Advertisement

The Keys to Making a Confident Diagnosis of IPF

  • Jamie Sheth
  • Anish Wadhwa
  • Kevin R. FlahertyEmail author
Chapter
Part of the Respiratory Medicine book series (RM)

Abstract

Diffuse parenchymal lung diseases (DPLDs) are characterized by injury primarily to the interstitium of the lung but which may involve alveolar spaces, airways, and blood vessels (American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias, Am J Respir Crit Care Med 165(2):277–304, 2002). Many DPLDs are idiopathic (referred to as idiopathic interstitial pneumonias, or IIPs), but DPLD can develop secondary to other factors including connective tissue disease (CTD), environmental exposures, drugs/toxins, etc. (American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias, Am J Respir Crit Care Med 165(2):277–304, 2002). The major IIPs include idiopathic pulmonary fibrosis (IPF), idiopathic nonspecific interstitial pneumonia (NSIP), respiratory bronchiolitis interstitial lung disease (RB-ILD), desquamative interstitial pneumonia (DIP), cryptogenic organizing pneumonia (COP), and acute interstitial pneumonia (AIP) (American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias, Am J Respir Crit Care Med 165(2):277–304, 2002; Travis et al., Am J Respir Crit Care Med 188(6):733–748, 2013). There is significant overlap in the clinical features of the IIPs including chronic dyspnea, interstitial changes on imaging studies, reduction in lung volumes, and impairment in diffusion capacity (DLCO) (American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias, Am J Respir Crit Care Med 165(2):277–304, 2002). Distinct radiographic and histopathological features can distinguish among the clinical entities, and establishing an accurate diagnosis is critical to determining treatment and understanding prognosis (Flaherty et al., Eur Respir J 19(2):275–283, 2002; Bjoraker et al., Am J Respir Crit Care Med 157(1):199–203, 1998).

Of the over 150 recognized types of DPLDs, IPF is the most common and has the worst prognosis (Flaherty et al., Eur Respir J 19(2):275–283, 2002; Bjoraker et al., Am J Respir Crit Care Med 157(1):199–203, 1998). IPF is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown etiology that occurs primarily in older adults, is limited to the lungs, and is associated with a histopathologic and/or radiologic pattern of usual interstitial pneumonia (UIP) (Raghu et al., Am J Respir Crit Care Med 183(6):788–824, 2011). As outlined in the ATS/ERS 2011 consensus statement, the diagnosis requires the exclusion of known causes of DPLD and the presence of a UIP pattern on high-resolution computed tomography (HRCT) or surgical lung biopsy (SLB) (Raghu et al., Am J Respir Crit Care Med 183(6):788–824, 2011). The incidence and prevalence of IPF increase with age, and the diagnosis should be considered in older adult patients who present with nonproductive cough, dyspnea, or bibasilar crackles (Raghu et al., Am J Respir Crit Care Med 174(7):810–816, 2006; Douglas et al., Am J Respir Crit Care Med 161(4 Pt 1):1172–1178, 2000; Fell et al. Am J Respir Crit Care Med 181(8):832–837, 2010).

This chapter reviews the key historical, physiologic, radiographic, and histopathological features that are key to establishing a confident diagnosis of IPF.

Keywords

Pulmonary fibrosis Phenotype Mimics Multidisciplinary Confidence 

References

  1. 1.
    American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. 2002;165(2):277–304.CrossRefGoogle Scholar
  2. 2.
    Travis WD, 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(6):733–48.PubMedPubMedCentralCrossRefGoogle Scholar
  3. 3.
    Flaherty KR, et al. Clinical significance of histological classification of idiopathic interstitial pneumonia. Eur Respir J. 2002;19(2):275–83.PubMedPubMedCentralCrossRefGoogle Scholar
  4. 4.
    Bjoraker JA, et al. Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1998;157(1):199–203.PubMedCrossRefGoogle Scholar
  5. 5.
    Raghu G, 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(6):788–824.PubMedPubMedCentralCrossRefGoogle Scholar
  6. 6.
    Raghu G, et al. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2006;174(7):810–6.PubMedCrossRefGoogle Scholar
  7. 7.
    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(4 Pt 1):1172–8.PubMedCrossRefPubMedCentralGoogle Scholar
  8. 8.
    Fell CD, et al. Clinical predictors of a diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2010;181(8):832–7.PubMedPubMedCentralCrossRefGoogle Scholar
  9. 9.
    Wade JF 3rd, King TE Jr. Infiltrative and interstitial lung disease in the elderly patient. Clin Chest Med. 1993;14(3):501–21.PubMedGoogle Scholar
  10. 10.
    Raghu G, et al. Idiopathic pulmonary fibrosis in US Medicare beneficiaries aged 65 years and older: incidence, prevalence, and survival, 2001–11. Lancet Respir Med. 2014;2(7):566–72.PubMedCrossRefGoogle Scholar
  11. 11.
    Salisbury ML, et al. Predictors of idiopathic pulmonary fibrosis in absence of radiologic honeycombing: a cross sectional analysis in ILD patients undergoing lung tissue sampling. Respir Med. 2016;118:88–95.PubMedPubMedCentralCrossRefGoogle Scholar
  12. 12.
    Steele MP, et al. Clinical and pathologic features of familial interstitial pneumonia. Am J Respir Crit Care Med. 2005;172(9):1146–52.PubMedPubMedCentralCrossRefGoogle Scholar
  13. 13.
    Schwartz DA, et al. The influence of cigarette smoking on lung function in patients with idiopathic pulmonary fibrosis. Am Rev Respir Dis. 1991;144(3 Pt 1):504–6.PubMedCrossRefGoogle Scholar
  14. 14.
    Baumgartner KB, et al. Cigarette smoking: a risk factor for idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1997;155(1):242–8.PubMedCrossRefGoogle Scholar
  15. 15.
    Schwartz DA, et al. Determinants of progression in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1994;149(2 Pt 1):444–9.PubMedCrossRefGoogle Scholar
  16. 16.
    Lee JS, et al. Gastroesophageal reflux therapy is associated with longer survival in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;184(12):1390–4.PubMedPubMedCentralCrossRefGoogle Scholar
  17. 17.
    Raghu G, et al. High prevalence of abnormal acid gastro-oesophageal reflux in idiopathic pulmonary fibrosis. Eur Respir J. 2006;27(1):136–42.PubMedCrossRefPubMedCentralGoogle Scholar
  18. 18.
    Lee JS. The role of gastroesophageal reflux and microaspiration in idiopathic pulmonary fibrosis. Clin Pulm Med. 2014;21(2):81–5.PubMedPubMedCentralCrossRefGoogle Scholar
  19. 19.
    Lee JS, et al. Does chronic microaspiration cause idiopathic pulmonary fibrosis? Am J Med. 2010;123(4):304–11.PubMedPubMedCentralCrossRefGoogle Scholar
  20. 20.
    D’Ovidio F, et al. Prevalence of gastroesophageal reflux in end-stage lung disease candidates for lung transplant. Ann Thorac Surg. 2005;80(4):1254–60.PubMedCrossRefGoogle Scholar
  21. 21.
    Baumgartner KB, et al. Occupational and environmental risk factors for idiopathic pulmonary fibrosis: a multicenter case-control study. Collaborating centers. Am J Epidemiol. 2000;152(4):307–15.PubMedCrossRefGoogle Scholar
  22. 22.
    Hubbard R, et al. Occupational exposure to metal or wood dust and aetiology of cryptogenic fibrosing alveolitis. Lancet. 1996;347(8997):284–9.PubMedCrossRefGoogle Scholar
  23. 23.
    Kitamura H, 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(8):575–85.PubMedCrossRefGoogle Scholar
  24. 24.
    Selman M, et al. Accelerated variant of idiopathic pulmonary fibrosis: clinical behavior and gene expression pattern. PLoS One. 2007;2(5):e482.PubMedPubMedCentralCrossRefGoogle Scholar
  25. 25.
    Idiopathic Pulmonary Fibrosis Clinical Research, N, et al. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med. 2012;366(21):1968–77.CrossRefGoogle Scholar
  26. 26.
    Idiopathic Pulmonary Fibrosis Clinical Research, N, et al. Randomized trial of acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2093–101.CrossRefGoogle Scholar
  27. 27.
    Noble PW, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011;377(9779):1760–9.PubMedCrossRefGoogle Scholar
  28. 28.
    King TE Jr, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2083–92.PubMedCrossRefGoogle Scholar
  29. 29.
    Richeldi L, et al. Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med. 2011;365(12):1079–87.PubMedCrossRefGoogle Scholar
  30. 30.
    Richeldi L, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2071–82.PubMedCrossRefPubMedCentralGoogle Scholar
  31. 31.
    Daniels CE, Yi ES, Ryu JH. Autopsy findings in 42 consecutive patients with idiopathic pulmonary fibrosis. Eur Respir J. 2008;32(1):170–4.PubMedCrossRefGoogle Scholar
  32. 32.
    Panos RJ, et al. Clinical deterioration in patients with idiopathic pulmonary fibrosis – causes and assessment. Am J Med. 1990;88(4):396–404.PubMedCrossRefGoogle Scholar
  33. 33.
    Ley B, Collard HR, King TE Jr. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;183(4):431–40.PubMedCrossRefGoogle Scholar
  34. 34.
    Stewart JP, 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(4 Pt 1):1336–41.PubMedCrossRefGoogle Scholar
  35. 35.
    Konishi K, et al. Gene expression profiles of acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2009;180(2):167–75.PubMedPubMedCentralCrossRefGoogle Scholar
  36. 36.
    Ryerson CJ, et al. Acute exacerbation of idiopathic pulmonary fibrosis: shifting the paradigm. Eur Respir J. 2015;46(2):512–20.PubMedCrossRefGoogle Scholar
  37. 37.
    Atkins CP, Loke YK, Wilson AM. Outcomes in idiopathic pulmonary fibrosis: a meta-analysis from placebo controlled trials. Respir Med. 2014;108(2):376–87.PubMedCrossRefPubMedCentralGoogle Scholar
  38. 38.
    Lee JS, et al. Anti-acid treatment and disease progression in idiopathic pulmonary fibrosis: an analysis of data from three randomised controlled trials. Lancet Respir Med. 2013;1(5):369–76.PubMedPubMedCentralCrossRefGoogle Scholar
  39. 39.
    Chida M, et al. Subclinical idiopathic pulmonary fibrosis is also a risk factor of postoperative acute respiratory distress syndrome following thoracic surgery. Eur J Cardiothorac Surg. 2008;34(4):878–81.PubMedCrossRefPubMedCentralGoogle Scholar
  40. 40.
    Kondoh Y, et al. Risk factors of acute exacerbation of idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis. 2010;27(2):103–10.PubMedPubMedCentralGoogle Scholar
  41. 41.
    Utz JP, et al. High short-term mortality following lung biopsy for usual interstitial pneumonia. Eur Respir J. 2001;17(2):175–9.PubMedCrossRefPubMedCentralGoogle Scholar
  42. 42.
    Kim DS, et al. Acute exacerbation of idiopathic pulmonary fibrosis: frequency and clinical features. Eur Respir J. 2006;27(1):143–50.PubMedCrossRefGoogle Scholar
  43. 43.
    Martinez FJ, et al. The clinical course of patients with idiopathic pulmonary fibrosis. Ann Intern Med. 2005;142(12 Pt 1):963–7.PubMedCrossRefPubMedCentralGoogle Scholar
  44. 44.
    Song JW, et al. Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome. Eur Respir J. 2011;37(2):356–63.PubMedCrossRefPubMedCentralGoogle Scholar
  45. 45.
    Kondoh Y, et al. Risk factors for acute exacerbation of idiopathic pulmonary fibrosis – extended analysis of pirfenidone trial in Japan. Respir Investig. 2015;53(6):271–8.PubMedCrossRefPubMedCentralGoogle Scholar
  46. 46.
    King TE Jr, et al. Idiopathic pulmonary fibrosis: relationship between histopathologic features and mortality. Am J Respir Crit Care Med. 2001;164(6):1025–32.PubMedCrossRefPubMedCentralGoogle Scholar
  47. 47.
    Nicholson AG, et al. The relationship between individual histologic features and disease progression in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2002;166(2):173–7.PubMedCrossRefPubMedCentralGoogle Scholar
  48. 48.
    Tiitto L, et al. Relationship between histopathological features and the course of idiopathic pulmonary fibrosis/usual interstitial pneumonia. Thorax. 2006;61(12):1091–5.PubMedPubMedCentralCrossRefGoogle Scholar
  49. 49.
    King TE Jr, et al. Predicting survival in idiopathic pulmonary fibrosis: scoring system and survival model. Am J Respir Crit Care Med. 2001;164(7):1171–81.PubMedCrossRefPubMedCentralGoogle Scholar
  50. 50.
    Cottin V, Cordier JF. The syndrome of combined pulmonary fibrosis and emphysema. Chest. 2009;136(1):1–2.PubMedCrossRefGoogle Scholar
  51. 51.
    Doherty MJ, et al. Cryptogenic fibrosing alveolitis with preserved lung volumes. Thorax. 1997;52(11):998–1002.PubMedPubMedCentralCrossRefGoogle Scholar
  52. 52.
    Mejia M, et al. Idiopathic pulmonary fibrosis and emphysema: decreased survival associated with severe pulmonary arterial hypertension. Chest. 2009;136(1):10–5.PubMedCrossRefGoogle Scholar
  53. 53.
    Cottin V, et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J. 2005;26(4):586–93.PubMedCrossRefGoogle Scholar
  54. 54.
    Schmidt SL, et al. Pulmonary function measures predict mortality differently in IPF versus combined pulmonary fibrosis and emphysema. Eur Respir J. 2011;38(1):176–83.PubMedCrossRefPubMedCentralGoogle Scholar
  55. 55.
    Watters LC, 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(1):97–103.PubMedCrossRefGoogle Scholar
  56. 56.
    Wells AU, 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(7):962–9.PubMedCrossRefGoogle Scholar
  57. 57.
    Corte TJ, Wort SJ, Wells AU. Pulmonary hypertension in idiopathic pulmonary fibrosis: a review. Sarcoidosis Vasc Diffuse Lung Dis. 2009;26(1):7–19.PubMedGoogle Scholar
  58. 58.
    Andersen CU, et al. Pulmonary hypertension in interstitial lung disease: prevalence, prognosis and 6 min walk test. Respir Med. 2012;106(6):875–82.PubMedCrossRefPubMedCentralGoogle Scholar
  59. 59.
    D’Alonzo GE, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med. 1991;115(5):343–9.PubMedCrossRefPubMedCentralGoogle Scholar
  60. 60.
    Patel NM, et al. Pulmonary hypertension in idiopathic pulmonary fibrosis. Chest. 2007;132(3):998–1006.PubMedCrossRefPubMedCentralGoogle Scholar
  61. 61.
    Arcasoy SM, et al. Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease. Am J Respir Crit Care Med. 2003;167(5):735–40.PubMedCrossRefPubMedCentralGoogle Scholar
  62. 62.
    Nathan SD, et al. Serial development of pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Respiration. 2008;76(3):288–94.PubMedCrossRefPubMedCentralGoogle Scholar
  63. 63.
    Lettieri CJ, et al. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest. 2006;129(3):746–52.PubMedCrossRefPubMedCentralGoogle Scholar
  64. 64.
    Nathan SD, Noble PW, Tuder RM. Idiopathic pulmonary fibrosis and pulmonary hypertension: connecting the dots. Am J Respir Crit Care Med. 2007;175(9):875–80.PubMedCrossRefPubMedCentralGoogle Scholar
  65. 65.
    Hamada K, et al. Significance of pulmonary arterial pressure and diffusion capacity of the lung as prognosticator in patients with idiopathic pulmonary fibrosis. Chest. 2007;131(3):650–6.PubMedCrossRefPubMedCentralGoogle Scholar
  66. 66.
    Charbeneau RP, Peters-Golden M. Eicosanoids: mediators and therapeutic targets in fibrotic lung disease. Clin Sci (Lond). 2005;108(6):479–91.CrossRefGoogle Scholar
  67. 67.
    Richter A, et al. Impaired transforming growth factor-beta signaling in idiopathic pulmonary arterial hypertension. Am J Respir Crit Care Med. 2004;170(12):1340–8.PubMedCrossRefGoogle Scholar
  68. 68.
    Wright L, et al. 5-lipoxygenase and 5-lipoxygenase activating protein (FLAP) immunoreactivity in lungs from patients with primary pulmonary hypertension. Am J Respir Crit Care Med. 1998;157(1):219–29.PubMedCrossRefGoogle Scholar
  69. 69.
    Weitzenblum E, Chaouat A. Sleep and chronic obstructive pulmonary disease. Sleep Med Rev. 2004;8(4):281–94.PubMedCrossRefGoogle Scholar
  70. 70.
    Risk C, Epler GR, Gaensler EA. Exercise alveolar-arterial oxygen pressure difference in interstitial lung disease. Chest. 1984;85(1):69–74.PubMedCrossRefGoogle Scholar
  71. 71.
    O’Donnell D. Physiology of interstitial lung disease. In: Schwarz MI, King TE, editors. Interstitial lung disease. Hamilton: Marcel Dekker; 1998. p. 51–70.Google Scholar
  72. 72.
    Gottlieb D, Snider G. Lung function in pulmonary fibrosis. In: Phan S, Thrall R, editors. Lung biology in health and disease: pulmonary fibrosis. New York: Marcel Dekker; 1995. p. 85–135.Google Scholar
  73. 73.
    Collard HR, et al. Changes in clinical and physiologic variables predict survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2003;168(5):538–42.PubMedCrossRefGoogle Scholar
  74. 74.
    Flaherty KR, et al. Idiopathic pulmonary fibrosis: prognostic value of changes in physiology and six-minute-walk test. Am J Respir Crit Care Med. 2006;174(7):803–9.PubMedPubMedCentralCrossRefGoogle Scholar
  75. 75.
    Lama VN, et al. Prognostic value of desaturation during a 6-minute walk test in idiopathic interstitial pneumonia. Am J Respir Crit Care Med. 2003;168(9):1084–90.PubMedCrossRefGoogle Scholar
  76. 76.
    Fell CD, et al. The prognostic value of cardiopulmonary exercise testing in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2009;179(5):402–7.PubMedCrossRefGoogle Scholar
  77. 77.
    Latsi PI, et al. Fibrotic idiopathic interstitial pneumonia: the prognostic value of longitudinal functional trends. Am J Respir Crit Care Med. 2003;168(5):531–7.PubMedCrossRefGoogle Scholar
  78. 78.
    du Bois RM, 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(9):1231–7.PubMedCrossRefPubMedCentralGoogle Scholar
  79. 79.
    du Bois RM, et al. Ascertainment of individual risk of mortality for patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;184(4):459–66.PubMedCrossRefGoogle Scholar
  80. 80.
    Flaherty KR, et al. Prognostic implications of physiologic and radiographic changes in idiopathic interstitial pneumonia. Am J Respir Crit Care Med. 2003;168(5):543–8.PubMedCrossRefGoogle Scholar
  81. 81.
    Jegal Y, et al. Physiology is a stronger predictor of survival than pathology in fibrotic interstitial pneumonia. Am J Respir Crit Care Med. 2005;171(6):639–44.PubMedCrossRefGoogle Scholar
  82. 82.
    Ley B, et al. A multidimensional index and staging system for idiopathic pulmonary fibrosis. Ann Intern Med. 2012;156(10):684–91.PubMedCrossRefGoogle Scholar
  83. 83.
    Orens JB, et al. The sensitivity of high-resolution CT in detecting idiopathic pulmonary fibrosis proved by open lung biopsy. A prospective study. Chest. 1995;108(1):109–15.PubMedCrossRefGoogle Scholar
  84. 84.
    Hansell DM, et al. Fleischner society: glossary of terms for thoracic imaging. Radiology. 2008;246(3):697–722.PubMedCrossRefGoogle Scholar
  85. 85.
    Akira M, Sakatani M, Ueda E. Idiopathic pulmonary fibrosis: progression of honeycombing at thin-section CT. Radiology. 1993;189(3):687–91.PubMedCrossRefGoogle Scholar
  86. 86.
    Kazerooni EA, et al. Thin-section CT obtained at 10-mm increments versus limited three-level thin-section CT for idiopathic pulmonary fibrosis: correlation with pathologic scoring. AJR Am J Roentgenol. 1997;169(4):977–83.PubMedCrossRefGoogle Scholar
  87. 87.
    Lynch DA, et al. High-resolution computed tomography in idiopathic pulmonary fibrosis: diagnosis and prognosis. Am J Respir Crit Care Med. 2005;172(4):488–93.PubMedCrossRefGoogle Scholar
  88. 88.
    Travis WD, et al. Idiopathic nonspecific interstitial pneumonia: report of an American Thoracic Society project. Am J Respir Crit Care Med. 2008;177(12):1338–47.PubMedCrossRefGoogle Scholar
  89. 89.
    Flaherty KR, et al. Radiological versus histological diagnosis in UIP and NSIP: survival implications. Thorax. 2003;58(2):143–8.PubMedPubMedCentralCrossRefGoogle Scholar
  90. 90.
    du Bois R, King TE Jr. Challenges in pulmonary fibrosis x 5: the NSIP/UIP debate. Thorax. 2007;62(11):1008–12.PubMedPubMedCentralCrossRefGoogle Scholar
  91. 91.
    Arakawa H, Honma K. Honeycomb lung: history and current concepts. AJR Am J Roentgenol. 2011;196(4):773–82.PubMedCrossRefGoogle Scholar
  92. 92.
    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(2 Pt 1):646–64.Google Scholar
  93. 93.
    Ohshimo S, et al. Significance of bronchoalveolar lavage for the diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2009;179(11):1043–7.PubMedCrossRefGoogle Scholar
  94. 94.
    Berbescu EA, et al. Transbronchial biopsy in usual interstitial pneumonia. Chest. 2006;129(5):1126–31.PubMedPubMedCentralCrossRefGoogle Scholar
  95. 95.
    Shim HS, Park MS, Park IK. Histopathologic findings of transbronchial biopsy in usual interstitial pneumonia. Pathol Int. 2010;60(5):373–7.PubMedCrossRefGoogle Scholar
  96. 96.
    Tomassetti S, et al. Transbronchial biopsy is useful in predicting UIP pattern. Respir Res. 2012;13:96.PubMedPubMedCentralCrossRefGoogle Scholar
  97. 97.
    Sheth JS, et al. Utility of transbronchial vs surgical lung biopsy in the diagnosis of suspected fibrotic interstitial lung disease. Chest. 2017;151(2):389–99.PubMedCrossRefPubMedCentralGoogle Scholar
  98. 98.
    Casoni GL, et al. Transbronchial lung cryobiopsy in the diagnosis of fibrotic interstitial lung diseases. PLoS One. 2014;9(2):e86716.PubMedPubMedCentralCrossRefGoogle Scholar
  99. 99.
    Poletti V, Hetzel J. Transbronchial cryobiopsy in diffuse parenchymal lung disease: need for procedural standardization. Respiration. 2015;90(4):275–8.PubMedCrossRefGoogle Scholar
  100. 100.
    Ravaglia C, et al. Safety and diagnostic yield of transbronchial lung cryobiopsy in diffuse parenchymal lung diseases: a comparative study versus video-assisted thoracoscopic lung biopsy and a systematic review of the literature. Respiration. 2016;91(3):215–27.PubMedCrossRefGoogle Scholar
  101. 101.
    Gasparini S, Bonifazi M. Cryobiopsy for interstitial lung diseases. J Bronchol Interv Pulmonol. 2016;23(1):4–6.CrossRefGoogle Scholar
  102. 102.
    Tomassetti S, et al. Bronchoscopic lung cryobiopsy increases diagnostic confidence in the multidisciplinary diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2016;193(7):745–52.PubMedCrossRefPubMedCentralGoogle Scholar
  103. 103.
    Hunninghake GW, et al. Utility of a lung biopsy for the diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2001;164(2):193–6.PubMedCrossRefPubMedCentralGoogle Scholar
  104. 104.
    Lettieri CJ, et al. Outcomes and safety of surgical lung biopsy for interstitial lung disease. Chest. 2005;127(5):1600–5.PubMedCrossRefGoogle Scholar
  105. 105.
    Kreider ME, et al. Complications of video-assisted thoracoscopic lung biopsy in patients with interstitial lung disease. Ann Thorac Surg. 2007;83(3):1140–4.PubMedCrossRefGoogle Scholar
  106. 106.
    Tiitto L, et al. Thoracoscopic lung biopsy is a safe procedure in diagnosing usual interstitial pneumonia. Chest. 2005;128(4):2375–80.PubMedCrossRefGoogle Scholar
  107. 107.
    Canver CC, Mentzer RM Jr. The role of open lung biopsy in early and late survival of ventilator-dependent patients with diffuse idiopathic lung disease. J Cardiovasc Surg. 1994;35(2):151–5.Google Scholar
  108. 108.
    Hazelrigg SR, Nunchuck SK, LoCicero J 3rd. Video assisted thoracic surgery study group data. Ann Thorac Surg. 1993;56(5):1039–43. discussion 1043–4PubMedCrossRefGoogle Scholar
  109. 109.
    Nicod P, Moser KM. Primary pulmonary hypertension. The risk and benefit of lung biopsy. Circulation. 1989;80(5):1486–8.PubMedCrossRefGoogle Scholar
  110. 110.
    Hutchinson JP, et al. In-hospital mortality after surgical lung biopsy for interstitial lung disease in the United States. 2000 to 2011. Am J Respir Crit Care Med. 2016;193(10):1161–7.PubMedCrossRefGoogle Scholar
  111. 111.
    Hunninghake GW, et al. Radiologic findings are strongly associated with a pathologic diagnosis of usual interstitial pneumonia. Chest. 2003;124(4):1215–23.PubMedCrossRefGoogle Scholar
  112. 112.
    Raghu G, 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(5):1168–74.PubMedCrossRefGoogle Scholar
  113. 113.
    Park JH, et al. Mortality and risk factors for surgical lung biopsy in patients with idiopathic interstitial pneumonia. Eur J Cardiothorac Surg. 2007;31(6):1115–9.PubMedCrossRefGoogle Scholar
  114. 114.
    Kadokura M, et al. Pathologic comparison of video-assisted thoracic surgical lung biopsy with traditional open lung biopsy. J Thorac Cardiovasc Surg. 1995;109(3):494–8.PubMedCrossRefGoogle Scholar
  115. 115.
    Park JH, et al. Prognosis of fibrotic interstitial pneumonia: idiopathic versus collagen vascular disease-related subtypes. Am J Respir Crit Care Med. 2007;175(7):705–11.PubMedCrossRefPubMedCentralGoogle Scholar
  116. 116.
    Monaghan H, et al. Prognostic implications of histologic patterns in multiple surgical lung biopsies from patients with idiopathic interstitial pneumonias. Chest. 2004;125(2):522–6.PubMedCrossRefGoogle Scholar
  117. 117.
    Qureshi RA, et al. Does lung biopsy help patients with interstitial lung disease? Eur J Cardiothorac Surg. 2002;21(4):621–6. discussion 626PubMedCrossRefGoogle Scholar
  118. 118.
    Meyer EC, Liebow AA. Relationship of interstitial pneumonia honeycombing and atypical epithelial proliferation to cancer of the lung. Cancer. 1965;18:322–51.PubMedCrossRefGoogle Scholar
  119. 119.
    Johkoh T, et al. Nonspecific interstitial pneumonia: correlation between thin-section CT findings and pathologic subgroups in 55 patients. Radiology. 2002;225(1):199–204.PubMedCrossRefGoogle Scholar
  120. 120.
    Katzenstein AL, et al. Clinically occult interstitial fibrosis in smokers: classification and significance of a surprisingly common finding in lobectomy specimens. Hum Pathol. 2010;41(3):316–25.PubMedCrossRefGoogle Scholar
  121. 121.
    Pimentel JC. Tridimensional photographic reconstruction in a study of the pathogenesis of honeycomb lung. Thorax. 1967;22(5):444–52.PubMedPubMedCentralCrossRefGoogle Scholar
  122. 122.
    Katzenstein AL. Smoking-related interstitial fibrosis (SRIF): pathologic findings and distinction from other chronic fibrosing lung diseases. J Clin Pathol. 2013;66(10):882–7.PubMedCrossRefGoogle Scholar
  123. 123.
    Flaherty KR, et al. Fibroblastic foci in usual interstitial pneumonia: idiopathic versus collagen vascular disease. Am J Respir Crit Care Med. 2003;167(10):1410–5.PubMedCrossRefPubMedCentralGoogle Scholar
  124. 124.
    Nicholson AG, et al. Inter-observer variation between pathologists in diffuse parenchymal lung disease. Thorax. 2004;59(6):500–5.PubMedPubMedCentralCrossRefGoogle Scholar
  125. 125.
    Costabel U, Bonella F, Guzman J. Chronic hypersensitivity pneumonitis. Clin Chest Med. 2012;33(1):151–63.PubMedCrossRefGoogle Scholar
  126. 126.
    Hanak V, et al. High-resolution CT findings of parenchymal fibrosis correlate with prognosis in hypersensitivity pneumonitis. Chest. 2008;134(1):133–8.PubMedPubMedCentralCrossRefGoogle Scholar
  127. 127.
    Sahin H, et al. Chronic hypersensitivity pneumonitis: CT features comparison with pathologic evidence of fibrosis and survival. Radiology. 2007;244(2):591–8.PubMedCrossRefGoogle Scholar
  128. 128.
    Kishi M, et al. Pathogenesis of cBFL in common with IPF? Correlation of IP-10/TARC ratio with histological patterns. Thorax. 2008;63(9):810–6.PubMedCrossRefGoogle Scholar
  129. 129.
    Coleman A, Colby TV. Histologic diagnosis of extrinsic allergic alveolitis. Am J Surg Pathol. 1988;12(7):514–8.PubMedCrossRefGoogle Scholar
  130. 130.
    Churg A, et al. Chronic hypersensitivity pneumonitis. Am J Surg Pathol. 2006;30(2):201–8.PubMedCrossRefGoogle Scholar
  131. 131.
    Johannson KA, et al. A diagnostic model for chronic hypersensitivity pneumonitis. Thorax. 2016;71(10):951–4.PubMedPubMedCentralCrossRefGoogle Scholar
  132. 132.
    Lynch DA, et al. Can CT distinguish hypersensitivity pneumonitis from idiopathic pulmonary fibrosis? AJR Am J Roentgenol. 1995;165(4):807–11.PubMedCrossRefPubMedCentralGoogle Scholar
  133. 133.
    Silva CI, et al. Chronic hypersensitivity pneumonitis: differentiation from idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia by using thin-section CT. Radiology. 2008;246(1):288–97.PubMedCrossRefGoogle Scholar
  134. 134.
    Travis WD, et al. 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(1):19–33.PubMedCrossRefPubMedCentralGoogle Scholar
  135. 135.
    Park JS, et al. Nonspecific interstitial pneumonia with fibrosis: radiographic and CT findings in seven patients. Radiology. 1995;195(3):645–8.PubMedCrossRefGoogle Scholar
  136. 136.
    Kim DS, et al. The major histopathologic pattern of pulmonary fibrosis in scleroderma is nonspecific interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis. 2002;19(2):121–7.PubMedGoogle Scholar
  137. 137.
    Lee HK, et al. Histopathologic pattern and clinical features of rheumatoid arthritis-associated interstitial lung disease. Chest. 2005;127(6):2019–27.PubMedCrossRefPubMedCentralGoogle Scholar
  138. 138.
    Kim EJ, et al. Usual interstitial pneumonia in rheumatoid arthritis-associated interstitial lung disease. Eur Respir J. 2010;35(6):1322–8.PubMedCrossRefPubMedCentralGoogle Scholar
  139. 139.
    Gochuico BR, et al. Progressive preclinical interstitial lung disease in rheumatoid arthritis. Arch Intern Med. 2008;168(2):159–66.PubMedCrossRefGoogle Scholar
  140. 140.
    Mori S, et al. A simultaneous onset of organizing pneumonia and rheumatoid arthritis, along with a review of the literature. Mod Rheumatol. 2008;18(1):60–6.PubMedCrossRefPubMedCentralGoogle Scholar
  141. 141.
    Biederer J, et al. Correlation between HRCT findings, pulmonary function tests and bronchoalveolar lavage cytology in interstitial lung disease associated with rheumatoid arthritis. Eur Radiol. 2004;14(2):272–80.PubMedCrossRefPubMedCentralGoogle Scholar
  142. 142.
    Garcia JG, et al. Lower respiratory tract abnormalities in rheumatoid interstitial lung disease. Potential role of neutrophils in lung injury. Am Rev Respir Dis. 1987;136(4):811–7.PubMedCrossRefPubMedCentralGoogle Scholar
  143. 143.
    Komocsi A, et al. Alveolitis may persist during treatment that sufficiently controls muscle inflammation in myositis. Rheumatol Int. 2001;20(3):113–8.PubMedCrossRefPubMedCentralGoogle Scholar
  144. 144.
    Nagasawa Y, et al. Inflammatory cells in lung disease associated with rheumatoid arthritis. Intern Med. 2009;48(14):1209–17.PubMedCrossRefPubMedCentralGoogle Scholar
  145. 145.
    Costabel U, et al. Bronchoalveolar lavage in other interstitial lung diseases. Semin Respir Crit Care Med. 2007;28(5):514–24.PubMedCrossRefPubMedCentralGoogle Scholar
  146. 146.
    Ramirez P, Valencia M, Torres A. Bronchoalveolar lavage to diagnose respiratory infections. Semin Respir Crit Care Med. 2007;28(5):525–33.PubMedCrossRefPubMedCentralGoogle Scholar
  147. 147.
    Schnabel A, et al. Bronchoalveolar lavage cell profile in methotrexate induced pneumonitis. Thorax. 1997;52(4):377–9.PubMedPubMedCentralCrossRefGoogle Scholar
  148. 148.
    Kim EJ, Collard HR, King TE Jr. Rheumatoid arthritis-associated interstitial lung disease: the relevance of histopathologic and radiographic pattern. Chest. 2009;136(5):1397–405.PubMedPubMedCentralCrossRefGoogle Scholar
  149. 149.
    Dawson JK, et al. Predictors of progression of HRCT diagnosed fibrosing alveolitis in patients with rheumatoid arthritis. Ann Rheum Dis. 2002;61(6):517–21.PubMedPubMedCentralCrossRefGoogle Scholar
  150. 150.
    Stack BH, Grant IW. Rheumatoid interstitial lung disease. Br J Dis Chest. 1965;59(4):202–11.PubMedCrossRefGoogle Scholar
  151. 151.
    Kocheril SV, et al. Comparison of disease progression and mortality of connective tissue disease-related interstitial lung disease and idiopathic interstitial pneumonia. Arthritis Rheum. 2005;53(4):549–57.PubMedCrossRefPubMedCentralGoogle Scholar
  152. 152.
    Kligerman SJ, et al. Nonspecific interstitial pneumonia: radiologic, clinical, and pathologic considerations. Radiographics. 2009;29(1):73–87.PubMedCrossRefGoogle Scholar
  153. 153.
    Leslie KO, Trahan S, Gruden J. Pulmonary pathology of the rheumatic diseases. Semin Respir Crit Care Med. 2007;28(4):369–78.PubMedCrossRefPubMedCentralGoogle Scholar
  154. 154.
    Douglas WW, et al. Polymyositis-dermatomyositis-associated interstitial lung disease. Am J Respir Crit Care Med. 2001;164(7):1182–5.PubMedCrossRefPubMedCentralGoogle Scholar
  155. 155.
    Tansey D, et al. Variations in histological patterns of interstitial pneumonia between connective tissue disorders and their relationship to prognosis. Histopathology. 2004;44(6):585–96.PubMedCrossRefPubMedCentralGoogle Scholar
  156. 156.
    Shelton DN, et al. Microarray analysis of replicative senescence. Curr Biol. 1999;9(17):939–45.PubMedCrossRefPubMedCentralGoogle Scholar
  157. 157.
    Fischer A, et al. An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J. 2015;46(4):976–87.PubMedCrossRefPubMedCentralGoogle Scholar
  158. 158.
    Thannickal VJ, Loyd JE. Idiopathic pulmonary fibrosis: a disorder of lung regeneration? Am J Respir Crit Care Med. 2008;178(7):663–5.PubMedCrossRefPubMedCentralGoogle Scholar
  159. 159.
    Selman M, Pardo A. Revealing the pathogenic and aging-related mechanisms of the enigmatic idiopathic pulmonary fibrosis. An integral model. Am J Respir Crit Care Med. 2014;189(10):1161–72.PubMedCrossRefGoogle Scholar
  160. 160.
    Castriotta RJ, et al. Workshop on idiopathic pulmonary fibrosis in older adults. Chest. 2010;138(3):693–703.PubMedPubMedCentralCrossRefGoogle Scholar
  161. 161.
    Sakamoto K, et al. Acute exacerbation of IPF following diagnostic bronchoalveolar lavage procedures. Respir Med. 2012;106(3):436–42.PubMedCrossRefPubMedCentralGoogle Scholar
  162. 162.
    Aziz ZA, et al. HRCT diagnosis of diffuse parenchymal lung disease: inter-observer variation. Thorax. 2004;59(6):506–11.PubMedPubMedCentralCrossRefGoogle Scholar
  163. 163.
    Flaherty KR, et al. Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis? Am J Respir Crit Care Med. 2004;170(8):904–10.PubMedCrossRefGoogle Scholar
  164. 164.
    Flaherty KR, et al. Idiopathic interstitial pneumonia: do community and academic physicians agree on diagnosis? Am J Respir Crit Care Med. 2007;175(10):1054–60.PubMedPubMedCentralCrossRefGoogle Scholar
  165. 165.
    Katzenstein AL. Smoking-related interstitial fibrosis (SRIF), pathogenesis and treatment of usual interstitial pneumonia (UIP), and transbronchial biopsy in UIP. Mod Pathol. 2012;25(Suppl 1):S68–78.PubMedCrossRefPubMedCentralGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Division of Pulmonary and Critical Care MedicineUniversity of Michigan Health SystemAnn ArborUSA
  2. 2.Pulmonary Clinics of Southern MichiganJacksonUSA

Personalised recommendations