"skipMainNavigation"
closeDrawerMenuopenDrawerMenu
Search
Quick Search in Journals
Quick Search anywhere
Advanced Search
Radiology
Menu
  • Information
    • Previous
    Next
    ORIGINAL RESEARCH

    Biopsy-proved Idiopathic Pulmonary Fibrosis: Spectrum of Nondiagnostic Thin-Section CT Diagnoses

    Published Online:Feb 8 2010https://doi.org/10.1148/radiol.0990898

    Abstract

    Idiopathic pulmonary fibrosis may be misdiagnosed at thin-section CT as nonspecific interstitial pneumonia, chronic hypersensitivity pneumonitis, or sarcoidosis.

    Purpose

    To document the spectrum of misleading thin-section computed tomographic (CT) diagnoses in patients with biopsy-proved idiopathic pulmonary fibrosis (IPF).

    Materials and Methods

    This study had institutional review board approval, and patient consent was not required. Three observers, blinded to any clinical information and the purpose of the study, recorded thin-section CT differential diagnoses and assigned a percentage likelihood to each for a group of 123 patients (79 men, 44 women; age range, 27–82 years) with various chronic interstitial lung diseases, including a core group of 55 biopsy-proved cases of IPF. Patients with IPF in the core group, in whom IPF was diagnosed as low-grade probability (<30%) by at least two observers, were considered to have atypical IPF cases, and the alternative diagnoses were analyzed further.

    Results

    Thirty-four (62%) of 55 biopsy-proved IPF cases were regarded as alternative diagnoses. In these atypical IPF cases, the first-choice diagnoses, expressed with high degree of probability, were nonspecific interstitial pneumonia (NSIP; 18 [53%] of 34), chronic hypersensitivity pneumonitis (HP; four [12%] of 34), sarcoidosis (three [9%] of 34), and organizing pneumonia (one [3%] of 34); in eight (23%) of 34 cases, no single diagnosis was favored by more than one observer. Frequent differential diagnoses, although not always the first-choice diagnosis, were NSIP (n = 29), chronic HP (n = 23), and sarcoidosis (n = 9).

    Conclusion

    In the correct clinical setting, a diagnosis of IPF is not excluded by thin-section CT appearances more suggestive of NSIP, chronic HP, or sarcoidosis.

    © RSNA, 2010

    References

    • 1 Kim DS, Collard HR, King TE. Classification and natural history of the idiopathic interstitial pneumonias. Proc Am Thorac Soc 2006;3(4):285–292. Crossref, Medline, Google Scholar
    • 2 Maher TM, Wells AU, Laurent GJ. Idiopathic pulmonary fibrosis: multiple causes and multiple mechanisms?. Eur Respir J 2007;30(5):835–839. Crossref, Medline, Google Scholar
    • 3 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.[Published correction appears in Am J Respir Crit Care Med 2002;166(3):426.]. Crossref, Medline, Google Scholar
    • 4 Mueller-Mang C, Grosse C, Schmid K, Stiebellehner L, Bankier AA. What every radiologist should know about idiopathic interstitial pneumonias. RadioGraphics 2007;27(3):595–615. Link, Google Scholar
    • 5 Lynch DA, Travis WD, Müller NLet al.. Idiopathic interstitial pneumonias: CT features. Radiology 2005;236(1):10–21. Link, Google Scholar
    • 6 Hunninghake GW, Zimmerman MB, Schwartz DAet al.. Utility of a lung biopsy for the diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2001;164(2):193–196. Crossref, Medline, Google Scholar
    • 7 Flaherty KR, Thwaite EL, Kazerooni EAet al.. Radiological versus histological diagnosis in UIP and NSIP: survival implications. Thorax 2003;58(2):143–148. Crossref, Medline, Google Scholar
    • 8 Lynch DA, Godwin JD, Safrin Set al.. High-resolution computed tomography in idiopathic pulmonary fibrosis: diagnosis and prognosis. Am J Respir Crit Care Med 2005;172(4):488–493. Crossref, Medline, Google Scholar
    • 9 Sumikawa H, Johkoh T, Colby TVet al.. Computed tomography findings in pathological usual interstitial pneumonia: relationship to survival. Am J Respir Crit Care Med 2008;177(4):433–439. Crossref, Medline, Google Scholar
    • 10 MacDonald SL, Rubens MB, Hansell DMet al.. Nonspecific interstitial pneumonia and usual interstitial pneumonia: comparative appearances at and diagnostic accuracy of thin-section CT. Radiology 2001;221(3):600–605. Link, Google Scholar
    • 11 Coblentz CL, Babcook CJ, Alton D, Riley BJ, Norman G. Observer variation in detecting the radiologic features associated with bronchiolitis. Invest Radiol 1991;26(2):115–118. Crossref, Medline, Google Scholar
    • 12 Flaherty KR, Mumford JA, Murray Set al.. Prognostic implications of physiologic and radiographic changes in idiopathic interstitial pneumonia. Am J Respir Crit Care Med 2003;168(5):543–548. Crossref, Medline, Google Scholar
    • 13 Elliot TL, Lynch DA, Newell JDet al.. High-resolution computed tomography features of nonspecific interstitial pneumonia and usual interstitial pneumonia. J Comput Assist Tomogr 2005;29(3):339–345. Crossref, Medline, Google Scholar
    • 14 Sumikawa H, Johkoh T, Ichikado Ket al.. Usual interstitial pneumonia and chronic idiopathic interstitial pneumonia: analysis of CT appearance in 92 patients. Radiology 2006;241(1):258–266. Link, Google Scholar
    • 15 Silva CI, Müller NL, Hansell DM, Lee KS, Nicholson AG, Wells AU. Nonspecific interstitial pneumonia and idiopathic pulmonary fibrosis: changes in pattern and distribution of disease over time. Radiology 2008;247(1):251–259. Link, Google Scholar
    • 16 Silva CI, Müller NL, Lynch DAet al.. Chronic hypersensitivity pneumonitis: differentiation from idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia by using thin-section CT. Radiology 2008;246(1):288–297. Link, Google Scholar
    • 17 Zompatori M, Calabrò E, Chetta A, Chiari G, Marangio E, Olivieri D. Chronic hypersensitivity pneumonitis or idiopathic pulmonary fibrosis? Diagnostic role of high resolution computed tomography (HRCT). Radiol Med 2003;106(3):135–146. Medline, Google Scholar
    • 18 Johkoh T, Müller NL, Cartier Yet al.. Idiopathic interstitial pneumonias: diagnostic accuracy of thin-section CT in 129 patients. Radiology 1999;211(2):555–560. Link, Google Scholar
    • 19 Padley SP, Padhani AR, Nicholson A, Hansell DM. Pulmonary sarcoidosis mimicking cryptogenic fibrosing alveolitis on CT. Clin Radiol 1996;51(11):807–810. Crossref, Medline, Google Scholar
    • 20 Demedts M, Behr J, Buhl Ret al.. High-dose acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med 2005;353(21):2229–2242. Crossref, Medline, Google Scholar
    • 21 Latsi PI, du Bois RM, Nicholson AGet al.. Fibrotic idiopathic interstitial pneumonia: the prognostic value of longitudinal functional trends. Am J Respir Crit Care Med 2003;168(5):531–537. Crossref, Medline, Google Scholar
    • 22 Flaherty KR, Andrei AC, King TEet al.. Idiopathic interstitial pneumonia: do community and academic physicians agree on diagnosis?. Am J Respir Crit Care Med 2007;175(10):1054–1060. Crossref, Medline, Google Scholar

    Article History

    Received May 22, 2009; revision requested July 12; final revision received September 18; accepted September 29; final version accepted October 5. M.C.
    Published online: Feb 8 2010
    Published in print: Mar 2010