Clinical course and prediction of survival in idiopathic pulmonary fibrosis

American Journal of Respiratory and Critical Care Medicine

Volumn 183, Issue 4, 2011, Pages 431-440

  Ley, Brett ^a   Collard, Harold R ^a   King Jr , Talmadge E ^a  

^a UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

Clinical course; Idiopathic pulmonary fibrosis; Predictors; Prognosis; Survival

Indexed keywords

ALBUMIN; AMINO TERMINAL PRO BRAIN NATRIURETIC PEPTIDE; GELATINASE B; GLYCOPROTEIN; KREBS VONDEN LUNGEN 6; MATRILYSIN; NEUTROPHIL COLLAGENASE; STROMELYSIN; UNCLASSIFIED DRUG;

ADULT RESPIRATORY DISTRESS SYNDROME; AGE; CAUSE OF DEATH; CIGARETTE SMOKING; CLUBBING FINGERS; COMPUTER ASSISTED TOMOGRAPHY; CORONARY ARTERY DISEASE; DISEASE COURSE; DISEASE EXACERBATION; DYSPNEA; EMPHYSEMA; ETHNICITY; FIBROSING ALVEOLITIS; GENERAL CONDITION DETERIORATION; HEART CATHETERIZATION; HUMAN; INTERSTITIAL LUNG DISEASE; INTERSTITIAL PNEUMONIA; LUNG ALVEOLUS CELL; LUNG BIOPSY; LUNG EMBOLISM; LUNG FUNCTION; LUNG FUNCTION TEST; LUNG GAS EXCHANGE; LUNG VOLUME; PHYSICAL EXAMINATION; PREDICTION; PRIORITY JOURNAL; PROGNOSIS; PULMONARY HYPERTENSION; REVIEW; SEX DIFFERENCE; SMOKING HABIT; SPIROMETRY; SURVIVAL; SURVIVAL RATE; THORAX RADIOGRAPHY;

CAUSE OF DEATH; DISEASE PROGRESSION; HUMANS; IDIOPATHIC PULMONARY FIBROSIS; RISK FACTORS; SURVIVAL ANALYSIS;

EID: 79951844263     PISSN: 1073449X     EISSN: 15354970     Source Type: Journal    
DOI: 10.1164/rccm.201006-0894CI     Document Type: Review

References (137)

1. American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American thoracic society (ATS), and the European Respiratory Society (ERS). Am J Respir Crit Care Med 2000;161:646-664.
2. American Thoracic Society/European Respiratory Society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 2002;165:277-304.
3. Fernandez Perez ER, Daniels CE, Schroeder DR, St Sauver J, Hartman TE, Bartholmai BJ, Yi ES, Ryu JH. Incidence, prevalence, and clinical course of idiopathic pulmonary fibrosis: a population-based study. Chest 2010;137:129-137.
4. Martinez FJ, Safrin S, Weycker D, Starko KM, Bradford WZ, King TE Jr, Flaherty KR, Schwartz DA, Noble PW, Raghu G, et al. The clinical course of patients with idiopathic pulmonary fibrosis. Ann Intern Med 2005;142:963-967.
5. Swigris JJ, Kuschner WG, Kelsey JL, Gould MK. Idiopathic pulmonary fibrosis: challenges and opportunities for the clinician and investigator. Chest 2005;127:275-283. (Pubitemid 41724588)
6. Bjoraker JA, Ryu JH, Edwin MK, Myers JL, Tazelaar HD, Schroeder DR, Offord KP. Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1998;157:199-203. (Pubitemid 28071283)
7. Nagai S, Kitaichi M, Hamada K, Nagao T, Hoshino Y, Miki H, Izumi T. Hospital-based historical cohort study of 234 histologically proven Japanese patients with IPF. Sarcoidosis Vasc Diffuse Lung Dis 1999;16:209-214. (Pubitemid 29469102)
8. Nicholson AG, Colby TV, Dubois RM, Hansell DM, Wells AU. 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. (Pubitemid 32051689)
9. King TE Jr, Schwarz MI, Brown K, Tooze JA, Colby TV, Waldron JA Jr, Flint A, Thurlbeck WM, Cherniack RM. Idiopathic pulmonary fibrosis. Relationship between histopathologic features and mortality. Am J Respir Crit Care Med 2001;164:1025-1032. (Pubitemid 32971653)
10. Collard HR, King TE Jr, Bartelson BB, Vourlekis JS, Schwarz MI, Brown KK. Changes in clinical and physiologic variables predict survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2003;168:538-542. (Pubitemid 37088595)
11. Jegal Y, Kim DS, Shim TS, Lim CM, Do Lee S, Koh Y, Kim WS, Kim WD, Lee JS, Travis WD, 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. (Pubitemid 40365421)
12. Nagai S, Nagao T, Kitaichi M, Izumi T. Clinical courses of asymptomatic cases with idiopathic pulmonary fibrosis and a histology of usual interstitial pneumonia. Eur Respir J 1998;11:131s.
13. El-Chemaly S, Ziegler SG, Wilson K, Gahl WA, Moss J, Gochuico BR. Familial pulmonary fibrosis: natural history of preclinical disease. Am J Respir Crit Care Med 2010;181:A2980.
14. Steele MP, Speer MC, Loyd JE, Brown KK, Herron A, Slifer SH, Burch LH, Wahidi MM, Phillips JA III, Sporn TA, et al. Clinical and pathologic features of familial interstitial pneumonia. Am J Respir Crit Care Med 2005;172:1146-1152. (Pubitemid 43066651)
15. Rosas IO, Ren P, Avila NA, Chow CK, Franks TJ, Travis WD, McCoy JP Jr, May RM, Wu HP, Nguyen DM, et al. Early interstitial lung disease in familial pulmonary fibrosis. Am J Respir Crit Care Med 2007;176:698-705. (Pubitemid 47503078)
16. Baumgartner KB, Samet J, Stidley CA, Colby TV, Waldron JA, and the Collaborating Centers. Cigarette smoking: a risk factor for idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1997;155:242-248. (Pubitemid 27053171)
17. Lederer DJ, Enright PL, Kawut SM, Hoffman EA, Hunninghake G, van Beek EJ, Austin JH, Jiang R, Lovasi GS, Barr RG. Cigarette smoking is associated with subclinical parenchymal lung disease: the multi-ethnic study of atherosclerosis (MESA)-lung study. Am J Respir Crit Care Med 2009;180:407-414.
18. Washko GR, Lynch DA, Matsuoka S, Ross JC, Umeoka S, Diaz A, Sciurba FC, Hunninghake GM, Raull San Jose E, Silverman EK, et al. Identification of early interstitial lung disease in smokers from the COPDgene study. Acad Radiol 2010;17:48-53.
19. Chida M, Ono S, Hoshikawa Y, Kondo T. Subclinical idiopathic pulmonary fibrosis is also a risk factor of postoperative acute respiratory distress syndrome following thoracic surgery. Eur J Cardiothorac Surg 2008;34:878-881.
20. Jun A, Yoshinori K, Park J, Takashi U, Hideo O, Yutaka S. Clinically occult subpleural fibrosis and acute interstitial pneumonia a precursor to idiopathic pulmonary fibrosis? Respirology 2008;13:408-412.
21. Takeda A, Enomoto T, Sanuki N, Nakajima T, Takeda T, Sayama K, Kunieda E. Acute exacerbation of subclinical idiopathic pulmonary fibrosis triggered by hypofractionated stereotactic body radiotherapy in a patient with primary lung cancer and slightly focal honey-combing. Radiat Med 2008;26:504-507.
22. King TE Jr, Tooze JA, Schwarz MI, Brown K, Cherniack RM. Predicting survival in idiopathic pulmonary fibrosis. Scoring system and survival model. Am J Respir Crit Care Med 2001;164:1171-1181. (Pubitemid 33052246)
23. Selman M, Carrillo G, Estrada A, Mejia M, Becerril C, Cisneros J, Gaxiola M, Perez-Padilla R, Navarro C, Richards T, et al. Accelerated variant of idiopathic pulmonary fibrosis: clinical behavior and gene expression pattern. PLoS ONE 2007;2:e482.
24. King TE Jr, Behr J, Brown KK, du Bois RM, Lancaster L, de Andrade JA, Stahler G, Leconte I, Roux S, Raghu G. Build-1: a randomized placebo-controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2008;177:75-81.
25. Raghu G, Brown KK, Bradford WZ, Starko K, Noble PW, Schwartz DA, King TE Jr. The Idiopathic Pulmonary Fibrosis Study Group. A placebo-controlled trial of interferon gamma-1b in patients with idiopathic pulmonary fibrosis. N Engl J Med 2004;350:125-133. (Pubitemid 38056223)
26. Raghu G, Brown KK, Costabel U, Cottin V, du Bois RM, Lasky JA, Thomeer M, Utz JP, Khandker RK, McDermott L, et al. Treatment of idiopathic pulmonary fibrosis with etanercept: an exploratory, placebo-controlled trial. Am J Respir Crit Care Med 2008;178:948-955.
27. Azuma A, Nukiwa T, Tsuboi E, Suga M, Abe S, Nakata K, Taguchi Y, Nagai S, Itoh H, Ohi M, 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. (Pubitemid 40616955)
28. Demedts M, Behr J, Costabel U, Wallaert B, Van den Bosch J, Dekhuijzen PNR, Jansen HM, MacNee W, Buhl R, Thomeer M, et al. IFEGENIA: an international study of n-acetylcysteine (NAC) in IPF. Am J Respir Crit Care Med 2001;163:A708.
29. Daniels CE, Lasky JA, Limper AH, Mieras K, Gabor E, Schroeder DR. Imatinib treatment for idiopathic pulmonary fibrosis: randomized placebo-controlled trial results. Am J Respir Crit Care Med 2010;181:604-610.
30. Taniguchi H, Ebina M, Kondoh Y, Ogura T, Azuma A, Suga M, Taguchi Y, Takahashi H, Nakata K, Sato A, et al. Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J 2010;35:821-829.
31. Boon K, Bailey NW, Yang J, Steel MP, Groshong S, Kervitsky D, Brown KK, Schwarz MI, Schwartz DA. Molecular phenotypes distinguish patients with relatively stable from progressive idiopathic pulmonary fibrosis (IPF). PLoS ONE 2009;4:e5134.
32. Collard HR, Moore BB, Flaherty KR, Brown KK, Kaner RJ, King TE Jr, Lasky JA, Loyd JE, Noth I, Olman MA, et al. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2007;176:636-643. (Pubitemid 47503070)
33. Tomioka H, Sakurai T, Hashimoto K, Iwasaki H. Acute exacerbation of idiopathic pulmonary fibrosis: Role of Chlamydophila pneumoniae infection. Respirology 2007;12:700-706. (Pubitemid 47414875)
34. Silva CIS, Muller NL, Fujimoto K, Kato S, Ichikado K, Taniguchi H, Kondoh Y, Johkoh T, Churg A. Acute exacerbation of chronic interstitial pneumonia: high-resolution computed tomography and pathologic findings. J Thorac Imaging 2007;22:221-229. (Pubitemid 47329825)
35. Olson AL, Swigris JJ, Raghu G, Brown KK. Seasonal variation: mortality from pulmonary fibrosis is greatest in the winter. Chest 2009;136:16-22.
36. Flaherty KR, Toews GB, Travis WD, Colby TV, Kazerooni EA, Gross BH, Jain A, Strawderman RL III, Paine R, Flint A, et al. Clinical significance of histological classification of idiopathic interstitial pneumonia. Eur Respir J 2002;19:275-283. (Pubitemid 34162828)
37. Nagai S, Kitaichi M, Itoh H, Nishimura K, Izumi T, Colby TV. Idiopathic nonspecific interstitial pneumonia/fibrosis: comparison with idiopathic pulmonary fibrosis and BOOP. Eur Respir J 1998;12:1010-1019. (Pubitemid 28545604)
38. Daniil ZD, Gilchrist FC, Nicholson AG, Hansell DM, Harris J, Colby TV, du Bois RM. A histologic pattern of nonspecific interstitial pneumonia is associated with a better prognosis than usual interstitial pneumonia in patients with cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med 1999;160:899-905. (Pubitemid 29442028)
39. Park CS, Chung SW, Ki SY, Lim GI, Uh ST, Kim YH, Choi DI, Park JS, Lee DW, Kitaichi M. Increased levels of interleukin-6 are associated with lymphocytosis in bronchoalveolar lavage fluids of idiopathic nonspecific interstitial pneumonia. Am J Respir Crit Care Med 2000;162:1162-1168.
40. 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.
41. Panos RJ, Mortenson R, Niccoli SA, King TE Jr. Clinical deterioration in patients with idiopathic pulmonary fibrosis. Causes and assessment. Am J Med 1990;88:396-404. (Pubitemid 20147276)
42. Olson AL, Swigris JJ, Lezotte DC, Norris JM, Wilson CG, Brown KK. Mortality from pulmonary fibrosis increased in the United States from 1992 to 2003. Am J Respir Crit Care Med 2007;176:277-284.
43. King TE Jr, Albera C, Bradford WZ, Costabel U, Hormel P, Lancaster L, Noble PW, Sahn SA, Szwarcberg J, Thomeer M, 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.
44. Hubbard RB, Smith C, Le Jeune I, Gribbin J, Fogarty AW. The association between idiopathic pulmonary fibrosis and vascular disease: a population-based study. Am J Respir Crit Care Med 2008;178:1257-1261.
45. Alakhras M, Decker PA, Nadrous HF, Collazo-Clavell M, Ryu JH. Body mass index and mortality in patients with idiopathic pulmonary fibrosis. Chest 2007;131:1448-1453. (Pubitemid 46762351)
46. Nadrous HF, Pellikka PA, Krowka MJ, Swanson KL, Chaowalit N, Decker PA, Ryu JH. Pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Chest 2005;128:2393-2399. (Pubitemid 41507585)
47. Raghu G, Weycker D, Edelsberg J, Bradford WZ, Oster G. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2006;174:810-816. (Pubitemid 44511663)
48. Erbes R, Schaberg T, Loddenkemper R. Lung function tests in patients with idiopathic pulmonary fibrosis. Are they helpful for predicting outcome? Chest 1997;111:51-57.
49. Fell CD, Martinez FJ, Liu LX, Murray S, Han MK, Kazerooni EA, Gross BH, Myers J, Travis WD, Colby TV, et al. Clinical predictors of a diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2010;181:832-837.
50. Collard HR. The age of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2010;181:771-772.
51. Schwartz DA, Helmers RA, Galvin JR, Van Fossen DS, Frees KL, Dayton CS, Burmeister LF, Hunninghake GW. Determinants of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1994;149:450-454. (Pubitemid 24068994)
52. Han MK, Murray S, Fell CD, Flaherty KR, Toews GB, Myers J, Colby TV, Travis WD, Kazerooni EA, Gross BH, et al. Sex differences in physiological progression of idiopathic pulmonary fibrosis. Eur Respir J 2008;31:1183-1188.
53. Lederer DJ, Caplan-Shaw CE, O'Shea MK, Wilt JS, Basner RC, Bartels MN, Sonett JR, Arcasoy SM, Kawut SM. Racial and ethnic disparities in survival in lung transplant candidates with idiopathic pulmonary fibrosis. Am J Transplant 2006;6:398-403. (Pubitemid 43382178)
54. Lederer DJ, Arcasoy SM, Barr RG, Wilt JS, Bagiella E, D'Ovidio F, Sonett JR, Kawut SM. Racial and ethnic disparities in idiopathic pulmonary fibrosis: a UNOS/OPTN database analysis. Am J Transplant 2006;6:2436-2442. (Pubitemid 44359431)
55. Antoniou KM, Hansell DM, Rubens MB, Marten K, Desai SR, Siafakas NM, Nicholson AG, du Bois RM, Wells AU. Idiopathic pulmonary fibrosis: outcome in relation to smoking status. Am J Respir Crit Care Med 2008;177:190-194.
56. Becklake MR, Lalloo U. The "healthy smoker": a phenomenon of health selection? Respiration 1990;57:137-144.
57. Manali ED, Stathopoulos GT, Kollintza A, Kalomenidis I, Emili JM, Sotiropoulou C, Daniil Z, Roussos C, Papiris SA. The medical research council chronic dyspnea score predicts the survival of patients with idiopathic pulmonary fibrosis. Respir Med 2008;102:586-592.
58. Lettieri CJ, Nathan SD, Barnett SD, Ahmad S, Shorr AF. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest 2006;129:746-752.
59. Hamada K, Nagai S, Tanaka S, Handa T, Shigematsu M, Nagao T, Mishima M, Kitaichi M, Izumi T. Significance of pulmonary arterial pressure and diffusion capacity of the lung as prognosticator in patients with idiopathic pulmonary fibrosis. Chest 2007;131:650-656. (Pubitemid 46446853)
60. Fell CD, Martinez FJ. The impact of pulmonary arterial hypertension on idiopathic pulmonary fibrosis. Chest 2007;131:641-643. (Pubitemid 46446849)
61. 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. (Pubitemid 46684695)
62. Nathan SD, Shlobin OA, Ahmad S, Koch J, Barnett SD, Ad N, Burton N, Leslie K. Serial development of pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Respiration 2008;76:288-294.
63. Song JW, Song JK, Kim DS. Echocardiography and brain natriuretic peptide as prognostic indicators in idiopathic pulmonary fibrosis. Respir Med 2009;103:180-186.
64. Nathan SD, Shlobin OA, Barnett SD, Saggar R, Belperio JA, Ross DJ, Ahmad S, Saggar R, Libre E, Lynch JP III, et al. Right ventricular systolic pressure by echocardiography as a predictor of pulmonary hypertension in idiopathic pulmonary fibrosis. Respir Med 2008;102:1305-1310.
65. Zisman DA, Karlamangla AS, Ross DJ, Keane MP, Belperio JA, Saggar R, Lynch JP III, Ardehali A, Goldin J. High-resolution chest CT findings do not predict the presence of pulmonary hypertension in advanced idiopathic pulmonary fibrosis. Chest 2007;132:773-779.
66. Zisman DA, Karlamangla AS, Kawut SM, Shlobin OA, Saggar R, Ross DJ, Schwarz MI, Belperio JA, Ardehali A, Lynch JP III, et al. Validation of a method to screen for pulmonary hypertension in advanced idiopathic pulmonary fibrosis. Chest 2008;133:640-645. (Pubitemid 351367371)
67. Grubstein A, Bendayan D, Schactman I, Cohen M, Shitrit D, Kramer MR. Concomitant upper-lobe bullous emphysema, lower-lobe interstitial fibrosis and pulmonary hypertension in heavy smokers: report of eight cases and review of the literature. Respir Med 2005;99:948-954. (Pubitemid 40796537)
68. Mura M, Zompatori M, Pacilli AM, Fasano L, Schiavina M, Fabbri M. The presence of emphysema further impairs physiologic function in patients with idiopathic pulmonary fibrosis. Respir Care 2006;51:257-265. (Pubitemid 46888402)
69. Jankowich MD, Polsky M, Klein M, Rounds S. Heterogeneity in combined pulmonary fibrosis and emphysema. Respiration 2008;75:411-417. (Pubitemid 351685432)
70. Casas JP, Abbona H, Robles A, Lopez AM. [Normal lung volumes in patients with idiopathic pulmonary fibrosis and emphysema.] Medicina (B Aires) 2008;68:282-284.
71. Silva DR, Gazzana MB, Barreto SS, Knorst MM. Idiopathic pulmonary fibrosis and emphysema in smokers. J Bras Pneumol 2008;34:779-786.
72. Akagi T, Matsumoto T, Harada T, Tanaka M, Kuraki T, Fujita M, Watanabe K. Coexistent emphysema delays the decrease of vital capacity in idiopathic pulmonary fibrosis. Respir Med 2009;103:1209-1215.
73. Cottin V, Nunes H, Brillet PY, Delaval P, Devouassoux G, Tillie-Leblond I, Israel-Biet D, Court-Fortune I, Valeyre D, Cordier JF. Combined pulmonary fibrosis and emphysema: a distinct under-recognised entity. Eur Respir J 2005;26:586-593. (Pubitemid 41448654)
74. Mejia M, Carrillo G, Rojas-Serrano J, Estrada A, Suarez T, Alonso D, Barrientos E, Gaxiola M, Navarro C, Selman M. Idiopathic pulmonary fibrosis and emphysema: decreased survival associated with severe pulmonary arterial hypertension. Chest 2009;136:10-15.
75. Cottin V, Le Pavec J, Prevot G, Mal H, Humbert M, Simonneau G, Cordier JF. Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome. Eur Respir J 2010;35:105-111.
76. Millar AB, Denison DM. Vertical gradients of lung density in supine subjects with fibrosing alveolitis or pulmonary emphysema. Thorax 1990;45:602-605. (Pubitemid 20270478)
77. 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. (Pubitemid 20287410)
78. Hiwatari N, Shimura S, Takishima T. Pulmonary emphysema followed by pulmonary fibrosis of undetermined cause. Respiration 1993;60:354-358. (Pubitemid 23348646)
79. Doherty MJ, Pearson MG, O'Grady EA, Pellegrini V, Calverley PM. Cryptogenic fibrosing alveolitis with preserved lung volumes. Thorax 1997;52:998-1002.
80. Tobin RW, Pope CE II, Pellegrini CA, Emond MJ, Sillery J, Raghu G. Increased prevalence of gastroesophageal reflux in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1998;158:1804-1808. (Pubitemid 29008818)
81. Patti MG, Tedesco P, Golden J, Hays S, Hoopes C, Meneghetti A, Damani T, Way LW. Idiopathic pulmonary fibrosis: How often is it really idiopathic? J Gastrointest Surg 2005;9:1053-1056; discussion 1056-1058.
82. Raghu G, Freudenberger TD, Yang S, Curtis JR, Spada C, Hayes J, Sillery JK, Pope CE II, Pellegrini CA. High prevalence of abnormal acid gastro-oesophageal reflux in idiopathic pulmonary fibrosis. Eur Respir J 2006;27:136-142. (Pubitemid 43092677)
83. Sweet MP, Patti MG, Leard LE, Golden JA, Hays SR, Hoopes C, Theodore PR. Gastroesophageal reflux in patients with idiopathic pulmonary fibrosis referred for lung transplantation. J Thorac Cardiovasc Surg 2007;133:1078-1084. (Pubitemid 46435907)
84. Lee JS, Collard HR, Raghu G, Sweet MP, Hays SR, Campos GM, Golden JA, King TE Jr. Does chronic microaspiration cause idiopathic pulmonary fibrosis? Am J Med 2010;123:304-311.
85. Nathan SD, Basavaraj A, Reichner C, Shlobin OA, Ahmad S, Kiernan J, Burton N, Barnett SD. Prevalence and impact of coronary artery disease in idiopathic pulmonary fibrosis. Respir Med 2010;104:1035-1041.
86. Bouros D, Hatzakis K, Labrakis H, Zeibecoglou K. Association of malignancy with diseases causing interstitial pulmonary changes. Chest 2002;121:1278-1289. (Pubitemid 34280454)
87. Mogulkoc N, Brutsche MH, Bishop PW, Greaves SM, Horrocks AW, Egan JJ. Pulmonary function in idiopathic pulmonary fibrosis and referral for lung transplantation. Am J Respir Crit Care Med 2001;164:103-108. (Pubitemid 33151306)
88. Battista G, Zompatori M, Fasano L, Pacilli A, Basile B. Progressive worsening of idiopathic pulmonary fibrosis. High resolution computed tomography (HRCT) study with functional correlations. Radiol Med (Torino) 2003;105:2-11. (Pubitemid 36559771)
89. Lynch DA, David Godwin J, Safrin S, Starko KM, Hormel P, Brown KK, Raghu G, King TE Jr, Bradford WZ, Schwartz DA, et al. High-resolution computed tomography in idiopathic pulmonary fibrosis: diagnosis and prognosis. Am J Respir Crit Care Med 2005;172:488-493. (Pubitemid 41117395)
90. Shin KM, Lee KS, Chung MP, Han J, Bae YA, Kim TS, Chung MJ. Prognostic determinants among clinical, thin-section CT, and histopathologic findings for fibrotic idiopathic interstitial pneumonias: tertiary hospital study. Radiology 2008;249:328-337.
91. Best AC, Meng J, Lynch AM, Bozic CM, Miller D, Grunwald GK, Lynch DA. Idiopathic pulmonary fibrosis: physiologic tests, quantitative CT indexes, and CT visual scores as predictors of mortality. Radiology 2008;246:935-940.
92. Sumikawa H, Johkoh T, Colby TV, Ichikado K, Suga M, Taniguchi H, Kondoh Y, Ogura T, Arakawa H, Fujimoto K, et al. Computed tomography findings in pathological usual interstitial pneumonia: relationship to survival. Am J Respir Crit Care Med 2008;177:433-439. (Pubitemid 351317587)
93. Iwasawa T, Asakura A, Sakai F, Kanauchi T, Gotoh T, Ogura T, Yazawa T, Nishimura J, Inoue T. Assessment of prognosis of patients with idiopathic pulmonary fibrosis by computer-aided analysis of CT images. J Thorac Imaging 2009;24:216-222.
94. Flaherty KR, Thwaite EL, Kazerooni EA, Gross BH, Toews GB, Colby TV, Travis WD, Mumford JA, Murray S, Flint A, et al. Radiological versus histological diagnosis in UIP and NSIP: survival implications. Thorax 2003;58:143-148. (Pubitemid 36183002)
95. Wells AU, Desai SR, Rubens MB, Goh NS, Cramer D, Nicholson AG, Colby TV, du Bois RM, Hansell DM. 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. (Pubitemid 36408161)
96. Latsi PI, du Bois RM, Nicholson AG, Colby TV, Bisirtzoglou D, Nikolakopoulou A, Veeraraghavan S, Hansell DM, Wells AU. Fibrotic idiopathic interstitial pneumonia: the prognostic value of longitudinal functional trends. Am J Respir Crit Care Med 2003;168:531-537.
97. Flaherty KR, Andrei A-C, Murray S, Fraley C, Colby TV, Travis WD, Lama V, Kazerooni EA, Gross BH, Toews GB, et al. Idiopathic pulmonary fibrosis: prognostic value of changes in physiology and six-minute-walk test. Am J Respir Crit Care Med 2006;174:803-809. (Pubitemid 44511662)
98. Hanson D, Winterbauer RH, Kirtland SH, Wu R. Changes in pulmonary function test results after 1 year of therapy as predictors of survival in patients with idiopathic pulmonary fibrosis. Chest 1995;108:305-310.
99. Zappala CJ, Latsi PI, Nicholson AG, Colby TV, Cramer D, Renzoni EA, Hansell DM, du Bois RM, Wells AU. Marginal decline in forced vital capacity is associated with a poor outcome in idiopathic pulmonary fibrosis. Eur Respir J 2010;35:830-836.
100. Du Bois RM, Albera C, Bradford WZ, Costabel U, Kartashov A, Noble PW, Raghu G, Szwarcberg J, Thomeer M, Valeyre D, et al. Ascertainment of individual risk of mortality for patients with idiopathic pulmonary fibrosis (IPF). Am J Respir Crit Care Med 2010;181:A2499.
101. Fulmer JD, Roberts WC, von Gal ER, Crystal RG. Morphologic-physiologic correlates of the severity of fibrosis and degree of cellularity in idiopathic pulmonary fibrosis. J Clin Invest 1979;63:665-676. (Pubitemid 9167034)
102. Keogh BA, Crystal RG. Pulmonary function testing in interstitial pulmonary disease. What does it tell us? Chest 1980;78:856-865. (Pubitemid 11209630)
103. Eaton T, Young P, Milne D, Wells AU. Six-minute walk, maximal exercise tests: reproducibility in fibrotic interstitial pneumonia. Am J Respir Crit Care Med 2005;171:1150-1157. (Pubitemid 40663728)
104. Kadikar A, Maurer J, Kesten S. The six-minute walk test: a guide to assessment for lung transplantation. J Heart Lung Transplant 1997;16:313-319. (Pubitemid 27138296)
105. Lederer DJ, Arcasoy SM, Wilt JS, D'Ovidio F, Sonett JR, Kawut SM. Six-minute-walk distance predicts waiting list survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2006;174:659-664. (Pubitemid 44395333)
106. Caminati A, Bianchi A, Cassandro R, Mirenda MR, Harari S. Walking distance on 6-MWT is a prognostic factor in idiopathic pulmonary fibrosis. Respir Med 2009;103:117-123.
107. Hallstrand TS, Boitano LJ, Johnson WC, Spada CA, Hayes JG, Raghu G. The timed walk test as a measure of severity and survival in idiopathic pulmonary fibrosis. Eur Respir J 2005;25:96-103. (Pubitemid 40207737)
108. Lettieri CJ, Nathan SD, Browning RF, Barnett SD, Ahmad S, Shorr AF. The distance-saturation product predicts mortality in idiopathic pulmonary fibrosis. Respir Med 2006;100:1734-1741. (Pubitemid 44283761)
109. Swigris JJ, Swick J, Wamboldt FS, Sprunger D, du Bois R, Fischer A, Cosgrove GP, Frankel SK, Fernandez-Perez ER, Kervitsky D, et al. Heart rate recovery after 6-min walk test predicts survival in patients with idiopathic pulmonary fibrosis. Chest 2009;136:841-848.
110. Du Bois RM, Albera C, Costabel U, Bradford WZ, Kartashov A, Noble PW, Szwarcberg J, Thomeer M, Valeyre D, Weycker D, et al. 6-minute walk test distance (6MWD) is a reliable, valid, and responsive outcome measure that predicts mortality in patients with ipf. Am J Respir Crit Care Med 2010;181:A1103.
111. Swigris JJ, Wamboldt FS, Behr J, du Bois RM, King TE, Raghu G, Brown KK. The 6 minute walk in idiopathic pulmonary fibrosis: longitudinal changes and minimum important difference. Thorax 2010;65:173-177.
112. Shitrit D, Rusanov V, Peled N, Amital A, Fuks L, Kramer MR. The 15-step oximetry test: a reliable tool to identify candidates for lung transplantation among patients with idiopathic pulmonary fibrosis. J Heart Lung Transplant 2009;28:328-333.
113. Stephan S, de Castro Pereira CA, Coletta EM, Ferreira RG, Otta JS, Nery LE. Oxygen desaturation during a 4-minute step test: predicting survival in idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis 2007;24:70-76. (Pubitemid 350177775)
114. Flaherty KR, Travis WD, Colby TV, Toews GB, Kazerooni EA, Gross BH, Jain A, Strawderman RL, Flint A, Lynch JP, et al. Histopathologic variability in usual and nonspecific interstitial pneumonias. Am J Respir Crit Care Med 2001;164:1722-1727. (Pubitemid 33124165)
115. Monaghan H, Wells AU, Colby TV, du Bois RM, Hansell DM, Nicholson AG. Prognostic implications of histologic patterns in multiple surgical lung biopsies from patients with idiopathic interstitial pneumonias. Chest 2004;125:522-526.
116. Nicholson AG, Fulford LG, Colby TV, Dubois RM, Hansell DM, Wells AU. The relationship between individual histologic features and disease progression in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2002;166:173-177. (Pubitemid 34765453)
117. Enomoto N, Suda T, Kato M, Kaida Y, Nakamura Y, Imokawa S, Ida M, Chida K. Quantitative analysis of fibroblastic foci in usual interstitial pneumonia. Chest 2006;130:22-29. (Pubitemid 44121414)
118. Collard HR, Cool CD, Leslie KO, Curran-Everett D, Groshong S, Brown KK. Organizing pneumonia and lymphoplasmacytic inflammation predict treatment response in idiopathic pulmonary fibrosis. Histopathology 2007;50:258-265. (Pubitemid 46046395)
119. Zisman DA, Kawut SM, Lederer DJ, Belperio JA, Lynch JP III, Schwarz MI, Tayek JA, Reuben DB, Karlamangla AS. Serum albumin concentration and waiting list mortality in idiopathic interstitial pneumonia. Chest 2009;135:929-935.
120. Yokoyama A, Kondo K, Nakajima M, Matsushima T, Takahashi T, Nishimura M, Bando M, Sugiyama Y, Totani Y, Ishizaki T, et al. Prognostic value of circulating KL-6 in idiopathic pulmonary fibrosis. Respirology 2006;11:164-168. (Pubitemid 43356896)
121. Greene KE, Wright JR, Steinberg KP, Ruzinski JT, Caldwell E, Wong WB, Hull W, Whitsett JA, Akino T, Kuroki Y, et al. Serial changes in surfactant-associated proteins in lung and serum before and after onset of ARDS. Am J Respir Crit Care Med 1999;160:1843-1850. (Pubitemid 30004685)
122. Greene KE, King TE Jr, Kuroki Y, Bucher-Bartelson B, Hunninghake GW, Newman LS, Nagae H, Mason RJ. Serum surfactant proteins-A and -D as biomarkers in idiopathic pulmonary fibrosis. Eur Respir J 2002;19:439-446. (Pubitemid 34256957)
123. McCormack FX, King TE Jr, Bucher BL, Nielsen L, Mason RJ. Surfactant protein A predicts survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1995;152:751-759.
124. Takahashi H, Fujishima T, Koba H, Murakami S, Kurokawa K, Shibuya Y, Shiratori M, Kuroki Y, Abe S. Serum surfactant proteins a and d as prognostic factors in idiopathic pulmonary fibrosis and their relationship to disease extent. Am J Respir Crit Care Med 2000;162:1109-1114.
125. Barlo N, van Moorsel C, Ruven H, Zanen P, van den Bosch J, Grutters J. Surfactant protein-D predicts survival in patients with idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis 2009;26:155-161.
126. Kinder BW, Brown KK, McCormack FX, Ix JH, Kervitsky A, Schwarz MI, King TE. Serum surfactant protein-a is a strong predictor of early mortality in idiopathic pulmonary fibrosis. Chest 2009;135:1557-1563.
127. McKeown S, Richter AG, O'Kane C, McAuley DF, Thickett DR. Mmp expression and abnormal lung permeability are important determinants of outcome in IPF. Eur Respir J 2009;33:77-84.
128. Rosas IO, Richards TJ, Konishi K, Zhang Y, Gibson K, Lokshin AE, Lindell KO, Cisneros J, Macdonald SD, Pardo A, et al. Mmp1 and mmp7 as potential peripheral blood biomarkers in idiopathic pulmonary fibrosis. PLoS Med 2008;5:e93.
129. Prasse A, Probst C, Bargagli E, Zissel G, Toews GB, Flaherty KR, Olschewski M, Rottoli P, Muller-Quernheim J. Serum CC-chemokine ligand 18 concentration predicts outcome in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2009;179:717-723.
130. Shinoda H, Tasaka S, Fujishima S, Yamasawa W, Miyamoto K, Nakano Y, Kamata H, Hasegawa N, Ishizaka A. Elevated CC chemokine level in bronchoalveolar lavage fluid is predictive of a poor outcome of idiopathic pulmonary fibrosis. Respiration 2009;78:285-292.
131. Moeller A, Gilpin SE, Ask K, Cox G, Cook D, Gauldie J, Margetts PJ, Farkas L, Dobranowski J, Boylan C, et al. Circulating fibrocytes are an indicator of poor prognosis in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2009;179:588-594.
132. Kinder BW, Brown KK, Schwarz MI, Ix JH, Kervitsky A, King TE Jr. Baseline bronchoalveolar lavage neutrophilia predicts early mortality in idiopathic pulmonary fibrosis. Chest 2008;133:226-232.
133. Toll DB, Janssen KJ, Vergouwe Y, Moons KG. Validation, updating and impact of clinical prediction rules: a review. J Clin Epidemiol 2008;61:1085-1094.
134. Harrell FE Jr, Lee KL, Mark DB. Multivariable prognostic models: issues in developing models, evaluating assumptions and adequacy, and measuring and reducing errors. Stat Med 1996;15:361-387. (Pubitemid 26072233)
135. Laupacis A, Sekar N, Stiell IG. Clinical prediction rules. A review and suggested modifications of methodological standards. JAMA 1997;277:488-494.
136. Swigris JJ, Kuschner WG, Jacobs SS, Wilson SR, Gould MK. Health-related quality of life in patients with idiopathic pulmonary fibrosis: a systematic review. Thorax 2005;60:588-594. (Pubitemid 40966214)
137. The Idiopathic Pulmonary Fibrosis Clinical Research Network. A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. N Engl J Med 2010;363:620-628.