73-year-old man with 50 pack-year smoking history, progressive dyspnea, and nonproductive cough


Idiopathic pulmonary fibrosis (IPF)1


Patients are generally older than 50, with long-standing respiratory disease.

Progressive nonproductive cough and dyspnea; smoking may contribute to pathogenesis of IPF.

Median survival: two to four years; poorer prognosis compared with other interstitial lung diseases (eg, nonspecific interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia).

Key Diagnostic Features (High-Resolution CT)

Low lung volumes, subpleural reticular opacities, macrocystic honeycombing ,and traction bronchiectasis

Basilar predominance (apicobasal gradient)

Characteristic features and appropriate clinical history obviates need for biopsy: High-resolution CT pattern is virtually diagnostic. (For more information, reference ATS/ERS Multidisciplinary Consensus Statement regarding the major and minor criteria for establishing diagnosis of IPF without lung biopsy.)

Differential Diagnosis

Important to exclude potential underlying causes of pulmonary fibrosis (eg, asbestos exposure, drugs, connective tissue disorders).


Does not respond to high-dose steroids; might even be contraindicated.

Early diagnosis warrants consideration for lung transplantation.

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  1. 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.

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a. Diagnosis
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Section Editor: Rahul V. Pawar, MD, DABR
Department of Radiology, Division of Neuroradiology
Saint Barnabas Medical Center/Barnabas Ambulatory Care Center