurundkar D, et al. Novel mechanisms for the antifibrotic action of nintedanib. Am J Respir Cell Mol Biol. 2016;54:51–59.
   PubMed Web of Science ®Google Scholar
• Raghu G, Martinez FJ, Brown KK, et al. CC-chemokine ligand 2 inhibition in idiopathic pulmonary fibrosis: a phase 2 trial of carlumab. Eur Respir J. 2015;46(6):1740–1750.
   Web of Science ®Google Scholar
• Chien JW, Richards TJ, Gibson KF, et al. Serum lysyl oxidase-like 2 levels and idiopathic pulmonary fibrosis disease progression. Eur Respir J. 2014;43:1430–1438.
   PubMed Web of Science ®Google Scholar
• Yoshida K, Kuwano K, Hagimoto N, et al. MAP kinase activation and apoptosis in lung tissues from patients with idiopathic pulmonary fibrosis. J Pathol. 2002;198:388–396.
   PubMed Web of Science ®Google Scholar
• Shulgina L, Cahn AP, Chilvers ER, et al. Treating idiopathic pulmonary fibrosis with the addition of co-trimoxazole: a randomised controlled trial. Thorax. 2013;68:155–162.
   PubMed Web of Science ®Google Scholar
• Wuyts WA, Agostini C, Antoniou KM, et al. The pathogenesis of pulmonary fibrosis: a moving target. Eur Respir J. 2013;41:1207–1218.
   PubMed Web of Science ®Google Scholar
• Kreuter M, Kirsten D, Bahmer T, et al. Screening for helicobacter pylori in idiopathic pulmonary fibrosis lung biopsies. Respiration. 2016;91(1):3–8.
   PubMed Web of Science ®Google Scholar
• Lee JS, Ryu JH, Elicker BM, et al. Gastroesophageal reflux therapy is associated with longer survival in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;184:1390–1394.
   PubMed Web of Science ®Google Scholar
• Lee JS, Collard HR, Anstrom KJ, et al. Anti-acid treatment and disease progression in idiopathic pulmonary fibrosis: an analysis of data from three randomised controlled trials. Lancet Respir Med. 2013;1:369–376.
   PubMed Web of Science ®Google Scholar
• Tashiro J, Elliot SJ, Gerth DJ, et al. Therapeutic benefits of young, but not old, adipose-derived mesenchymal stem cells in a chronic mouse model of bleomycin-induced pulmonary fibrosis. Transl Res. 2015;166:554–567.
   PubMed Web of Science ®Google Scholar
• Pilling D, Roife D, Wang M, et al. Reduction of bleomycin-induced pulmonary fibrosis by serum amyloid P. J Immunol. 2007;179:4035–4044.
   PubMed Web of Science ®Google Scholar
• van den Blink B, Dillingh MR, Ginns LC, et al. Recombinant human pentraxin-2 therapy in patients with idiopathic pulmonary fibrosis: safety, pharmacokinetics and exploratory efficacy. Eur Respir J. 2016;47(3):889–897.
   PubMed Web of Science ®Google Scholar
• Funke M, Zhao Z, Xu Y, et al. The lysophosphatidic acid receptor LPA1 promotes epithelial cell apoptosis after lung injury. Am J Respir Cell Mol Biol. 2012;46:355–364.
   PubMed Web of Science ®Google Scholar
• Saini G, Porte J, Weinreb PH, et al. alphavbeta6 integrin may be a potential prognostic biomarker in interstitial lung disease. Eur Respir J. 2015;46:486–494.
   PubMed Web of Science ®Google Scholar
• Moshai EF, Wemeau-Stervinou L, Cigna N, et al. Targeting the hedgehog-glioma-associated oncogene homolog pathway inhibits bleomycin-induced lung fibrosis in mice. Am J Respir Cell Mol Biol. 2014;51:11–25.
   PubMed Web of Science ®Google Scholar
• Murray LA, Zhang H, Oak SR, et al. Targeting interleukin-13 with tralokinumab attenuates lung fibrosis and epithelial damage in a humanized SCID idiopathic pulmonary fibrosis model. Am J Respir Cell Mol Biol. 2014;50:985–994.
   PubMed Web of Science ®Google Scholar
• Yamashita CM, Dolgonos L, Zemans RL, et al. Matrix metalloproteinase 3 is a mediator of pulmonary fibrosis. Am J Pathol. 2011;179:1733–1745.
   PubMed Web of Science ®Google Scholar
• Craig VJ, Zhang L, Hagood JS, et al. Matrix metalloproteinases as therapeutic targets for idiopathic pulmonary fibrosis. Am J Respir Cell Mol Biol. 2015;53:585–600.
   PubMed Web of Science ®Google Scholar
• Staab-Weijnitz CA, Fernandez IE, Knuppel L, et al. FK506-binding protein 10, a potential novel drug target for idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2015;192:455–467.
   PubMed Web of Science ®Google Scholar
• Huang LS, Mathew B, Li H, et al. The mitochondrial cardiolipin remodeling enzyme lysocardiolipin acyltransferase is a novel target in pulmonary fibrosis. Am J Respir Crit Care Med. 2014;189:1402–1415.
   PubMed Web of Science ®Google Scholar
• Bardou O, Menou A, Francois C, et al. Membrane-anchored serine protease matriptase is a trigger of pulmonary fibrogenesis. Am J Respir Crit Care Med. 2015. DOI:10.1164/rccm.201502-0299OC
   PubMed Web of Science ®Google Scholar
• Konigshoff M, Kramer M, Balsara N, et al. WNT1-inducible signaling protein-1 mediates pulmonary fibrosis in mice and is upregulated in humans with idiopathic pulmonary fibrosis. J Clin Invest. 2009;119:772–787.
   PubMed Web of Science ®Google Scholar
• Kim TH, Kim SH, Seo JY, et al. Blockade of the Wnt/beta-catenin pathway attenuates bleomycin-induced pulmonary fibrosis. Tohoku J Exp Med. 2011;223:45–54.
   PubMed Web of Science ®Google Scholar
• Rajasekaran S, Rajaguru P, Sudhakar Gandhi PS. MicroRNAs as potential targets for progressive pulmonary fibrosis. Front Pharmacol. 2015;6:254.
   PubMed Web of Science ®Google Scholar
• Zhou Y, Huang X, Hecker L, et al. Inhibition of mechanosensitive signaling in myofibroblasts ameliorates experimental pulmonary fibrosis. J Clin Invest. 2013;123:1096–1108.
   PubMed Web of Science ®Google Scholar
• Liu F, Lagares D, Choi KM, et al. Mechanosignaling through YAP and TAZ drives fibroblast activation and fibrosis. Am J Physiol Lung Cell Mol Physiol. 2015;308:L344–L357.
   PubMed Web of Science ®Google Scholar
• Jose RJ, Williams AE, Chambers RC. Proteinase-activated receptors in fibroproliferative lung disease. Thorax. 2014;69:190–192.
   PubMed Web of Science ®Google Scholar
• Lin C, von der Thusen J, Daalhuisen J, et al. Protease-activated receptor (PAR)-2 is required for PAR-1 signalling in pulmonary fibrosis. J Cell Mol Med. 2015;19:1346–1356.
   PubMed Web of Science ®Google Scholar
• Wettstein G, Bellaye PS, Kolb M, et al. Inhibition of HSP27 blocks fibrosis development and EMT features by promoting Snail degradation. FASEB J. 2013;27:1549–1560.
   PubMed Web of Science ®Google Scholar
• Guillamat-Prats R, Gay-Jordi G, Xaubet A, et al. Alveolar type II cell transplantation restores pulmonary surfactant protein levels in lung fibrosis. J Heart Lung Transplant. 2014;33:758–765.
   PubMed Web of Science ®Google Scholar
• Serrano-Mollar A, Nacher M, Gay-Jordi G, et al. Intratracheal transplantation of alveolar type II cells reverses bleomycin-induced lung fibrosis. Am J Respir Crit Care Med. 2007;176:1261–1268.
   PubMed Web of Science ®Google Scholar
• Degryse AL, Lawson WE. Progress toward improving animal models for idiopathic pulmonary fibrosis. Am J Med Sci. 2011;341:444–449.
   PubMed Web of Science ®Google Scholar
• Degryse AL, Tanjore H, Xu XC, et al. Repetitive intratracheal bleomycin models several features of idiopathic pulmonary fibrosis. Am J Physiol Lung Cell Mol Physiol. 2010;299:L442–L452.
   PubMed Web of Science ®Google Scholar
• Liang M, Lv J, Zou L, et al. A modified murine model of systemic sclerosis: bleomycin given by pump infusion induced skin and pulmonary inflammation and fibrosis. Lab Invest. 2015;95:342–350.
   PubMed Web of Science ®Google Scholar
• Parra ER, Boufelli G, Bertanha F, et al. Temporal evolution of epithelial, vascular and interstitial lung injury in an experimental model of idiopathic pulmonary fibrosis induced by butyl-hydroxytoluene. Int J Exp Pathol. 2008;89:350–357.
   PubMed Web of Science ®Google Scholar
• Parra ER, Pincelli MS, Teodoro WR, et al. Modeling pulmonary fibrosis by abnormal expression of telomerase/apoptosis/collagen V in experimental usual interstitial pneumonia. Braz J Med Biol Res. 2014;47:567–575.
   PubMed Web of Science ®Google Scholar
• Decologne N, Kolb M, Margetts PJ, et al. TGF-beta1 induces progressive pleural scarring and subpleural fibrosis. J Immunol. 2007;179:6043–6051.
   PubMed Web of Science ®Google Scholar
• Povedano JM, Martinez P, Flores JM, et al. Mice with pulmonary fibrosis driven by telomere dysfunction. Cell Rep. 2015;12:286–299.
   PubMed Web of Science ®Google Scholar
• Jenkins G, Blanchard A, Borok Z, et al. In search of the fibrotic epithelial cell: opportunities for a collaborative network. Thorax. 2012;67:179–182.
   PubMed Web of Science ®Google Scholar
• Costabel U, Inoue Y, Richeldi L, et al. Efficacy of nintedanib in idiopathic pulmonary fibrosis across prespecified subgroups in INPULSIS. Am J Respir Crit Care Med. 2016;193:178–185.
   PubMed Web of Science ®Google Scholar
• Naik PK, Bozyk PD, Bentley JK, et al. Periostin promotes fibrosis and predicts progression in patients with idiopathic pulmonary fibrosis. Am J Physiol Lung Cell Mol Physiol. 2012;303:L1046–L1056.
   PubMed Web of Science ®Google Scholar
• Tominaga J, Sakai F, Johkoh T, et al. Diagnostic certainty of idiopathic pulmonary fibrosis/usual interstitial pneumonia: the effect of the integrated clinico-radiological assessment. Eur J Radio L. 2015;84(12):2640–2645.
   Google Scholar
• Behr J, Kreuter M, Hoeper MM, et al. Management of patients with idiopathic pulmonary fibrosis in clinical practice: the INSIGHTS-IPF registry. Eur Respir J. 2015;46:186–196.
   PubMed Web of Science ®Google Scholar
• Xaubet A, Serrano-Mollar A, Ancochea J. Pirfenidone for the treatment of idiopathic pulmonary fibrosis. Expert Opin Pharmacother. 2014;15:275–281.
   PubMed Web of Science ®Google Scholar
• Wuyts WA, Antoniou KM, Borensztajn K, et al. Combination therapy: the future of management for idiopathic pulmonary fibrosis? Lancet Respir Med. 2014;2:933–942.
   PubMed Web of Science ®Google Scholar
• Loveman E, Copley VR, Colquitt J, et al. The clinical effectiveness and cost-effectiveness of treatments for idiopathic pulmonary fibrosis: a systematic review and economic evaluation. Health Technol Assess. 2015;19:i–xxiv, 1–336.
   Web of Science ®Google Scholar
• Loveman E, Copley VR, Colquitt JL, et al. The effectiveness and cost-effectiveness of treatments for idiopathic pulmonary fibrosis: systematic review, network meta-analysis and health economic evaluation. BMC Pharmacol Toxicol. 2014;15:63.
   PubMed Web of Science ®Google Scholar
• Bonella F, Wijsenbeek M, Molina-Molina M, et al. European IPF patient charter: unmet needs and a call to action for healthcare policymakers. Eur Respir J. 2016;47:597–606.
   PubMed Web of Science ®Google Scholar
• Crestani B. European IPF patient charter: an SOS to the world. Eur Respir J. 2016;47:403–405.
   PubMed Web of Science ®Google Scholar