What's in a name? That which we call IPF, by any other name would act the same

European Respiratory Journal

Volumn 51, Issue 5, 2018, Pages

  Wells, Athol U ^a   Brown, Kevin K ^b   Flaherty, Kevin R ^c   Kolb, Martin ^d   Thannickal, Victor J ^e  

^a ROYAL BROMPTON HOSPITAL   (United Kingdom)

^b DEPARTMENT OF MEDICINE   (United States)

^c UNIVERSITY OF MICHIGAN HEALTH SYSTEM   (United States)

^d MCMASTER UNIVERSITY   (Canada)

^e University of Alabama at Birmingham   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

TELOMERASE REVERSE TRANSCRIPTASE;

DISEASE CLASSIFICATION; ENVIRONMENTAL FACTOR; FIBROSING ALVEOLITIS; GENE; GENE MUTATION; GENETIC PREDISPOSITION; HEREDITY; HUMAN; MOLECULAR PATHOLOGY; MUTATIONAL ANALYSIS; OXIDATIVE STRESS; PARN GENE; PERSONALIZED MEDICINE; PHENOTYPE; PRIORITY JOURNAL; REVIEW; RTEL1 GENE; SFTPA1 GENE; SFTPA2 GENE; SFTPC GENE; SINGLE NUCLEOTIDE POLYMORPHISM; TERC GENE; TERT GENE; CLASSIFICATION; DISEASE EXACERBATION; HISTORY; NOMENCLATURE; PATHOPHYSIOLOGY;

DISEASE PROGRESSION; HISTORY, 20TH CENTURY; HISTORY, 21ST CENTURY; HUMANS; IDIOPATHIC PULMONARY FIBROSIS; PHENOTYPE; TERMINOLOGY AS TOPIC;

EID: 85051490518     PISSN: 09031936     EISSN: 13993003     Source Type: Journal    
DOI: 10.1183/13993003.00692-2018     Document Type: Review

References (105)

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. 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.
3. 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.
4. Prescott HC, Calfee CS, Thompson BT, et al. Toward smarter lumping and smarter splitting: Rethinking strategies for sepsis and acute respiratory distress syndrome clinical trial design. Am J Respir Crit Care Med 2016; 194: 147-155.
5. Wolters PJ, Blackwell TS, Eickelberg O, et al. Time for a change: Is idiopathic pulmonary fibrosis still idiopathic and only fibrotic? Lancet Respir Med 2018; 6: 154-160.
6. Thannickal VJ, Wells A, Kolb M. Idiopathic pulmonary fibrosis: Idiopathic no more? Lancet Respir Med 2018; 6: 84-85.
7. Seibold MA, Wise AL, Speer MC, et al. A common MUC5B promoter polymorphism and pulmonary fibrosis. N Engl J Med 2011; 364: 1503-1512.
8. Selman M, King TE, Pardo A, et al. Idiopathic pulmonary fibrosis: Prevailing and evolving hypotheses about its pathogenesis and implications for therapy. Ann Intern Med 2001; 134: 136-151.
9. Thannickal VJ, Zhou Y, Gaggar A, et al. Fibrosis: Ultimate and proximate causes. J Clin Invest 2014; 124: 4673-4677.
10. Borie R, Kannengiesser C, Sicre de Fontbrune F, et al. Management of suspected monogenic lung fibrosis in a specialised centre. Eur Respir Rev 2017; 26: 160122.
11. Selman M, López-Otín C, Pardo A. Age-driven developmental drift in the pathogenesis of idiopathic pulmonary fibrosis. Eur Respir J 2016; 48: 538-552.
12. Korfei M, Skwarna S, Henneke I, et al. Aberrant expression and activity of histone deacetylases in sporadic idiopathic pulmonary fibrosis. Thorax 2015; 70: 1022-1032.
13. Feghali-Bostwick CA, Tsai CG, Valentine VG, et al. Cellular and humoral autoreactivity in idiopathic pulmonary fibrosis. J Immunol 2007; 179: 2592-2599.
14. Donahoe M, Valentine VG, Chien N, et al. Autoantibody-targeted treatments for acute exacerbations of idiopathic pulmonary fibrosis. PLoS One 2015; 10: E0127771.
15. Zhou Y, Huang X, Hecker L, et al. Inhibition of mechanosensitive signaling in myofibroblasts ameliorates experimental pulmonary fibrosis. J Clin Invest 2013; 123: 1096-1108.
16. Parker MW, Rossi D, Peterson M, et al. Fibrotic extracellular matrix activates a profibrotic positive feedback loop. J Clin Invest 2014; 124: 1622-1635.
17. Hecker L, Logsdon NJ, Kurundkar D, et al. Reversal of persistent fibrosis in aging by targeting Nox4-Nrf2 redox imbalance. Sci Transl Mede 2014; 6: 231ra47.
18. Chanda D, Kurundkar A, Rangarajan S, et al. Developmental reprogramming in mesenchymal stromal cells of human subjects with idiopathic pulmonary fibrosis. Sci Rep 2016; 6: 37445.
19. Travis WD, Costabel U, Hansell DM, et al. An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 2013; 188: 733-748.
20. Fingerlin TE, Murphy E, Zhang W, et al. Genome-wide association study identifies multiple susceptibility loci for pulmonary fibrosis. Nat Genet 2013; 45: 613-620.
21. Oldham JM, Ma SF, Martinez FJ, et al. TOLLIP, MUC5B, and the response to N-Acetylcysteine among individuals with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2015; 192: 1475-1482.
22. 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.
23. DePianto DJ, Chandriani S, Abbas AR, et al. Heterogeneous gene expression signatures correspond to distinct lung pathologies and biomarkers of disease severity in idiopathic pulmonary fibrosis. Thorax 2015; 70: 48-56.
24. Pennathur S, Vivekanandan-Giri A, Locy ML, et al. Oxidative modifications of protein tyrosyl residues are increased in plasma of human subjects with interstitial lung disease. Am J Respir Crit Care Med 2016; 193: 861-868.
25. Surolia R, Li FJ, Wang Z, et al. 3D pulmospheres serve as a personalized and predictive multicellular model for assessment of antifibrotic drugs. JCI Insight 2017; 2: E91377.
26. White ES, Xia M, Murray S, et al. Plasma surfactant protein-D, matrix metalloproteinase-7, and osteopontin index distinguishes idiopathic pulmonary fibrosis from other idiopathic interstitial pneumonias. Am J Respir Crit Care Med 2016; 194: 1242-1251.
27. Mathai SK, Newton CA, Schwartz DA, et al. Pulmonary fibrosis in the era of stratified medicine. Thorax 2016; 71: 1154-1160.
28. Spagnolo P, Cottin V. Genetics of idiopathic pulmonary fibrosis: From mechanistic pathways to personalised medicine. J Med Genet 2017; 54: 93-99.
29. Spagnolo P, Tzouvelekis A, Maher TM. Personalized medicine in idiopathic pulmonary fibrosis: Facts and promises. Curr Opin Pulm Med 2015; 21: 470-478.
30. Sato S, Kolb MRJ. Personalised medicine for IPF: Getting closer, but not there yet. Lancet Respir Med 2017; 5: 836-837.
31. Ramsey BW, Davies J, McElvaney NG, et al. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med 2011; 365: 1663-1672.
32. Bel EH, Wenzel SE, Thompson PJ, et al. Oral glucocorticoid-sparing effect of mepolizumab in eosinophilic asthma. N Engl J Med 2014; 371: 1189-1197.
33. Nair P, Wenzel S, Rabe KF, et al. Oral glucocorticoid-sparing effect of benralizumab in severe asthma. N Engl J Med 2017; 376: 2448-2458.
34. Corren J, Lemanske RF, Hanania NA, et al. Lebrikizumab treatment in adults with asthma. N Engl J Med 2011; 365: 1088-1098.
35. Hanania NA, Korenblat P, Chapman KR, et al. Efficacy and safety of lebrikizumab in patients with uncontrolled asthma (LAVOLTA I and LAVOLTA II): Replicate, phase 3, randomised, double-blind, placebo-controlled trials. Lancet Respir Med 2016; 4: 781-796.
36. 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.
37. Kahloon RA, Xue J, Bhargava A, et al. Patients with idiopathic pulmonary fibrosis with antibodies to heat shock protein 70 have poor prognoses. Am J Respir Crit Care Med 2013; 187: 768-775.
38. King TE Jr, Bradford WZ, Castro-Bernardini S, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med 2014; 370: 2083-2092.
39. Raghu G, Wells AU, Nicholson AG, et al. Effect of nintedanib in subgroups of idiopathic pulmonary fibrosis by diagnostic criteria. Am J Respir Crit Care Med 2017; 195: 78-85.
40. Wells AU. Approach to diagnosis of diffuse lung disease. In: Albert RK, Spiro SG, Jett JR, eds. Clinical Respiratory Medicine. 2nd Edn. Mosby, Elsevier Science, 2004.
41. Tashkin DP, Elashoff R, Clements PJ, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 2006; 354: 2655-2666.
42. Tashkin DP, Roth MD, Clements PJ, et al. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): A randomised controlled, double-blind, parallel group trial. Lancet Respir Med 2016; 4: 708-719.
43. Fischer A, Brown KK, Du Bois RM, et al. Mycophenolate mofetil improves lung function in connective tissue disease-associated interstitial lung disease. J Rheumatol 2013; 40: 640-646.
44. Morisset J, Johannson KA, Vittinghoff E, et al. Use of mycophenolate mofetil or azathioprine for the management of chronic hypersensitivity pneumonitis. Chest 2017; 151: 619-625.
45. Adegunsoye A, Oldham JM, Fernández Pérez ER, et al. Outcomes of immunosuppressive therapy in chronic hypersensitivity pneumonitis. ERJ Open Res 2017; 3: 00016-2017.
46. Gaxiola M, Buendía-Roldán I, Mejía M, et al. Morphologic diversity of chronic pigeon breeder's disease: Clinical features and survival. Respir Med 2011; 105: 608-614.
47. Wang P, Jones KD, Urisman A, et al. Pathologic findings and prognosis in a large prospective cohort of chronic hypersensitivity pneumonitis. Chest 2017; 152: 502-509.
48. Pérez-Padilla R, Salas J, Chapela R, et al. Mortality in Mexican patients with chronic pigeon breeder's lung compared with those with usual interstitial pneumonia. Am Rev Respir Dis 1993; 148: 49-53.
49. Ohtani Y, Saiki S, Kitaichi M, et al. Chronic bird fancier's lung: Histopathological and clinical correlation. An application of the 2002 ATS/ERS consensus classification of the idiopathic interstitial pneumonias. Thorax 2005; 60: 665-671.
50. Churg A, Sin DD, Everett D, et al. Pathologic patterns and survival in chronic hypersensitivity pneumonitis. Am J Surg Pathol 2009; 33: 1765-1770.
51. Walsh SL, Sverzellati N, Devaraj A, et al. Chronic hypersensitivity pneumonitis: High resolution computed tomography patterns and pulmonary function indices as prognostic determinants. Eur Radiol 2012; 22: 1672-1679.
52. 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.
53. Tsuchiya Y, Takayanagi N, Sugiura H, et al. Lung diseases directly associated with rheumatoid arthritis and their relationship to outcome. Eur Respir J 2011; 37: 1411-1417.
54. Moua T, Zamora Martinez AC, Baqir M, et al. Predictors of diagnosis and survival in idiopathic pulmonary fibrosis and connective tissue disease-related usual interstitial pneumonia. Respir Res 2014; 15: 154.
55. Nakamura Y, Suda T, Kaida Y, et al. Rheumatoid lung disease: Prognostic analysis of 54 biopsy-proven cases. Respir Med 2012; 106: 1164-1169.
56. Song JW, Lee HK, Lee CK, et al. Clinical course and outcome of rheumatoid arthritis-related usual interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis 2013; 30: 103-112.
57. Kim EJ, Elicker BM, Maldonado F, et al. Usual interstitial pneumonia in rheumatoid arthritis-associated interstitial lung disease. Eur Respir J 2010; 35: 1322-1328.
58. Kelly CA, Saravanan V, Nisar M, et al. Rheumatoid arthritis-related interstitial lung disease: Associations, prognostic factors and physiological and radiological characteristics-a large multicentre UK study. Rheumatology (Oxford) 2014; 53: 1676-1682.
59. Solomon JJ, Chung JH, Cosgrove GP, et al. Predictors of mortality in rheumatoid arthritis-associated interstitial lung disease. Eur Respir J 2016; 47: 588-596.
60. Yunt ZX, Chung JH, Hobbs S, et al. High resolution computed tomography pattern of usual interstitial pneumonia in rheumatoid arthritis-associated interstitial lung disease: Relationship to survival. Respir Med 2017; 126: 100-104.
61. Nurmi HM, Kettunen HP, Suoranta SK, et al. Several high-resolution computed tomography findings associate with survival and clinical features in rheumatoid arthritis-associated interstitial lung disease. Respir Med 2018; 134: 24-30.
62. Fischer A, Antoniou KM, Brown KK, et al. An official European Respiratory Society/American Thoracic Society research statement: Interstitial pneumonia with autoimmune features. Eur Respir J 2015; 46: 976-987.
63. Oldham JM, Adegunsoye A, Valenzi E, et al. Characterisation of patients with interstitial pneumonia with autoimmune features. Eur Respir J 2016; 47: 1767-1775.
64. Assayag D, Elicker BM, Urbania TH, et al. Rheumatoid arthritis-associated interstitial lung disease: Radiologic identification of usual interstitial pneumonia pattern. Radiology 2014; 270: 583-588.
65. Jacob J, Bartholmai BJ, Rajagopalan S, et al. Unclassifiable-interstitial lung disease: Outcome prediction using CT and functional indices. Respir Med 2017; 130: 43-51.
66. Ryerson CJ, Urbania TH, Richeldi L, et al. Prevalence and prognosis of unclassifiable interstitial lung disease. Eur Respir J 2013; 42: 750-757.
67. Thomeer MJ, Vansteenkiste J, Verbeken EK, et al. Interstitial lung diseases: Characteristics at diagnosis and mortality risk assessment. Respir Med 2004; 98: 567-573.
68. 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.
69. 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.
70. Sleijfer S. Bleomycin-induced pneumonitis. Chest 2001; 120: 617-624.
71. Nardi A, Brillet PY, Letoumelin P, et al. Stage IV sarcoidosis: Comparison of survival with the general population and causes of death. Eur Respir J 2011; 38: 1368-1373.
72. Gimenez A, Storrer K, Kuranishi L, et al. Change in FVC and survival in chronic fibrotic hypersensitivity pneumonitis. Thorax 2018; 73: 391-392.
73. Goh NS, Hoyles RK, Denton CP, et al. Short-term pulmonary function trends are predictive of mortality in interstitial lung disease associated with systemic sclerosis. Arthritis Rheumatol 2017; 69: 1670-1678.
74. Kulkarni T, de Andrade J, Zhou Y, et al. Alveolar epithelial disintegrity in pulmonary fibrosis. Am J Physiol Lung Cell Mol Physiol 2016; 311: L185-L191.
75. Cao Z, Lis R, Ginsberg M, et al. Targeting of the pulmonary capillary vascular niche promotes lung alveolar repair and ameliorates fibrosis. Nat Med 2016; 22: 154-162.
76. Juge PA, Borie R, Kannengiesser C, et al. Shared genetic predisposition in rheumatoid arthritis-interstitial lung disease and familial pulmonary fibrosis. Eur Respir J 2017; 49: 1602314.
77. Snetselaar R, van Batenburg AA, van Oosterhout MFM, et al. Short telomere length in IPF lung associates with fibrotic lesions and predicts survival. PLoS One 2017; 12: E0189467.
78. Ley B, Newton CA, Arnould I, et al. The MUC5B promoter polymorphism and telomere length in patients with chronic hypersensitivity pneumonitis: An observational cohort-control study. Lancet Respir Med 2017; 5: 639-647.
79. Kennedy B, Branagan P, Moloney F, et al. Biomarkers to identify ILD and predict lung function decline in scleroderma lung disease or idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis 2015; 32: 228-236.
80. Luckhardt TR, Thannickal VJ. Systemic sclerosis-associated fibrosis: An accelerated aging phenotype? Curr Opin Rheumatol 2015; 27: 571-576.
81. Thannickal VJ. Mechanistic links between aging and lung fibrosis. Biogerontology 2013; 14: 609-615.
82. Chilosi M, Doglioni C, Murer B, et al. Epithelial stem cell exhaustion in the pathogenesis of idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis 2010; 27: 7-18.
83. Birch J, Barnes PJ, Passos JF. Mitochondria, telomeres and cell senescence: Implications for lung ageing and disease. Pharmacol Ther 2018; 183: 34-49.
84. Stock CJ, Sato H, Fonseca C, et al. Mucin 5B promoter polymorphism is associated with idiopathic pulmonary fibrosis but not with development of lung fibrosis in systemic sclerosis or sarcoidosis. Thorax 2013; 68: 436-441.
85. Peljto AL, Zhang Y, Fingerlin TE, et al. Association between the MUC5B promoter polymorphism and survival in patients with idiopathic pulmonary fibrosis. JAMA 2013; 309: 2232-2239.
86. Noth I, Zhang Y, Ma SF, et al. Genetic variants associated with idiopathic pulmonary fibrosis susceptibility and mortality: A genome-wide association study. Lancet Respir Med 2013; 1: 309-317.
87. Behr J, Neuser P, Prasse A, et al. Exploring efficacy and safety of oral pirfenidone for progressive, non-IPF lung fibrosis (RELIEF) - a randomized, double-blind, placebo-controlled, parallel group, multi-center, phase II trial. BMC Pulm Med 2017; 17: 122.
88. Flaherty KR, Brown KK, Wells AU, et al. Design of the PF-ILD trial: A double-blind, randomised, placebo-controlled phase III trial of nintedanib in patients with progressive fibrosing interstitial lung disease. BMJ Open Respir Res 2017; 4: E000212.
89. Hamman L, Rich AR. Fulminating diffuse interstitial fibrosis of the lungs. Trans Am Clin Climat Assoc 1935; 51: 154-163.
90. Liebow AA. New concepts and entities in pulmonary disease. In: Liebow AA, Smith DE, eds. The Lung. Baltimore, The Williams and Wilkins Company; 1968; pp. 332-365.
91. 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.
92. Grinblat J, Mechlis S, Lewitus Z. Organizing pneumonia-like process: An unusual observation in steroid responsive cases with features of chronic interstitial pneumonia. Chest 1981; 80: 259-263.
93. Davison AG, Heard BE, McAllister WAC, et al. Cryptogenic organizing pneumonitis. Q J Med 1983; 52: 382-394.
94. Epler GR, Colby TV, McLoud TC, et al. Bronchiolitis obliterans organizing pneumonia. N Engl J Med 1985; 312: 152-158.
95. Crystal RG, Bitterman PB, Rennard SI, et al. Interstitial lung diseases of unknown cause: Disorders characterized by chronic inflammation of the lower respiratory tract. N Engl J Med 1984; 310: 154-166.
96. Ohori NP, Sciurba FC, Owens GR, et al. Giant-cell interstitial pneumonia and hard-metal pneumoconiosis. a clinicopathologic study of four cases and review of the literature. Am J Surg Pathol 1989; 13: 581-587.
97. Abraham JL, Burnett BR, Hunt A. Development and use of a pneumoconiosis database of human pulmonary inorganic particulate burden in over 400 lungs. Scanning Microsc 1991; 5: 95-104.
98. Katzenstein ALA, Fiorelli RF. Nonspecific interstitial pneumonia/fibrosis. Histological features and clinical significance. Am J Surg Pathol 1994; 18: 136-147.
99. 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.
100. Beasley MB, Franks TJ, Galvin JR, et al. 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.
101. Cottin V, Nunes H, Brillet PY, et al. Combined pulmonary fibrosis and emphysema: A distinct underrecognised entity. Eur Respir J 2005; 26: 586-593.
102. Armanios MY, Chen JJL, Cogan JD, et al. Telomerase mutations in families with idiopathic pulmonary fibrosis. N Engl J Med 2007; 356: 1317-1326.
103. Richeldi L, du Bois RM, Raghu G, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med 2014; 370: 2071-2082.
104. Raghu G, Rochwerg B, Zhang Y, et al. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. An Update of the 2011 Clinical Practice Guideline. Am J Respir Crit Care Med 2015; 192: E3-e19.
105. Lynch DA, Sverzellati N, Travis WD, et al. Diagnostic criteria for idiopathic pulmonary fibrosis: A Fleischner Society White Paper. Lancet Respir Med 2018; 6: 138-153.