Semin Respir Crit Care Med 2006; 27(6): 623-633
DOI: 10.1055/s-2006-957334
Copyright © 2006 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

High-Resolution Computed Tomography of Interstitial Pulmonary Fibrosis

Robert D. Suh1 , Jonathan G. Goldin1
  • 1Department of Radiology, David Geffen School of Medicine at UCLA, Los Angeles, California
Further Information

Publication History

Publication Date:
29 December 2006 (online)

ABSTRACT

Idiopathic pulmonary fibrosis (IPF), also referred to as cryptogenic fibrosing alveolitis (CFA), is the clinical-radiological-pathological syndrome associated with the most common form of fibrosing interstitial pneumonia, usual interstitial pneumonia (UIP). Unfortunately, the diagnosis of IPF carries with it a relatively poor prognosis. The characteristic features on high-resolution computed tomography (HRCT), consisting of reticular abnormality and honeycombing with basal and peripheral predominance, are virtually diagnostic within the correct clinical context. The extent of fibrosis on HRCT is an important prognostic indicator. When ground-glass attenuation is seen in patients with IPF, it commonly progresses to fibrosis and honeycombing. Complications of IPF include accelerated progression and deterioration, lung cancer, spontaneous air leak, and secondary infection. This article discusses the key imaging features as correlated with histopathology, differential diagnosis, complications, and follow-up evaluation of idiopathic pulmonary fibrosis.

REFERENCES

  • 1 Katzenstein A L, Myers J L. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification.  Am J Respir Crit Care Med. 1998;  157 1301-1315
  • 2 Muller N L, Coiby T V. Idiopathic interstitial pneumonias: high-resolution CT and histologic findings.  Radiographics. 1997;  17 1016-1022
  • 3 Ryu J H, Colby T V, Hartman T E. Idiopathic pulmonary fibrosis: current concepts.  Mayo Clin Proc. 1998;  73 1085-1101
  • 4 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;  161 646-664
  • 5 Coultas D B, Zumwalt R E, Black W C, Sobonya R E. The epidemiology of interstitial lung diseases.  Am J Respir Crit Care Med. 1994;  150 967-972
  • 6 Johnston I, Britton J, Kinnear W, Logan R. Rising mortality from cryptogenic fibrosing alveolitis.  BMJ. 1990;  301 1017-1021
  • 7 Schwarz M I. Idiopathic pulmonary fibrosis.  West J Med. 1988;  149 199-203
  • 8 Bjoraker J A, Ryu J H, Edwin M K et al.. Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis.  Am J Respir Crit Care Med. 1998;  157 199-203
  • 9 Katzenstein A L, Myers J L. Nonspecific interstitial pneumonia and the other idiopathic interstitial pneumonias: classification and diagnostic criteria.  Am J Surg Pathol. 2000;  24 1-3
  • 10 Crystal R G, Fulmer J D, Roberts W C, Moss M L, Line B R, Reynolds H Y. Idiopathic pulmonary fibrosis: clinical, histologic, radiographic, physiologic, scintigraphic, cytologic and biochemical aspects.  Ann Intern Med. 1976;  85 769-788
  • 11 Mathieson J R, Mayo J R, Staples C A, Muller N L. Chronic diffuse infiltrative lung disease: comparison of diagnostic accuracy of CT and chest radiography.  Radiology. 1989;  171 111-116
  • 12 Muller N L, Guerry-Force M L, Staples C A et al.. Differential diagnosis of bronchiolitis obliterans with organizing pneumonia and usual interstitial pneumonia: clinical, functional and radiologic findings.  Radiology. 1987;  162 151-156
  • 13 Nishimura K, Kitiaichi M, Izumi T, Nagai S, Kanaoka M, Itoh H. Usual interstitial pneumonia: histologic correlation with high-resolution CT.  Radiology. 1992;  182 337-342
  • 14 Hiwatari N, Shimura S, Takishima T. Pulmonary emphysema followed by pulmonary fibrosis of undetermined cause.  Respiration (Int'l Rev Thorac Dis). 1993;  60 354-358
  • 15 Goldin J G. Quantitative CT of the lung.  Radiol Clin North Am. 2002;  40 145-162
  • 16 Marchiori E, Souza A SJ, Franquet T, Muller N L. Diffuse high-attenuation pulmonary abnormalities: a pattern-oriented diagnostic approach on high-resolution CT.  AJR Am J Roentgenol. 2005;  184 273-282
  • 17 Orens J B, Kazerooni E A, Martinez F J et al.. The sensitivity of high-resolution CT in detecting idiopathic pulmonary fibrosis proved by open lung biopsy: a prospective study.  Chest. 1995;  108 109-115
  • 18 Hunninghake G W, Lynch D A, Galvin J R et al.. Radiologic findings are strongly associated with a pathologic diagnosis of usual interstitial pneumonia.  Chest. 2003;  124(4) 1215-1223
  • 19 Franquet T, Gimenez A, Alegret X, Rodriguez-Arias J M. Mediastinal lymphadenopathy in cryptogenic fibrosing alveolitis: the effect of steroid therapy on the prevalence of nodal enlargement.  Clin Radiol. 1998;  53 435-438
  • 20 Niimi H, Kang E Y, Kwong J S, Carignan S, Muller N L. CT of chronic infiltrative lung disease: prevalence of mediastinal lymphadenopathy.  J Comput Assist Tomogr. 1996;  20 305-308
  • 21 Bergin C J, Coblentz C L, Chiles C et al.. Chronic lung diseases: specific diagnosis by using CT.  AJR Am J Roentgenol. 1989;  152 1183-1188
  • 22 Grenier P, Chevret S, Beigelman C et al.. Chronic diffuse infiltrative lung disease: determination of the diagnostic value of clinical data, chest radiography, and CT and Bayesian analysis.  Radiology. 1994;  191 383-390
  • 23 Mathieson J R, Mayo J R, Staples C A, Muller N L. Chronic diffuse infiltrative lung disease: comparison of diagnostic accuracy of CT and chest radiography.  Radiology. 1989;  171 111-116
  • 24 Padley S P, Hansell D M, Flower C D, Jennings P. Comparative accuracy of high resolution computed tomography and chest radiography in the diagnosis of chronic diffuse infiltrative lung disease.  Clin Radiol. 1991;  44 222-226
  • 25 Tung K T, Wells A U, Rubens M B, Kirk J M, du Bois R M, Hansell D M. Accuracy of the typical computed tomographic appearances of fibrosing alveolitis.  Thorax. 1993;  48 334-338
  • 26 Grenier P, Valeyre D, Cluzel P, Brauner M W, Lenoir S, Chastang C. Chronic diffuse interstitial lung disease: diagnostic value of chest radiography and high-resolution CT.  Radiology. 1991;  179 123-132
  • 27 Lynch D A, Newell J D, Logan P M, King Jr T E, Muller N L. Can CT distinguish hypersensitivity pneumonitis from idiopathic pulmonary fibrosis?.  AJR Am J Roentgenol. 1995;  165(4) 807-811
  • 28 Wells A U, Hansell D M, Rubens M B, Cullinan P, Black C M, du Bois R M. The predictive value of appearances on thin-section computed tomography in fibrosing alveolitis.  Am Rev Respir Dis. 1993;  148 1076-1082
  • 29 Raghu G, Mageto Y N, Lockhart D, Schmidt R A, Wood D E, Godwin J D. The accuracy of the clinical diagnosis of new-onset idiopathic pulmonary fibrosis and other interstitial lung disease: a prospective study.  Chest. 1999;  116 1168-1174
  • 30 Hunninghake G W, Zimmerman M B, Schwartz D A et al.. Utility of a lung biopsy for the diagnosis of idiopathic pulmonary fibrosis.  Am J Respir Crit Care Med. 2001;  164 193-196
  • 31 Swensen S J, Aughenbaugh G L, Myers J L. Diffuse lung disease: diagnostic accuracy of CT in patients undergoing surgical biopsy of the lung.  Radiology. 1997;  205 229-234
  • 32 Collins C D, Wells A U, Hansell D M et al.. Observer variation in pattern type and extent of disease in fibrosing alveolitis on thin section computed tomography and chest radiography.  Clin Radiol. 1994;  49 236-240
  • 33 Kazerooni E A, Martinez F J, Flint A 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;  169 977-983
  • 34 Herold C J, Brown R H, Mitzner W, Links J M, Hirshman C A, Zerhouni E A. Assessment of pulmonary airway reactivity with high-resolution CT.  Radiology. 1991;  181 369-374
  • 35 Coxson H O, Hogg J C, Mayo J R et al.. Quantification of idiopathic pulmonary fibrosis using computed tomography and histology.  Am J Respir Crit Care Med. 1997;  155 1649-1656
  • 36 Johkoh T, Ikezoe J, Kohno N et al.. High-resolution CT and pulmonary function tests in collagen vascular disease: comparison with idiopathic pulmonary fibrosis.  Eur J Radiol. 1994;  18 113-121
  • 37 Chan T Y, Hansell D M, Rubens M B, du Bois R M, Wells A U. Cryptogenic fibrosing alveolitis and the fibrosing alveolitis of systemic sclerosis: morphological differences on computed tomographic scans.  Thorax. 1997;  52 265-270
  • 38 Aberle D R, Gamsu G, Ray C S. High-resolution CT of benign asbestos-related diseases: clinical and radiographic correlation.  AJR Am J Roentgenol. 1988;  151 883-891
  • 39 Padley S P, Padhani A R, Nicholson A, Hansell D M. Pulmonary sarcoidosis mimicking cryptogenic fibrosing alveolitis on CT.  Clin Radiol. 1996;  51 807-810
  • 40 Akira M, Hamada H, Sakatani M, Kobayashi C, Nishioka M, Yamamoto S. CT findings during phase of accelerated deterioration in patients with idiopathic pulmonary fibrosis.  AJR Am J Roentgenol. 1997;  168 79-83
  • 41 Lee H J, Im J G, Ahn J M, Yeon K M. Lung cancer in patients with idiopathic pulmonary fibrosis: CT findings.  J Comput Assist Tomogr. 1996;  20 979-982
  • 42 Mizushima Y, Kobayashi M. Clinical characteristics of synchronous multiple lung cancer associated with idiopathic pulmonary fibrosis: a review of Japanese cases.  Chest. 1995;  108 1272-1277
  • 43 Akira M, Sakatani M, Ueda E. Idiopathic pulmonary fibrosis: progression of honeycombing at thin-section CT.  Radiology. 1993;  189 687-691
  • 44 Lee J S, Gong G, Song K S, Kim D S, Lim T H. Usual interstitial pneumonia: relationship between disease activity and the progression of honeycombing at thin-section computed tomography.  J Thorac Imaging. 1998;  13 199-203
  • 45 Terriff B A, Kwan S Y, Chan-Yeung M M, Muller N L. Fibrosing alveolitis: chest radiography and CT as predictors of clinical and functional impairment at follow-up in 26 patients.  Radiology. 1992;  184 445-449
  • 46 Wells A U, Rubens M B, du Bois R M, Hansell D M. Serial CT in fibrosing alveolitis: prognostic significance of the initial pattern.  AJR Am J Roentgenol. 1993;  161 1159-1165
  • 47 Gay S E, Kazerooni E A, Toews G B et al.. Idiopathic pulmonary fibrosis: predicting response to therapy and survival.  Am J Respir Crit Care Med. 1998;  157 1063-1072
  • 48 Hartman T E, Primack S L, Kang E Y et al.. Disease progression in usual interstitial pneumonia compared with desquamative interstitial pneumonia: assessment with serial CT.  Chest. 1996;  110 378-382

Jonathan G GoldinMBChB Ph.D. 

Department of Radiology, David Geffen School of Medicine at UCLA

10833 Le Conte Ave., B2-165 CHS, Los Angeles, CA 90095-1721

Email: jgoldin@mednet.ucla.edu

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