An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias

American Journal of Respiratory and Critical Care Medicine

Volumn 188, Issue 6, 2013, Pages 733-748

  Travis, William D ^a   Costabel, Ulrich ^a   Hansell, David M ^a   King Jr , Talmadge E ^a   Lynch, David A ^a   Nicholson, Andrew G ^a   Ryerson, Christopher J ^a   Ryu, Jay H ^a   Selman, Moisés ^a   Wells, Athol U ^a   Behr, Jurgen ^a   Bouros, Demosthenes ^a   Brown, Kevin K ^a   Colby, Thomas V ^a   Collard, Harold R ^a   Cordeiro, Carlos Robalo ^a   Cottin, Vincent ^a   Crestani, Bruno ^a   Drent, Marjolein ^a   Dudden, Rosalind F ^a   Egan, Jim ^a   Flaherty, Kevin ^a   Hogaboam, Cory ^a   Inoue, Yoshikazu ^a   Johkoh, Takeshi ^a   Kim, Dong Soon ^a   Kitaichi, Masanori ^a   Loyd, James ^a   Martinez, Fernando J ^a   Myers, Jeffrey ^a   Protzko, Shandra ^a   Raghu, Ganesh ^a   Richeldi, Luca ^a   Sverzellati, Nicola ^a   Swigris, Jeffrey ^a   Valeyre, Dominique ^a   more..

^a NONE

Author keywords

Acute interstitial pneumonia; Cryptogenic organizing pneumonia; Desquamative interstitial pneumonia; Idiopathic interstitial pneumonia; Nonspecific interstitial pneumonia; Respiratory bronchiolitis; Usual interstitial pneumonia

Indexed keywords

BIOLOGICAL MARKER; COLLAGEN;

ALLERGIC PNEUMONITIS; ARTICLE; BRONCHIOLITIS; DIFFERENTIAL DIAGNOSIS; DISEASE CLASSIFICATION; DISEASE EXACERBATION; HISTOPATHOLOGY; HUMAN; IDIOPATHIC LYMPHOID INTERSTITIAL PNEUMONIA; IDIOPATHIC PLEUROPARENCHYMAL FIBROELASTOSIS; INTERSTITIAL LUNG DISEASE; INTERSTITIAL PNEUMONIA; LUNG FIBROSIS; LUNG INJURY; LUNG LAVAGE; MEDICAL SOCIETY; PRIORITY JOURNAL; PROGNOSIS; SMOKING; VASCULAR DISEASE;

DIAGNOSIS, DIFFERENTIAL; EUROPE; HUMANS; IDIOPATHIC INTERSTITIAL PNEUMONIAS; SOCIETIES, MEDICAL; UNITED STATES;

EID: 84881669432     PISSN: 1073449X     EISSN: 15354970     Source Type: Journal    
DOI: 10.1164/rccm.201308-1483ST     Document Type: Article

References (166)

1. American Thoracic Society, European Respiratory Society. 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.
2. Travis WD, Hunninghake G, King TE Jr, Lynch DA, Colby TV, Galvin JR, Brown KK, Chung MP, Cordier J-F, du Bois RM, et al. Idiopathic nonspecific interstitial pneumonia: report of an American Thoracic Society project. Am J Respir Crit Care Med 2008;177:1338-1347.
3. Aziz ZA, Wells AU, Hansell DM, Bain GA, Copley SJ, Desai SR, Ellis SM, Gleeson FV, Grubnic S, Nicholson AG, et al. HRCT diagnosis of diffuse parenchymal lung disease: inter-observer variation. Thorax 2004;59:506-511.
4. Flaherty KR, King TE Jr, Raghu G, Lynch JP I I I, Colby TV, Travis WD, Gross BH, Kazerooni EA, Toews GB, Long Q, 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.
5. Nicholson AG, Addis BJ, Bharucha H, Clelland CA, Corrin B, Gibbs AR, Hasleton PS, Kerr KM, Ibrahim N B N, Stewart S, et al. Interobserver variation between pathologists in diffuse parenchymal lung disease. Thorax 2004;59:500-505.
6. Thomeer M, Demedts M, Behr J, Buhl R, Costabel U, Flower C D R, Verschakelen J, Laurent F, Nicholson AG, Verbeken EK, et al.; Idiopathic Pulmonary Fibrosis International Group Exploring NAcetylcysteine I Annual (IFIGENIA) Study Group. Multidisciplinary interobserver agreement in the diagnosis of idiopathic pulmonary fibrosis. Eur Respir J 2008;31:585-591.
7. Flaherty KR, Andrei A-C, King TE Jr, Raghu G, Colby TV, Wells A, Bassily N, Brown K, du Bois R, Flint A, et al. Idiopathic interstitial pneumonia: do community and academic physicians agree on diagnosis? Am J Respir Crit Care Med 2007;175:1054-1060.
8. Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, Colby TV, Cordier JF, Flaherty KR, Lasky JA, et al.; ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. 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.
9. Selman M. Hypersensitivity pneumonitis: a multifaceted deceiving disorder. Clin Chest Med 2004;25:531-547, vi.
10. Lynch DA, Newell JD, Logan PM, King TE Jr, Müller NL. Can CT distinguish hypersensitivity pneumonitis from idiopathic pulmonary fibrosis? AJR Am J Roentgenol 1995;165:807-811.
11. Silva CI, Churg A, Müller NL. Hypersensitivity pneumonitis: spectrum of high-resolution CT and pathologic findings. AJR Am J Roentgenol 2007;188:334-344.
12. Silva C I S, Müller NL, Lynch DA, Curran-Everett D, Brown KK, Lee KS, Chung MP, Churg A. Chronic hypersensitivity pneumonitis: differentiation from idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia by using thin-section CT. Radiology 2008;246:288-297.
13. Akashi T, Takemura T, Ando N, Eishi Y, Kitagawa M, Takizawa T, Koike M, Ohtani Y, Miyazaki Y, Inase N, et al. Histopathologic analysis of sixteen autopsy cases of chronic hypersensitivity pneumonitis and comparison with idiopathic pulmonary fibrosis/usual interstitial pneumonia. Am J Clin Pathol 2009;131:405-415.
14. Churg A, Muller NL, Flint J, Wright JL. Chronic hypersensitivity pneumonitis. Am J Surg Pathol 2006;30:201-208.
15. Churg A, Sin DD, Everett D, Brown K, Cool C. Pathologic patterns and survival in chronic hypersensitivity pneumonitis. Am J Surg Pathol 2009;33:1765-1770.
16. Fenton ME, Cockcroft DW, Wright JL, Churg A. Hypersensitivity pneumonitis as a cause of airway-centered interstitial fibrosis. Ann Allergy Asthma Immunol 2007;99:465-466.
17. Sahin H, Brown KK, Curran-Everett D, Hale V, Cool CD, Vourlekis JS, Lynch DA. Chronic hypersensitivity pneumonitis: CT features comparison with pathologic evidence of fibrosis and survival. Radiology 2007;244:591-598.
18. Hanak V, Golbin JM, Ryu JH. Causes and presenting features in 85 consecutive patients with hypersensitivity pneumonitis. Mayo Clin Proc 2007;82:812-816.
19. Felício CH, Parra ER, Capelozzi VL. Idiopathic and collagen vascular disease nonspecific interstitial pneumonia: clinical significance of remodeling process. Lung 2007;185:39-46.
20. Park JH, Kim DS, Park IN, Jang SJ, Kitaichi M, Nicholson AG, Colby TV. Prognosis of fibrotic interstitial pneumonia: idiopathic versus collagen vascular disease-related subtypes. Am J Respir Crit Care Med 2007;175:705-711.
21. Hwang J-H, Misumi S, Sahin H, Brown KK, Newell JD, Lynch DA. 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.
22. Song JW, Do K-H, Kim M-Y, Jang SJ, Colby TV, Kim DS. Pathologic and radiologic differences between idiopathic and collagen vascular disease-related usual interstitial pneumonia. Chest 2009;136:23-30.
23. Corte TJ, Copley SJ, Desai SR, Zappala CJ, Hansell DM, Nicholson AG, Colby TV, Renzoni E, Maher TM, Wells AU. Significance of connective tissue disease features in idiopathic interstitial pneumonia. Eur Respir J 2012;39:661-668.
24. Suda T, Kono M, Nakamura Y, Enomoto N, Kaida Y, Fujisawa T, Imokawa S, Yasuda K, Hashizume H, Yokomura K, et al. Distinct prognosis of idiopathic nonspecific interstitial pneumonia (NSIP) fulfilling criteria for undifferentiated connective tissue disease (UCTD). Respir Med 2010;104:1527-1534.
25. Kinder BW, Collard HR, Koth L, Daikh DI, Wolters PJ, Elicker B, Jones KD, King TE Jr. Idiopathic nonspecific interstitial pneumonia: lung manifestation of undifferentiated connective tissue disease? Am J Respir Crit Care Med 2007;176:691-697.
26. García-Sancho C, Buendía-Roldán I, Fernández-Plata MR, Navarro C, Pérez-Padilla R, Vargas MH, Loyd JE, Selman M. Familial pulmonary fibrosis is the strongest risk factor for idiopathic pulmonary fibrosis. Respir Med 2011;105:1902-1907.
27. 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.
28. Marshall RP, Puddicombe A, Cookson WO, Laurent GJ. Adult familial cryptogenic fibrosing alveolitis in the United Kingdom. Thorax 2000;55:143-146.
29. Barlo NP, van Moorsel C H M, Ruven H J T, Zanen P, van den Bosch J M M, Grutters JC. Surfactant protein-D predicts survival in patients with idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis 2009;26:155-161.
30. Nogee LM, Dunbar AE I I I, Wert SE, Askin F, Hamvas A, Whitsett JA. A mutation in the surfactant protein C gene associated with familial interstitial lung disease. N Engl J Med 2001;344:573-579.
31. Kirwan M, Dokal I. Dyskeratosis congenita, stem cells and telomeres. Biochim Biophys Acta 2009;1792:371-379.
32. van Moorsel CH, van Oosterhout MF, Barlo NP, de Jong PA, van der Vis JJ, Ruven HJ, van Es HW, van den Bosch JM, Grutters JC. 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 2010;182:1419-1425.
33. Alder JK, Chen J J L, Lancaster L, Danoff S, Su SC, Cogan JD, Vulto I, Xie M, Qi X, Tuder RM, et al. Short telomeres are a risk factor for idiopathic pulmonary fibrosis. Proc Natl Acad Sci USA 2008;105:13051-13056.
34. Cronkhite JT, Xing C, Raghu G, Chin KM, Torres F, Rosenblatt RL, Garcia CK. Telomere shortening in familial and sporadic pulmonary fibrosis. Am J Respir Crit Care Med 2008;178:729-737.
35. Lawson WE, Crossno PF, Polosukhin VV, Roldan J, Cheng D-S, Lane KB, Blackwell TR, Xu C, Markin C, Ware LB, et al. Endoplasmic reticulum stress in alveolar epithelial cells is prominent in IPF: association with altered surfactant protein processing and herpesvirus infection. Am J Physiol Lung Cell Mol Physiol 2008;294:L1119-L1126.
36. Seibold MA, Wise AL, Speer MC, Steele MP, Brown KK, Loyd JE, Fingerlin TE, Zhang W, Gudmundsson G, Groshong SD, et al. A common MUC5B promoter polymorphism and pulmonary fibrosis. N Engl J Med 2011;364:1503-1512.
37. Zhang Y, Noth I, Garcia JG, Kaminski N. A variant in the promoter of MUC5B and idiopathic pulmonary fibrosis. N Engl J Med 2011;364:1576-1577.
38. 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.
39. 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.
40. Ryu JH, Colby TV, Hartman TE, Vassallo R. Smoking-related interstitial lung diseases: a concise review. Eur Respir J 2001;17:122-132.
41. Aubry MC, Wright JL, Myers JL. The pathology of smoking-related lung diseases. Clin Chest Med 2000;21:11-35, vii.
42. Vassallo R, Jensen EA, Colby TV, Ryu JH, Douglas WW, Hartman TE, Limper AH. The overlap between respiratory bronchiolitis and desquamative interstitial pneumonia in pulmonary Langerhans cell histiocytosis: high-resolution CT, histologic, and functional correlations. Chest 2003;124:1199-1205.
43. Cottin V, Le Pavec J, Prévot G, Mal H, Humbert M, Simonneau G, Cordier JF; GERM "O" P. Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome. Eur Respir J 2010;35:105-111.
44. Cottin V, Cordier JF. The syndrome of combined pulmonary fibrosis and emphysema. Chest 2009;136:1-2.
45. Cottin V, Nunes H, Brillet PY, Delaval P, Devouassoux G, Tillie-Leblond I, Israel-Biet D, Court-Fortune I, Valeyre D, Cordier JF; Groupe d'Etude et de Recherche sur les Maladies Orphelines Pulmonaires (GERM "O" P). Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J 2005;26:586-593.
46. Mejía M, Carrillo G, Rojas-Serrano J, Estrada A, Suárez 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.
47. Akira M, Inoue Y, Kitaichi M, Yamamoto S, Arai T, Toyokawa K. Usual interstitial pneumonia and nonspecific interstitial pneumonia with and without concurrent emphysema: thin-section CT findings. Radiology 2009;251:271-279.
48. 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.
49. 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.
50. Sverzellati N, Wells AU, Tomassetti S, Desai SR, Copley SJ, Aziz ZA, Zompatori M, Chilosi M, Nicholson AG, Poletti V, et al. Biopsyproved idiopathic pulmonary fibrosis: spectrum of nondiagnostic thin-section CT diagnoses. Radiology 2010;254:957-964.
51. 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.
52. Silva CI, Müller NL, Hansell DM, Lee KS, Nicholson AG, Wells AU. Nonspecific interstitial pneumonia and idiopathic pulmonary fibrosis: changes in pattern and distribution of disease over time. Radiology 2008;247:251-259.
53. Lynch DA, Godwin JD, Safrin S, Starko KM, Hormel P, Brown KK, Raghu G, King TE Jr, Bradford WZ, Schwartz DA, 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.
54. Kim EY, Lee KS, Chung MP, Kwon OJ, Kim TS, Hwang JH. Nonspecific interstitial pneumonia with fibrosis: serial highresolution CT findings with functional correlation. AJR Am J Roentgenol 1999;173:949-953.
55. Hartman TE, Swensen SJ, Hansell DM, Colby TV, Myers JL, Tazelaar HD, Nicholson AG, Wells AU, Ryu JH, Midthun DE, et al. Nonspecific interstitial pneumonia: variable appearance at high-resolution chest CT. Radiology 2000;217:701-705.
56. [Published erratum appears in Radiology 218:606.]
57. Park JS, Lee KS, Kim JS, Park CS, Suh YL, Choi DL, Kim KJ. Nonspecific interstitial pneumonia with fibrosis: radiographic and CT findings in seven patients. Radiology 1995;195:645-648.
58. Cottin V, Donsbeck AV, Revel D, Loire R, Cordier JF. Nonspecific interstitial pneumonia: individualization of a clinicopathologic entity in a series of 12 patients. Am J Respir Crit Care Med 1998;158:1286-1293.
59. 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.
60. Johkoh T, Müller NL, Colby TV, Ichikado K, Taniguchi H, Kondoh Y, Fujimoto K, Kinoshita M, Arakawa H, Yamada H, et al. Nonspecific interstitial pneumonia: correlation between thin-section CT findings and pathologic subgroups in 55 patients. Radiology 2002;225:199-204.
61. Johkoh T, Müller NL, Cartier Y, Kavanagh PV, Hartman TE, Akira M, Ichikado K, Ando M, Nakamura H. Idiopathic interstitial pneumonias: diagnostic accuracy of thin-section CT in 129 patients. Radiology 1999;211:555-560.
62. Nishiyama O, Kondoh Y, Taniguchi H, Yamaki K, Suzuki R, Yokoi T, Takagi K. Serial high resolution CT findings in nonspecific interstitial pneumonia/fibrosis. J Comput Assist Tomogr 2000;24:41-46.
63. MacDonald SL, Rubens MB, Hansell DM, Copley SJ, Desai SR, du Bois RM, Nicholson AG, Colby TV, Wells AU. Nonspecific interstitial pneumonia and usual interstitial pneumonia: comparative appearances at and diagnostic accuracy of thin-section CT. Radiology 2001;221:600-605.
64. Akira M, Inoue Y, Arai T, Okuma T, Kawata Y. Long-term follow-up high-resolution CT findings in non-specific interstitial pneumonia. Thorax 2011;66:61-65.
65. Katzenstein AL, Fiorelli RF. Nonspecific interstitial pneumonia/fibrosis: histologic features and clinical significance. Am J Surg Pathol 1994;18:136-147.
66. Travis WD, Matsui K, Moss J, Ferrans VJ. Idiopathic nonspecific interstitial pneumonia: prognostic significance of cellular and fibrosing patterns: survival comparison with usual interstitial pneumonia and desquamative interstitial pneumonia. Am J Surg Pathol 2000;24:19-33.
67. Tsubamoto M, Müller NL, Johkoh T, Ichikado K, Taniguchi H, Kondoh Y, Fujimoto K, Arakawa H, Koyama M, Kozuka T, et al. Pathologic subgroups of nonspecific interstitial pneumonia: differential diagnosis from other idiopathic interstitial pneumonias on high-resolution computed tomography. J Comput Assist Tomogr 2005;29:793-800.
68. Park IN, Jegal Y, Kim DS, Do KH, Yoo B, Shim TS, Lim CM, Lee SD, Koh Y, Kim WS, et al. Clinical course and lung function change of idiopathic nonspecific interstitial pneumonia. Eur Respir J 2009;33:68-76.
69. Hidalgo A, Franquet T, Giménez A, Bordes R, Pineda R, Madrid M. Smoking-related interstitial lung diseases: radiologic-pathologic correlation. Eur Radiol 2006;16:2463-2470.
70. Craig PJ, Wells AU, Doffman S, Rassl D, Colby TV, Hansell DM, Du Bois RM, Nicholson AG. Desquamative interstitial pneumonia, respiratory bronchiolitis and their relationship to smoking. Histopathology 2004;45:275-282.
71. Fraig M, Shreesha U, Savici D, Katzenstein AL. Respiratory bronchiolitis: a clinicopathologic study in current smokers, ex-smokers, and never-smokers. Am J Surg Pathol 2002;26:647-653.
72. Vassallo R, Ryu JH. Tobacco smoke-related diffuse lung diseases. Semin Respir Crit Care Med 2008;29:643-650.
73. Portnoy J, Veraldi KL, Schwarz MI, Cool CD, Curran-Everett D, Cherniack RM, King TE Jr, Brown KK. Respiratory bronchiolitisinterstitial lung disease: long-term outcome. Chest 2007;131:664-671.
74. Doan ML, Guillerman RP, Dishop MK, Nogee LM, Langston C, Mallory GB, Sockrider MM, Fan LL. Clinical, radiological and pathological features of ABCA3 mutations in children. Thorax 2008;63:366-373.
75. Bullard JE, Wert SE, Whitsett JA, Dean M, Nogee LM. ABCA3 mutations associated with pediatric interstitial lung disease. Am J Respir Crit Care Med 2005;172:1026-1031.
76. Kawabata Y, Hoshi E, Murai K, Ikeya T, Takahashi N, Saitou Y, Kurashima K, Ubukata M, Takayanagi N, Sugita H, et al. Smoking-related changes in the background lung of specimens resected for lung cancer: a semiquantitative study with correlation to postoperative course. Histopathology 2008;53:707-714.
77. Katzenstein AL, Mukhopadhyay S, Zanardi C, Dexter E. Clinically occult interstitial fibrosis in smokers: classification and significance of a surprisingly common finding in lobectomy specimens. Hum Pathol 2010;41:316-325.
78. Snider GL, Kleinerman J, Thurlbeck WM, Bengali ZH. The definition of emphysema: report of a National Heart, Lung, and Blood Institute, Division of Lung Diseases workshop. Am Rev Respir Dis 1985;132:182-185.
79. King TE Jr, Mortenson RL. Cryptogenic organizing pneumonitis: the North American experience. Chest 1992;102 (1, Suppl) 8S-13S.
80. Lee JW, Lee KS, Lee HY, Chung MP, Yi CA, Kim TS, Chung MJ. Cryptogenic organizing pneumonia: serial high-resolution CT findings in 22 patients. AJR Am J Roentgenol 2010;195:916-922.
81. Sen T, Udwadia ZF. Cryptogenic organizing pneumonia: clinical profile in a series of 34 admitted patients in a hospital in India. J Assoc Physicians India 2008;56:229-232.
82. Oymak FS, Demirba̧ HM, Mavili E, Akgun H, Gulmez I, Demir R, Ozesmi M. Bronchiolitis obliterans organizing pneumonia: clinical and roentgenological features in 26 cases. Respiration 2005;72:254-262.
83. Lee JS, Lynch DA, Sharma S, Brown KK, Müller NL. Organizing pneumonia: prognostic implication of high-resolution computed tomography features. J Comput Assist Tomogr 2003;27:260-265.
84. Lee KS, Kullnig P, Hartman TE, Müller NL. Cryptogenic organizing pneumonia: CT findings in 43 patients. AJR Am J Roentgenol 1994;162:543-546.
85. Müller NL, Staples CA, Miller RR. Bronchiolitis obliterans organizing pneumonia: CT features in 14 patients. AJR Am J Roentgenol 1990;154:983-987.
86. Ujita M, Renzoni EA, Veeraraghavan S, Wells AU, Hansell DM. Organizing pneumonia: perilobular pattern at thin-section CT. Radiology 2004;232:757-761.
87. Kim SJ, Lee KS, Ryu YH, Yoon YC, Choe KO, Kim TS, Sung KJ. Reversed halo sign on high-resolution CT of cryptogenic organizing pneumonia: diagnostic implications. AJR Am J Roentgenol 2003;180:1251-1254.
88. Epler GR, Colby TV, McLoud TC, Carrington CB, Gaensler EA. Bronchiolitis obliterans organizing pneumonia. N Engl J Med 1985;312:152-158.
89. Lohr RH, Boland BJ, Douglas WW, Dockrell DH, Colby TV, Swensen SJ, Wollan PC, Silverstein MD. Organizing pneumonia: features and prognosis of cryptogenic, secondary, and focal variants. Arch Intern Med 1997;157:1323-1329.
90. Lazor R, Vandevenne A, Pelletier A, Leclerc P, Court-Fortune I, Cordier JF; Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM "O" P). Cryptogenic organizing pneumonia: characteristics of relapses in a series of 48 patients. Am J Respir Crit Care Med 2000;162:571-577.
91. Fischer A, Swigris JJ, du Bois RM, Lynch DA, Downey GP, Cosgrove GP, Frankel SK, Fernandez-Perez ER, Gillis JZ, Brown KK. Anti-synthetase syndrome in ANA and anti-Jo-1 negative patients presenting with idiopathic interstitial pneumonia. Respir Med 2009;103:1719-1724.
92. Bouros D, Nicholson AC, Polychronopoulos V, du Bois RM. Acute interstitial pneumonia. Eur Respir J 2000;15:412-418.
93. Vourlekis JS, Brown KK, Cool CD, Young DA, Cherniack RM, King TE, Schwarz MI. Acute interstitial pneumonitis: case series and review of the literature. Medicine (Baltimore) 2000;79:369-378.
94. Ichikado K, Suga M, Müller NL, Taniguchi H, Kondoh Y, Akira M, Johkoh T, Mihara N, Nakamura H, Takahashi M, et al. Acute interstitial pneumonia: comparison of high-resolution computed tomography findings between survivors and nonsurvivors. Am J Respir Crit Care Med 2002;165:1551-1556.
95. Primack SL, Hartman TE, Ikezoe J, Akira M, Sakatani M, Müller NL. Acute interstitial pneumonia: radiographic and CT findings in nine patients. Radiology 1993;188:817-820.
96. Johkoh T, Müller NL, Taniguchi H, Kondoh Y, Akira M, Ichikado K, Ando M, Honda O, Tomiyama N, Nakamura H. Acute interstitial pneumonia: thin-section CT findings in 36 patients. Radiology 1999;211:859-863.
97. Desai SR, Wells AU, Rubens MB, Evans TW, Hansell DM. Acute respiratory distress syndrome: CT abnormalities at long-term followup. Radiology 1999;210:29-35.
98. Ichikado K, Suga M, Muranaka H, Gushima Y, Miyakawa H, Tsubamoto M, Johkoh T, Hirata N, Yoshinaga T, Kinoshita Y, et al. Prediction of prognosis for acute respiratory distress syndrome with thin-section CT: validation in 44 cases. Radiology 2006;238:321-329.
99. Araya J, Kawabata Y, Jinho P, Uchiyama T, Ogata H, Sugita Y. Clinically occult subpleural fibrosis and acute interstitial pneumonia a precursor to idiopathic pulmonary fibrosis? Respirology 2008;13:408-412.
100. Rice AJ, Wells AU, Bouros D, du Bois RM, Hansell DM, Polychronopoulos V, Vassilakis D, Kerr JR, Evans TW, Nicholson AG. Terminal diffuse alveolar damage in relation to interstitial pneumonias: an autopsy study. Am J Clin Pathol 2003;119:709-714.
101. Song JW, Hong SB, Lim CM, Koh Y, Kim DS. Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome. Eur Respir J 2011;37:356-363.
102. Agarwal R, Jindal SK. Acute exacerbation of idiopathic pulmonary fibrosis: a systematic review. Eur J Intern Med 2008;19:227-235.
103. Silva C I S, Müller 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.
104. Churg A, Müller NL, Silva C I S, Wright JL. 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.
105. Suda T, Kaida Y, Nakamura Y, Enomoto N, Fujisawa T, Imokawa S, Hashizume H, Naito T, Hashimoto D, Takehara Y, et al. Acute exacerbation of interstitial pneumonia associated with collagen vascular diseases. Respir Med 2009;103:846-853.
106. Park IN, Kim DS, Shim TS, Lim C-M, Lee SD, Koh Y, Kim WS, Kim WD, Jang SJ, Colby TV. Acute exacerbation of interstitial pneumonia other than idiopathic pulmonary fibrosis. Chest 2007;132:214-220.
107. Miyazaki Y, Tateishi T, Akashi T, Ohtani Y, Inase N, Yoshizawa Y. Clinical predictors and histologic appearance of acute exacerbations in chronic hypersensitivity pneumonitis. Chest 2008;134:1265-1270.
108. Collard HR, Moore BB, Flaherty KR, Brown KK, Kaner RJ, King TE Jr, Lasky JA, Loyd JE, Noth I, Olman MA, 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.
109. Akira M, Kozuka T, Yamamoto S, Sakatani M. Computed tomography findings in acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2008;178:372-378.
110. Akira M, Hamada H, Sakatani M, Kobayashi C, Nishioka M, Yamamoto S. CT findings during phase of accelerated deterioration in patients with idiopathic pulmonary fibrosis. AJR Am J Roentgenol 1997;168:79-83.
111. Fujimoto K, Taniguchi H, Johkoh T, Kondoh Y, Ichikado K, Sumikawa H, Ogura T, Kataoka K, Endo T, Kawaguchi A, et al. Acute exacerbation of idiopathic pulmonary fibrosis: high-resolution CT scores predict mortality. Eur Radiol 2012;22:83-92.
112. Cha SI, Fessler MB, Cool CD, Schwarz MI, Brown KK. Lymphoid interstitial pneumonia: clinical features, associations and prognosis. Eur Respir J 2006;28:364-369.
113. Johkoh T, Müller NL, Pickford HA, Hartman TE, Ichikado K, Akira M, Honda O, Nakamura H. Lymphocytic interstitial pneumonia: thin-section CT findings in 22 patients. Radiology 1999;212:567-572.
114. Reddy TL, Tominaga M, Hansell DM, von der Thusen J Jr, Rassl D, Parfrey H, Guy S, Twentyman O, Rice A, Maher TM, et al. Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur Respir J 2012;40:377-385.
115. Becker CD, Gil J, Padilla ML. Idiopathic pleuroparenchymal fibroelastosis: an unrecognized or misdiagnosed entity? Mod Pathol 2008;21:784-787.
116. Frankel SK, Cool CD, Lynch DA, Brown KK. Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity. Chest 2004;126:2007-2013.
117. von der Thüsen JH, Hansell DM, Tominaga M, Veys PA, Ashworth MT, Owens CM, Nicholson AG. Pleuroparenchymal fibroelastosis in patients with pulmonary disease secondary to bone marrow transplantation. Mod Pathol 2011;24:1633-1639.
118. Amitani R, Niimi A, Kuze F. Idiopathic pulmonary upper lobe fibrosis (IPUF) [in Japanese]. Kokyu 1992;11:693-699.
119. Watanabe K, Nagata N, Kitasato Y, Wakamatsu K, Nabeshima K, Harada T, Hirota T, Shiraishi M, Fujita M. Rapid decrease in forced vital capacity in patients with idiopathic pulmonary upper lobe fibrosis. Respir Investig 2012;50:88-97.
120. Beasley MB, Franks TJ, Galvin JR, Gochuico B, Travis WD. Acute fibrinous and organizing pneumonia: a histological pattern of lung injury and possible variant of diffuse alveolar damage. Arch Pathol Lab Med 2002;126:1064-1070.
121. Balduin R, Giacometti C, Saccarola L, Marulli G, Rea F, Bartoli M, Agostini C, Calabrese F. Acute fibrinous and organizing pneumonia in a patient with collagen vascular disease "stigma." Sarcoidosis Vasc Diffuse Lung Dis 2007;24:78-80.
122. Hariri LP, Mino-Kenudson M, Shea B, Digumarthy S, Onozato M, Yagi Y, Fraire AE, Matsubara O, Mark EJ. Distinct histopathology of acute onset or abrupt exacerbation of hypersensitivity pneumonitis. Hum Pathol 2012;43:660-668.
123. Yokogawa N, Alcid DV. Acute fibrinous and organizing pneumonia as a rare presentation of abacavir hypersensitivity reaction. AIDS 2007;21:2116-2117.
124. Churg A, Myers J, Suarez T, Gaxiola M, Estrada A, Mejia M, Selman M. Airway-centered interstitial fibrosis: a distinct form of aggressive diffuse lung disease. Am J Surg Pathol 2004;28:62-68.
125. Fukuoka J, Franks TJ, Colby TV, Flaherty KR, Galvin JR, Hayden D, Gochuico BR, Kazerooni EA, Martinez F, Travis WD. Peribronchiolar metaplasia: a common histologic lesion in diffuse lung disease and a rare cause of interstitial lung disease: clinicopathologic features of 15 cases. Am J Surg Pathol 2005;29:948-954.
126. Yousem SA, Dacic S. Idiopathic bronchiolocentric interstitial pneumonia. Mod Pathol 2002;15:1148-1153.
127. Mark EJ, Ruangchira Urai R. Bronchiolitis interstitial pneumonitis: a pathologic study of 31 lung biopsies with features intermediate between bronchiolitis obliterans organizing pneumonia and usual interstitial pneumonitis, with clinical correlation. Ann Diagn Pathol 2008;12:171-180.
128. Ryerson CJ, Urbania TH, Richeldi L, Mooney JJ, Lee JS, Jones KD, Elicker BM, Koth LL, King TE Jr, Wolters PJ, et al. Prevalence and prognosis of unclassifiable interstitial lung disease. Eur Respir J 2013;42:750-757.
129. Kinder BW, Brown KK, McCormack FX, Ix JH, Kervitsky A, Schwarz MI, King TE Jr. Serum surfactant protein-A is a strong predictor of early mortality in idiopathic pulmonary fibrosis. Chest 2009;135:1557-1563.
130. Satoh H, Kurishima K, Ishikawa H, Ohtsuka M. Increased levels of KL-6 and subsequent mortality in patients with interstitial lung diseases. J Intern Med 2006;260:429-434.
131. Prasse A, Probst C, Bargagli E, Zissel G, Toews GB, Flaherty KR, Olschewski M, Rottoli P, Müller-Quernheim J. Serum CC-chemokine ligand 18 concentration predicts outcome in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2009;179:717-723.
132. Prasse A, Pechkovsky DV, Toews GB, Schäfer M, Eggeling S, Ludwig C, Germann M, Kollert F, Zissel G, Müller-Quernheim J. CCL18 as an indicator of pulmonary fibrotic activity in idiopathic interstitial pneumonias and systemic sclerosis. Arthritis Rheum 2007;56:1685-1693.
133. Ishii H, Mukae H, Kadota J, Kaida H, Nagata T, Abe K, Matsukura S, Kohno S. High serum concentrations of surfactant protein A in usual interstitial pneumonia compared with non-specific interstitial pneumonia. Thorax 2003;58:52-57.
134. Ohnishi H, Yokoyama A, Kondo K, Hamada H, Abe M, Nishimura K, Hiwada K, Kohno N. Comparative study of KL-6, surfactant protein-A, surfactant protein-D, and monocyte chemoattractant protein-1 as serum markers for interstitial lung diseases. Am J Respir Crit Care Med 2002;165:378-381.
135. Casoni GL, Ulivi P, Mercatali L, Chilosi M, Tomassetti S, Romagnoli M, Ravaglia C, Gurioli C, Gurioli C, Zoli W, et al. Increased levels of free circulating DNA in patients with idiopathic pulmonary fibrosis. Int J Biol Markers 2010;25:229-235.
136. Pignatti P, Brunetti G, Moretto D, Yacoub M-R, Fiori M, Balbi B, Balestrino A, Cervio G, Nava S, Moscato G. Role of the chemokine receptors CXCR3 and CCR4 in human pulmonary fibrosis. Am J Respir Crit Care Med 2006;173:310-317.
137. Ishii H, Mukae H, Kadota J, Fujii T, Abe K, Ashitani J, Kohno S. Increased levels of interleukin-18 in bronchoalveolar lavage fluid of patients with idiopathic nonspecific interstitial pneumonia. Respiration 2005;72:39-45.
138. Choi ES, Pierce EM, Jakubzick C, Carpenter KJ, Kunkel SL, Evanoff H, Martinez FJ, Flaherty KR, Moore BB, Toews GB, et al. Focal interstitial CC chemokine receptor 7 (CCR7) expression in idiopathic interstitial pneumonia. J Clin Pathol 2006;59:28-39.
139. Choi ES, Jakubzick C, Carpenter KJ, Kunkel SL, Evanoff H, Martinez FJ, Flaherty KR, Toews GB, Colby TV, Kazerooni EA, et al. Enhanced monocyte chemoattractant protein-3/CC chemokine ligand-7 in usual interstitial pneumonia. Am J Respir Crit Care Med 2004;170:508-515.
140. Yang IV, Burch LH, Steele MP, Savov JD, Hollingsworth JW, McElvania-Tekippe E, Berman KG, Speer MC, Sporn TA, Brown KK, et al. Gene expression profiling of familial and sporadic interstitial pneumonia. Am J Respir Crit Care Med 2007;175:45-54.
141. Lawson WE, Grant SW, Ambrosini V, Womble KE, Dawson EP, Lane KB, Markin C, Renzoni E, Lympany P, Thomas AQ, et al. Genetic mutations in surfactant protein C are a rare cause of sporadic cases of IPF. Thorax 2004;59:977-980.
142. Kim KK, Flaherty KR, Long Q, Hattori N, Sisson TH, Colby TV, Travis WD, Martinez FJ, Murray S, Simon RH. A plasminogen activator inhibitor-1 promoter polymorphism and idiopathic interstitial pneumonia. Mol Med 2003;9:52-56.
143. Markart P, Ruppert C, Wygrecka M, Schmidt R, Korfei M, Harbach H, Theruvath I, Pison U, Seeger W, Guenther A, et al. Surfactant protein C mutations in sporadic forms of idiopathic interstitial pneumonias. Eur Respir J 2007;29:134-137.
144. Zorzetto M, Ferrarotti I, Campo I, Trisolini R, Poletti V, Scabini R, Ceruti M, Mazzola P, Crippa E, Ottaviani S, et al. NOD2/CARD15 gene polymorphisms in idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis 2005;22:180-185.
145. Hutyrová B, Pantelidis P, Drábek J, Zùrková M, Kolek V, Lenhart K, Welsh KI, Du Bois RM, Petrek M. Interleukin-1 gene cluster polymorphisms in sarcoidosis and idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2002;165:148-151.
146. Renzoni E, Lympany P, Sestini P, Pantelidis P, Wells A, Black C, Welsh K, Bunn C, Knight C, Foley 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.
147. Zorzetto M, Ferrarotti I, Trisolini R, Lazzari Agli L, Scabini R, Novo M, De Silvestri A, Patelli M, Martinetti M, Cuccia M, et al. Complement receptor 1 gene polymorphisms are associated with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2003;168:330-334.
148. Hodgson U, Tukiainen P, Laitinen T. The polymorphism C5507G of complement receptor 1 does not explain idiopathic pulmonary fibrosis among the Finns. Respir Med 2005;99:265-267.
149. Kubistova Z, Mrazek F, Lympany PA, Lagan AL, Arakelyan A, Kriegova E, Welsh KI, Kolek V, Zatloukal J, Hutyrova B, et al. The CR1 C5507G polymorphism is not involved in susceptibility to idiopathic pulmonary fibrosis in two European populations. Tissue Antigens 2008;72:483-486.
150. Mushiroda T, Wattanapokayakit S, Takahashi A, Nukiwa T, Kudoh S, Ogura T, Taniguchi H, Kubo M, Kamatani N, Nakamura Y; Pirfenidone Clinical Study Group. 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.
151. Selman M, Lin H-M, Montaño M, Jenkins AL, Estrada A, Lin Z, Wang G, Di Angelo SL, Guo X, Umstead TM, et al. Surfactant protein A and B genetic variants predispose to idiopathic pulmonary fibrosis. Hum Genet 2003;113:542-550.
152. Falfán-Valencia R, Camarena A, Juárez A, Becerril C, Montaño M, Cisneros J, Mendoza F, Granados J, Pardo A, Selman M. Major histocompatibility complex and alveolar epithelial apoptosis in idiopathic pulmonary fibrosis. Hum Genet 2005;118:235-244.
153. Checa M, Ruiz V, Montaño M, Velázquez-Cruz R, Selman M, Pardo A. MMP-1 polymorphisms and the risk of idiopathic pulmonary fibrosis. Hum Genet 2008;124:465-472.
154. Matsushima H, Takayanagi N, Sakamoto T, Kawata I, Motegi M, Ubukata M, Yanagisawa T, Sugita Y, Kanazawa M, Kawabata Y. Pathologic findings both before and after steroid therapy in a case of desquamative interstitial pneumonia [article in Japanese]. Nihon Kokyuki Gakkai Zasshi 2001;39:609-614.
155. 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.
156. 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.
157. 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.
158. 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.
159. Takahashi H, Shiratori M, Kanai A, Chiba H, Kuroki Y, Abe S. Monitoring markers of disease activity for interstitial lung diseases with serum surfactant proteins A and D. Respirology 2006;11:S51-S54.
160. Daniil ZD, Papageorgiou E, Koutsokera A, Kostikas K, Kiropoulos T, Papaioannou AI, Gourgoulianis KI. Serum levels of oxidative stress as a marker of disease severity in idiopathic pulmonary fibrosis. Pulm Pharmacol Ther 2008;21:26-31.
161. Rosas IO, Richards TJ, Konishi K, Zhang Y, Gibson K, Lokshin AE, Lindell KO, Cisneros J, Macdonald SD, Pardo A, Sciurba F, Dauber J, Selman M, Gochuico BR, Kaminski N. MMP1 and MMP7 as potential peripheral blood biomarkers in idiopathic pulmonary fibrosis. PLoS Med 2008;5:e93.
162. Gilani SR, Vuga LJ, Lindell KO, Gibson KF, Xue J, Kaminski N, Valentine VG, Lindsay EK, George MP, Steele C, et al. CD28 down-regulation on circulating CD4 T-cells is associated with poor prognoses of patients with idiopathic pulmonary fibrosis. PLoS One 2010;5:e8959.
163. Richards TJ, Kaminski N, Baribaud F, Flavin S, Brodmerkel C, Horowitz D, Li K, Choi J, Vuga LJ, Lindell KO, Klesen M, Zhang Y, Gibson KF. Peripheral blood proteins predict mortality in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2012;185:67-76.
164. 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.
165. 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.
166. Richter AG, McKeown S, Rathinam S, Harper L, Rajesh P, McAuley DF, Heljasvaara R, Thickett DR. Soluble endostatin is a novel inhibitor of epithelial repair in idiopathic pulmonary fibrosis. Thorax 2009;64:156-161.