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Diffuse interstitial lung disease: overlaps and uncertainties

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Abstract

Histopathological analysis of lung biopsy material allows the diagnosis of idiopathic interstitial pneumonias; however, the strength of this diagnosis is sometimes subverted by interobserver variation and sampling. The American Thoracic Society and European Respiratory Society recommendations of 2002 provide a framework for the diagnosis of interstitial lung disease (ILD) and proposed an integrated clinical, radiological and histopathological approach. These recommendations represent a break with tradition by replacing the ‘gold standard’ of histopathology with the combined ‘silver standards’ of clinical, imaging and histopathological information. One of the pitfalls of a rigid classification system for the diagnosis of interstitial lung disease is its failure to accommodate the phenomenon of overlapping disease patterns. This article reviews the various ways that interstitial lung disease may be classified and discusses their applicability. In addition the issue of overlap disease patterns is considered in the context of histopathological interobserver variation and sampling error and how a pigeonhole approach to disease classification may overlook these hybrid entities.

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References

  1. Coultas DB, Zumwalt RE, Black WC, Sobonya RE (1994) The epidemiology of interstitial lung diseases. Am J Respir Crit Care Med 150:967–672

    CAS  PubMed  Google Scholar 

  2. Bjoraker JA, Ryu JH, Edwin MK et al (1998) Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 157:199–203

    CAS  PubMed  Google Scholar 

  3. Visscher DW, Myers JL (2006) Histologic spectrum of idiopathic interstitial pneumonias. Proc Am Thorac Soc 3:322–329

    Article  PubMed  Google Scholar 

  4. Swensen SJ, Aughenbaugh GL, Myers JL (1997) Diffuse lung disease. Diagnostic accuracy of CT in patients undergoing surgical biopsy of the lung. Radiology 205:229–234

    CAS  PubMed  Google Scholar 

  5. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias (2002) 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 165:277–304

    Google Scholar 

  6. American Thoracic Society (2000) 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 161:646–664

    Google Scholar 

  7. Collard HR, King TE Jr (2003) Demystifying idiopathic interstitial pneumonia. Arch Intern Med 163:17–29

    Article  PubMed  Google Scholar 

  8. Travis WD, Hunninghake G, King TE Jr et al (2008) Idiopathic nonspecific interstitial pneumonia: report of an American Thoracic Society project. Am J Respir Crit Care Med 177:1338–1347

    Article  PubMed  Google Scholar 

  9. Liebow AA (1969) The interstitial pneumonias. Grune & Stratton, New York

  10. Ohori NP, Sciurba FC, Owens GR, Hodgson MJ, Yousem SA (1989) Giant-cell interstitial pneumonia and hard-metal pneumoconiosis. A clinicopathologic study of four cases and review of the literature. Am J Surg Pathol 13:581–587

    Article  CAS  PubMed  Google Scholar 

  11. Nicholson AG (2002) Classification of idiopathic interstitial pneumonias: making sense of the alphabet soup. Histopathology 41:381–391

    Article  CAS  PubMed  Google Scholar 

  12. Katzenstein AL, Fiorelli RF (1994) Nonspecific interstitial pneumonia/fibrosis. Histologic features and clinical significance. Am J Surg Pathol 18:136–147

    Article  CAS  PubMed  Google Scholar 

  13. Raghu G, Mageto YN, Lockhart D et al (1999) The accuracy of the clinical diagnosis of new-onset idiopathic pulmonary fibrosis and other interstitial lung disease: a prospective study. Chest 116:1168–1174

    Article  CAS  PubMed  Google Scholar 

  14. Hunninghake GW, Zimmerman MB, Schwartz DA et al (2001) Utility of a lung biopsy for the diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 164:193–196

    CAS  PubMed  Google Scholar 

  15. Churg A, Muller NL (2006) Cellular vs fibrosing interstitial pneumonias and prognosis: a practical classification of the idiopathic interstitial pneumonias and pathologically/radiologically similar conditions. Chest 130:1566–1570

    Article  PubMed  Google Scholar 

  16. Craig PJ, Wells AU, Doffman S et al (2004) Desquamative interstitial pneumonia, respiratory bronchiolitis and their relationship to smoking. Histopathology 45:275–282

    Article  CAS  PubMed  Google Scholar 

  17. Wells AU (2008) In: Albert RK, Spiro SG, Jett JR (eds) Clinical respiratory medicine. Mosby, London, pp 655–666

  18. Goh NS, Desai SR, Veeraraghavan S et al (2008) Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med 177:1248–1254

    Article  PubMed  Google Scholar 

  19. Flaherty KR, Travis WD, Colby TV et al (2001) Histopathologic variability in usual and nonspecific interstitial pneumonias. Am J Respir Crit Care Med 164:1722–1727

    CAS  PubMed  Google Scholar 

  20. Flint A, Martinez FJ, Young ML et al (1995) Influence of sample number and biopsy site on the histologic diagnosis of diffuse lung disease. Ann Thorac Surg 60:1605–1607; discussion 1607–8

    Article  CAS  PubMed  Google Scholar 

  21. Flaherty KR, Thwaite EL, Kazerooni EA et al (2003) Radiological versus histological diagnosis in UIP and NSIP: survival implications. Thorax 58:143–148

    Article  CAS  PubMed  Google Scholar 

  22. Nicholson AG, Addis BJ, Bharucha H et al (2004) Inter-observer variation between pathologists in diffuse parenchymal lung disease. Thorax 59:500–505

    Article  CAS  PubMed  Google Scholar 

  23. Wells AU (2004) Histopathologic diagnosis in diffuse lung disease: an ailing gold standard. Am J Respir Crit Care Med 170:828–829

    Article  PubMed  Google Scholar 

  24. Flaherty KR, King TE Jr, Raghu G et al (2004) Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis? Am J Respir Crit Care Med 170:904–910

    Article  PubMed  Google Scholar 

  25. Heyneman LE, Ward S, Lynch DA et al (1999) Respiratory bronchiolitis, respiratory bronchiolitis-associated interstitial lung disease, and desquamative interstitial pneumonia: different entities or part of the spectrum of the same disease process? AJR Am J Roentgenol 173:1617–1622

    CAS  PubMed  Google Scholar 

  26. Vassallo R, Jensen EA, Colby TV et al (2003) The overlap between respiratory bronchiolitis and desquamative interstitial pneumonia in pulmonary Langerhans cell histiocytosis: high-resolution CT, histologic, and functional correlations. Chest 124:1199–1205

    Article  PubMed  Google Scholar 

  27. Ryu JH, Myers JL, Capizzi SA et al (2005) Desquamative interstitial pneumonia and respiratory bronchiolitis-associated interstitial lung disease. Chest 127:178–184

    Article  PubMed  Google Scholar 

  28. Remy-Jardin M, Edme JL, Boulenguez C et al (2002) Longitudinal follow-up study of smoker’s lung with thin-section CT in correlation with pulmonary function tests. Radiology 222:261–270

    Article  PubMed  Google Scholar 

  29. Soejima K, Yamaguchi K, Kohda E et al (2000) Longitudinal follow-up study of smoking-induced lung density changes by high-resolution computed tomography. Am J Respir Crit Care Med 161:1264–1273

    CAS  PubMed  Google Scholar 

  30. Howling SJ, Evans TW, Hansell DM (1998) The significance of bronchial dilatation on CT in patients with adult respiratory distress syndrome. Clin Radiol 53:105–109

    Article  CAS  PubMed  Google Scholar 

  31. Park JS, Lee KS, Kim JS et al (1995) Nonspecific interstitial pneumonia with fibrosis: radiographic and CT findings in seven patients. Radiology 195:645–648

    CAS  PubMed  Google Scholar 

  32. Cottin V, Donsbeck AV, Revel D, Loire R, Cordier JF (1998) Nonspecific interstitial pneumonia. Individualization of a clinicopathologic entity in a series of 12 patients. Am J Respir Crit Care Med 158:1286–1293

    CAS  PubMed  Google Scholar 

  33. Tazelaar HD, Viggiano RW, Pickersgill J, Colby TV (1990) Interstitial lung disease in polymyositis and dermatomyositis. Clinical features and prognosis as correlated with histologic findings. Am Rev Respir Dis 141:727–733

    CAS  PubMed  Google Scholar 

  34. Mino M, Noma S, Taguchi Y et al (1997) Pulmonary involvement in polymyositis and dermatomyositis: sequential evaluation with CT. AJR Am J Roentgenol 169:83–87

    CAS  PubMed  Google Scholar 

  35. Franquet T, Hansell DM, Senbanjo T, Remy-Jardin M, Muller NL (2003) Lung cysts in subacute hypersensitivity pneumonitis. J Comput Assist Tomogr 27:475–478

    Article  PubMed  Google Scholar 

  36. Agarwal R, Aggarwal AN, Gupta D (2007) Another cause of reverse halo sign: Wegener’s granulomatosis. Br J Radiol 80:849–850

    Article  CAS  PubMed  Google Scholar 

  37. Travis WD, Matsui K, Moss J, Ferrans VJ (2000) 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 24:19–33

    Article  CAS  PubMed  Google Scholar 

  38. Hartman TE, Swensen SJ, Hansell DM et al (2000) Nonspecific interstitial pneumonia: variable appearance at high-resolution chest CT. Radiology 217:701–705

    CAS  PubMed  Google Scholar 

  39. Liebow AA (1977) Definition and classification of interstitial pneumonias in human pathology. Prog Respir Res:1–33

  40. Moriyama H, Kobayashi M, Takada T et al (2007) Two-dimensional analysis of elements and mononuclear cells in hard metal lung disease. Am J Respir Crit Care Med 176:70–77

    Article  PubMed  Google Scholar 

  41. Johkoh T, Muller NL, Cartier Y et al (1999) Idiopathic interstitial pneumonias: diagnostic accuracy of thin-section CT in 129 patients. Radiology 211:555–560

    CAS  PubMed  Google Scholar 

  42. MacDonald SL, Rubens MB, Hansell DM et al (2001) Nonspecific interstitial pneumonia and usual interstitial pneumonia: comparative appearances at and diagnostic accuracy of thin-section CT. Radiology 221:600–605

    Article  CAS  PubMed  Google Scholar 

  43. Flaherty KR, Andrei AC, King TE Jr et al (2007) Idiopathic interstitial pneumonia: do community and academic physicians agree on diagnosis? Am J Respir Crit Care Med 175:1054–1060

    Article  PubMed  Google Scholar 

  44. Park IN, Jegal Y, Kim DS et al (2009) Clinical course and lung function change of idiopathic nonspecific interstitial pneumonia. Eur Respir J 33:68–76

    Article  CAS  PubMed  Google Scholar 

  45. Flaherty KR, Toews GB, Travis WD et al (2002) Clinical significance of histological classification of idiopathic interstitial pneumonia. Eur Respir J 19:275–283

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Radiology, Royal Brompton Hospital, Sydney Street, London, SW3 6NP, UK

    Simon L. F. Walsh & David M. Hansell

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  1. Simon L. F. Walsh
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  2. David M. Hansell
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Correspondence to David M. Hansell.

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Walsh, S.L.F., Hansell, D.M. Diffuse interstitial lung disease: overlaps and uncertainties. Eur Radiol 20, 1859–1867 (2010). https://doi.org/10.1007/s00330-010-1737-3

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  • DOI: https://doi.org/10.1007/s00330-010-1737-3

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