S2K-Leitlinie zur Diagnostik und Therapie der idiopathischen Lungenfibrose

 

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Zusammenfassung

Die idiopathische pulmonale Fibrose (IPF) ist eine schwerwiegende und in der Regel zum Tod führende Erkrankung, die bisher nur unzureichend behandelt werden kann. Empfehlungen zur Diagnostik und Therapie wurden erstmals im ATS-ERS-Statement im Jahr 2000 publiziert [1]. Seither haben sich die diagnostischen Standards geändert und es liegen zahlreiche Therapiestudien zu diesem Krankheitsbild vor, die es erforderlich machten, die bestehenden Empfehlungen zu überarbeiten und eine wissenschaftlich begründete Leitlinie zu erstellen. Diese wurde von einer internationalen Expertengruppe in den Jahren 2006 – 2010 erarbeitet und publiziert [2]. Die folgenden Ausführungen beinhalten eine Übersetzung wesentlicher Inhalte der Originalleitlinie sowie die Interpretation und Adaptation der Empfehlungen an die speziellen Belange des deutschen Gesundheitssystems, wobei auch neue wissenschaftliche Erkenntnisse Berücksichtigung fanden. Die Deutsche Leitlinie zur Diagnostik und Therapie der IPF beruht auf einer Initiative deutscher Experten unter der Schirmherrschaft der Deutschen Gesellschaft für Pneumologie und Beatmungsmedizin (DGP) und auf den Ergebnissen einer Konsensuskonferenz, die am 3. 12. 2011 in Bochum unter Supervision der „Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften (AWMF)“ abgehalten wurde. Die Mehrzahl der Empfehlungen der internationalen Leitlinie konnten dabei übernommen werden. Basierend auf aktuellen Studienergebnissen, die zum Zeitpunkt der Verabschiedung der internationalen Leitlinie noch nicht bzw. nicht vollständig vorlagen wurden die schwach negativen Empfehlungen für die Antikoagulantientherapie und die Kombinationstherapie mit Prednison, Azathioprin und N-Acteylcystein in stark negative Empfehlungen umgewandelt, während für Pirfenidon, welches inzwischen in der Europäischen Union zugelassen ist, eine schwach positive Therapieempfehlung ausgesprochen wurde.

Abstract

Idiopathic pulmonary fibrosis is a fatal lung disease with a variable and unpredictable natural history and limited treatment options. Since publication of the ATS-ERS statement on IPF in the year 2000 diagnostic standards have improved and a considerable number of randomized controlled treatment trials have been published necessitating a revision. In the years 2006 – 2010 an international panel of IPF experts produced an evidence-based guideline on diagnosis and treatment of IPF, which was published in 2011. In order to implement this evidence-based guideline into the German Health System a group of German IPF experts translated and commented the international guideline, also including new publications in the field. A consensus conference was held in Bochum on December 3^rd 2011 under the protectorate of the “Deutsche Gesellschaft für Pneumologie und Beatmungsmedizin (DGP)” and supervised by the “Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften” (AWMF). Most recommendations of the international guideline were found to be appropriate for the german situation. Based on recent clinical studies “weak negative” treatment recommendations for pirfenidone and anticoagulation were changed into “weak positive” for pirfenidone and “strong negative” for anticoagulation. Based on negative results from the PANTHER-trial the recommendation for the combination therapy of prednisone plus azathiorpine plus N-acetlycsteine was also changed into strong negative für patients with definite IPF. This document summarizes essential parts of the international IPF guideline and the comments and recommendations of the German IPF consensus conference.

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• Literatur

• 1 American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment: international consensus statement. Am J Respir Crit Care Med 2000; 161: 646-664
  CrossrefPubMedGoogle Scholar
• 2 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
  CrossrefPubMedGoogle Scholar
• 3 Schunemann HJ, Jaeschke R, Cook DJ et al. An official ATS statement: grading the quality of evidence and strength of recommendations in ATS guidelines and recommendations. Am J Respir Crit Care Med 2006; 174: 605-614
  CrossrefPubMedGoogle Scholar
• 4 American Thoracic Society. Consensus Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med 2002; 165: 277-304
  CrossrefPubMedGoogle Scholar
• 5 Visscher DW, Myers JL. Histologic spectrum of idiopathic interstitial pneumonias. Proc Am Thorac Soc 2006; 3: 322-329
  CrossrefPubMedGoogle Scholar
• 6 Douglas WW, Ryu JH, Schroeder DR. Idiopathic pulmonary fibrosis: Impact of oxygen and colchicine, prednisone, or no therapy on survival. Am J Respir Crit Care Med 2000; 161: 1172-1178
  CrossrefPubMedGoogle Scholar
• 7 King Jr TE, Tooze JA, Schwarz MI et al. Predicting survival in idiopathic pulmonary fibrosis: scoring system and survival model. Am J Respir Crit Care Med 2001; 164: 1171-1181
  CrossrefPubMedGoogle Scholar
• 8 Gribbin J, Hubbard RB, Le Jeune I et al. Incidence and mortality of idiopathic pulmonary fibrosis and sarcoidosis in the UK. Thorax 2006; 61: 980-985
  CrossrefPubMedGoogle Scholar
• 9 Scott J, Johnston I, Britton J. What causes cryptogenic fibrosing alveolitis? A case-control study of environmental exposure to dust. BMJ 1990; 301: 1015-1017
  CrossrefPubMedGoogle Scholar
• 10 Mannino DM, Etzel RA, Parrish RG. Pulmonary fibrosis deaths in the United States, 1979–1991: an analysis of multiple-cause mortality data. Am J Respir Crit Care Med 1996; 153: 1548-1552
  CrossrefPubMedGoogle Scholar
• 11 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
  CrossrefPubMedGoogle Scholar
• 12 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
  CrossrefPubMedGoogle Scholar
• 13 Nadrous HF, Myers JL, Decker PA et al. Idiopathic pulmonary fibrosis in patients younger than 50 years. Mayo Clin Proc 2005; 80: 37-40
  CrossrefPubMedGoogle Scholar
• 14 Iwai K, Mori T, Yamada N et al. Idiopathic pulmonary fibrosis: epidemiologic approaches to occupational exposure. Am J Respir Crit Care Med 1994; 150: 670-675
  CrossrefPubMedGoogle Scholar
• 15 Coultas DB, Zumwalt RE, Black WC et al. The epidemiology of interstitial lung diseases. Am J Respir Crit Care Med 1994; 150: 967-972
  CrossrefPubMedGoogle Scholar
• 16 von Plessen C, Grinde O, Gulsvik A. Incidence and prevalence of cryptogenic fibrosing alveolitis in a Norwegian community. Respir Med 2003; 97: 428-435
  CrossrefPubMedGoogle Scholar
• 17 Karakatsani A, Papakosta D, Rapti A et al. Hellenic Interstitial Lung Diseases Group. Epidemiology of interstitial lung diseases in Greece. Respir Med 2009; 103: 1122-1129
  CrossrefPubMedGoogle Scholar
• 18 Hubbard R, Lewis S, Richards K et al. Occupational exposure to metal or wood dust and aetiology of cryptogenic fibrosing alveolitis. Lancet 1996; 347: 284-289
  CrossrefPubMedGoogle Scholar
• 19 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
  CrossrefPubMedGoogle Scholar
• 20 Enomoto T, Usuki J, Azuma A et al. Diabetes mellitus may increase risk for idiopathic pulmonary fibrosis. Chest 2003; 123: 2007-2011
  CrossrefPubMedGoogle Scholar
• 21 Steele MP, Speer MC, Loyd JE et al. Clinical and pathologic features of familial interstitial pneumonia. Am J Respir Crit Care Med 2005; 172: 1146-1152
  CrossrefPubMedGoogle Scholar
• 22 Miyake Y, Sasaki S, Yokoyama T et al. Occupational and environmental factors and idiopathic pulmonary fibrosis in Japan. Ann Occup Hyg 2005; 49: 259-265
  CrossrefPubMedGoogle Scholar
• 23 Taskar VS, Coultas DB. Is idiopathic pulmonary fibrosis an environmental disease?. Proc Am Thorac Soc 2006; 3: 293-298
  CrossrefPubMedGoogle Scholar
• 24 Johnston ID, Prescott RJ, Chalmers JC et al. British Thoracic Society study of cryptogenic fibrosing alveolitis: current presentation and initial management. Fibrosing Alveolitis Subcommittee of the Research Committee of the British Thoracic Society. Thorax 1997; 52: 38-44
  CrossrefPubMedGoogle Scholar
• 25 Hubbard R, Cooper M, Antoniak M et al. Risk of cryptogenic fibrosing alveolitis in metal workers. Lancet 2000; 355: 466-467
  CrossrefPubMedGoogle Scholar
• 26 Gustafson T, Dahlman-Hoglund A, Nilsson K et al. Occupational exposure and severe pulmonary fibrosis. Respir Med 2007; 101: 2207-2212
  CrossrefPubMedGoogle Scholar
• 27 Kitamura H, Ichinose S, Hosoya T et al. Inhalation of inorganic particles as a risk factor for idiopathic pulmonary fibrosis: elemental microanalysis of pulmonary lymph nodes obtained at autopsy cases. Pathol Res Pract 2007; 203: 575-585
  CrossrefPubMedGoogle Scholar
• 28 Ueda T, Ohta K, Suzuki N et al. Idiopathic pulmonary fibrosis and high prevalence of serum antibodies to hepatitis C virus. Am Rev Respir Dis 1992; 146: 266-268
  CrossrefPubMedGoogle Scholar
• 29 Irving WL, Day S, Johnston ID. Idiopathic pulmonary fibrosis and hepatitis C virus infection. Am Rev Respir Dis 1993; 148: 1683-1684
  CrossrefPubMedGoogle Scholar
• 30 Egan JJ, Stewart JP, Hasleton PS et al. Epstein-Barr virus replication within pulmonary epithelial cells in cryptogenic fibrosing alveolitis. Thorax 1995; 50: 1234-1239
  CrossrefPubMedGoogle Scholar
• 31 Meliconi R, Andreone P, Fasano L et al. Incidence of hepatitis C virus infection in Italian patients with idiopathic pulmonary fibrosis. Thorax 1996; 51: 315-317
  CrossrefPubMedGoogle Scholar
• 32 Kuwano K, Nomoto Y, Kunitake R et al. Detection of adenovirus E1A DNA in pulmonary fibrosis using nested polymerase chain reaction. Eur Respir J 1997; 10: 1445-1449
  CrossrefPubMedGoogle Scholar
• 33 Wangoo A, Shaw RJ, Diss TC et al. Cryptogenic fibrosing alveolitis: lack of association with Epstein-Barr virus infection. Thorax 1997; 52: 888-891
  CrossrefPubMedGoogle Scholar
• 34 Yamaguchi S, Kubo K, Fujimoto K et al. Analysis of bronchoalveolar lavage fluid in patients with chronic hepatitis C before and after treatment with interferon alpha. Thorax 1997; 52: 33-37
  CrossrefPubMedGoogle Scholar
• 35 Yonemaru M, Kasuga I, Kusumoto H et al. Elevation of antibodies to cytomegalovirus and other herpes viruses in pulmonary fibrosis. Eur Respir J 1997; 10: 2040-2045
  CrossrefPubMedGoogle Scholar
• 36 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
  CrossrefPubMedGoogle Scholar
• 37 Tsukamoto K, Hayakawa H, Sato A et al. Involvement of Epstein-Barr virus latent membrane protein 1 in disease progression in patients with idiopathic pulmonary fibrosis. Thorax 2000; 55: 958-961
  CrossrefPubMedGoogle Scholar
• 38 Lok SS, Stewart JP, Kelly BG et al. Epstein-Barr virus and wild p53 in idiopathic pulmonary fibrosis. Respir Med 2001; 95: 787-791
  CrossrefPubMedGoogle Scholar
• 39 Idilman R, Cetinkaya H, Savas I et al. Bronchoalveolar lavage fluid analysis in individuals with chronic hepatitis C. J Med Virol 2002; 66: 34-39
  CrossrefPubMedGoogle Scholar
• 40 Kelly BG, Lok SS, Hasleton PS et al. A rearranged form of Epstein-Barr virus DNA is associated with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2002; 166: 510-513
  CrossrefPubMedGoogle Scholar
• 41 Arase Y, Ikeda K, Tsubota A et al. Usefulness of serum KL-6 for early diagnosis of idiopathic pulmonary fibrosis in patients with hepatitis C virus. Hepatol Res 2003; 27: 89-94
  CrossrefPubMedGoogle Scholar
• 42 Tang YW, Johnson JE, Browning PJ et al. Herpesvirus DNA is consistently detected in lungs of patients with idiopathic pulmonary fibrosis. J Clin Microbiol 2003; 41: 2633-2640
  CrossrefPubMedGoogle Scholar
• 43 Procop GW, Kohn DJ, Johnson JE et al. BK and JC polyomaviruses are not associated with idiopathic pulmonary fibrosis. J Clin Microbiol 2005; 43: 1385-1386
  CrossrefPubMedGoogle Scholar
• 44 Zamo A, Poletti V, Reghellin D et al. HHV-8 and EBV are not commonly found in idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis 2005; 22: 123-128
  PubMedGoogle Scholar
• 45 Tobin RW, Pope II CE, Pellegrini CA et al. Increased prevalence of gastroesophageal reflux in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1998; 158: 1804-1808
  CrossrefPubMedGoogle Scholar
• 46 Patti MG, Tedesco P, Golden J et al. Idiopathic pulmonary fibrosis: how often is it really idiopathic?. J Gastrointest Surg 2005; 9: 1053-1056
  CrossrefPubMedGoogle Scholar
• 47 el-Serag HB, Sonnenberg A. Comorbid occurrence of laryngeal or pulmonary disease with esophagitis in United States military veterans. Gastroenterology 1997; 113: 755-760
  CrossrefPubMedGoogle Scholar
• 48 D’Ovidio F, Singer LG, Hadjiliadis D et al. Prevalence of American Thoracic Society Documents 817 gastroesophageal reflux in end-stage lung disease candidates for lung transplant. Ann Thorac Surg 2005; 80: 1254-1260
  CrossrefPubMedGoogle Scholar
• 49 Gribbin J, Hubbard R, Smith C. Role of diabetes mellitus and gastrooesophageal reflux in the aetiology of idiopathic pulmonary fibrosis. Respir Med 2009; 103: 927-931
  CrossrefPubMedGoogle Scholar
• 50 Bitterman PB, Rennard SI, Keogh BA et al. Familial idiopathic pulmonary fibrosis: evidence of lung inflammation in unaffected family members. N Engl J Med 1986; 314: 1343-1347
  CrossrefPubMedGoogle Scholar
• 51 Marshall RP, Puddicombe A, Cookson WO et al. Adult familial cryptogenic fibrosing alveolitis in the UK. Thorax 2000; 55: 143-146
  CrossrefPubMedGoogle Scholar
• 52 Hodgson U, Laitinen T, Tukiainen P. Nationwide prevalence of sporadic and familial idiopathic pulmonary fibrosis: evidence of founder effect among multiplex families in Finland. Thorax 2002; 57: 338-342
  CrossrefPubMedGoogle Scholar
• 53 Allam JS, Limper AH. Idiopathic pulmonary fibrosis: is it a familial disease?. Curr Opin Pulm Med 2006; 12: 312-317
  CrossrefPubMedGoogle Scholar
• 54 Lee H, Ryu JH, Wittmer MH et al. Familial idiopathic pulmonary fibrosis: clinical features and outcome. Chest 2005; 127: 2034-2041
  CrossrefPubMedGoogle Scholar
• 55 Raghu G, Hert R. Interstitial lung diseases: genetic predisposition and inherited interstitial lung diseases. Sem Respir Med 1993; 14: 323-332
  Article in Thieme ConnectPubMedGoogle Scholar
• 56 Mageto YN, Raghu G. Genetic predisposition of idiopathic pulmonary fibrosis. Curr Opin Pulm Med 1997; 3: 336-340
  CrossrefPubMedGoogle Scholar
• 57 Yang IV, Burch LH, Steele MP et al. Gene expression profiling of familial and sporadic interstitial pneumonia. Am J Respir Crit Care Med 2007; 175: 45-54
  CrossrefPubMedGoogle Scholar
• 58 Hodgson U, Pulkkinen V, Dixon M et al. ELMOD2 is a candidate gene for familial idiopathic pulmonary fibrosis. Am J Hum Genet 2006; 79: 149-154
  CrossrefPubMedGoogle Scholar
• 59 Rosas IO, Ren P, Avila NA et al. Early interstitial lung disease in familial pulmonary fibrosis. Am J Respir Crit Care Med 2007; 176: 698-705
  CrossrefPubMedGoogle Scholar
• 60 Musk AW, Zilko PJ, Manners P et al. Genetic studies in familial fibrosing alveolitis. Possible linkage with immunoglobulin allotypes (Gm). Chest 1986; 89: 206-210
  CrossrefPubMedGoogle Scholar
• 61 Thomas AQ, Lane K, Phillips III J et al. Heterozygosity for a surfactant protein C gene mutation associated with usual interstitial pneumonitis and cellular nonspecific interstitial pneumonitis in one kindred. Am J Respir Crit Care Med 2002; 165: 1322-1328
  CrossrefPubMedGoogle Scholar
• 62 Selman M, Lin H, Montano M et al. Surfactant protein A and B genetic variants predispose to idiopathic pulmonary fibrosis. Hum Genet 2003; 113: 542-550
  CrossrefPubMedGoogle Scholar
• 63 Lawson WE, Grant SW, Ambrosini V et al. Genetic mutations in surfactant protein C are a rare cause of sporadic cases of IPF. Thorax 2004; 59: 977-980
  CrossrefPubMedGoogle Scholar
• 64 Markart P, Ruppert C, Wygrecka M et al. Surfactant protein C mutations in sporadic forms of idiopathic interstitial pneumonias. Eur Respir J 2007; 29: 134-137
  PubMedGoogle Scholar
• 65 Wang Y, Kuan PJ, Xing C et al. Genetic defects in surfactant protein A2 are associated with pulmonary fibrosis and lung cancer. Am J Hum Genet 2009; 84: 52-59
  CrossrefPubMedGoogle Scholar
• 66 Armanios MY, Chen JJ, Cogan JD et al. Telomerase mutations in families with idiopathic pulmonary fibrosis. N Engl J Med 2007; 356: 1317-1326
  CrossrefPubMedGoogle Scholar
• 67 Tsakiri KD, Cronkhite JT, Kuan PJ et al. Adult-onset pulmonary fibrosis caused by mutations in telomerase. Proc Natl Acad Sci USA 2007; 104: 7552-7557
  CrossrefPubMedGoogle Scholar
• 68 Cronkhite JT, Xing C, Raghu G et al. Telomere shortening in familial and sporadic pulmonary fibrosis. Am J Respir Crit Care Med 2008; 178: 729-737
  CrossrefPubMedGoogle Scholar
• 69 Alder JK, Chen JJ, Lancaster L et al. Short telomeres are a risk factor for idiopathic pulmonary fibrosis. Proc Natl Acad Sci USA 2008; 105: 13051-13056
  CrossrefPubMedGoogle Scholar
• 70 Mushiroda T, Wattanapokayakit S, Takahashi A et al. A genome-wide association study identifies an association of a common variant in TERT with susceptibility to idiopathic pulmonary fibrosis. J Med Genet 2008; 45: 654-656
  CrossrefPubMedGoogle Scholar
• 71 Renzoni E, Lympany P, Sestini P et al. Distribution of novel polymorphisms of the interleukin-8 and CXC receptor 1 and 2 genes in systemic sclerosis and cryptogenic fibrosing alveolitis. Arthritis Rheum 2000; 43: 1633-1640
  CrossrefPubMedGoogle Scholar
• 72 Whyte M, Hubbard R, Meliconi R et al. Increased risk of fibrosing alveolitis associated with interleukin-1 receptor antagonist and tumor necrosis factor-alpha gene polymorphisms. Am J Respir Crit Care Med 2000; 162: 755-758
  CrossrefPubMedGoogle Scholar
• 73 Freeburn RW, Kendall H, Dobson L et al. The 39 untranslated region of tumor necrosis factor-alpha is highly conserved in idiopathic pulmonary fibrosis (IPF). Eur Cytokine Netw 2001; 12: 33-38
  PubMedGoogle Scholar
• 74 Pantelidis P, Fanning GC, Wells AU et al. Analysis of tumor necrosis factor-alpha, lymphotoxin-alpha, tumor necrosis factor receptor II, and interleukin-6 polymorphisms in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2001; 163: 1432-1436
  CrossrefPubMedGoogle Scholar
• 75 Hutyrova B, Pantelidis P, Drabek J et al. Interleukin-1 gene cluster polymorphisms in sarcoidosis and idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2002; 165: 148-151
  CrossrefPubMedGoogle Scholar
• 76 Latsi P, Pantelidis P, Vassilakis D et al. Analysis of IL-12 p40 subunit gene and IFN-gamma G5644A polymorphisms in Idiopathic Pulmonary Fibrosis. Respir Res 2003; 4: 6
  CrossrefPubMedGoogle Scholar
• 77 Whittington HA, Freeburn RW, Godinho SIH et al. Analysis of an IL-10 polymorphism in idiopathic pulmonary fibrosis. Genes Immun 2003; 4: 258-264
  CrossrefPubMedGoogle Scholar
• 78 Riha RL, Yang IA, Rabnott GC et al. Cytokine gene polymorphisms in idiopathic pulmonary fibrosis. Intern Med J 2004; 34: 126-129
  CrossrefPubMedGoogle Scholar
• 79 Vasakova M, Striz I, Slavcev A et al. Correlation of IL-1alpha and IL-4 gene polymorphisms and clinical parameters in idiopathic pulmonary fibrosis. Scand J Immunol 2007; 65: 265-270
  CrossrefPubMedGoogle Scholar
• 80 Vasakova M, Striz I, Slavcev A et al. Th1/Th2 cytokine gene polymorphisms in patients with idiopathic pulmonary fibrosis. Tissue Antigens 2006; 67: 229-232
  CrossrefPubMedGoogle Scholar
• 81 Geddes DM, Webley M, Brewerton DA et al. alpha 1-antitrypsin phenotypes in fibrosing alveolitis and rheumatoid arthritis. Lancet 1977; 2: 1049-1051
  PubMedGoogle Scholar
• 82 Hubbard R, Baoku Y, Kalsheker N et al. Alpha1-antitrypsin phenotypes in patients with cryptogenic fibrosing alveolitis: a case-control study. Eur Respir J 1997; 10: 2881-2883
  CrossrefPubMedGoogle Scholar
• 83 Morrison CD, Papp AC, Hejmanowski AQ et al. Increased D allele frequency of the angiotensin-converting enzyme gene in pulmonary fibrosis. Hum Pathol 2001; 32: 521-528
  CrossrefPubMedGoogle Scholar
• 84 Xaubet A, Marin-Arguedas A, Lario S et al. Transforming growth factor-beta1 gene polymorphisms are associated with disease progression in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2003; 168: 431-435
  CrossrefPubMedGoogle Scholar
• 85 Zorzetto M, Ferrarotti I, Campo I et al. NOD2/CARD15 gene polymorphisms in idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis 2005; 22: 180-185
  PubMedGoogle Scholar
• 86 Checa M, Ruiz V, Montano M et al. MMP-1 polymorphisms and the risk of idiopathic pulmonary fibrosis. Hum Genet 2008; 124: 465-472
  CrossrefPubMedGoogle Scholar
• 87 Falfan-Valencia R, Camarena A, Juarez A et al. Major histocompatibility complex and alveolar epithelial apoptosis in idiopathic pulmonary fibrosis. Hum Genet 2005; 118: 235-244
  CrossrefPubMedGoogle Scholar
• 88 Lederer DJ, Arcasoy SM, Barr RG et al. Racial and ethnic disparities in idiopathic pulmonary fibrosis: A UNOS/OPTN database analysis. Am J Transplant 2006; 6: 2436-2442
  CrossrefPubMedGoogle Scholar
• 89 Zuo F, Kaminski N, Eugui E et al. Gene expression analysis reveals matrilysin as a key regulator of pulmonary fibrosis in mice and humans. Proc Natl Acad Sci USA 2002; 99: 6292-6297
  CrossrefPubMedGoogle Scholar
• 90 Seibold MA, Wise AL, Speer MC et al. A common MUC5B promoter polymorphism and pulmonary fibrosis. N Engl J Med 2011; 364: 1503-1512
  CrossrefPubMedGoogle Scholar
• 91 Zhang Y, Noth I, Garcia JG et al. A variant in the promoter of MUC5B and idiopathic pulmonary fibrosis. N Engl J Med 2011; 364: 1576-1577
  CrossrefPubMedGoogle Scholar
• 92 Armanios M. Telomerase and idiopathic pulmonary fibrosis. Mutat Res 2012; 730: 52-58
  CrossrefPubMedGoogle Scholar
• 93 Lawson WE, Loyd JE, Degryse AL. Genetics in pulmonary fibrosis -- familial cases provide clues to the pathogenesis of idiopathic pulmonary fibrosis. Am J Med Sci 2011; 341: 439-443
  CrossrefPubMedGoogle Scholar
• 94 Diaz de Leon A, Cronkhite J, Katzenstein A et al. Telomere Lengths, Pulmonary Fibrosis and Telomerase (TERT) Mutations. PLOS One 2010; 5 e 10680
  CrossrefPubMedGoogle Scholar
• 95 van Moorsel C, van Oosterhout M, Barlo N et al. Surfactant Protein C Mutations Are the Basis of a Significant Portion of Adult Familial Pulmonary Fibrosis in a Dutch Cohort. Am J Respir Crit Care Med 2011; 182: 1419-1425
  PubMedGoogle Scholar
• 96 Nishimura K, Kitaichi M, Izumi T et al. Usual interstitial pneumonia: histologic correlation with high-resolution CT. Radiology 1992; 182: 337-342
  CrossrefPubMedGoogle Scholar
• 97 Johkoh T, Muller NL, Cartier Y et al. Idiopathic interstitial pneumonias: diagnostic accuracy of thin-section CT in 129 patients. Radiology 1999; 211: 555-560
  CrossrefPubMedGoogle Scholar
• 98 Hansell DM, Bankier AA, Macmahon H et al. Fleischner Society: Glossary of terms for thoracic imaging. Radiology 2008; 246: 697-722
  CrossrefPubMedGoogle Scholar
• 99 Hwang JH, Misumi S, Sahin H et al. Computed tomographic features of idiopathic fibrosing interstitial pneumonia: comparison with pulmonary fibrosis related to collagen vascular disease. J Comput Assist Tomogr 2009; 33: 410-415
  CrossrefPubMedGoogle Scholar
• 100 Souza CA, Muller NL, Lee KS et al. Idiopathic interstitial pneumonias: prevalence of mediastinal lymph node enlargement in 206 patients. AJR Am J Roentgenol 2006; 186: 995-999
  CrossrefPubMedGoogle Scholar
• 101 Mathieson JR, Mayo JR, Staples CA et al. Chronic diffuse infiltrative lung disease: comparison of diagnostic accuracy of CT and chest radiography. Radiology 1989; 171: 111-116
  CrossrefPubMedGoogle Scholar
• 102 Hunninghake GW, Zimmerman MB, Schwartz DA et al. Utility of a lung biopsy for the diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2001; 164: 193-196
  CrossrefPubMedGoogle Scholar
• 103 Raghu G, Mageto YN, Lockhart D et al. The accuracy of the clinical diagnosis of new-onset idiopathic pulmonary fibrosis and other interstitial lung disease: A prospective study. Chest 1999; 116: 1168-1174
  CrossrefPubMedGoogle Scholar
• 104 Grenier P, Valeyre D, Cluzel P et al. Chronic diffuse interstitial lung disease: diagnostic value of chest radiography and high-resolution CT. Radiology 1991; 179: 123-132
  PubMedGoogle Scholar
• 105 Lee KS, Primack SL, Staples CA et al. Chronic infiltrative lung disease: comparison of diagnostic accuracies of radiography and low- and conventional-dose thin-section CT. Radiology 1994; 191: 669-673
  PubMedGoogle Scholar
• 106 Swensen SJ, Aughenbaugh GL, Myers JL. Diffuse lung disease: diagnostic accuracy of CT in patients undergoing surgical biopsy of the lung. Radiology 1997; 205: 229-234
  CrossrefPubMedGoogle Scholar
• 107 Flaherty KR, Thwaite EL, Kazerooni EA et al. Radiological versus histological diagnosis in UIP and NSIP: survival implications. Thorax 2003; 58: 143-148
  CrossrefPubMedGoogle Scholar
• 108 Quadrelli S, Molinari L, Ciallella L et al. Radiological versus histopathological diagnosis of usual interstitial pneumonia in the clinical practice: does it have any survival difference?. Respiration 2010; 79: 32-37
  CrossrefPubMedGoogle Scholar
• 109 Flaherty KR, King Jr TE, Raghu G et al. Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis?. Am J Respir Crit Care Med 2004; 170: 904-910
  CrossrefPubMedGoogle Scholar
• 110 Lynch III JP, Saggar R, Weigt SS et al. Usual interstitial pneumonia. Semin Respir Crit Care Med 2006; 27: 634-651
  Article in Thieme ConnectPubMedGoogle Scholar
• 111 Trahan S, Hanak V, Ryu JH et al. Role of surgical lung biopsy in separating chronic hypersensitivity pneumonia from usual interstitial pneumonia/idiopathic pulmonary fibrosis: analysis of 31 biopsies from 15 patients. Chest 2008; 134: 126-132
  CrossrefPubMedGoogle Scholar
• 112 Silva CI, Muller NL, Lynch DA et al. Chronic hypersensitivity pneumonitis: differentiation from idiopathic pulmonary fibrosis and non-specific interstitial pneumonia by using thin-section CT. Radiology 2008; 246: 288-297
  CrossrefPubMedGoogle Scholar
• 113 Monaghan H, Wells AU, Colby TV et al. Prognostic implications of histologic patterns in multiple surgical lung biopsies from patients with idiopathic interstitial pneumonias. Chest 2004; 125: 522-526
  CrossrefPubMedGoogle Scholar
• 114 Flaherty KR, Travis WD, Colby TV et al. Histopathologic variability in usual and nonspecific interstitial pneumonias. Am J Respir Crit Care Med 2001; 164: 1722-1727
  CrossrefPubMedGoogle Scholar
• 115 Bensard DD, McIntyre Jr RC, Waring BJ et al. Comparison of video thoracoscopic lung biopsy to open lung biopsy in the diagnosis of interstitial lung disease. Chest 1993; 103: 765-770
  CrossrefPubMedGoogle Scholar
• 116 Miller JD, Urschel JD, Cox G et al. A randomized, controlled trial comparing thoracoscopy and limited thoracotomy for lung biopsy in interstitial lung disease. Ann Thorac Surg 2000; 70: 1647-1650
  CrossrefPubMedGoogle Scholar
• 117 Carnochan FM, Walker WS, Cameron EW. Efficacy of video assisted thoracoscopic lung biopsy: an historical comparison with open lung biopsy. Thorax 1994; 49: 361-363
  CrossrefPubMedGoogle Scholar
• 118 Ferson PF, Landreneau RJ, Dowling RD et al. Comparison of open versus thoracoscopic lung biopsy for diffuse infiltrative pulmonary disease. J Thorac Cardiovasc Surg 1993; 106: 194-199
  PubMedGoogle Scholar
• 119 Vourlekis JS, Schwarz MI, Cherniack RM et al. The effect of pulmonary fibrosis on survival in patients with hypersensitivity pneumonitis. Am J Med 2004; 116: 662-668
  CrossrefPubMedGoogle Scholar
• 120 Ohshimo S, Bonella F, Cui A et al. Significance of bronchoalveolar lavage for the diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2009; 179: 1043-1047
  CrossrefPubMedGoogle Scholar
• 121 Berbescu EA, Katzenstein AA, Snow JL et al. Transbronchial biopsy in usual interstitial pneumonia. Chest 2006; 129: 1126-1131
  CrossrefPubMedGoogle Scholar
• 122 Park JH, Kim DS, Park IN et al. Prognosis of fibrotic interstitial pneumonia: idiopathic versus collagen vascular disease-related subtypes. Am J Respir Crit Care Med 2007; 175: 705-711
  CrossrefPubMedGoogle Scholar
• 123 Wasicek CA, Reichlin M, Montes M et al. Polymyositis and interstitial lung disease in a patient with anti-Jo1 prototype. Am J Med 1984; 76: 538-544
  CrossrefPubMedGoogle Scholar
• 124 Flaherty KR, Andrei A, King Jr TE et al. Idiopathic interstitial pneumonia: do community and academic physicians agree on diagnosis?. Am J Respir Crit Care Med 2007; 175: 1054-1060
  CrossrefPubMedGoogle Scholar
• 125 Carrington CB, Gaensler EA, Coutu RE et al. Natural history and treated course of usual and desquamative interstitial pneumonia. N Engl J Med 1978; 298: 801-809
  CrossrefPubMedGoogle Scholar
• 126 Tukiainen P, Taskinen E, Holsti P et al. Prognosis of cryptogenic fibrosing alveolitis. Thorax 1983; 38: 349-355
  CrossrefPubMedGoogle Scholar
• 127 Gross TJ, Hunninghake GW. Idiopathic pulmonary fibrosis. N Engl J Med 2001; 345: 517-525
  CrossrefPubMedGoogle Scholar
• 128 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
  CrossrefPubMedGoogle Scholar
• 129 Bjoraker JA, Ryu JH, Edwin MK et al. Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1998; 157: 199-203
  CrossrefPubMedGoogle Scholar
• 130 Flaherty KR, Toews GB, Travis WD et al. Clinical significance of histological classification of idiopathic interstitial pneumonia. Eur Respir J 2002; 19: 275-283
  CrossrefPubMedGoogle Scholar
• 131 Nicholson AG, Colby TV, du Bois RM et al. The prognostic significance of the histologic pattern of interstitial pneumonia in patients presenting with the clinical entity of cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med 2000; 162: 2213-2217
  CrossrefPubMedGoogle Scholar
• 132 Rudd RM, Prescott RJ, Chalmers JC et al. British Thoracic Society Study on cryptogenic fibrosing alveolitis: Response to treatment and survival. Thorax 2007; 62: 62-66
  CrossrefPubMedGoogle Scholar
• 133 King Jr TE, Schwarz MI, Brown K et al. Idiopathic pulmonary fibrosis: relationship between histopathologic features and mortality. Am J Respir Crit Care Med 2001; 164: 1025-1032
  CrossrefPubMedGoogle Scholar
• 134 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
  CrossrefPubMedGoogle Scholar
• 135 King Jr TE, Albera C, Bradford WZ et al. INSPIRE Study Group. Effect of interferon gamma-1b on survival in patients with idiopathic pulmonary fibrosis (INSPIRE): a multicentre, randomised, placebo-controlled trial. Lancet 2009; 374: 222-228
  CrossrefPubMedGoogle Scholar
• 136 King Jr TE, 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 2008; 177: 75-81
  CrossrefPubMedGoogle Scholar
• 137 Raghu G. Idiopathic pulmonary fibrosis: a rational clinical approach. Chest 1987; 92: 148-154
  CrossrefPubMedGoogle Scholar
• 138 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
  CrossrefPubMedGoogle Scholar
• 139 Mejia M, Carrillo G, Rojas-Serrano J et al. Idiopathic pulmonary fibrosis and emphysema: decreased survival associated with severe pulmonary arterial hypertension. Chest 2009; 136: 10-15
  CrossrefPubMedGoogle Scholar
• 140 Wells AU, Desai SR, Rubens MB et al. Idiopathic pulmonary fibrosis: a composite physiologic index derived from disease extent observed by computed tomography. Am J Respir Crit Care Med 2003; 167: 962-969
  CrossrefPubMedGoogle Scholar
• 141 Lettieri CJ, Nathan SD, Barnett SD et al. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest 2006; 129: 746-752
  CrossrefPubMedGoogle Scholar
• 142 Collard HR, Moore BB, Flaherty KR et al. Idiopathic Pulmonary Fibrosis Clinical Research Network Investigators. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2007; 176: 636-643
  CrossrefPubMedGoogle Scholar
• 143 Azuma A, Nukiwa T, Tsuboi E et al. Double-blind, placebo-controlled trial of pirfenidone in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2005; 171: 1040-1047
  CrossrefPubMedGoogle Scholar
• 144 Song JW, Hong S-B, Koh Y et al. Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome. Eur Respir J 2011; 37: 356-363
  CrossrefPubMedGoogle Scholar
• 145 Martinez FJ, Safrin S, Weycker D et al. IPF Study Group. The clinical course of patients with idiopathic pulmonary fibrosis. Ann Intern Med 2005; 142: 963-967
  CrossrefPubMedGoogle Scholar
• 146 Panos RJ, Mortenson RL, Niccoli SA et al. Clinical deterioration in patients with idiopathic pulmonary fibrosis: causes and assessment. Am J Med 1990; 88: 396-404
  CrossrefPubMedGoogle Scholar
• 147 Akira M, Hamada H, Sakatani M et al. CT findings during phase of accelerated deterioration in patients with idiopathic pulmonary fibrosis. AJR Am J Roentgenol 1997; 168: 79-83
  CrossrefPubMedGoogle Scholar
• 148 Ambrosini V, Cancellieri A, Chilosi M et al. Acute exacerbation of idiopathic pulmonary fibrosis: report of a series. Eur Respir J 2003; 22: 821-826
  CrossrefPubMedGoogle Scholar
• 149 Kim DS, Park JH, Park BK et al. Acute exacerbation of idiopathic pulmonary fibrosis: frequency and clinical features. Eur Respir J 2006; 27: 143-150
  CrossrefPubMedGoogle Scholar
• 150 Kondoh Y, Taniguchi H, Kawabata Y et al. Acute exacerbation in idiopathic pulmonary fibrosis: analysis of clinical and pathologic findings in three cases. Chest 1993; 103: 1808-1812
  CrossrefPubMedGoogle Scholar
• 151 Kondoh Y, Taniguchi H, Yokoi T et al. Cyclophosphamide and low-dose prednisolone in idiopathic pulmonary fibrosis and fibrosing nonspecific interstitial pneumonia. Eur Respir J 2005; 25: 528-533
  CrossrefPubMedGoogle Scholar
• 152 Kubo H, Nakayama K, Yanai M et al. Anticoagulant therapy for idiopathic pulmonary fibrosis. Chest 2005; 128: 1475-1482
  CrossrefPubMedGoogle Scholar
• 153 Parambil JG, Myers JL, Ryu JH. Histopathologic features and outcome of patients with acute exacerbation of idiopathic pulmonary fibrosis undergoing surgical lung biopsy. Chest 2005; 128: 3310-3315
  CrossrefPubMedGoogle Scholar
• 154 Tiitto L, Bloigu R, Heiskanen U et al. Relationship between histopathological features and the course of idiopathic pulmonary fibrosis/usual interstitial pneumonia. Thorax 2006; 61: 1091-1095
  CrossrefPubMedGoogle Scholar
• 155 Rice AJ, Wells AU, Bouros D et al. Terminal diffuse alveolar damage in relation to interstitial pneumonias: an autopsy study. Am J Clin Pathol 2003; 119: 709-714
  CrossrefPubMedGoogle Scholar
• 156 Churg A, Muller NL, Silva CIS et al. Acute exacerbation (acute lung injury of unknown cause) in UIP and other forms of fibrotic interstitial pneumonias. Am J Surg Pathol 2007; 31: 277-284
  CrossrefPubMedGoogle Scholar
• 157 Kondo A, Saiki S. Acute exacerbation in idiopathic interstitial pneumonia (IIP). In: Harasawa M, Fukuchi Y, Morinari H, eds. Interstitial pneumonia of unknown etiology. Tokyo: University of Tokyo Press; 1989: 33-42
  Google Scholar
• 158 Konishi K, Gibson KF, Lindell KO et al. Gene expression profiles of acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2009; 180: 167-175
  CrossrefPubMedGoogle Scholar
• 159 Wootton SC, Kim DS, Kondoh Y et al. Viral infection in acute exacerbation of Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med 2011; 183: 1698-1702
  CrossrefPubMedGoogle Scholar
• 160 Sakamoto K, Taniguchi H, Kondoh Y et al. Acute exacerbation of idiopathic pulmonary fibrosis as the initial presentation of the disease. Eur Respir Rev 2009; 18: 129-132
  CrossrefPubMedGoogle Scholar
• 161 Kondoh Y, Taniguchi H, Kataoka K et al. Prognostic factors in rapidly progressive interstitial pneumonia. Respirology 2010; 15: 257-264
  CrossrefPubMedGoogle Scholar
• 162 Kumar P, Goldstraw P, Yamada K et al. Pulmonary fibrosis and lung cancer: risk and benefit analysis of pulmonary resection. J Thorac Cardiovasc Surg 2003; 125: 1321-1327
  CrossrefPubMedGoogle Scholar
• 163 Yuksel M, Ozyurtkan MO, Bostanci K et al. Acute exacerbation of interstitial fibrosis after pulmonary resection. Ann Thorac Surg 2006; 82: 336-338
  CrossrefPubMedGoogle Scholar
• 164 Utz JP, Ryu JH, Douglas WW et al. High short-term mortality following lung biopsy for usual interstitial pneumonia. Eur Respir J 2001; 17: 175-179
  CrossrefPubMedGoogle Scholar
• 165 Zegdi R, Azorin J, Tremblay B et al. Videothoracoscopic lung biopsy in diffuse infiltrative lung diseases: a 5-year surgical experience. Ann Thorac Surg 1998; 66: 1170-1173
  CrossrefPubMedGoogle Scholar
• 166 Kondoh Y, Taniguchi H, Kitaichi M et al. Acute exacerbation of interstitial pneumonia following surgical lung biopsy. Respir Med 2006; 100: 1753-1759
  CrossrefPubMedGoogle Scholar
• 167 Hiwatari N, Shimura S, Takishima T et al. Bronchoalveolar lavage as a possible cause of acute exacerbation in idiopathic pulmonary fibrosis patients. Tohoku J Exp Med 1994; 174: 379-386
  CrossrefPubMedGoogle Scholar
• 168 Olson AL, Swigris JJ, Lezotte DC et al. Mortality from pulmonary fibrosis increased in the United States from 1992 to 2003. Am J Respir Crit Care Med 2007; 176: 277-284
  CrossrefPubMedGoogle Scholar
• 169 Navaratnam V, Fleming KM, West J et al. The rising incidence of idiopathic pulmonary fibrosis in the UK. Thorax 2011; 66: 462-467
  CrossrefPubMedGoogle Scholar
• 170 Jemal A, Siegel R, Ward E et al. Cancer statistics, 2007. CA Cancer J Clin 2007; 57: 43-66
  CrossrefPubMedGoogle Scholar
• 171 Olson AL, Swigris JJ, Raghu G et al. Seasonal variation: mortality from pulmonary fibrosis is greatest in the winter. Chest 2009; 136: 16-22
  CrossrefPubMedGoogle Scholar
• 172 Fernandez Perez ER, Daniels CE, Schroeder DR et al. Incidence, prevalence and clinical course of Idiopathic Pulmonary Fibrosis: a population-based study. Chest 2010; 137: 129-37
  CrossrefPubMedGoogle Scholar
• 173 Hubbard RB, Smith C, Le Jeune I et al. The association between idiopathic pulmonary fibrosis and vascular disease: a population-based study. Am J Respir Crit Care Med 2008; 178: 1257-1261
  CrossrefPubMedGoogle Scholar
• 174 Nadrous HF, Pellikka PA, Krowka MJ et al. Pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Chest 2005; 128: 2393-2399
  CrossrefPubMedGoogle Scholar
• 175 Collard HR, Ryu JH, Douglas WW et al. Combined corticosteroid and cyclophosphamide therapy does not alter survival in idiopathic pulmonary fibrosis. Chest 2004; 125: 2169-2174
  CrossrefPubMedGoogle Scholar
• 176 Hubbard R, Venn A, Smith C et al. Exposure to commonly prescribed drugs and the etiology of cryptogenic fibrosing alveolitis: a case-control study. Am J Respir Crit Care Med 1998; 157: 743-747
  CrossrefPubMedGoogle Scholar
• 177 Schwartz DA, Van Fossen DS, Davis CS et al. Determinants of progression in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1994; 149: 444-449
  CrossrefPubMedGoogle Scholar
• 178 Enomoto N, Suda T, Kato M et al. Quantitative analysis of fibroblastic foci in usual interstitial pneumonia. Chest 2006; 130: 22-29
  CrossrefPubMedGoogle Scholar
• 179 Nadrous HF, Pellikka PA, Krowka MJ et al. The impact of pulmonary hypertension on survival in patients with idiopathic pulmonary fibrosis. Chest 2005; 128: 616-617
  CrossrefPubMedGoogle Scholar
• 180 Jegal Y, Kim DS, Shim TS et al. Physiology is a stronger predictor of survival than pathology in fibrotic interstitial pneumonia. Am J Respir Crit Care Med 2005; 171: 639-644
  CrossrefPubMedGoogle Scholar
• 181 Wells AU, Hogaboam CM. Update in diffuse parenchymal lung disease 2007. Am J Respir Crit Care Med 2008; 177: 580-584
  CrossrefPubMedGoogle Scholar
• 182 du Bois RM, Weycker D, Albera C et al. Ascertainment of individual risk of mortality for patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011; 184: 459-466
  CrossrefPubMedGoogle Scholar
• 183 Turner-Warwick M, Burrows B, Johnson A. Cryptogenic fibrosing alveolitis: response to corticosteroid treatment and its effect on survival. Thorax 1980; 35: 593-599
  CrossrefPubMedGoogle Scholar
• 184 Schwartz DA, Helmers RA, Galvin JR et al. Determinants of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1994; 149: 450-454
  CrossrefPubMedGoogle Scholar
• 185 Nishiyama O, Taniguchi H, Kondoh Y et al. Health-related quality of life in patients with idiopathic pulmonary fibrosis: what is the main contributing factor?. Respir Med 2005; 99: 408-414
  CrossrefPubMedGoogle Scholar
• 186 Nishiyama O, Taniguchi H, Kondoh Y et al. A simple assessment of dyspnea as a prognostic indicator in idiopathic pulmonary fibrosis. Eur Respir J 2010; 36: 1067-1072
  CrossrefPubMedGoogle Scholar
• 187 Watters LC, King Jr TE, Schwarz MI et al. A clinical, radiographic, and physiologic scoring system for the longitudinal assessment of patients with idiopathic pulmonary fibrosis. Am Rev Respir Dis 1986; 133: 97-103
  CrossrefPubMedGoogle Scholar
• 188 Witek Jr TJ, Mahler DA. Minimal important difference of the transition dyspnoea index in a multinational clinical trial. Eur Respir J 2003; 21: 267-272
  CrossrefPubMedGoogle Scholar
• 189 Kupferberg DH, Kaplan RM, Slymen DJ et al. Minimal clinically important difference for the UCSD Shortness of Breath Questionnaire. J Cardiopulm Rehabil 2005; 25: 370-377
  CrossrefPubMedGoogle Scholar
• 190 Collard HR, King Jr TE, Bartelson BB et al. Changes in clinical and physiologic variables predict survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2003; 168: 538-542
  CrossrefPubMedGoogle Scholar
• 191 King Jr TE, Safrin S, Starko KM et al. Analyses of efficacy end points in a controlled trial of interferon-gamma1b for idiopathic pulmonary fibrosis. Chest 2005; 127: 171-177
  CrossrefPubMedGoogle Scholar
• 192 Hallstrand TS, Boitano LJ, Johnson WC et al. The timed walk test as a measure of severity and survival in idiopathic pulmonary fibrosis. Eur Respir J 2005; 25: 96-103
  CrossrefPubMedGoogle Scholar
• 193 Hamada K, Nagai S, Tanaka S et al. Significance of pulmonary arterial pressure and diffusion capacity of the lung as prognosticator in patients with idiopathic pulmonary fibrosis. Chest 2007; 131: 650-656
  CrossrefPubMedGoogle Scholar
• 194 Egan JJ, Martinez FJ, Wells AU et al. Lung function estimates in idiopathic pulmonary fibrosis: the potential for a simple classification. Thorax 2005; 60: 270-273
  CrossrefPubMedGoogle Scholar
• 195 Latsi PI, du Bois RM, Nicholson AG et al. Fibrotic idiopathic interstitial pneumonia: the prognostic value of longitudinal functional trends. Am J Respir Crit Care Med 2003; 168: 531-537
  CrossrefPubMedGoogle Scholar
• 196 Fell CD, Liu LX, Motika C et al. The prognostic value of cardiopulmonary exercise testing in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2009; 179: 402-407
  CrossrefPubMedGoogle Scholar
• 197 Flaherty KR, Mumford JA, Murray S et al. Prognostic implications of physiologic and radiographic changes in idiopathic interstitial pneumonia. Am J Respir Crit Care Med 2003; 168: 543-548
  CrossrefPubMedGoogle Scholar
• 198 du Bois RM, Weycker D, Albera C et al. Forced vital capacity in patients with idiopathic pulmonary fibrosis: test properties and minimal clinically important difference. Am J Respir Crit Care Med 2011; 184: 1382-1389
  CrossrefPubMedGoogle Scholar
• 199 Lynch DA, Godwin JD, Safrin S et al. Idiopathic Pulmonary Fibrosis Study Group. High-resolution computed tomography in idiopathic pulmonary fibrosis: diagnosis and prognosis. Am J Respir Crit Care Med 2005; 172: 488-493
  CrossrefPubMedGoogle Scholar
• 200 Best AC, Meng J, Lynch AM et al. Idiopathic pulmonary fibrosis: physiologic tests, quantitative CT indexes, and CT visual scores as predictors of mortality. Radiology 2008; 246: 935-940
  CrossrefPubMedGoogle Scholar
• 201 Jeong YJ, Lee KS, Muller NL et al. Usual interstitial pneumonia and non-specific interstitial pneumonia: serial thin-section CT findings correlated with pulmonary function. Korean J Radiol 2005; 6: 143-152
  CrossrefPubMedGoogle Scholar
• 202 Shin KM, Lee KS, Chung MP et al. Prognostic determinants among clinical, thin-section CT, and histopathologic findings for fibrotic idiopathic interstitial pneumonias: tertiary hospital study. Radiology 2008; 249: 328-337
  CrossrefPubMedGoogle Scholar
• 203 Sumikawa H, Johkoh T, Colby TV et al. Computed tomography findings in pathological usual interstitial pneumonia: relationship to survival. Am J Respir Crit Care Med 2008; 177: 433-439
  CrossrefPubMedGoogle Scholar
• 204 Lama VN, Flaherty KR, Toews GB et al. Prognostic value of desaturation during a 6-minute walk test in idiopathic interstitial pneumonia. Am J Respir Crit Care Med 2003; 168: 1084-1090
  CrossrefPubMedGoogle Scholar
• 205 Enright PL. The six-minute walk test. Respir Care 2003; 48: 783-785
  PubMedGoogle Scholar
• 206 Lederer DJ, Arcasoy SM, Wilt JS et al. Six-minute-walk distance predicts waiting list survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2006; 174: 659-664
  CrossrefPubMedGoogle Scholar
• 207 Swigris JJ, Swick J, Wamboldt FS et al. Heart rate recovery after 6-min walk test predicts survival in patients with idiopathic pulmonary fibrosis. Chest 2009; 136: 841-848
  CrossrefPubMedGoogle Scholar
• 208 Caminati A, Bianchi A, Cassandro R et al. Walking distance on 6-MWT is a prognostic factor in idiopathic pulmonary fibrosis. Respir Med 2009; 103: 117-123
  CrossrefPubMedGoogle Scholar
• 209 Eaton T, Young P, Milne D et al. Six-minute walk, maximal exercise tests: reproducibility in fibrotic interstitial pneumonia. Am J Respir Crit Care Med 2005; 171: 1150-1157
  CrossrefPubMedGoogle Scholar
• 210 du Bois RM, Weycker D, Albera C et al. Six-minute-walk test in idiopathic pulmonary fibrosis: test validation and minimal clinically important difference. Am J Respir Crit Care Med 2011; 183: 1231-1237
  CrossrefPubMedGoogle Scholar
• 211 Nicholson AG, Fulford LG, Colby TV et al. The relationship between individual histologic features and disease progression in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2002; 166: 173-177
  CrossrefPubMedGoogle Scholar
• 212 Flaherty KR, Colby TV, Toews GB et al. Differential presence of fibroblastic foci in UIP patients with or without conntective tissue disease. Am J Respir Crit Care Med 2001; 163: A983
  CrossrefPubMedGoogle Scholar
• 213 Hanak V, Ryu JH, de Carvalho E et al. Profusion of fibroblast foci in patients with idiopathic pulmonary fibrosis does not predict outcome. Respir Med 2008; 102: 852-856
  CrossrefPubMedGoogle Scholar
• 214 Arcasoy SM, Christie JD, Ferrari VA et al. Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease. Am J Respir Crit Care Med 2003; 167: 735-740
  CrossrefPubMedGoogle Scholar
• 215 Nathan SD, Shlobin OA, Barnett SD et al. Right ventricular systolic pressure by echocardiography as a predictor of pulmonary hypertension in idiopathic pulmonary fibrosis. Respir Med 2008; 102: 1305-1310
  CrossrefPubMedGoogle Scholar
• 216 Fisher MR, Forfia PR, Chamera E et al. Accuracy of Doppler echocardiography in the hemodynamic assessment of pulmonary hypertension. Am J Respir Crit Care Med 2009; 179: 615-621
  CrossrefPubMedGoogle Scholar
• 217 Modrykamien AM, Gudavalli R, McCarthy K et al. Echocardiography, 6-minute walk distance, and distance saturation product as predictors of pulmonary arterial hypertension in Idiopathic Pulmonary Fibrosis. Respir Care 2010; 55: 584-88
  PubMedGoogle Scholar
• 218 Corte TJ, Wort SJ, Gatzoulis MA et al. Pulmonary vascular resistance predicts early mortality in patients with diffuse fibrotic lung disease and suspected pulmonary hypertension. Thorax 2009; 64: 883-888
  CrossrefPubMedGoogle Scholar
• 219 Hoeper MM, Andreas S, Bastian A et al. Pulmonary hypertension due to chronic lung disease: updated recommendations of the Cologne Consensus Conference 2011. Int J Cardiol 2011; 152S: 45-53
  PubMedGoogle Scholar
• 220 Cottin V, Nunes H, Brillet PY et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J 2005; 26: 586-593
  CrossrefPubMedGoogle Scholar
• 221 Nathan SD, Basavaraj A, Reichner C et al. Prevalence and impact of coronary artery disease in idiopathic pulmonary fibrosis. Respir Med 2010; 104: 1035-1040
  CrossrefPubMedGoogle Scholar
• 222 Lee JS, Collard HR, Raghu G et al. Does chronic microaspiration cause idiopathic pulmonary fibrosis. Am J Med 2010; 123: 304-311
  CrossrefPubMedGoogle Scholar
• 223 Bouros D, Hatzakis K, Labrakis H et al. Association of malignancy with diseases causing interstitial pulmonary changes. Chest 2002; 212: 1278-1289
  PubMedGoogle Scholar
• 224 Mermigkis C, Stagaki E, Amfilochiou A et al. Sleep quality and associated daytime consequences in patients with idiopathic pulmonary fibrosis. Med Princ Pract 2009; 18: 10-15
  CrossrefPubMedGoogle Scholar
• 225 Stahel RA, Gilks WR, Lehmann HP et al. Third International Workshop on Lung Tumor and Differentiation Antigens: overview of the results of the central data analysis. Int J Cancer Suppl 1994; 8: 6-26
  PubMedGoogle Scholar
• 226 Kohno N, Kyoizumi S, Awaya Y et al. New serum indicator of interstitial pneumonitis activity: sialylated carbohydrate antigen KL-6. Chest 1989; 96: 68-73
  CrossrefPubMedGoogle Scholar
• 227 Yokoyama A, Kondo K, Nakajima M et al. Prognostic value of circulating KL-6 in idiopathic pulmonary fibrosis. Respirology 2006; 11: 164-168
  CrossrefPubMedGoogle Scholar
• 228 Greene KE, King Jr TE, Kuroki Y et al. Serum surfactant proteins-A and -D as biomarkers in idiopathic pulmonary fibrosis. Eur Respir J 2002; 19: 439-446
  CrossrefPubMedGoogle Scholar
• 229 Kinder BW, Brown KK, McCormack FX et al. Serum surfactant protein-A is a strong predictor of early mortality in idiopathic pulmonary fibrosis. Chest 2009; 135: 1557-1563
  CrossrefPubMedGoogle Scholar
• 230 Takahashi H, Fujishima T, Koba H et al. Serum surfactant proteins A and D as prognostic factors in idiopathic pulmonary fibrosis and American Thoracic Society Documents 821 their relationship to disease extent. Am J Respir Crit Care Med 2000; 162: 1109-1114
  CrossrefPubMedGoogle Scholar
• 231 Prasse A, Probst C, Bargagli E et al. Serum CC-chemokine ligand 18 concentration predicts outcome in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2009; 179: 717-723
  CrossrefPubMedGoogle Scholar
• 232 Leuchte HH, Baumgartner RA, Nounou ME et al. Brain natriuretic peptide is a prognostic parameter in chronic lung disease. Am J Respir Crit Care Med 2006; 173: 744-750
  CrossrefPubMedGoogle Scholar
• 233 Leuchte HH, Neurohr C, Baumgartner R et al. Brain natriuretic peptide and exercise capacity in lung fibrosis and pulmonary hypertension. Am J Respir Crit Care Med 2004; 170: 360-365
  CrossrefPubMedGoogle Scholar
• 234 Song JW, Song JK, Kim DS. Echocardiography and brain natriuretic peptide as prognostic indicators in idiopathic pulmonary fibrosis. Respir Med 2009; 103: 180-186
  CrossrefPubMedGoogle Scholar
• 235 Shinoda H, Tasaka S, Fujishima S et al. Elevated CC chemokine level in bronchoalveolar lavage fluid is predictive of a poor outcome of idiopathic pulmonary fibrosis. Respiration 2009; 78: 285-292
  CrossrefPubMedGoogle Scholar
• 236 Rosas IO, Richards TJ, Konishi K et al. MMP1 and MMP7 as potential peripheral blood biomarkers in idiopathic pulmonary fibrosis. PLoS Med 2008; 5: e93
  CrossrefPubMedGoogle Scholar
• 237 Phelps DS, Umstead TM, Mejia M et al. Increased surfactant protein-A levels in patients with newly diagnosed idiopathic pulmonary fibrosis. Chest 2004; 125: 617-625
  CrossrefPubMedGoogle Scholar
• 238 McCormack FX, King Jr TE, Bucher BL et al. Surfactant protein A predicts survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1995; 152: 751-759
  CrossrefPubMedGoogle Scholar
• 239 Kinder BW, Brown KK, Schwarz MI et al. Baseline BAL neutrophilia predicts early mortality in idiopathic pulmonary fibrosis. Chest 2008; 133: 226-232
  CrossrefPubMedGoogle Scholar
• 240 Moeller A, Gilpin SE, Ask K et al. Circulating fibrocytes are an indicator of poor prognosis in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2009; 179: 588-594
  CrossrefPubMedGoogle Scholar
• 241 Richards TJ, Kaminski N, Baribaud F et al. Peripheral blood proteins predict mortality in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2012; 185: 67-76
  CrossrefPubMedGoogle Scholar
• 242 Davies HR, Richeldi L, Walters EH. Immunomodulatory agents for idiopathic pulmonary fibrosis. Cochrane Database Syst Rev 2003; 03 CD003134
  PubMedGoogle Scholar
• 243 Richeldi L, Davies HR, Ferrara G et al. Corticosteroids for idiopathic pulmonary fibrosis. Cochrane Database Syst Rev 2003; 3 CD002880
  PubMedGoogle Scholar
• 244 Turner-Warwick M, Burrows B, Johnson A. Cryptogenic fibrosing alveolitis: response to corticosteroid treatment and its effect on survival. Thorax 1980; 35: 593-599
  CrossrefPubMedGoogle Scholar
• 245 Gay SE, Kazerooni EA, Toews GB et al. Idiopathic pulmonary fibrosis: predicting response to therapy and survival. Am J Respir Crit Care Med 1998; 157: 1063-1072
  CrossrefPubMedGoogle Scholar
• 246 Flaherty KR, Toews GB, Lynch III JP et al. Steroids in idiopathic pulmonary fibrosis: a prospective assessment of adverse reactions, response to therapy, and survival. Am J Med 2001; 110: 278-282
  CrossrefPubMedGoogle Scholar
• 247 Nagai S, Kitaichi M, Hamada K et al. Hospital-based historical cohort study of 234 histologically proven Japanese patients with IPF. Sarcoidosis Vasc Diffuse Lung Dis 1999; 16: 209-214
  PubMedGoogle Scholar
• 248 Entzian P, Schlaak M, Seitzer U et al. Antiinflammatory and antifibrotic properties of colchicine: implications for idiopathic pulmonary fibrosis. Lung 1997; 175: 41-51
  CrossrefPubMedGoogle Scholar
• 249 Peters SG, McDougall JC, Douglas WW et al. Colchicine in the treatment of pulmonary fibrosis. Chest 1993; 103: 101-104
  CrossrefPubMedGoogle Scholar
• 250 Selman M, Carrillo G, Salas J et al. Colchicine, D-penicillamine, and prednisone in the treatment of idiopathic pulmonary fibrosis: a controlled clinical trial. Chest 1998; 114: 507-512
  CrossrefPubMedGoogle Scholar
• 251 Fiorucci E, Lucantoni G, Paone G et al. Colchicine, cyclophosphamide and prednisone in the treatment of mild-moderate idiopathic pulmonary fibrosis: comparison of three currently available therapeutic regimens. Eur Rev Med Pharmacol Sci 2008; 12: 105-111
  PubMedGoogle Scholar
• 252 Antoniou KM, Nicholson AG, Dimadi M et al. Longterm clinical effects of interferon gamma-1b and colchicine in idiopathic pulmonary fibrosis. Eur Respir J 2006; 28: 496-504
  CrossrefPubMedGoogle Scholar
• 253 Douglas WW, Ryu JH, Swensen SJ et al. Colchicine versus prednisone in the treatment of idiopathic pulmonary fibrosis: a randomized prospective study. Am J Respir Crit Care Med 1998; 158: 220-225
  CrossrefPubMedGoogle Scholar
• 254 Alton EW, Johnson M, Turner-Warwick M. Advanced cryptogenic fibrosing alveolitis: preliminary report on treatment with cyclosporin A. Respir Med 1989; 83: 277-279
  CrossrefPubMedGoogle Scholar
• 255 Moolman JA, Bardin PG, Rossouw DJ et al. Cyclosporin as a treatment for interstitial lung disease of unknown aetiology. Thorax 1991; 46: 592-595
  CrossrefPubMedGoogle Scholar
• 256 Homma S, Sakamoto S, Kawabata M et al. Cyclosporin treatment in steroid-resistant and acutely exacerbated interstitial pneumonia. Intern Med 2005; 44: 1144-1150
  CrossrefPubMedGoogle Scholar
• 257 Grgic A, Lausberg H, Heinrich M et al. Progression of fibrosis in usual interstitial pneumonia: serial evaluation of the native lung after single lung transplantation. Respiration 2008; 76: 139-145
  CrossrefPubMedGoogle Scholar
• 258 Wahidi MM, Ravenel J, Palmer SM et al. Progression of idiopathic pulmonary fibrosis in native lungs after single lung transplantation. Chest 2002; 121: 2072-2076
  CrossrefPubMedGoogle Scholar
• 259 Winterbauer RH, Hammar SP, Hallman KO et al. Diffuse interstitial pneumonitis: clinicopathologic correlations in 20 patients treated with prednisone/azathioprine. Am J Med 1978; 65: 661-672
  CrossrefPubMedGoogle Scholar
• 260 Raghu G, Depaso WJ, Cain K et al. Azathioprine combined with prednisone in the treatment of idiopathic pulmonary fibrosis: a prospective double-blind, randomized, placebo-controlled clinical trial. Am Rev Respir Dis 1991; 144: 291-296
  CrossrefPubMedGoogle Scholar
• 261 Johnson MA, Kwan S, Snell NJ et al. Randomised controlled trial comparing prednisolone alone with cyclophosphamide and low dose prednisolone in combination in cryptogenic fibrosing alveolitis. Thorax 1989; 44: 80-288
  PubMedGoogle Scholar
• 262 Collard HR, Ryu JH, Douglas WW et al. Combined corticosteroid and cyclophosphamide therapy does not alter survival in idiopathic pulmonary fibrosis. Chest 2004; 125: 2169-2174
  CrossrefPubMedGoogle Scholar
• 263 Pereira CAC, Malheiros T, Coletta EM et al. Survival in idiopathic pulmonary fibrosiscytotoxic agents compared to corticosteroids. Respir Med 2006; 100: 340-347
  CrossrefPubMedGoogle Scholar
• 264 Cantin AM, Hubbard RC, Crystal RG. Glutathione deficiency in the epithelial lining fluid of the lower respiratory tract in idiopathic pulmonary fibrosis. Am Rev Respir Dis 1989; 139: 370-372
  CrossrefPubMedGoogle Scholar
• 265 Behr J, Maier K, Degenkolb B et al. Antioxidative and clinical effects of high-dose N-acetylcysteine in fibrosing alveolitis: adjunctive therapy to maintenance immunosuppression. Am J Respir Crit Care Med 1997; 156: 1897-1901
  CrossrefPubMedGoogle Scholar
• 266 Demedts M, Behr J, Buhl R et al. IFIGENIA Study Group. High-dose acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med 2005; 353: 2229-2242
  CrossrefPubMedGoogle Scholar
• 267 Hunninghake GW. Antioxidant therapy for idiopathic pulmonary fibrosis. N Engl J Med 2005; 353: 2285-2287
  CrossrefPubMedGoogle Scholar
• 268 Wells AU. Antioxidant therapy in idiopathic pulmonary fibrosis: hope is kindled. Eur Respir J 2006; 27: 664-666
  CrossrefPubMedGoogle Scholar
• 269 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
  CrossrefPubMedGoogle Scholar
• 270 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
  CrossrefPubMedGoogle Scholar
• 271 King Jr TE, Albera C, Bradford WZ. INSPIRE Study Group et al. Effect of interferon gamma-1b on survival in patients with idiopathic pulmonary fibrosis (INSPIRE): a multicentre, randomised, placebo-controlled trial. Lancet 2009; 374: 222-228
  CrossrefPubMedGoogle Scholar
• 272 Thannickal VJ, Flaherty KR, Martinez FJ et al. Idiopathic pulmonary fibrosis: emerging concepts on pharmacotherapy. Expert Opin Pharmacother 2004; 5: 1671-1686
  CrossrefPubMedGoogle Scholar
• 273 King Jr TE, 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 2008; 177: 75-81
  CrossrefPubMedGoogle Scholar
• 274 Raghu G, King Jr TE, Behr J et al. Quality of life and dyspnoea in patients treated with bosentan for idiopathic pulmonary fibrosis (BUILD-1). Eur Respir J 2010; 35: 118-123
  CrossrefPubMedGoogle Scholar
• 275 Mohler KM, Torrance DS, Smith CA et al. Soluble tumor necrosis factor (TNF) receptors are effective therapeutic agents in lethal endotoxemia and function simultaneously as both TNF carriers and TNF antagonists. J Immunol 1993; 151: 1548-1561
  PubMedGoogle Scholar
• 276 Sime PJ, Marr RA, Gauldie D et al. Transfer of tumor necrosis factor-alpha to rat lung induces severe pulmonary inflammation and patchy interstitial fibrogenesis with induction of transforming growth factor-beta1 and myofibroblasts. Am J Pathol 1998; 153: 825-832
  CrossrefPubMedGoogle Scholar
• 277 Piguet PF, Ribaux C, Karpuz V et al. Expression and localization of tumor necrosis factor-alpha and its mRNA in idiopathic pulmonary fibrosis. Am J Pathol 1993; 143: 651-655
  PubMedGoogle Scholar
• 278 Raghu G, Brown KK, Costabel U et al. Treatment of idiopathic pulmonary fibrosis with etanercept: an exploratory, placebo-controlled trial. Am J Respir Crit Care Med 2008; 178: 948-955
  CrossrefPubMedGoogle Scholar
• 279 Kubo H, Nakayama K, Yanai M et al. Anticoagulant therapy for idiopathic pulmonary fibrosis. Chest 2005; 128: 1475-1482
  CrossrefPubMedGoogle Scholar
• 280 Raghu G, Johnson WC, Lockhart D et al. Treatment of idiopathic pulmonary fibrosis with a new antifibrotic agent, pirfenidone: results of a prospective, open-label Phase II study. Am J Respir Crit Care Med 1999; 159: 1061-1069
  CrossrefPubMedGoogle Scholar
• 281 Nagai S, Hamada K, Shigematsu M et al. Open-label compassionate use one year-treatment with pirfenidone to patients with chronic pulmonary fibrosis. Intern Med 2002; 41: 1118-1123
  CrossrefPubMedGoogle Scholar
• 282 Azuma A, Nukiwa T, Tsuboi E et al. Double-blind, placebo-controlled trial of pirfenidone in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2005; 171: 1040-1047
  CrossrefPubMedGoogle Scholar
• 283 Taniguchi H, Ebina M, Kondoh Y et al. Pirfenidone Clinical Study Group in Japan. Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J 2010; 35: 821-829
  CrossrefPubMedGoogle Scholar
• 284 Seymour S. Briefing Information for the March 9, 2010 Meeting of the Pulmonary-Allergy Drugs Advisory Committee. NDA 22-535. Washington, D.C.: US Food and Drug Administration; 2010 Available from: http://www.fda.gov/downloads/AdvisoryCommittees/CommitteesMeetingMaterials/Drugs/Pulmonary-AllergyDrugsAdvisoryCommittee/UCM203081.pdf [accessed 2010 May 10]
  PubMedGoogle Scholar
• 285 Seymour S. Division Summary: Overview of the FDA background materials for New Drug Application (NDA) 22-535, Esbriet (pirfenidone) for the treatment of patients with idiopathic pulmonary fibrosis (IPF) to reduce the decline in lung function. In: Seymour S, ed. FDA Briefing Information for the March 9, 2010 Meeting of the Pulmonary-Allergy Drugs Advisory Committee (PADAC). NDA 22-535. Washington, D.C.: US Food and Drug Administration; 2010: 2-20 Available from: http://www.fda.gov/downloads/AdvisoryCommittees/CommitteesMeetingMaterials/Drugs/Pulmonary-AllergyDrugsAdvisoryCommittee/UCM203081.pdf [accessed 2010 May 10]
  Google Scholar
• 286 Karimi-Shah B. Esbriet (pirfenidone) 2403 mg/day to reduce the decline in lung function in patients with idiopathic pulmonary fibrosis (Clinical Briefing Document). In: Seymour S, ed. FDA Briefing Information for the March 9, 2010 Meeting of the Pulmonary-Allergy Drugs Advisory Committee (PADAC). NDA 22-535. Washington, D.C.: US Food and Drug Administration; Available from: http://www.fda.gov/downloads/AdvisoryCommittees/CommitteesMeetingMaterials/Drugs/Pulmonary-AllergyDrugsAdvisoryCommittee/UCM203081.pdf [accessed 2010 May 10]
  Google Scholar
• 287 Zhou F. Pirfenidone capsules [three 267-mg capsules TID] for treatment of patients with idiopathic pulmonary fibrosis to reduce decline in lung function (Statistical Briefing Document). In: Seymour S, ed. FDA Briefing Information for the March 9, 2010 Meeting of the Pulmonary-Allergy Drugs Advisory Committee (PADAC). NDA 22-535. Washington, D.C.: US Food and Drug Administration; 2010: 110-158 Available from: http://www.fda.gov/downloads/AdvisoryCommittees/CommitteesMeetingMaterials/Drugs/Pulmonary-AllergyDrugsAdvisoryCommittee/UCM203081.pdf [accessed 2010 May 10]
  Google Scholar
• 288 King Jr TE, Brown KK, Raghu G et al. BUILD-3: A Randomized, Controlled Trial of Bosentan in Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med 2011; 184: 92-99
  CrossrefPubMedGoogle Scholar
• 289 Behr J, Costabel U, Worth H. Stellungnahme der DGP zur Pressemitteilung des National Heart Lung and Blood Institute zur PANTHER-Studie bei IPF-Patienten vom 21.10.2011. Pneumologie 2011; 67: 724-725
  Article in Thieme ConnectPubMedGoogle Scholar
• 290 Noble PW, Albera C, Bradford WZ et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet 2011; 377: 1760-1769
  CrossrefPubMedGoogle Scholar
• 291 Sagnolo P, Del Giovane C, Luppi F et al. Non-steroid agents for idiopathic pulmonary fibrosis. Cochrane Datbase Syst Rev 2010; 9 CD003134
  PubMedGoogle Scholar
• 292 Daniels CE, Lasky JA, Limper AH et al. Imatinib treatment for idiopathic pulmonary fibrosis: randomized placebo-controlled trial results. Am J Respir Crit Care Med 2010; 181: 604-610
  CrossrefPubMedGoogle Scholar
• 293 Richeldi L, Costabel U, Selman M et al. Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. New Engl J Med 2011; 365: 1079-1087
  CrossrefPubMedGoogle Scholar
• 294 Morrison DA, Stovall JR. Increased exercise capacity in hypoxemic patients after long-term oxygen therapy. Chest 1992; 102: 542-550
  CrossrefPubMedGoogle Scholar
• 295 Nocturnal Oxygen Therapy Trial Group. Continuous or nocturnal oxygen therapy in hypoxemic chronic obstructive lung disease: a clinical trial. Ann Intern Med 1980; 93: 391-398
  CrossrefPubMedGoogle Scholar
• 296 Longterm domiciliary oxygen therapy in chronic hypoxic cor pulmonale complicating chronic bronchitis and emphysema: report of the Medical Research Council Working Party. Lancet 1981; 1: 681-686
  PubMedGoogle Scholar
• 297 Mason DP, Brizzio ME, Alster JM et al. Lung transplantation for idiopathic pulmonary fibrosis. Ann Thorac Surg 2007; 84: 1121-1128
  CrossrefPubMedGoogle Scholar
• 298 Keating D, Levvey B, Kotsimbos T et al. Lung transplantation in pulmonary fibrosis: challenging early outcomes counterbalanced by surprisingly good outcomes beyond 15 years. Transplant Proc 2009; 41: 289-291
  CrossrefPubMedGoogle Scholar
• 299 Christie JD, Edwards LB, Kucheryavaya AY et al. The Registry of the International Society for Heart and Lung Transplantation: Twenty-eighth Adult Lung and Heart-Lung Transplant Report -- 2011. J Heart Lung Transplant 2011; 30: 1104-1122
  CrossrefPubMedGoogle Scholar
• 300 Thabut G, Mal H, Castier Y et al. Survival benefit of lung transplantation for patients with idiopathic pulmonary fibrosis. J Thorac Cardiovasc Surg 2003; 126: 469-475
  CrossrefPubMedGoogle Scholar
• 301 Orens J, Estenne M, Arcasoy S et al. International guidelines for the selection of lung transplant candidates: 2006 update -- a consensus report from the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant 2006; 25: 745-55
  CrossrefPubMedGoogle Scholar
• 302 Thabut G, Christie JD, Ravaud P et al. Survival after bilateral versus single-lung transplantation for idiopathic pulmonary fibrosis. Ann Intern Med 2009; 151: 767-774
  CrossrefPubMedGoogle Scholar
• 303 Kim DS, Park JH, Park BK et al. Acute exacerbation of idiopathic pulmonary fibrosis: frequency and clinical features. Eur Respir J 2006; 27: 143-150
  CrossrefPubMedGoogle Scholar
• 304 Blivet S, Philit F, Sab JM et al. Outcome of patients with idiopathic pulmonary fibrosis admitted to the ICU for respiratory failure. Chest 2001; 120: 209-212
  CrossrefPubMedGoogle Scholar
• 305 Molina-Molina M, Badia JR, Marin-Arguedas A et al. Outcomes and clinical characteristics of patients with pulmonary fibrosis and respiratory failure admitted to an intensive care unit: a study of 20 cases. Med Clin (Barc) 2003; 121: 63-67
  CrossrefPubMedGoogle Scholar
• 306 Saydain G, Islam A, Afessa B et al. Outcome of patients with idiopathic pulmonary fibrosis admitted to the intensive care unit. Am J Respir Crit Care Med 2002; 166: 839-842
  CrossrefPubMedGoogle Scholar
• 307 Nava S, Rubini F. Lung and chest wall mechanics in ventilated patients with end stage idiopathic pulmonary fibrosis. Thorax 1999; 54: 390-395
  CrossrefPubMedGoogle Scholar
• 308 Stern JB, Mal H, Groussard O et al. Prognosis of patients with advanced idiopathic pulmonary fibrosis requiring mechanical ventilation for acute respiratory failure. Chest 2001; 120: 213-219
  CrossrefPubMedGoogle Scholar
• 309 Al-Hameed FM, Sharma S. Outcome of patients admitted to the intensive care unit for acute exacerbation of idiopathic pulmonary fibrosis. Can Respir J 2004; 11: 117-122
  PubMedGoogle Scholar
• 310 Fumeaux T, Rothmeier C, Jolliet P. Outcome of mechanical ventilation for acute respiratory failure in patients with pulmonary fibrosis. Intensive Care Med 2001; 27: 1868-1874
  CrossrefPubMedGoogle Scholar
• 311 Pitsiou G, Ioannis Trigonis I, Tsiata E et al. Outcome of patients with pulmonary fibrosis admitted to the ICU for acute respiratory failure. Eur Respir J Suppl 2006; 28: E650
  PubMedGoogle Scholar
• 312 Mollica C, Paone G, Conti V et al. Mechanical ventilation in patients with end-stage idiopathic pulmonary fibrosis. Respiration 2010; 79: 209-215
  CrossrefPubMedGoogle Scholar
• 313 Rangappa P, Moran JL. Outcomes of patients admitted to the intensive care unit with idiopathic pulmonary fibrosis. Crit Care Resusc 2009; 11: 102-109
  PubMedGoogle Scholar
• 314 Mallick S. Outcome of patients with idiopathic pulmonary fibrosis (IPF) ventilated in intensive care unit. Respir Med 2008; 102: 1355-1359
  CrossrefPubMedGoogle Scholar
• 315 Mason DP, Thuita L, Nowicki ER et al. Should lung transplantation be performed for patients on mechanical respiratory support? The US experience. J Thorac Cardiovasc Surg 2010; 139: 765-773
  CrossrefPubMedGoogle Scholar
• 316 Holland AE, Hill CJ, Conron M et al. Short term improvement in exercise capacity and symptoms following exercise training in interstitial lung disease. Thorax 2008; 63: 549-554
  CrossrefPubMedGoogle Scholar
• 317 Nishiyama O, Kondoh Y, Kimura T et al. Effects of pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis. Respirology 2008; 13: 394-399
  CrossrefPubMedGoogle Scholar
• 318 Ferreira A, Garvey C, Connors GL et al. Pulmonary rehabilitation in interstitial lung disease: benefits and predictors of response. Chest 2009; 135: 442-447
  CrossrefPubMedGoogle Scholar
• 319 Ferreira G, Feuerman M, Spiegler P. Results of an 8-week, outpatient pulmonary rehabilitation program on patients with and without chronic obstructive pulmonary disease. J Cardiopulm Rehabil 2006; 26: 54-60
  CrossrefPubMedGoogle Scholar
• 320 Jastrzebski D, Gumola A, Gawlik R et al. Dyspnea and quality of life in patients with pulmonary fibrosis after six weeks of respiratory rehabilitation. J Physiol Pharmacol 2006; 57: 139-148
  PubMedGoogle Scholar
• 321 Naji NA, Connor MC, Donnelly SC et al. Effectiveness of pulmonary rehabilitation in restrictive lung disease. J Cardiopulm Rehabil 2006; 26: 237-243
  CrossrefPubMedGoogle Scholar
• 322 Magnussen H, Kirsten AM, Köhler D et al. Deutsche Gesellschaft Für Pneumologie und Beatmungsmedizin e.V. Guidelines for long-term-oxygen therapy. German Society for Pneumology and Respiratory Medicine. Pneumologie 2008; 62: 748-756
  Article in Thieme ConnectPubMedGoogle Scholar
• 323 Fischer S, Hoeper MM, Bein T et al. Interventional lung assist: a new concept of protective ventilation in bridge to lung transplantation. ASAIO J 2008; 54 : 3-10
  CrossrefPubMedGoogle Scholar
• 324 Lancaster LH, Mason WR, Parnell JA et al. Obstructive sleep apnea is common in idiopathic pulmonary fibrosis. Chest 2009; 136: 772-778
  CrossrefPubMedGoogle Scholar
• 325 Collard HR, Moore BB, Flaherty KR et al. Idiopathic Pulmonary Fibrosis Clinical Research Network Investigators. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2007; 176: 636-643
  CrossrefPubMedGoogle Scholar
• 326 Akira M, Hamada H, Sakatani M et al. CT findings during phase of accelerated deterioration in patients with idiopathic pulmonary fibrosis. AJR Am J Roentgenol 1997; 168: 79-83
  CrossrefPubMedGoogle Scholar
• 327 Ambrosini V, Cancellieri A, Chilosi M et al. Acute exacerbation of idiopathic pulmonary fibrosis: report of a series. Eur Respir J 2003; 22: 821-826
  CrossrefPubMedGoogle Scholar
• 328 Parambil JG, Myers JL, Ryu JH. Histopathologic features and outcome of patients with acute exacerbation of idiopathic pulmonary fibrosis undergoing surgical lung biopsy. Chest 2005; 128: 3310-3315
  CrossrefPubMedGoogle Scholar
• 329 Rice AJ, Wells AU, Bouros D et al. Terminal diffuse alveolar damage in relation to interstitial pneumonias: an autopsy study. Am J Clin Pathol 2003; 119: 709-714
  CrossrefPubMedGoogle Scholar
• 330 Kondo A, Saiki S. Acute exacerbation in idiopathic interstitial pneumonia (IIP). In: Harasawa M, Fukuchi Y, Morinari H, ed. Interstitial pneumonia of unknown etiology. Tokyo: University of Tokyo Press; 1989: 33-42
  Google Scholar
• 331 Kim DS, Collard HR, King Jr TE. Classification and natural history of the idiopathic interstitial pneumonias. Proc Am Thorac Soc 2006; 3: 285-292
  CrossrefPubMedGoogle Scholar
• 332 Sakamoto S, Homma S, Miyamoto A et al. Cyclosporin A in the treatment of acute exacerbation of idiopathic pulmonary fibrosis. Intern Med 2010; 49: 109-115
  CrossrefPubMedGoogle Scholar
• 333 Olschewski H, Ghofrani HA, Walmrath D et al. Inhaled prostacyclin and iloprost in severe pulmonary hypertension secondary to lung fibrosis. Am J Respir Crit Care Med 1999; 160: 600-607
  CrossrefPubMedGoogle Scholar
• 334 Minai OA, Sahoo D, Chapman JT et al. Vaso-active therapy can improve 6-min walk distance in patients with pulmonary hypertension and fibrotic interstitial lung disease. Respir Med 2008; 102: 1015-1020
  CrossrefPubMedGoogle Scholar
• 335 Ghofrani HA, Wiedemann R, Rose F et al. Sildenafil for treatment of lung fibrosis and pulmonary hypertension: a randomised controlled trial. Lancet 2002; 360: 895-900
  CrossrefPubMedGoogle Scholar
• 336 Madden BP, Allenby M, Loke T et al. A potential role for sildenafil in the management of pulmonary hypertension in patients with parenchymal lung disease. Vascul Pharmacol 2006; 44: 372-376
  CrossrefPubMedGoogle Scholar
• 337 Collard HR, Anstrom KJ, Schwarz MI et al. Sildenafil improves walk distance in idiopathic pulmonary fibrosis. Chest 2007; 131: 897-899
  CrossrefPubMedGoogle Scholar
• 338 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
  CrossrefPubMedGoogle Scholar
• 339 Tobin RW, Pope II CE, Pellegrini CA et al. Increased prevalence of gastroesophageal reflux in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1998; 158: 1804-1808
  CrossrefPubMedGoogle Scholar
• 340 Sweet MP, Patti MG, Leard LE et al. Gastroesophageal reflux in patients with idiopathic pulmonary fibrosis referred for lung transplantation. J Thorac Cardiovasc Surg 2007; 133: 1078-1084
  CrossrefPubMedGoogle Scholar
• 341 Marik PE. Aspiration pneumonitis and aspiration pneumonia. N Engl J Med 2001; 344: 665-671
  CrossrefPubMedGoogle Scholar
• 342 Barnes TW, Vassallo R, Tazelaar HD et al. Diffuse bronchiolar disease due to chronic occult aspiration. Mayo Clin Proc 2006; 81: 172-176
  CrossrefPubMedGoogle Scholar
• 343 Davis Jr RD, Lau CL, Eubanks S et al. Improved lung allograft function after fundoplication in patients with gastroesophageal reflux disease undergoing lung transplantation. J Thorac Cardiovasc Surg 2003; 125: 533-542
  CrossrefPubMedGoogle Scholar
• 344 Linden PA, Gilbert RJ, Yeap BY et al. Laparoscopic fundoplication in patients with end-stage lung disease awaiting transplantation. J Thorac Cardiovasc Surg 2006; 131: 438-446
  CrossrefPubMedGoogle Scholar
• 345 Hope-Gill BDM, Hilldrup S, Davies C et al. A study of the cough reflex in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2003; 168: 995-1002
  CrossrefPubMedGoogle Scholar
• 346 Horton MR, Danoff SK, Lechtzin N. Thalidomide inhibits the intractable cough of idiopathic pulmonary fibrosis. Thorax 2008; 63: 749
  CrossrefPubMedGoogle Scholar
• 347 Allen S, Raut S, Woollard J et al. Low dose diamorphine reduces breathlessness without causing a fall in oxygen saturation in elderly patients with end-stage idiopathic pulmonary fibrosis. Palliat Med 2005; 19: 128-130
  CrossrefPubMedGoogle Scholar
• 348 Jegal Y, Kim DS, Shim TS et al. Physiology is a stronger predictor of survival than pathology in fibrotic interstitial pneumonia. Am J Respir Crit Care Med 2005; 171: 639-644
  CrossrefPubMedGoogle Scholar
• 349 Collard HR, King Jr TE, Bartelson BB et al. Changes in clinical and physiologic variables predict survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2003; 168: 538-542
  CrossrefPubMedGoogle Scholar
• 350 King Jr TE, Safrin S, Starko KM et al. Analyses of efficacy end points in a controlled trial of interferon-gamma1b for idiopathic pulmonary fibrosis. Chest 2005; 127: 171-177
  CrossrefPubMedGoogle Scholar
• 351 Latsi PI, du Bois RM, Nicholson AG et al. Fibrotic idiopathic interstitial pneumonia: the prognostic value of longitudinal functional trends. Am J Respir Crit Care Med 2003; 168: 531-537
  CrossrefPubMedGoogle Scholar
• 352 Flaherty KR, Mumford JA, Murray S et al. Prognostic implications of physiologic and radiographic changes in idiopathic interstitial pneumonia. Am J Respir Crit Care Med 2003; 168: 543-548
  CrossrefPubMedGoogle Scholar
• 353 Zappala CJ, Latsi PI, Nicholson AG et al. Marginal decline in forced vital capacity is associated with a poor outcome in idiopathic pulmonary fibrosis. Eur Respir J 2010; 35: 830-836
  CrossrefPubMedGoogle Scholar
• 354 Miller MR, Crapo R, Hankinson J et al. ATS/ERS Task Force. General considerations for lung function testing. Eur Respir J 2005; 26: 153-161
  CrossrefPubMedGoogle Scholar
• 355 Miller MR, Hankinson J, Brusasco V et al. ATS/ERS Task Force. Standardisation of spirometry. Eur Respir J 2005; 26: 319-338
  CrossrefPubMedGoogle Scholar
• 356 Wanger J, Clausen JL, Coates A et al. Standardisation of the measurement of lung volumes. Eur Respir J 2005; 26: 511-522
  CrossrefPubMedGoogle Scholar
• 357 Macintyre N, Crapo RO, Viegi G et al. Standardisation of the single-breath determination of carbon monoxide uptake in the lung. Eur Respir J 2005; 26: 720-735
  CrossrefPubMedGoogle Scholar
• 358 Pellegrino R, Viegi G, Brusasco V et al. Interpretative strategies for lung function tests. Eur Respir J 2005; 26: 948-968
  CrossrefPubMedGoogle Scholar
• 359 Selman M, King Jr TE, Pardo A. American Thoracic Society. Idiopathic pulmonary fibrosis: prevailing and evolving hypotheses about its pathogenesis and implications for therapy. Ann Intern Med 2001; 134: 136-151
  CrossrefPubMedGoogle Scholar
• 360 Akagi T, Matsumoto T, Harada T et al. Coexistent emphysema delays the decrease of vital capacity in idiopathic pulmonary fibrosis. Respir Med 2009; 103: 1209-1215
  CrossrefPubMedGoogle Scholar
• 361 Mura M, Zompatori M, Pacilli AMG et al. The presence of emphysema further impairs physiologic function in patients with idiopathic pulmonary fibrosis. Respir Care 2006; 51: 257-265
  PubMedGoogle Scholar
• 362 Cottin V, Cordier JF. Combined pulmonary fibrosis and emphysema: an experimental and clinically relevant phenotype. Am J Respir Crit Care Med 2005; 172: 1605-1606
  CrossrefPubMedGoogle Scholar
• 363 Wiggins J, Strickland B, Turner-Warwick M. Combined cryptogenic fibrosing alveolitis and emphysema: the value of high resolution computed tomography in assessment. Respir Med 1990; 84: 365-369
  CrossrefPubMedGoogle Scholar
• 364 Martinez FJ, Safrin S, Weycker D. IPF Study Group et al. The clinical course of patients with idiopathic pulmonary fibrosis. Ann Intern Med 2005; 142: 963-967
  CrossrefPubMedGoogle Scholar
• 365 ATS Committee on Proficiency Standards for Clinical Pulmonary Function Laboratories. ATS statement: guidelines for the six-minute walk test. Am J Respir Crit Care Med 2002; 166: 111-117
  CrossrefPubMedGoogle Scholar
• 366 Arcasoy SM, Christie JD, Ferrari VA et al. Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease. Am J Respir Crit Care Med 2003; 167: 735-740
  CrossrefPubMedGoogle Scholar
• 367 Fisher MR, Forfia PR, Chamera E et al. Accuracy of Doppler echocardiography in the hemodynamic assessment of pulmonary hypertension. Am J Respir Crit Care Med 2009; 179: 615-621
  CrossrefPubMedGoogle Scholar
• 368 Nathan SD, Noble PW, Tuder RM. Idiopathic pulmonary fibrosis and pulmonary hypertension: connecting the dots. Am J Respir Crit Care Med 2007; 175: 875-880
  CrossrefPubMedGoogle Scholar
• 369 Leuchte HH, Baumgartner RA, Nounou ME et al. Brain natriuretic peptide is a prognostic parameter in chronic lung disease. Am J Respir Crit Care Med 2006; 173: 744-750
  CrossrefPubMedGoogle Scholar
• 370 Leuchte HH, Neurohr C, Baumgartner R et al. Brain natriuretic peptide and exercise capacity in lung fibrosis and pulmonary hypertension. Am J Respir Crit Care Med 2004; 170: 360-365
  CrossrefPubMedGoogle Scholar
• 371 Zisman DA, Karlamangla AS, Kawut SM et al. Validation of a method to screen for pulmonary hypertension in advanced idiopathic pulmonary fibrosis. Chest 2008; 133: 640-645
  CrossrefPubMedGoogle Scholar
• 372 Egan JJ, Adamali H et al. Ganciclovir Antiviral Therapy in Advanced Idiopathic Pulmonary Fibrosis: An Open Pilot Study. Pulmonary Medicine 2011; doi 10.1155/2011/24080, Epub 2011 Apr 7
  PubMedGoogle Scholar
• 373 Zisman DA, Schwarz M, Anstrom KJ. Idiopathic Pulmonary Fibrosis Clinical Research Network et al. A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. N Engl J Med 2010; 363: 620-628
  CrossrefPubMedGoogle Scholar
• 374 Hoeper MM, Halank M, Wilkens H et al. Riociguat for patients with pulmonary hypertension associated with interstitial lung disease. http://www.phaonlineuniv.org/node/5134
  PubMedGoogle Scholar
• 375 The Idiopathic Pulmonary Fibrosis Clinical Research Network. Prednisone, Azathiorpine, and n-Acetylcysteine for pulmonary fibrosis. N Engl J Med 2012; 366: 1968-1977
  CrossrefPubMedGoogle Scholar
• 376 Behr J. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med 2012; 367: 869 (letter), author reply 870 – 871
  CrossrefPubMedGoogle Scholar
• 377 Wells AU, Behr J, Costabel U et al. Triple therapy in idiopathic pulmonary fibrosis: an alarming press release. Eur Respir J 2012; 39: 805-806
  CrossrefPubMedGoogle Scholar
• 378 Noth I, Anstrom SB, de Andrade J et al. A Placebo-Controlled Randomized Trial of Warfarin in Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med 2012; 186: 88-95
  CrossrefPubMedGoogle Scholar
• 379 Müller-Quernheim J, Wells A. Evidence-based recommendations in idiopathic pulmonary fibrosis: a year is a long time in interstitial lung disease. Am J Respir Crit Care Med 2012; 186: 5-7
  CrossrefPubMedGoogle Scholar