DOI: 10.1148/rg.273065130
What Every Radiologist Should Know about Idiopathic Interstitial Pneumonias1
Christina Mueller-Mang, MD,
Claudia Grosse, MD,
Katharina Schmid, MD,
Leopold Stiebellehner, MD, and
Alexander A. Bankier, MD
1 From the Departments of Radiology (C.M.M., C.G., A.A.B.), Pathology (K.S.), and Pulmonology (L.S.), Medical University of Vienna, Waehringer Guertel 18-20, A-1090 Vienna, Austria. Presented as an education exhibit at the 2005 RSNA Annual Meeting. Received July 12, 2006; revision requested October 25 and received November 27; accepted December 4. All authors have no financial relationships to disclose.
View larger version (183K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 2a. Histologic features of UIP. (a) Photomicrograph (original magnification, x40; hematoxylin-eosin stain) shows patchy fibrosis with remodeling of the lung architecture. Interstitial chronic inflammation is mild, with only a few lymphoid aggregates (thin arrow). Cystically dilated airspaces that produce a honeycomb pattern (arrowhead) and areas of relatively unaffected lung (thick arrow) are present. (b) Photomicrograph (original magnification, x200; hematoxylin-eosin stain) shows a fibroblastic focus of loose organizing connective tissue (arrowheads), which is the hallmark of UIP.
|
|
View larger version (177K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 2b. Histologic features of UIP. (a) Photomicrograph (original magnification, x40; hematoxylin-eosin stain) shows patchy fibrosis with remodeling of the lung architecture. Interstitial chronic inflammation is mild, with only a few lymphoid aggregates (thin arrow). Cystically dilated airspaces that produce a honeycomb pattern (arrowhead) and areas of relatively unaffected lung (thick arrow) are present. (b) Photomicrograph (original magnification, x200; hematoxylin-eosin stain) shows a fibroblastic focus of loose organizing connective tissue (arrowheads), which is the hallmark of UIP.
|
|
View larger version (27K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 3a. Distribution (a), CT image (b), and CT pattern (c) of UIP. The distribution is subpleural with an apicobasal gradient (red area in a). CT shows honeycombing (green areas in c), reticular opacities (blue areas in c), traction bronchiectasis (red area in c), and focal ground-glass opacity (gray area in c).
|
|
View larger version (122K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 3b. Distribution (a), CT image (b), and CT pattern (c) of UIP. The distribution is subpleural with an apicobasal gradient (red area in a). CT shows honeycombing (green areas in c), reticular opacities (blue areas in c), traction bronchiectasis (red area in c), and focal ground-glass opacity (gray area in c).
|
|
View larger version (16K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 3c. Distribution (a), CT image (b), and CT pattern (c) of UIP. The distribution is subpleural with an apicobasal gradient (red area in a). CT shows honeycombing (green areas in c), reticular opacities (blue areas in c), traction bronchiectasis (red area in c), and focal ground-glass opacity (gray area in c).
|
|
View larger version (130K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 4a. IPF in a 64-year-old man. (a) High-resolution CT image obtained at presentation shows reticular opacities, honeycombing (arrowhead), and focal ground-glass opacity (thick arrow). Moderate traction bronchiectasis is present (thin arrow). These findings are consistent with the UIP pattern. (b) Follow-up CT image obtained 12 months later shows marked progression of the honeycombing (arrowheads) and traction bronchiectasis (arrows).
|
|
View larger version (137K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 4b. IPF in a 64-year-old man. (a) High-resolution CT image obtained at presentation shows reticular opacities, honeycombing (arrowhead), and focal ground-glass opacity (thick arrow). Moderate traction bronchiectasis is present (thin arrow). These findings are consistent with the UIP pattern. (b) Follow-up CT image obtained 12 months later shows marked progression of the honeycombing (arrowheads) and traction bronchiectasis (arrows).
|
|
View larger version (152K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 5a. IPF in a 67-year-old man. (a) High-resolution CT image shows areas of relatively unaffected lung parenchyma with only ground-glass opacity (arrow) next to fibrotic areas with honeycombing and traction bronchiectasis (arrowhead), an appearance typical of UIP. (b) Coronal CT image shows an obvious apicobasal gradient of the lung alterations.
|
|
View larger version (167K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 5b. IPF in a 67-year-old man. (a) High-resolution CT image shows areas of relatively unaffected lung parenchyma with only ground-glass opacity (arrow) next to fibrotic areas with honeycombing and traction bronchiectasis (arrowhead), an appearance typical of UIP. (b) Coronal CT image shows an obvious apicobasal gradient of the lung alterations.
|
|
View larger version (190K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 6a. Histologic features of NSIP. (a) Photomicrograph (original magnification, x100; hematoxylin-eosin stain) of cellular NSIP shows a uniform appearance of interstitial inflammation (arrow), which consists of lymphocytes and plasma cells. (b) Photomicrograph (original magnification, x100; hematoxylin-eosin stain) of fibrosing NSIP shows areas of fibrosis (arrow) in addition to uniform inflammation.
|
|
View larger version (158K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 6b. Histologic features of NSIP. (a) Photomicrograph (original magnification, x100; hematoxylin-eosin stain) of cellular NSIP shows a uniform appearance of interstitial inflammation (arrow), which consists of lymphocytes and plasma cells. (b) Photomicrograph (original magnification, x100; hematoxylin-eosin stain) of fibrosing NSIP shows areas of fibrosis (arrow) in addition to uniform inflammation.
|
|
View larger version (51K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 7a. Distribution (a), CT image (b), and CT pattern (c) of NSIP. The distribution is subpleural with no obvious gradient (red area in a). CT shows ground-glass opacity (gray areas in c), irregular linear and reticular opacities (blue areas in c), micronodules (red areas in c), and microcystic honeycombing (green areas in c).
|
|
View larger version (144K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 7b. Distribution (a), CT image (b), and CT pattern (c) of NSIP. The distribution is subpleural with no obvious gradient (red area in a). CT shows ground-glass opacity (gray areas in c), irregular linear and reticular opacities (blue areas in c), micronodules (red areas in c), and microcystic honeycombing (green areas in c).
|
|
View larger version (26K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 7c. Distribution (a), CT image (b), and CT pattern (c) of NSIP. The distribution is subpleural with no obvious gradient (red area in a). CT shows ground-glass opacity (gray areas in c), irregular linear and reticular opacities (blue areas in c), micronodules (red areas in c), and microcystic honeycombing (green areas in c).
|
|
View larger version (147K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 8a. NSIP in a 60-year-old woman with mild dyspnea and fatigue. (a) High-resolution CT image of the lower lungs shows bilateral subpleural ground-glass opacities (arrowhead) and irregular linear opacities (arrow). The patient received corticosteroid treatment. (b) Follow-up CT image obtained 6 months later shows improvement, with partial resolution of the ground-glass opacities (arrowhead) and linear opacities (arrow).
|
|
View larger version (151K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 8b. NSIP in a 60-year-old woman with mild dyspnea and fatigue. (a) High-resolution CT image of the lower lungs shows bilateral subpleural ground-glass opacities (arrowhead) and irregular linear opacities (arrow). The patient received corticosteroid treatment. (b) Follow-up CT image obtained 6 months later shows improvement, with partial resolution of the ground-glass opacities (arrowhead) and linear opacities (arrow).
|
|
View larger version (141K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 9a. NSIP in a 53-year-old man with mild dyspnea. (a) Coronal CT image shows diffuse lung involvement consisting of peripherally located irregular linear opacities with ground-glass opacities (arrows). Small cystic lesions are seen (arrowhead). (b) Axial high-resolution CT image shows the small cystic lesions more clearly (arrowhead).
|
|
View larger version (154K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 9b. NSIP in a 53-year-old man with mild dyspnea. (a) Coronal CT image shows diffuse lung involvement consisting of peripherally located irregular linear opacities with ground-glass opacities (arrows). Small cystic lesions are seen (arrowhead). (b) Axial high-resolution CT image shows the small cystic lesions more clearly (arrowhead).
|
|
View larger version (173K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 11a. Comparison of high-resolution CT features between UIP and NSIP. (a) UIP is characterized by heterogeneous lung abnormalities consisting of subpleural honeycombing (arrowhead), reticular opacities, and traction bronchiectasis. (b) NSIP demonstrates homogeneous lung involvement with predominance of ground-glass opacity combined with sub-pleural linear opacities and micronodules. The microcysts in NSIP (arrowhead) are much smaller than the honeycombing in UIP.
|
|
View larger version (133K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 11b. Comparison of high-resolution CT features between UIP and NSIP. (a) UIP is characterized by heterogeneous lung abnormalities consisting of subpleural honeycombing (arrowhead), reticular opacities, and traction bronchiectasis. (b) NSIP demonstrates homogeneous lung involvement with predominance of ground-glass opacity combined with sub-pleural linear opacities and micronodules. The microcysts in NSIP (arrowhead) are much smaller than the honeycombing in UIP.
|
|
View larger version (178K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 12. Histologic features of COP. Photomicrograph (original magnification, x100; hematoxylin-eosin stain) shows polypoid fibroblastic foci in the alveolar ducts and alveoli (arrows). The organizing connective tissue is all the same age and shows moderate cellular proliferation.
|
|
View larger version (14K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 13a. Distribution (a), CT image (b), and CT pattern (c) of COP. The distribution is peripheral or peribronchial with a basal predominance (red areas in a). CT shows consolidation with air bronchograms (dark gray areas in c), ground-glass opacities (light gray areas in c), linear opacities (blue areas in c), and mild bronchial dilatation (red areas in c).
|
|
View larger version (103K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 13b. Distribution (a), CT image (b), and CT pattern (c) of COP. The distribution is peripheral or peribronchial with a basal predominance (red areas in a). CT shows consolidation with air bronchograms (dark gray areas in c), ground-glass opacities (light gray areas in c), linear opacities (blue areas in c), and mild bronchial dilatation (red areas in c).
|
|
View larger version (13K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 13c. Distribution (a), CT image (b), and CT pattern (c) of COP. The distribution is peripheral or peribronchial with a basal predominance (red areas in a). CT shows consolidation with air bronchograms (dark gray areas in c), ground-glass opacities (light gray areas in c), linear opacities (blue areas in c), and mild bronchial dilatation (red areas in c).
|
|
View larger version (150K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 14a. COP in a 54-year-old woman. (a) Coronal CT image shows extensive bilateral peribronchial consolidation and ground-glass opacities (arrows). An endotracheal tube is present (arrowhead), indicating the need for mechanical ventilation. (b) CT image obtained after 3 weeks of corticosteroid and supportive treatment shows subtotal resolution of the lung abnormalities (arrows).
|
|
View larger version (138K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 14b. COP in a 54-year-old woman. (a) Coronal CT image shows extensive bilateral peribronchial consolidation and ground-glass opacities (arrows). An endotracheal tube is present (arrowhead), indicating the need for mechanical ventilation. (b) CT image obtained after 3 weeks of corticosteroid and supportive treatment shows subtotal resolution of the lung abnormalities (arrows).
|
|
View larger version (113K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 16a. Atypical appearances of COP. (a) CT image shows bizarrely shaped nodules, some of which are cavitating (arrow). (b) CT image shows perilobular opacities that resemble thickened interlobular septa (arrow).
|
|
View larger version (118K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 16b. Atypical appearances of COP. (a) CT image shows bizarrely shaped nodules, some of which are cavitating (arrow). (b) CT image shows perilobular opacities that resemble thickened interlobular septa (arrow).
|
|
View larger version (140K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 17. Histologic features of RB-ILD. Photomicrograph (original magnification, x100; hematoxylin-eosin stain) shows pigmented alveolar macrophages in a terminal bronchiole and the adjacent alveoli (arrows). Moderate peribronchiolar inflammation and fibrosis are present (arrowhead).
|
|
View larger version (22K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 18a. Distribution (a), CT image (b), and CT pattern (c) of RB-ILD. RB-ILD has an upper lung predominance (red area in a). CT shows ground-glass opacity (gray area in c) and centrilobular nodules (red areas in c).
|
|
View larger version (104K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 18b. Distribution (a), CT image (b), and CT pattern (c) of RB-ILD. RB-ILD has an upper lung predominance (red area in a). CT shows ground-glass opacity (gray area in c) and centrilobular nodules (red areas in c).
|
|
View larger version (16K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 18c. Distribution (a), CT image (b), and CT pattern (c) of RB-ILD. RB-ILD has an upper lung predominance (red area in a). CT shows ground-glass opacity (gray area in c) and centrilobular nodules (red areas in c).
|
|
View larger version (116K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 19. RB-ILD in a 44-year-old woman with a 20 pack-year smoking history. High-resolution CT image of the upper lung lobes shows centrilobular nodules (white arrows) and patchy ground-glass opacities (black arrow). Mild coexisting centrilobular emphysema is seen (arrowhead).
|
|
View larger version (163K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 20. Histologic features of DIP. Photomicrograph (original magnification, x200; hematoxylin-eosin stain) shows diffuse filling of the alveolar spaces with alveolar macrophages and a few desquamated alveolar epithelial cells (arrow) (inset). Mild interstitial fibrosis is present (arrowhead).
|
|
View larger version (21K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 21a. Distribution (a), CT image (b), and CT pattern (c) of DIP. DIP has a peripheral predominance (red areas in a). CT shows ground-glass opacity (gray area in c), irregular linear opacities (blue areas in c), and cysts (green areas in c).
|
|
View larger version (98K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 21b. Distribution (a), CT image (b), and CT pattern (c) of DIP. DIP has a peripheral predominance (red areas in a). CT shows ground-glass opacity (gray area in c), irregular linear opacities (blue areas in c), and cysts (green areas in c).
|
|
View larger version (18K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 21c. Distribution (a), CT image (b), and CT pattern (c) of DIP. DIP has a peripheral predominance (red areas in a). CT shows ground-glass opacity (gray area in c), irregular linear opacities (blue areas in c), and cysts (green areas in c).
|
|
View larger version (161K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 22. DIP in a 55-year-old man. High-resolution CT image of the lower lung lobes shows extensive bilateral ground-glass opacities (arrowhead). Coexisting moderate bronchial wall thickening is present (arrow).
|
|
View larger version (159K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 23. DIP in a 43-year-old man with a history of smoking. High-resolution CT image of the lower lung zones shows patchy ground-glass opacities in both lungs, predominantly in the subpleural region (arrowheads). Small cystic spaces are present in these areas (arrow).
|
|
View larger version (160K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 24. Histologic features of LIP. Photomicrograph (original magnification, x200; hematoxylin-eosin stain) shows widening of alveolar septa by lymphoid infiltrates (arrow) (inset), which consist of mature lymphocytes, plasma cells, and histiocytes.
|
|
View larger version (37K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 25a. Distribution (a), CT image (b), and CT pattern (c) of LIP. The distribution is diffuse (red area in a). CT shows ground-glass opacity (gray area in c) and perivascular cysts (green areas in c).
|
|
View larger version (50K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 25b. Distribution (a), CT image (b), and CT pattern (c) of LIP. The distribution is diffuse (red area in a). CT shows ground-glass opacity (gray area in c) and perivascular cysts (green areas in c).
|
|
View larger version (22K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 25c. Distribution (a), CT image (b), and CT pattern (c) of LIP. The distribution is diffuse (red area in a). CT shows ground-glass opacity (gray area in c) and perivascular cysts (green areas in c).
|
|
View larger version (132K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 26a. LIP in a 47-year-old woman. (a) High-resolution CT image shows diffuse ground-glass opacity (arrow) with multiple perivascular cysts (arrowheads) and reticular abnormalities (*). (b) CT image obtained after corticosteroid therapy shows improvement, with partial resolution of the ground-glass and reticular opacities and better demarcation of the perivascular cysts (arrowheads).
|
|
View larger version (122K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 26b. LIP in a 47-year-old woman. (a) High-resolution CT image shows diffuse ground-glass opacity (arrow) with multiple perivascular cysts (arrowheads) and reticular abnormalities (*). (b) CT image obtained after corticosteroid therapy shows improvement, with partial resolution of the ground-glass and reticular opacities and better demarcation of the perivascular cysts (arrowheads).
|
|
View larger version (156K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 27. Histologic features of the exudative phase of AIP. The alveolar septa are diffusely thickened by hyaline membranes (arrow). Fibrin deposition and inflammatory cells are present in the alveoli (arrowhead).
|
|
View larger version (27K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 28a. Distribution (a), CT image (b), and CT pattern (c) of AIP. AIP has a basal predominance (red area in a). CT shows airspace consolidation (dark gray areas in c), ground-glass opacities (light gray areas in c), and bronchial dilatation (red areas in c).
|
|
View larger version (108K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 28b. Distribution (a), CT image (b), and CT pattern (c) of AIP. AIP has a basal predominance (red area in a). CT shows airspace consolidation (dark gray areas in c), ground-glass opacities (light gray areas in c), and bronchial dilatation (red areas in c).
|
|
View larger version (19K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 28c. Distribution (a), CT image (b), and CT pattern (c) of AIP. AIP has a basal predominance (red area in a). CT shows airspace consolidation (dark gray areas in c), ground-glass opacities (light gray areas in c), and bronchial dilatation (red areas in c).
|
|
View larger version (131K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 29. Exudative phase of AIP in a 22-year-old man. High-resolution CT image shows bilateral ground-glass opacities (arrowheads) and consolidation (arrow) in the dependent areas of the lungs. The anterior zones of the lungs are relatively spared.
|
|
View larger version (146K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 30. Fibrotic phase of AIP in a 53-year-old woman who survived the acute phase of the disease. CT image shows fibrotic changes with traction bronchiectasis and architectural distortion predominantly in the nondependent areas of the lungs (arrow). A coexisting right pleural effusion is seen (arrowhead).
|
|
Copyright © 2007 by the Radiological Society of North America.
