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.
KEYWORDS
High-resolution computed tomography - idiopathic pulmonary fibrosis - usual interstitial
pneumonia (UIP)
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
