Interstitial Lung Diseases Associated with Collagen Vascular Diseases: Radiologic and Histopathologic Findings1
Eun A Kim, MD,
Kyung Soo Lee, MD,
Takeshi Johkoh, MD, PhD,
Tae Sung Kim, MD,
Gee Young Suh, MD,
O Jung Kwon, MD and
Joungho Han, MD
1 From the Departments of Radiology (E.A.K., K.S.L., T.S.K.), Medicine (G.Y.S., O.J.K.), and Diagnostic Pathology (J.H.), Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Ilwon-Dong, Kangnam-Ku, Seoul 135-710, Korea; and the Department of Radiology, Osaka University Graduate School of Medicine, Osaka, Japan (T.J.). Presented as an education exhibit at the 2001 RSNA scientific assembly. Received January 21, 2002; revision requested February 20 and received April 4; accepted April 4. Address correspondence to K.S.L. (e-mail: kslee@smc.samsung.co.kr).
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Figure 1a. Systemic lupus erythematosus and acute lupus pneumonitis in a 45-year-old man. (a, b) CT scans (3-mm collimation) obtained with lung windowing at the levels of the aortic arch (a) and lingular segmental bronchus (b) show airspace consolidation in the right upper lobe and ground-glass attenuation in the left upper lobe. Bronchial dilatation is seen in the consolidation in the right upper lobe (arrows in a). (c) Photomicrograph (original magnification, x40; hematoxylin-eosin [H-E] stain) shows prominent interstitial fibroblastic proliferation and alveolar type 2 pneumocyte hyperplasia (large arrows) that cause alveolar collapse. There is also intraalveolar macrophage collection (small arrows). These findings are compatible with the organizing stage of diffuse alveolar damage.
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Figure 1b. Systemic lupus erythematosus and acute lupus pneumonitis in a 45-year-old man. (a, b) CT scans (3-mm collimation) obtained with lung windowing at the levels of the aortic arch (a) and lingular segmental bronchus (b) show airspace consolidation in the right upper lobe and ground-glass attenuation in the left upper lobe. Bronchial dilatation is seen in the consolidation in the right upper lobe (arrows in a). (c) Photomicrograph (original magnification, x40; hematoxylin-eosin [H-E] stain) shows prominent interstitial fibroblastic proliferation and alveolar type 2 pneumocyte hyperplasia (large arrows) that cause alveolar collapse. There is also intraalveolar macrophage collection (small arrows). These findings are compatible with the organizing stage of diffuse alveolar damage.
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Figure 1c. Systemic lupus erythematosus and acute lupus pneumonitis in a 45-year-old man. (a, b) CT scans (3-mm collimation) obtained with lung windowing at the levels of the aortic arch (a) and lingular segmental bronchus (b) show airspace consolidation in the right upper lobe and ground-glass attenuation in the left upper lobe. Bronchial dilatation is seen in the consolidation in the right upper lobe (arrows in a). (c) Photomicrograph (original magnification, x40; hematoxylin-eosin [H-E] stain) shows prominent interstitial fibroblastic proliferation and alveolar type 2 pneumocyte hyperplasia (large arrows) that cause alveolar collapse. There is also intraalveolar macrophage collection (small arrows). These findings are compatible with the organizing stage of diffuse alveolar damage.
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Figure 2a. Systemic lupus erythematosus and nonspecific interstitial pneumonia (fibrosing pattern) in a 67-year-old woman. (a) Thin-section (1-mm collimation) CT scan obtained at the level of the inferior pulmonary vein shows ground-glass attenuation and irregular linear hyperattenuating areas in the subpleural areas of both lower lung zones, as well as traction bronchiectasis (arrows). (b) Photomicrograph (original magnification, x12; H-E stain) shows uniform interstitial fibrous thickening with infiltration of a few mononuclear cells.
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Figure 2b. Systemic lupus erythematosus and nonspecific interstitial pneumonia (fibrosing pattern) in a 67-year-old woman. (a) Thin-section (1-mm collimation) CT scan obtained at the level of the inferior pulmonary vein shows ground-glass attenuation and irregular linear hyperattenuating areas in the subpleural areas of both lower lung zones, as well as traction bronchiectasis (arrows). (b) Photomicrograph (original magnification, x12; H-E stain) shows uniform interstitial fibrous thickening with infiltration of a few mononuclear cells.
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Figure 3. Systemic lupus erythematosus and BOOP in a 23-year-old woman. Thin-section (1-mm collimation) CT scan obtained at the level of the liver dome shows subpleural consolidation and ground-glass attenuation in the basal areas of both lungs.
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Figure 4. Systemic lupus erythematosus and usual interstitial pneumonia in a 51-year-old woman. Thin-section (1-mm collimation) CT scan obtained at the level of the liver dome shows subpleural honeycombing, irregular linear hyperattenuating areas, and ground-glass attenuation in the basal areas of both lungs.
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Figure 5a. Rheumatoid arthritis and BOOP in a 68-year-old man. (a) Thin-section (1-mm collimation) CT scan obtained at the level of the inferior pulmonary vein shows patchy areas of ground-glass attenuation and consolidation with a subpleural or peribronchovascular distribution in both lungs. (b) Photomicrograph (original magnification, x40; H-E stain) shows intraalveolar fibroblastic plugging (arrows) in alveolar spaces and alveolar ducts.
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Figure 5b. Rheumatoid arthritis and BOOP in a 68-year-old man. (a) Thin-section (1-mm collimation) CT scan obtained at the level of the inferior pulmonary vein shows patchy areas of ground-glass attenuation and consolidation with a subpleural or peribronchovascular distribution in both lungs. (b) Photomicrograph (original magnification, x40; H-E stain) shows intraalveolar fibroblastic plugging (arrows) in alveolar spaces and alveolar ducts.
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Figure 6a. Rheumatoid arthritis and diffuse alveolar damage (organizing phase) in a 68-year-old man. (a, b) Thin-section (1-mm collimation) CT scans obtained at the levels of the aortic arch (a) and the right upper lobar bronchus (b) show patchy ground-glass attenuation and consolidation bilaterally in the upper and middle lung zones. A small amount of bilateral pleural effusion is also apparent. (c) Photomicrograph (original magnification, x12; H-E stain) of a biopsy specimen obtained from the right upper lobe shows diffuse interstitial thickening with fibroblastic proliferation and mononuclear cell infiltration.
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Figure 6b. Rheumatoid arthritis and diffuse alveolar damage (organizing phase) in a 68-year-old man. (a, b) Thin-section (1-mm collimation) CT scans obtained at the levels of the aortic arch (a) and the right upper lobar bronchus (b) show patchy ground-glass attenuation and consolidation bilaterally in the upper and middle lung zones. A small amount of bilateral pleural effusion is also apparent. (c) Photomicrograph (original magnification, x12; H-E stain) of a biopsy specimen obtained from the right upper lobe shows diffuse interstitial thickening with fibroblastic proliferation and mononuclear cell infiltration.
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Figure 6c. Rheumatoid arthritis and diffuse alveolar damage (organizing phase) in a 68-year-old man. (a, b) Thin-section (1-mm collimation) CT scans obtained at the levels of the aortic arch (a) and the right upper lobar bronchus (b) show patchy ground-glass attenuation and consolidation bilaterally in the upper and middle lung zones. A small amount of bilateral pleural effusion is also apparent. (c) Photomicrograph (original magnification, x12; H-E stain) of a biopsy specimen obtained from the right upper lobe shows diffuse interstitial thickening with fibroblastic proliferation and mononuclear cell infiltration.
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Figure 7a. Progressive systemic sclerosis and nonspecific interstitial pneumonia (group 3) in a 50-year-old woman. (a) Thin-section (1-mm collimation) CT scan obtained at the level of the liver dome shows patchy areas of ground-glass attenuation, irregular linear hyperattenuating areas, and traction bronchiectasis. (b) Photomicrograph (original magnification, x40; H-E stain) shows diffuse uniform interstitial fibrous thickening, intraalveolar mucin, and inflammatory cell infiltration.
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Figure 7b. Progressive systemic sclerosis and nonspecific interstitial pneumonia (group 3) in a 50-year-old woman. (a) Thin-section (1-mm collimation) CT scan obtained at the level of the liver dome shows patchy areas of ground-glass attenuation, irregular linear hyperattenuating areas, and traction bronchiectasis. (b) Photomicrograph (original magnification, x40; H-E stain) shows diffuse uniform interstitial fibrous thickening, intraalveolar mucin, and inflammatory cell infiltration.
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Figure 8a. Progressive systemic sclerosis and usual interstitial pneumonia in a 41-year-old woman. (a) Thin-section (1-mm collimation) CT scan obtained at the level of the liver dome shows honeycombing, irregular linear hyperattenuating areas, and ground-glass attenuation in the basal areas of both lungs. (b) Photomicrograph (original magnification, x12; H-E stain) shows irregular interstitial fibrosis (solid arrow) with mononuclear cell infiltration and foci of fibroblastic proliferation (arrowheads). Intervening normal lung tissue is also seen (open arrows).
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Figure 8b. Progressive systemic sclerosis and usual interstitial pneumonia in a 41-year-old woman. (a) Thin-section (1-mm collimation) CT scan obtained at the level of the liver dome shows honeycombing, irregular linear hyperattenuating areas, and ground-glass attenuation in the basal areas of both lungs. (b) Photomicrograph (original magnification, x12; H-E stain) shows irregular interstitial fibrosis (solid arrow) with mononuclear cell infiltration and foci of fibroblastic proliferation (arrowheads). Intervening normal lung tissue is also seen (open arrows).
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Figure 9a. Progression of disease extent in a 51-year-old woman with progressive systemic sclerosis and nonspecific interstitial pneumonia. (a) Thin-section (1-mm collimation) CT scan obtained at the level of the basal segmental bronchi shows patchy distribution of irregular linear hyperattenuating areas and ground-glass attenuation with traction bronchiectasis (arrows) in both lungs. (b) Follow-up CT scan obtained at a similar level 54 months later shows increased extent of disease in both lungs. Traction bronchiectasis has progressed (arrows).
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Figure 9b. Progression of disease extent in a 51-year-old woman with progressive systemic sclerosis and nonspecific interstitial pneumonia. (a) Thin-section (1-mm collimation) CT scan obtained at the level of the basal segmental bronchi shows patchy distribution of irregular linear hyperattenuating areas and ground-glass attenuation with traction bronchiectasis (arrows) in both lungs. (b) Follow-up CT scan obtained at a similar level 54 months later shows increased extent of disease in both lungs. Traction bronchiectasis has progressed (arrows).
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Figure 10a. Dermatomyositis and usual interstitial pneumonia in a 64-year-old woman. (a, b) Initial thin-section (1-mm collimation) CT scans obtained at the levels of the bronchus intermedius (a) and liver dome (b) show patchy areas of ground-glass attenuation and irregular linear hyperattenuating areas with random distribution in both lungs. (c) Photomicrograph (original magnification, x12.5; H-E stain) shows irregular interstitial fibrosis (arrows) and mononuclear cell infiltration with subpleural predominance. Germinal center formation is also seen (arrowheads).
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Figure 10b. Dermatomyositis and usual interstitial pneumonia in a 64-year-old woman. (a, b) Initial thin-section (1-mm collimation) CT scans obtained at the levels of the bronchus intermedius (a) and liver dome (b) show patchy areas of ground-glass attenuation and irregular linear hyperattenuating areas with random distribution in both lungs. (c) Photomicrograph (original magnification, x12.5; H-E stain) shows irregular interstitial fibrosis (arrows) and mononuclear cell infiltration with subpleural predominance. Germinal center formation is also seen (arrowheads).
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Figure 10c. Dermatomyositis and usual interstitial pneumonia in a 64-year-old woman. (a, b) Initial thin-section (1-mm collimation) CT scans obtained at the levels of the bronchus intermedius (a) and liver dome (b) show patchy areas of ground-glass attenuation and irregular linear hyperattenuating areas with random distribution in both lungs. (c) Photomicrograph (original magnification, x12.5; H-E stain) shows irregular interstitial fibrosis (arrows) and mononuclear cell infiltration with subpleural predominance. Germinal center formation is also seen (arrowheads).
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Figure 11a. Dermatomyositis and nonspecific interstitial pneumonia in a 51-year-old man. (a) Thin-section (2.5-mm collimation) CT scan obtained at the level of the liver dome shows subpleural patchy areas of ground-glass attenuation and irregular linear areas of hyperattenuation. Traction bronchiectasis is also visible (arrows). (b) Coronal reformatted image (2-mm collimation of original axial helical CT data obtained with 2.5-mm collimation, 120 kVp, and 100 mA) of the posterior portion of the lung shows subpleural ground-glass attenuation and irregular linear hyperattenuating areas, predominantly in the lower lung zones. Traction bronchiectasis is also visible (arrows).
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Figure 11b. Dermatomyositis and nonspecific interstitial pneumonia in a 51-year-old man. (a) Thin-section (2.5-mm collimation) CT scan obtained at the level of the liver dome shows subpleural patchy areas of ground-glass attenuation and irregular linear areas of hyperattenuation. Traction bronchiectasis is also visible (arrows). (b) Coronal reformatted image (2-mm collimation of original axial helical CT data obtained with 2.5-mm collimation, 120 kVp, and 100 mA) of the posterior portion of the lung shows subpleural ground-glass attenuation and irregular linear hyperattenuating areas, predominantly in the lower lung zones. Traction bronchiectasis is also visible (arrows).
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Figure 12a. Sjögren syndrome and lymphocytic interstitial pneumonia in a 32-year-old woman. (a) Thin-section (1-mm collimation) CT scan obtained at the level of the carina shows centrilobular nodules and branching linear structures (straight arrow) in the right lung. Many thin-walled cysts (curved arrows) were seen in both lungs. (b) Photomicrograph (original magnification, x20; H-E stain) shows diffuse lymphocyte infiltration in the peribronchovascular interstitium and surrounding alveolar septa (arrows).
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Figure 12b. Sjögren syndrome and lymphocytic interstitial pneumonia in a 32-year-old woman. (a) Thin-section (1-mm collimation) CT scan obtained at the level of the carina shows centrilobular nodules and branching linear structures (straight arrow) in the right lung. Many thin-walled cysts (curved arrows) were seen in both lungs. (b) Photomicrograph (original magnification, x20; H-E stain) shows diffuse lymphocyte infiltration in the peribronchovascular interstitium and surrounding alveolar septa (arrows).
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Figure 13. Sjögren syndrome and usual interstitial pneumonia in a 30-year-old woman. Thin-section (1-mm collimation) CT scan obtained at the level of the lower lobar bronchus shows subpleural ground-glass attenuation, irregular linear hyperattenuating areas, and honeycombing. Traction bronchiectasis is also visible (arrows).
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Figure 14. Mixed connective tissue disease and usual interstitial pneumonia in a 54-year-old woman. Thin-section (1-mm collimation) CT scan obtained at the level of the liver dome shows subpleural areas of ground-glass attenuation, irregular linear hyperattenuating areas, and traction bronchiectasis and bronchiolectasis (arrows).
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Copyright © 2002 by the Radiological Society of North America.
