Skip to main content
SpringerLink
Log in

Histopathology of IPF and Related Disorders

  • Chapter
  • First Online:
Idiopathic Pulmonary Fibrosis

Part of the book series: Respiratory Medicine ((RM,volume 9))

  • 2088 Accesses

Abstract

Histopathologic classification schemes provide the underpinnings for separating idiopathic interstitial pneumonias into clinically meaningful groups. A number of multidisciplinary position papers and guidelines published over the last decade have cemented usual interstitial pneumonia (UIP) as the defining feature of idiopathic pulmonary fibrosis (IPF). Surgical lung biopsy is an important diagnostic tool in patients for whom imaging studies are inconclusive. Diagnosis of UIP on a surgical lung biopsy remains the single most important predictor of outcome at the time of diagnosis in patients with otherwise unexplained diffuse lung disease. Identifying patients with respiratory bronchiolitis interstitial lung disease (RBILD), desquamative interstitial pneumonia (DIP), and nonspecific interstitial pneumonia (NSIP) is more challenging and requires careful correlation of lung biopsy findings with other clinical and radiological data.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 109.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 139.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Myers J. Idiopathic interstitial pneumonias. In: Churg A, Myers J, Tazelaar HD, Wright JL, editors. Thurlbeck’s pathology of the lung. New York, NY: Thieme; 2004. p. 563–600.

    Google Scholar 

  2. Katzenstein AL. Katzenstein and askin’s surgical pathology of non-neoplastic lung disease. 4th ed. Philadelphia, PA: Saunders Elsevier; 2006.

    Google Scholar 

  3. Katzenstein AL, Myers JL. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification. Am J Respir Crit Care Med. 1998;157(4 Pt 1):1301–15.

    Article  PubMed  CAS  Google Scholar 

  4. Liebow AA, Steer A, Billingsley JG. Desquamative interstitial pneumonia. Am J Med. 1965;39:369–404.

    Article  PubMed  CAS  Google Scholar 

  5. 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(2):277–304.

    Article  Google Scholar 

  6. Travis W, Gostabel U, Hensell D, et al. An official American Thoracic Society/European Respiratory Society Statement: Update of the international multidisciplinary classification of the Idiopathic interstitional pneumonias. Am J Respir Crit Care Med. 2013 (in press).

    Google Scholar 

  7. Hamman BL, Rich AR. Acute diffuse interstitial fibrosis of the lung. Bull Johns Hopkins Hosp. 1944;74:177–212.

    Google Scholar 

  8. Katzenstein AL, Myers JL, Mazur MT. Acute interstitial pneumonia. A clinicopathologic, ultrastructural, and cell kinetic study. Am J Surg Pathol. 1986;10(4):256–67.

    Article  PubMed  CAS  Google Scholar 

  9. Bjoraker JA, Ryu JH, Edwin MK, Myers JL, Tazelaar HD, Schroeder DR, et al. Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1998;157(1):199–203.

    Article  PubMed  CAS  Google Scholar 

  10. Flaherty KR, Toews GB, Travis WD, Colby TV, Kazerooni EA, Gross BH, et al. Clinical significance of histological classification of idiopathic interstitial pneumonia. Eur Respir J. 2002;19(2):275–83.

    Article  PubMed  CAS  Google Scholar 

  11. Nicholson AG, Colby TV, du Bois RM, Hansell DM, Wells AU. The prognostic significance of the histologic pattern of interstitial pneumonia in patients presenting with the clinical entity of cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med. 2000;162(6):2213–7.

    Article  PubMed  CAS  Google Scholar 

  12. Riha RL, Duhig EE, Clarke BE, Steele RH, Slaughter RE, Zimmerman PV. Survival of patients with biopsy-proven usual interstitial pneumonia and nonspecific interstitial pneumonia. Eur Respir J. 2002;19(6):1114–8.

    Article  PubMed  CAS  Google Scholar 

  13. Idiopathic Pulmonary Fibrosis: diagnosis and treatment. Am J Respir Crit Care Med. 2000;161(2):646–64.

    Google Scholar 

  14. Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, 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.

    Article  PubMed  Google Scholar 

  15. Lee HL, Ryu JH, Wittmer MH, Hartman TE, Lymp JF, Tazelaar HD, et al. Familial idiopathic pulmonary fibrosis: clinical features and outcome. Chest. 2005;127(6):2034–41.

    Article  PubMed  Google Scholar 

  16. Steele MP, Speer MC, Loyd JE, Brown KK, Herron A, Slifer SH, et al. Clinical and pathologic features of familial interstitial pneumonia. Am J Respir Crit Care Med. 2005;172(9):1146–52.

    Article  PubMed  Google Scholar 

  17. Flaherty KR, Colby TV, Travis WD, Toews GB, Mumford J, Murray S, et al. Fibroblastic foci in usual interstitial pneumonia: idiopathic versus collagen vascular disease. Am J Respir Crit Care Med. 2003;167(10):1410–5.

    Article  PubMed  Google Scholar 

  18. Collard HR, Moore BB, Flaherty KR, Brown KK, Kaner RJ, King Jr TE, et al. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2007;176(7):636–43.

    Article  PubMed  Google Scholar 

  19. Parambil JG, Myers JL, Ryu JH. Histopathologic features and outcome of patients with acute exacerbation of idiopathic pulmonary fibrosis undergoing surgical lung biopsy. Chest. 2005;128(5):3310–5.

    Article  PubMed  Google Scholar 

  20. Azuma A, Nukiwa T, Tsuboi E, Suga M, Abe S, Nakata K, et al. Double-blind, placebo-controlled trial of pirfenidone in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2005;171(9):1040–7.

    Article  PubMed  Google Scholar 

  21. Martinez FJ, Safrin S, Weycker D, Starko KM, Bradford WZ, King Jr TE, et al. The clinical course of patients with idiopathic pulmonary fibrosis. Ann Intern Med. 2005;142(12 Pt 1):963–7.

    Article  PubMed  Google Scholar 

  22. Daniels C, Yi E, Ryu JH. Autopsy findings in 42 consecutive patients with idiopathic pulmonary fibrosis. Eur Respir J. 2008;32:170–4.

    Article  PubMed  CAS  Google Scholar 

  23. Flaherty KR, Thwaite EL, Kazerooni EA, Gross BH, Toews GB, Colby TV, et al. Radiological versus histological diagnosis in UIP and NSIP: survival implications. Thorax. 2003;58(2):143–8.

    Article  PubMed  CAS  Google Scholar 

  24. Hunninghake GW, Zimmerman MB, Schwartz DA, King Jr TE, Lynch J, Hegele R, 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.

    Article  PubMed  CAS  Google Scholar 

  25. Johkoh T, Muller NL, Cartier Y, Kavanagh PV, Hartman TE, Akira M, et al. Idiopathic interstitial pneumonias: diagnostic accuracy of thin-section CT in 129 patients. Radiology. 1999;211(2):555–60.

    PubMed  CAS  Google Scholar 

  26. Sumikawa H, Johkoh T, Ichikado K, Taniguchi H, Kondoh Y, Fujimoto K, et al. Usual interstitial pneumonia and chronic idiopathic interstitial pneumonia: analysis of CT appearance in 92 patients. Radiology. 2006;241(1):258–66.

    Article  PubMed  Google Scholar 

  27. Sumikawa H, Johkoh T, Colby TV, Ichikado K, Suga M, Taniguchi H, et al. Computed tomography findings in pathological usual interstitial pneumonia: relationship to survival. Am J Respir Crit Care Med. 2008;177(4):433–9.

    Article  PubMed  Google Scholar 

  28. Katzenstein AL, Mukhopadhyay S, Myers JL. Diagnosis of usual interstitial pneumonia and distinction from other fibrosing interstitial lung diseases. Hum Pathol. 2008;39(9):1275–94.

    Article  PubMed  Google Scholar 

  29. Katzenstein AL, Zisman DA, Litzky LA, Nguyen BT, Kotloff RM. Usual interstitial pneumonia: histologic study of biopsy and explant specimens. Am J Surg Pathol. 2002;26(12):1567–77.

    Article  PubMed  Google Scholar 

  30. Myers JL, Katzenstein AL. Beyond a consensus classification for idiopathic interstitial pneumonias: progress and controversies. Histopathology. 2009;54(1):90–103.

    Article  PubMed  Google Scholar 

  31. Myers JL, Katzenstein AL. Fibroblasts in focus. Am J Respir Crit Care Med. 2006;174(6):623–4.

    Article  PubMed  Google Scholar 

  32. Atkins SR, Turesson C, Myers JL, Tazelaar HD, Ryu JH, Matteson EL, et al. Morphologic and quantitative assessment of CD20+ B cell infiltrates in rheumatoid arthritis-associated nonspecific interstitial pneumonia and usual interstitial pneumonia. Arthritis Rheum. 2006;54(2):635–41.

    Article  PubMed  Google Scholar 

  33. Enomoto N, Suda T, Kato M, Kaida Y, Nakamura Y, Imokawa S, et al. Quantitative analysis of fibroblastic foci in usual interstitial pneumonia. Chest. 2006;130(1):22–9.

    Article  PubMed  Google Scholar 

  34. King Jr TE, Schwarz MI, Brown K, Tooze JA, Colby TV, Waldron Jr JA, et al. Idiopathic pulmonary fibrosis: relationship between histopathologic features and mortality. Am J Respir Crit Care Med. 2001;164(6):1025–32.

    Article  PubMed  Google Scholar 

  35. Nicholson AG, Fulford LG, Colby TV, du Bois RM, Hansell DM, Wells AU. The relationship between individual histologic features and disease progression in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2002;166(2):173–7.

    Article  PubMed  Google Scholar 

  36. Tiitto L, Bloigu R, Heiskanen U, Paakko P, Kinnula VL, Kaarteenaho-Wiik R. Relationship between histopathological features and the course of idiopathic pulmonary fibrosis/usual interstitial pneumonia. Thorax. 2006;61(12):1091–5.

    Article  PubMed  CAS  Google Scholar 

  37. Hanak V, Ryu JH, de Carvalho E, Limper A, Hartman TE, Decker PA, et al. Profusion of fibroblast foci in patients with idiopathic pulmonary fibrosis does not predict outcome. Respir Med. 2008;102:852–6.

    Article  PubMed  Google Scholar 

  38. Myers JL, Veal Jr CF, Shin MS, Katzenstein AL. Respiratory bronchiolitis causing interstitial lung disease. A clinicopathologic study of six cases. Am Rev Respir Dis. 1987;135(4):880–4.

    PubMed  CAS  Google Scholar 

  39. Ryu JH, Myers JL, Capizzi SA, Douglas WW, Vassallo R, Decker PA. Desquamative interstitial pneumonia and respiratory bronchiolitis-associated interstitial lung disease. Chest. 2005;127(1):178–84.

    Article  PubMed  Google Scholar 

  40. Myers JL. Nonspecific interstitial pneumonia: pathologic features and clinical implications. Semin Diagn Pathol. 2007;24(3):183–7.

    Article  PubMed  Google Scholar 

  41. Portnoy J, Veraldi KL, Schwarz MI, Cool CD, Curran-Everett D, Cherniack RM, et al. Respiratory bronchiolitis-interstitial lung disease: long-term outcome. Chest. 2007;131(3):664–71.

    Article  PubMed  Google Scholar 

  42. Craig PJ, Wells AU, Doffman S, Rassl D, Colby TV, Hansell DM, et al. Desquamative interstitial pneumonia, respiratory bronchiolitis and their relationship to smoking. Histopathology. 2004;45(3):275–82.

    Article  PubMed  CAS  Google Scholar 

  43. Yousem SA. Respiratory bronchiolitis-associated interstitial lung disease with fibrosis is a lesion distinct from fibrotic nonspecific interstitial pneumonia: a proposal. Mod Pathol. 2006;19(11):1474–9.

    PubMed  Google Scholar 

  44. 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.

    Article  PubMed  Google Scholar 

  45. Gaensler EA, Goff AM, Prowse CM. Desquamative interstitial pneumonia. N Engl J Med. 1966;274(3):113–28.

    Article  PubMed  CAS  Google Scholar 

  46. 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(5):647–53.

    Article  PubMed  Google Scholar 

  47. Wells AU, Nicholson AG, Hansell DM. Challenges in pulmonary fibrosis. 4: smoking-induced diffuse interstitial lung diseases. Thorax. 2007;62(10):904–10.

    Article  PubMed  Google Scholar 

  48. Katzenstein AL, Fiorelli RF. Nonspecific interstitial pneumonia/fibrosis. Histologic features and clinical significance. Am J Surg Pathol. 1994;18(2):136–47.

    Article  PubMed  CAS  Google Scholar 

  49. Travis WD, Hunninghake G, King Jr TE, Lynch DA, Colby TV, Galvin JR, et al. Idiopathic nonspecific interstitial pneumonia: report of an American Thoracic Society project. Am J Respir Crit Care Med. 2008;177(12):1338–47.

    Article  PubMed  Google Scholar 

  50. 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(5):1010–9 [erratum appears in Eur Respir J 1999 Mar;13(3):711].

    Article  PubMed  CAS  Google Scholar 

  51. 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(1):19–33.

    Article  PubMed  CAS  Google Scholar 

  52. Latsi PI, du Bois RM, Nicholson AG, Colby TV, Bisirtzoglou D, Nikolakopoulou A, et al. Fibrotic idiopathic interstitial pneumonia: the prognostic value of longitudinal functional trends. Am J Respir Crit Care Med. 2003;168(5):531–7.

    Article  PubMed  Google Scholar 

  53. Shin KM, Lee KS, Chung MP, Han J, Bae YA, Kim TS, et al. Prognostic determinants among clinical, thin-section CT, and histopathologic findings for fibrotic idiopathic interstitial pneumonia: tertiary hospital study. Radiology. 2008;249(1):328–37.

    Article  PubMed  Google Scholar 

  54. Jegal Y, Kim DS, Shim TS, Lim CM, Do Lee S, Koh 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.

    Article  PubMed  Google Scholar 

  55. Flaherty KR, Andrei AC, King Jr TE, Raghu G, Colby TV, Wells A, et al. Idiopathic interstitial pneumonia: do community and academic physicians agree on diagnosis? Am J Respir Crit Care Med. 2007;175(10):1054–60.

    Article  PubMed  Google Scholar 

  56. Flaherty KR, King Jr TE, Raghu G, Lynch 3rd JP, Colby TV, Travis WD, 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.

    Article  PubMed  Google Scholar 

  57. Hubbard R, Venn A. The impact of coexisting connective tissue disease on survival in patients with fibrosing alveolitis. Rheumatology. 2002;41(6):676–9.

    Article  PubMed  CAS  Google Scholar 

  58. Kocheril SV, Appleton BE, Somers EC, Kazerooni EA, Flaherty KR, Martinez FJ, 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.

    Article  PubMed  CAS  Google Scholar 

  59. Park JH, Kim DS, Park IN, Jang SJ, Kitaichi M, Nicholson AG, 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.

    Article  PubMed  Google Scholar 

  60. Copley SJ, Wells AU, Sivakumaran P, Rubens MB, Lee YC, Desai SR, et al. Asbestosis and idiopathic pulmonary fibrosis: comparison of thin-section CT features. Radiology. 2003;229(3):731–6.

    Article  PubMed  Google Scholar 

  61. Gaensler EA, Jederlinic PJ, Churg A. Idiopathic pulmonary fibrosis in asbestos-exposed workers. Am Rev Respir Dis. 1991;144(3 Pt 1):689–96.

    Article  PubMed  CAS  Google Scholar 

  62. Perez-Padilla R, Salas J, Chapela R, Sanchez M, Carrillo G, Perez 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(1):49–53.

    Article  PubMed  CAS  Google Scholar 

  63. Churg A, Sin DD, Everett D, Brown K, Cool C. Pathologic patterns and survival in chronic hypersensitivity pneumonitis. Am J Surg Pathol. 2009;33(12):1765–70.

    Article  PubMed  Google Scholar 

  64. Hayakawa H, Shirai M, Sato A, Yoshizawa Y, Todate A, Imokawa S, et al. Clinicopathological features of chronic hypersensitivity pneumonitis. Respirology. 2002;7(4):359–64.

    Article  PubMed  Google Scholar 

  65. Ohtani Y, Saiki S, Kitaichi M, Usui Y, Inase N, Costabel U, 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(8):665–71.

    Article  PubMed  CAS  Google Scholar 

  66. Trahan S, Hanak V, Ryu JH, Myers JL. Role of surgical lung biopsy in separating chronic hypersensitivity pneumonia from usual interstitial pneumonia/idiopathic pulmonary fibrosis: analysis of 31 biopsies from 15 patients. Chest. 2008;134(1):126–32.

    Article  PubMed  Google Scholar 

  67. Flaherty KR, Travis WD, Colby TV, Toews GB, Kazerooni EA, Gross BH, et al. Histopathologic variability in usual and nonspecific interstitial pneumonias. Am J Respir Crit Care Med. 2001;164(9):1722–7.

    Article  PubMed  CAS  Google Scholar 

  68. 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(2):522–6.

    Article  PubMed  Google Scholar 

  69. Berbescu EA, Katzenstein AL, Snow JL, Zisman DA. Transbronchial biopsy in usual interstitial pneumonia. Chest. 2006;129(5):1126–31.

    Article  PubMed  Google Scholar 

  70. Ensminger SA, Prakash UB. Is bronchoscopic lung biopsy helpful in the management of patients with diffuse lung disease? Eur Respir J. 2006;28(6):1081–4.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Divisions of Anatomic Pathology and MLabs, Department of Pathology, University of Michigan, 1500 East Medical Center Drive, 2G332UH, Ann Arbor, MI, 48109, USA

    Jeffrey L. Myers M.D.

Authors
  1. Jeffrey L. Myers M.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Jeffrey L. Myers M.D. .

Editor information

Editors and Affiliations

  1. Dept. Internal Medicine, Sect. Allergy Pulmonary & Critical Care Med., University of Wisconsin School of Medicine & Public Health, Madison, Wisconsin, USA

    Keith C. Meyer

  2. Advanced Lung Disease and Lung Transplant Program, Inova Fairfax Hospital, Falls Church, Virginia, USA

    Steven D. Nathan

Rights and permissions

Reprints and permissions

Copyright information

© 2014 Springer Science+Business Media New York

About this chapter

Cite this chapter

Myers, J.L. (2014). Histopathology of IPF and Related Disorders. In: Meyer, K., Nathan, S. (eds) Idiopathic Pulmonary Fibrosis. Respiratory Medicine, vol 9. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-62703-682-5_3

Download citation

  • DOI: https://doi.org/10.1007/978-1-62703-682-5_3

  • Published:

  • Publisher Name: Humana Press, Totowa, NJ

  • Print ISBN: 978-1-62703-681-8

  • Online ISBN: 978-1-62703-682-5

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation