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Differential Diagnosis of IPF

What Should We Particularly Keep in Mind in the Differential Diagnosis?

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Idiopathic Pulmonary Fibrosis
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Abstract

The idiopathic pulmonary fibrosis (IPF) guidelines were announced in the 2011 ATS/ERS/JRA/ALAT statement, and 6 items were listed as Summary Conclusions. Diagnosis is described in item 2, and both exhibiting a UIP pattern on high-resolution computed tomography (HRCT) scans, and that finding being consistent with the SLB findings are said to be necessary. The factors that are suspected of being associated with IPF and are important to the differential diagnosis have also been sorted out, and we consider them to mainly consist of three categories of factors and miscellaneous other factors. The first category is chronic hypersensitivity pneumonitis (CHP)/Feather duvet lung (FDL) caused by environmental factors. The second category is autoimmune-featured interstitial lung disease (AIF-ILD) in which lung lesions are observed first. The third category consists of PF that develops after a certain period of time has passed in patients treated with molecularly targeted drugs, anti-arrhythmia drugs, etc. The rest consist of PF caused by persistent infection. Because the time that has passed (sometimes as much as several years) after the corresponding episode is long, and the HRCT findings and SLB findings resemble the findings in a usual interstitial pneumonia (UIP), differentiation is difficult even when a diagnosis of PF has been made. A wide variety of possibilities are suspected when PF is encountered, and it is necessary to confirm the detail of past medical history and base on it to perform special tests (e.g., lymphocyte stimulation tests, antibody tests, virus DNA tests) in addition.

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References

  1. American Thoracic Society; European Respiratory Society: 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.

    Article  Google Scholar 

  2. Kondoh Y, Taniguchi H, Ogura T, Johkoh T, Fujimoto K, Sumikawa H, Kataoka K, Baba T, Colby TV, Kitaichi M. Disease progression in idiopathic pulmonary fibrosis without pulmonary function impairment. Respirology. 2013;18(5):820–6.

    Article  PubMed  Google Scholar 

  3. Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, Colby TV, Cordier JF, Flaherty KR, Lasky JA, Lynch DA, Ryu JH, Swigris JJ, Wells AU, Ancochea J, Bouros D, Carvalho C, Costabel U, Ebina M, Hansell DM, Johkoh T, Kim DS, King Jr TE, Kondoh Y, Myers J, Müller NL, Nicholson AG, Richeldi L, Selman M, Dudden RF, Griss BS, Protzko SL, Schünemann HJ. 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(6):788–824.

    Article  PubMed  Google Scholar 

  4. Travis WD, Costabel U, Hansell DM, King Jr TE, Lynch DA, Nicholson AG, Ryerson CJ, Ryu JH, Selman M, Wells AU, Behr J, Bouros D, Brown KK, Colby TV, Collard HR, Cordeiro CR, Cottin V, Crestani B, Drent M, Dudden RF, Egan J, Flaherty K, Hogaboam C, Inoue Y, Johkoh T, Kim DS, Kitaichi M, Loyd J, Martinez FJ, Myers J, Protzko S, Raghu G, Richeldi L, Sverzellati N, Swigris J, Valeyre D. ATS/ERS committee on idiopathic interstitial pneumonias: 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.

    Article  PubMed  Google Scholar 

  5. Wuyts WA, Cavazza A, Rossi G, Bonella F, Sverzellati N, Spagnolo P. Differential diagnosis of usual interstitial pneumonia: when is it truly idiopathic? Eur Respir Rev. 2014;23(133):308–19.

    Article  PubMed  Google Scholar 

  6. Margaritopoulos GA, Harari S, Caminati A, Antoniou KM. Smoking-related idiopathic interstitial pneumonia: A review. Respirology. 2015;2. doi:10.1111/resp.12576.

    Google Scholar 

  7. Sverzellati N, Guerci L, Giorgia R, Calabrò E, La Vecchia C, Marchianò A, Pesci A, Zompatori M, Pastorino U. Interstitial lung diseases in a lung cancer screening trial. Eur Respir J. 2011;38(2):392–400.

    Article  CAS  PubMed  Google Scholar 

  8. Washko GR, Hunninghake GM, Fernandez IE, Nishino M, Okajima Y, Yamashiro T, Ross JC, Estépar RS, Lynch DA, Brehm JM, Andriole KP, Diaz AA, Khorasani R, D’Aco K, Sciurba FC, Silverman EK, Hatabu H, Rosas IO, COPDGene Investigators. Lung volumes and emphysema in smokers with interstitial lung abnormalities. N Engl J Med. 2011;364(10):897–906.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  9. Jin GY, Lynch D, Chawla A, Garg K, Tammemagi MC, Sahin H, Misumi S, Kwon KS. Interstitial lung abnormalities in a CT lung cancer screening population: prevalence and progression rate. Radiology. 2013;268(2):563–71.

    Article  PubMed Central  PubMed  Google Scholar 

  10. Lacasse Y, Selman M, Costabel U, Dalphin JC, Morell F, Erkinjuntti-Pekkanen R, Mueller NL, Colby TV, Schuyler M, Jomphe V, Cormier Y, HP Study Group. Classification of hypersensitivity pneumonitis: a hypothesis. Int Arch Allergy Immunol. 2009;149(2):161–6.

    Article  PubMed  Google Scholar 

  11. Morell F, Villar A, Montero MÁ, Muñoz X, Colby TV, Pipvath S, Cruz MJ, Raghu G. Chronic hypersensitivity pneumonitis in patients diagnosed with idiopathic pulmonary fibrosis: a prospective case-cohort study. Lancet Respir Med. 2013;1(9):685–94.

    Article  PubMed  Google Scholar 

  12. Plessner M. A disease of feather sorters: duck fever [Article in French]. Arch Mal Prof. 1960;21:67–9.

    CAS  PubMed  Google Scholar 

  13. Haitjema T, van Velzen-Blad H, van den Bosch JM. Extrinsic allergic alveolitis caused by goose feathers in a duvet. Thorax. 1992;47(11):990–1.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  14. Inase N, Ohtani Y, Endo J, Miyake S, Yoshizawa Y. Feather duvet lung. Med Sci Monit. 2003;9(5):CS37–40.

    PubMed  Google Scholar 

  15. Morell F, Roger A, Reyes L, Cruz MJ, Murio C, Muñoz X. Bird fancier’s lung: a series of 86 patients. Med (Baltimore). 2008;87(2):110–30.

    Article  Google Scholar 

  16. Koschel D, Wittstruck H, Renck T, Müller-Wening D, Höffken G. Presenting features of feather duvet lung. Int Arch Allergy Immunol. 2010;152(3):264–70.

    Article  PubMed  Google Scholar 

  17. Akashi T, Takemura T, Ando N, Eishi Y, Kitagawa M, Takizawa T, Koike M, Ohtani Y, Miyazaki Y, Inase N, Yoshizawa Y. 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(3):405–15.

    Article  PubMed  Google Scholar 

  18. Takemura T, Akashi T, Kamiya H, Ikushima S, Ando T, Oritsu M, Sawahata M, Ogura T. Pathological differentiation of chronic hypersensitivity pneumonitis from idiopathic pulmonary fibrosis/usual interstitial pneumonia. Histopathology. 2012;61(6):1026–35.

    Article  PubMed  Google Scholar 

  19. Silva CI, 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(1):288–97.

    Article  PubMed  Google Scholar 

  20. Ohnishi H, Miyamoto S, Kawase S, Kubota T, Yokoyama A. Seasonal variation of serum KL-6 concentrations is greater in patients with hypersensitivity pneumonitis. BMC Pulm Med. 2014;7(14):129.

    Article  Google Scholar 

  21. Koschel D, Lützkendorf L, Wiedemann B, Höffken G. Antigen-specific IgG antibodies in feather duvet lung. Eur J Clin Invest. 2010;40(9):797–802.

    Article  CAS  PubMed  Google Scholar 

  22. Inase N, Ohtani Y, Sumi Y, Umino T, Usui Y, Miyake S, Yoshizawa Y. A clinical study of hypersensitivity pneumonitis presumably caused by feather duvets. Ann Allergy Asthma Immunol. 2006;96(1):98–104.

    Article  PubMed  Google Scholar 

  23. Cordeiro CR, Alfaro TM, Freitas S. Clinical case: differential diagnosis of idiopathic pulmonary fibrosis. BMC Res Notes. 2013;6(Suppl 1):S1.

    Google Scholar 

  24. Mosca M, Tavoni A, Neri R, Bencivelli W, Bombardieri S. Undifferentiated connective tissue diseases: the clinical and serological profiles of 91 patients followed for at least 1 year. Lupus. 1998;7(2):95–100.

    Article  CAS  PubMed  Google Scholar 

  25. Kinder BW, Collard HR, Koth L, Daikh DI, Wolters PJ, Elicker B, Jones KD, King Jr TE. Idiopathic nonspecific interstitial pneumonia: lung manifestation of undifferentiated connective tissue disease? Am J Respir Crit Care Med. 2007;176(7):691–7.

    Article  PubMed Central  PubMed  Google Scholar 

  26. 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(3):661–8.

    Article  CAS  PubMed  Google Scholar 

  27. Fischer A, West SG, Swigris JJ, Brown KK, du Bois RM. Connective tissue disease-associated interstitial lung disease: a call for clarification. Chest. 2010;138(2):251–6.

    Article  PubMed Central  PubMed  Google Scholar 

  28. Alhamad EH, Al-Kassimi FA, Alboukai AA, Raddaoui E, Al-Hajjaj MS, Hajjar W, Shaik SA. Comparison of three groups of patients with usual interstitial pneumonia. Respir Med. 2012;106(11):1575–85.

    Article  PubMed  Google Scholar 

  29. Vij R, Noth I, Strek ME. Autoimmune-featured interstitial lung disease: a distinct entity. Chest. 2011;140(5):1292–9.

    Article  PubMed Central  PubMed  Google Scholar 

  30. Kono M, Nakamura Y, Enomoto N, Hashimoto D, Fujisawa T, Inui N, Maekawa M, Suda T, Colby TV, Chida K. Usual interstitial pneumonia preceding collagen vascular disease: a retrospective case control study of patients initially diagnosed with idiopathic pulmonary fibrosis. PLoS One. 2014;9(4), e94775.

    Article  PubMed Central  PubMed  Google Scholar 

  31. Lee HK, Kim DS, Yoo B, Seo JB, Rho JY, Colby TV, Kitaichi M. Histopathologic pattern and clinical features of rheumatoid arthritis-associated interstitial lung disease. Chest. 2005;127(6):2019–27.

    Article  PubMed  Google Scholar 

  32. Hagiwara K, Sato T, Takagi-Kobayashi S, Hasegawa S, Shigihara N, Akiyama O. Acute exacerbation of preexisting interstitial lung disease after administration of etanercept for rheumatoid arthritis. J Rheumatol. 2007;34(5):1151–4.

    PubMed  Google Scholar 

  33. Thavarajah K, Wu P, Rhew EJ, Yeldandi AK, Kamp DW. Pulmonary complications of tumor necrosis factor-targeted therapy. Respir Med. 2009;103(5):661–9.

    Article  PubMed Central  PubMed  Google Scholar 

  34. Chatterjee S. Severe interstitial pneumonitis associated with infliximab therapy. Scand J Rheumatol. 2004;33(4):276–7.

    Article  CAS  PubMed  Google Scholar 

  35. Kramer N, Chuzhin Y, Kaufman LD, Ritter JM, Rosenstein ED. Methotrexate pneumonitis after initiation of infliximab therapy for rheumatoid arthritis. Arthritis Rheum. 2002;47(6):670–1.

    Article  PubMed  Google Scholar 

  36. Ando M, Okamoto I, Yamamoto N, Takeda K, Tamura K, Seto T, Ariyoshi Y, Fukuoka M. Predictive factors for interstitial lung disease, antitumor response, and survival in non-small-cell lung cancer patients treated with gefitinib. J Clin Oncol. 2006;24(16):2549–56.

    Article  CAS  PubMed  Google Scholar 

  37. Cohen MH, Williams GA, Sridhara R, Chen G, Pazdur R. FDA drug approval summary: gefitinib (ZD1839) (Iressa) tablets. Oncologist. 2003;8(4):303–6.

    Article  CAS  PubMed  Google Scholar 

  38. Ernawati DK, Stafford L, Hughes JD. Amiodarone-induced pulmonary toxicity. Br J Clin Pharmacol. 2008;66(1):82–7.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  39. Kang IS, Kim KJ, Kim Y, Park SH. The diagnostic utility of chest computed tomography scoring for the assessment of amiodarone-induced pulmonary toxicity. Korean J Intern Med. 2014;29(6):746–53.

    Article  PubMed Central  PubMed  Google Scholar 

  40. Nakajima M, Kawahara Y, Yoshida K, Miyashita N, Niki Y, Matsushima T. Serum KL-6 as a possible marker for amiodarone-induced pulmonary toxicity. Intern Med. 2000;39(12):1097–100.

    Article  CAS  PubMed  Google Scholar 

  41. Umetani K, Abe M, Kawabata K, Iida T, Kohno I, Sawanobori T, Kugiyama K. SP-D as a marker of amiodarone-induced pulmonary toxicity. Intern Med. 2002;41(9):709–12.

    Article  PubMed  Google Scholar 

  42. Naik PK, Moore BB. Viral infection and aging as cofactors for the development of pulmonary fibrosis. Expert Rev Respir Med. 2010;4(6):759–71.

    Article  PubMed Central  PubMed  Google Scholar 

  43. Ueda T, Ohta K, Suzuki N, Yamaguchi M, Hirai K, Horiuchi T, Watanabe J, Miyamoto T, Ito K. Idiopathic pulmonary fibrosis and high prevalence of serum antibodies to hepatitis C virus. Am Rev Respir Dis. 1992;146(1):266–8.

    Article  CAS  PubMed  Google Scholar 

  44. Kuwano K, Nomoto Y, Kunitake R, Hagimoto N, Matsuba T, Nakanishi Y, Hara N. Detection of adenovirus E1A DNA in pulmonary fibrosis using nested polymerase chain reaction. Eur Respir J. 1997;10(7):1445–9.

    Article  CAS  PubMed  Google Scholar 

  45. Dworniczak S, Ziora D, Kapral M, Mazurek U, Niepsuj G, Rauer R, Wilczok T, Kozielski J. Human cytomegalovirus DNA level in patients with idiopathic pulmonary fibrosis. J Physiol Pharmacol. 2004;55 Suppl 3:67–75.

    PubMed  Google Scholar 

  46. Tang YW, Johnson JE, Browning PJ, Cruz-Gervis RA, Davis A, Graham BS, Brigham KL, Oates Jr JA, Loyd JE, Stecenko AA. Herpesvirus DNA is consistently detected in lungs of patients with idiopathic pulmonary fibrosis. J Clin Microbiol. 2003;41(6):2633–40.

    Article  PubMed Central  PubMed  Google Scholar 

  47. Thannickal VJ, Horowitz JC. Evolving concepts of apoptosis in idiopathic pulmonary fibrosis. Proc Am Thorac Soc. 2006;3(4):350–6.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  48. Lawson WE, Crossno PF, Polosukhin VV, 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(6):L1119–26.

    Article  CAS  PubMed  Google Scholar 

  49. Tablan OC, Reyes MP. Chronic interstitial pulmonary fibrosis following Mycoplasma pneumoniae pneumonia. Am J Med. 1985;79(2):268–70.

    Article  CAS  PubMed  Google Scholar 

  50. Konishi K, Gibson KF, Lindell KO, et al. Gene expression profiles of acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2009;180(2):167–75.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  51. Zamo A, Poletti V, Reghellin D, et al. HHV-8 and EBV are not commonly found in idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis. 2005;22(2):123–8.

    PubMed  Google Scholar 

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Watanabe, H. (2016). Differential Diagnosis of IPF. In: Nakamura, H., Aoshiba, K. (eds) Idiopathic Pulmonary Fibrosis. Springer, Tokyo. https://doi.org/10.1007/978-4-431-55582-7_8

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  • DOI: https://doi.org/10.1007/978-4-431-55582-7_8

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