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Idiopathic Pulmonary Fibrosis: Spectrum of High-Resolution CT Findings

Carolina Althoff Souza1, Nestor L. Müller1, Julia Flint2, Joanne L. Wright2 and Andrew Churg2

1 Department of Radiology, Vancouver General Hospital, University of British Columbia, 899 W. 12th Ave., Vancouver, BC V5Z 1M9, Canada.
2 Department of Pathology, Vancouver General Hospital, University of British Columbia, Vancouver, BC V5Z 1M9, Canada.



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Fig. 1A Classic idiopathic pulmonary fibrosis in 70-year-old man. High-resolution CT shows bilateral subpleural reticulation, traction bronchiectasis (curved arrow), and honeycombing (straight arrows).

 


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Fig. 1B Classic idiopathic pulmonary fibrosis in 70-year-old man. Coronal reformatted image shows characteristic predominance of abnormalities in subpleural and basal regions.

 


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Fig. 2 Photomicrograph of histopathologic specimen of 57-year-old man with mild usual interstitial pneumonia shows paucicellular dense fibrosis concentrated in periphery of lobule (arrows). (H and E, x50)

 


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Fig. 3A 54-year-old man with severe idiopathic pulmonary fibrosis who underwent lung transplantation. Extremely low-power view of pathologic specimen from transplanted lung shows extensive honeycomb changes (curved arrows) and less severely affected areas (straight arrow). (H and E, x10)

 


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Fig. 3B 54-year-old man with severe idiopathic pulmonary fibrosis who underwent lung transplantation. Photomicrograph of histopathologic specimen (higher-power view) of less severely affected areas shows patchy interstitial fibrosis and occasional fibroblast foci (arrows). (H and E, x50)

 


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Fig. 4A 42-year-old woman with biopsy-proven idiopathic pulmonary fibrosis. High-resolution CT shows patchy bilateral ground-glass opacities and mild predominantly subpleural reticulation.

 


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Fig. 4B 42-year-old woman with biopsy-proven idiopathic pulmonary fibrosis. Photomicrograph of histopathologic specimen (low-power view) shows typical patchy interstitial fibrosis and areas of microscopic honeycombing (arrows). (H and E, x30)

 


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Fig. 5 High-resolution CT (HRCT) in 56-year-old woman with biopsy-proven idiopathic pulmonary fibrosis (IPF) shows subtle areas of ground-glass opacity involving both lungs and minimal subpleural reticulation. HRCT findings are more suggestive of hypersensitivity pneumonitis or nonspecific interstitial pneumonia than IPF.

 


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Fig. 6A 68-year-old man with biopsy-proven idiopathic pulmonary fibrosis (IPF). High-resolution CT shows patchy ground-glass opacities and fibrosis with reticulation and traction bronchiectasis (straight arrow). Some lobules appear relatively radiolucent, reflecting mosaic perfusion and air-trapping (curved arrows). Findings are more suggestive of hypersensitivity pneumonitis than IPF.

 


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Fig. 6B 68-year-old man with biopsy-proven idiopathic pulmonary fibrosis (IPF). Low-power view of autopsy specimen shows severe fibrosis and honeycombing consistent with idiopathic pulmonary fibrosis. Microscopic findings were characteristic of usual interstitial pneumonia; there was no microscopic evidence of hypersensitivity pneumonitis. (H and E, x10)

 


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Fig. 7A 61-year-old woman with biopsy-proven idiopathic pulmonary fibrosis. High-resolution CT shows subpleural ground-glass opacity (arrow) and mild reticulation.

 


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Fig. 7B 61-year-old woman with biopsy-proven idiopathic pulmonary fibrosis. Photomicrograph of histopathologic specimen obtained from region of ground-glass opacity on CT shows microscopic honeycombing with airspaces filled by mucus and inflammatory cells (arrows). (H and E, x150)

 


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Fig. 8A 57-year-old man with biopsy-proven idiopathic pulmonary fibrosis. High-resolution CT (HRCT) shows patchy bilateral areas of ground-glass opacity. Fine reticulation is observed in subpleural regions.

 


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Fig. 8B 57-year-old man with biopsy-proven idiopathic pulmonary fibrosis. HRCT at same approximate level as A, 2 years later, shows ground-glass opacities more prominent in subpleural regions, reticulation, and mild honeycombing.

 


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Fig. 8C 57-year-old man with biopsy-proven idiopathic pulmonary fibrosis. HRCT at same approximate level as A, 3 years later, shows extensive reticular opacities, traction bronchiectasis, and honeycombing in areas previously involved by ground-glass opacities.

 


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Fig. 8D 57-year-old man with biopsy-proven idiopathic pulmonary fibrosis. Gross pathologic specimen from autopsy shows predominantly lower lobe, peripheral, and subpleural fibrotic lesions that alternate with areas of normal lung (asterisks). Honeycombing cysts are seen in subpleural regions (arrow).

 


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Fig. 9A Accelerated idiopathic pulmonary fibrosis (IPF) in 62-year-old man. High-resolution CT (HRCT) shows patchy bilateral ground-glass opacities and subpleural reticulation.

 


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Fig. 9B Accelerated idiopathic pulmonary fibrosis (IPF) in 62-year-old man. HRCT obtained 10 days later shows extensive areas of ground-glass opacity and patchy consolidation involving both lungs.

 


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Fig. 9C Accelerated idiopathic pulmonary fibrosis (IPF) in 62-year-old man. Low-power view of lung at autopsy shows extensive fibrosis and honeycombing (arrow). (H and E, x30)

 


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Fig. 9D Accelerated idiopathic pulmonary fibrosis (IPF) in 62-year-old man. Another area of same lung shows hyaline membranes of diffuse alveolar damage (arrows). Changes of diffuse alveolar damage are typical microscopic finding in accelerated IPF. (H and E, x150)

 


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Fig. 10 High-resolution CT of 71-year-old man with idiopathic pulmonary fibrosis shows extensive reticulation, subpleural honeycombing, and architectural distortion. Subpleural irregular nodule (curved arrow) is seen within area of severe fibrosis in right lung. Diagnosis of pulmonary carcinoma was proven by biopsy.

 


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Fig. 11A 74 year-old-man with idiopathic pulmonary fibrosis and pulmonary ossification. High-resolution CT (HRCT) shows subpleural reticulation and mild ground-glass opacity.

 


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Fig. 11B 74 year-old-man with idiopathic pulmonary fibrosis and pulmonary ossification. HRCT image photographed using soft-tissue windows at same level as A shows bilateral small calcified nodules (curved arrows) within areas of fibrosis.

 


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Fig. 12A 63-year-old man with biopsy-proven desquamative interstitial pneumonia (DIP). High-resolution CT (HRCT) shows patchy bilateral areas of ground-glass opacity and mild reticulation.

 


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Fig. 12B 63-year-old man with biopsy-proven desquamative interstitial pneumonia (DIP). Photomicrograph of histopathologic specimen obtained by surgical biopsy shows mild thickening of alveolar septa and extensive airspace filling by macrophages (arrows). (H and E, x100) Inset: Higher-power view shows airspace macrophages and chronic interstitial inflammatory infiltrate (arrow). (H and E, x250) Findings are characteristic of DIP.

 


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Fig. 12C 63-year-old man with biopsy-proven desquamative interstitial pneumonia (DIP). HRCT scan at same approximate level as A 13 years later shows extensive fibrotic changes with irregular reticular opacities, traction bronchiectasis, and subpleural honeycombing (arrows). Findings are those of end-stage fibrosis and mimic those of idiopathic pulmonary fibrosis.

 

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