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Small-Vessel Diseases of the Lung: CT-Pathologic Correlates¹

¹ From the Department of Radiology, Royal Brompton Hospital, Sydney St, London SW3 6NP, England. Received September 6, 2001; revision requested November 8; revision received January 4, 2002; accepted January 22. Address correspondence to the author (e-mail: d.hansell@rbh.nthames.nhs.uk).

View larger version (43K): [in a new window]   Figure 1. Example of classification of pulmonary vascular disease. This list is a modification of that by Anderson (7).

View larger version (160K): [in a new window]   Figure 2. Maximum intensity projection image comprising a slab of seven contiguous transverse 1.5-mm-thick CT sections shows to advantage the branching morphology of vessels in the upper lobes.

View larger version (119K): [in a new window]   Figure 3. Transverse thin-section CT image through the upper lobes in a 64-year-old woman with chronic thromboembolic disease shows typical mosaic oligemia (mosaic attenuation pattern). Note the smaller caliber of vessels in the low-attenuating portion of (underperfused) lung, as compared with those in areas of increased attenuation (relatively overperfused lung). The proximal subsegmental and segmental pulmonary arteries are abnormally dilated.

View larger version (105K): [in a new window]   Figure 4. Transverse thin-section CT image through the mid-lung zones in a 44-year-old woman with mixed small-airways and small-vessel disease. The patient had constrictive obliterative bronchiolitis secondary to a severe viral lower respiratory tract infection; she subsequently developed chronic thromboembolic disease. In this case, it is impossible to be certain what proportion of the mosaic attenuation pattern is ascribable to small-airways, as opposed to small-vessels, disease.

View larger version (106K): [in a new window]   Figure 5. Transverse thin-section CT image through the lower lobes in a patient with severe chronic thromboembolic disease and presumed hypoxic bronchodilatation. There are extensive areas of relatively underperfused lung. Peripheral subpleural opacification in the right lower lobe likely reflects previous pulmonary infarction (arrowheads). Note the mildly dilated segmental and subsegmental bronchi (arrow) in the lower lobes, which were also present on adjacent sections (not shown).

View larger version (111K): [in a new window]   Figure 6. Standard transverse CT section obtained after intravenous administration of contrast material in a 56-year-old woman with Wegener granulomatosis shows an area of consolidation in the medial segment of the right middle lobe (arrowheads), thought to be an area of organizing pneumonia. Histologic examination of a lung biopsy specimen revealed Wegener granulomatosis.

View larger version (129K): [in a new window]   Figure 7. Transverse thin-section CT image through the upper lobes in a 39-year-old woman with severe primary pulmonary hypertension shows widespread subtle small nodules of relatively low attenuation throughout the lungs; these nodules correspond to focal extravasation of blood and/or cholesterol granuloma formation.

View larger version (123K): [in a new window]   Figure 8. Transverse thin-section CT image through the upper lobes in a patient with veno-occlusive disease. Thickening of the interlobular septa is the most prominent feature, and there are some poorly defined centrilobular nodules. Bilateral dependent pleural effusions (arrows) are visible.

View larger version (197K): [in a new window]   Figure 9a. Pulmonary tumor thrombotic microangiopathy. (a) Transverse thin-section CT image through the right lower lobe shows small branching opacities (arrows) representing enlarged peripheral arteries. (b) Cut surface of autopsy lung specimen shows pale nodules, which correspond to the pulmonary arteries thickened by the fibrotic reaction to tumor. (c) Photomicrograph demonstrates dense fibrocellular proliferation within an arteriolar lumen. The tumor emboli (arrows) are relatively small. (Image courtesy of Dr T. Franquet, Hospital Sant Pau, Barcelona, Spain.)

View larger version (148K): [in a new window]   Figure 9b. Pulmonary tumor thrombotic microangiopathy. (a) Transverse thin-section CT image through the right lower lobe shows small branching opacities (arrows) representing enlarged peripheral arteries. (b) Cut surface of autopsy lung specimen shows pale nodules, which correspond to the pulmonary arteries thickened by the fibrotic reaction to tumor. (c) Photomicrograph demonstrates dense fibrocellular proliferation within an arteriolar lumen. The tumor emboli (arrows) are relatively small. (Image courtesy of Dr T. Franquet, Hospital Sant Pau, Barcelona, Spain.)

View larger version (155K): [in a new window]   Figure 9c. Pulmonary tumor thrombotic microangiopathy. (a) Transverse thin-section CT image through the right lower lobe shows small branching opacities (arrows) representing enlarged peripheral arteries. (b) Cut surface of autopsy lung specimen shows pale nodules, which correspond to the pulmonary arteries thickened by the fibrotic reaction to tumor. (c) Photomicrograph demonstrates dense fibrocellular proliferation within an arteriolar lumen. The tumor emboli (arrows) are relatively small. (Image courtesy of Dr T. Franquet, Hospital Sant Pau, Barcelona, Spain.)

View larger version (117K): [in a new window]   Figure 10a. Histopathologic features of pulmonary hypertension. (a) Photomicrograph shows muscular hypertrophy and subendothelial fibrosis in two small pulmonary arteries. (Original magnification, x400.) (b) Photomicrograph shows typical plexiform lesion, with several abnormal vascular channels (arrows) in the damaged wall of a small pulmonary artery. (Original magnification, x200.)

View larger version (139K): [in a new window]   Figure 10b. Histopathologic features of pulmonary hypertension. (a) Photomicrograph shows muscular hypertrophy and subendothelial fibrosis in two small pulmonary arteries. (Original magnification, x400.) (b) Photomicrograph shows typical plexiform lesion, with several abnormal vascular channels (arrows) in the damaged wall of a small pulmonary artery. (Original magnification, x200.)

View larger version (154K): [in a new window]   Figure 11. Transverse thin-section CT image (soft-tissue window settings) in a patient with primary pulmonary hypertension. In this patient with a mean pulmonary artery pressure of more than 35 mm Hg, the main pulmonary artery (pa) is dilated and there is fluid within the anterior (double-headed arrow) and posterior (arrowhead) superior pericardial recesses. A pericardial effusion was evident on more caudal sections (not shown).

View larger version (73K): [in a new window]   Figure 12a. Severe small-vessel vasculopathy thought to be a variant of veno-occlusive disease at histologic examination. (a) Transverse thin-section CT image through the lower lobes shows numerous poorly defined nodules (arrows) ranging from 1 to 4 mm in diameter, with a generalized increase in attenuation of lung parenchyma. (b) Low-power photomicrograph shows that nodules seen at CT correspond to small, scattered, focal areas of hemorrhage, most obvious in the bottom right-hand corner. (Original magnification, x20.)

View larger version (187K): [in a new window]   Figure 12b. Severe small-vessel vasculopathy thought to be a variant of veno-occlusive disease at histologic examination. (a) Transverse thin-section CT image through the lower lobes shows numerous poorly defined nodules (arrows) ranging from 1 to 4 mm in diameter, with a generalized increase in attenuation of lung parenchyma. (b) Low-power photomicrograph shows that nodules seen at CT correspond to small, scattered, focal areas of hemorrhage, most obvious in the bottom right-hand corner. (Original magnification, x20.)

View larger version (103K): [in a new window]   Figure 13a. Capillary hemangiomatosis. (a) Transverse thin-section CT image shows mosaic attenuation pattern in the lower lobes, with clear demarcation between areas of lung of differing attenuation. More cephalic sections (not shown) demonstrated marked dilatation of the proximal pulmonary arteries. (Image courtesy of Dr L. Mitchell, Freeman Hospital, Newcastle upon Tyne, England.) (b) Low-power photomicrograph of lung biopsy specimen shows clear demarcation between abnormal capillary proliferation causing thickening of interstitium (on the left), as compared with relatively normal lung (on the right). Small pulmonary arteries (arrowheads) at top of field show features of pulmonary hypertension; the patient was initially thought to have primary pulmonary hypertension. (Original magnification, x40.)

View larger version (154K): [in a new window]   Figure 13b. Capillary hemangiomatosis. (a) Transverse thin-section CT image shows mosaic attenuation pattern in the lower lobes, with clear demarcation between areas of lung of differing attenuation. More cephalic sections (not shown) demonstrated marked dilatation of the proximal pulmonary arteries. (Image courtesy of Dr L. Mitchell, Freeman Hospital, Newcastle upon Tyne, England.) (b) Low-power photomicrograph of lung biopsy specimen shows clear demarcation between abnormal capillary proliferation causing thickening of interstitium (on the left), as compared with relatively normal lung (on the right). Small pulmonary arteries (arrowheads) at top of field show features of pulmonary hypertension; the patient was initially thought to have primary pulmonary hypertension. (Original magnification, x40.)

View larger version (131K): [in a new window]   Figure 14. Transverse thin-section CT image through the lower lobes in a patient with veno-occlusive disease shows combination of thickening of interlobular septa (arrows), ground-glass opacity, and pleural effusions. Fluid is seen tracking into the right oblique fissure (arrowheads). (Image courtesy of Dr S. Swensen, Mayo Clinic, Rochester, Minn.)

View larger version (96K): [in a new window]   Figure 15a. (a) Transverse thin-section CT image through the upper lobes in a ventilated 27-year-old woman with acute sickle cell crisis shows mosaic attenuation pattern. Vessels within areas of reduced attenuation are of decreased caliber (arrowheads). Combined dependent atelectasis and pleural effusions can be seen. (b) Photomicrograph of open lung biopsy specimen shows engorgement of alveolar capillaries resulting from sludging and occlusion by sickle cells, most obvious on the left-hand side of the image. (Original magnification, x200.)

View larger version (152K): [in a new window]   Figure 15b. (a) Transverse thin-section CT image through the upper lobes in a ventilated 27-year-old woman with acute sickle cell crisis shows mosaic attenuation pattern. Vessels within areas of reduced attenuation are of decreased caliber (arrowheads). Combined dependent atelectasis and pleural effusions can be seen. (b) Photomicrograph of open lung biopsy specimen shows engorgement of alveolar capillaries resulting from sludging and occlusion by sickle cells, most obvious on the left-hand side of the image. (Original magnification, x200.)

View larger version (129K): [in a new window]   Figure 16a. Transverse thin-section CT images through the lower zones of two patients with chronic changes of sickle cell anemia. (a) Irregular linear subpleural opacities in the lower lobes (arrowheads), with associated distortion of the lung architecture, indicate interstitial fibrosis in a patient with repeated sickle cell crises. (b) The striking abnormality is diffuse ground-glass opacity: The pathologic correlate of this pattern in chronic sickle cell disease is uncertain. (Images courtesy of Dr S. Desai, King’s College Hospital, London, England.)

View larger version (107K): [in a new window]   Figure 16b. Transverse thin-section CT images through the lower zones of two patients with chronic changes of sickle cell anemia. (a) Irregular linear subpleural opacities in the lower lobes (arrowheads), with associated distortion of the lung architecture, indicate interstitial fibrosis in a patient with repeated sickle cell crises. (b) The striking abnormality is diffuse ground-glass opacity: The pathologic correlate of this pattern in chronic sickle cell disease is uncertain. (Images courtesy of Dr S. Desai, King’s College Hospital, London, England.)

View larger version (141K): [in a new window]   Figure 17a. Langerhans cell histiocytosis. (a) Transverse thin-section CT image shows fine reticulonodular pattern in the upper lobes, with relative sparing of the lower lobes. (b) Transverse contrast-enhanced CT image shows marked dilatation of the main pulmonary artery (arrowheads) relative to the ascending aorta (a), indicating severe pulmonary hypertension. (c) Photomicrograph of biopsy specimen in another patient shows Langerhans cell histiocytosis nodule involving and partly obliterating a pulmonary artery (arrowheads). (Original magnification, x20.) (Fig 17c courtesy of Dr F. Capron, Assistance Hôpitaux Publique de Paris, France.)

View larger version (92K): [in a new window]   Figure 17b. Langerhans cell histiocytosis. (a) Transverse thin-section CT image shows fine reticulonodular pattern in the upper lobes, with relative sparing of the lower lobes. (b) Transverse contrast-enhanced CT image shows marked dilatation of the main pulmonary artery (arrowheads) relative to the ascending aorta (a), indicating severe pulmonary hypertension. (c) Photomicrograph of biopsy specimen in another patient shows Langerhans cell histiocytosis nodule involving and partly obliterating a pulmonary artery (arrowheads). (Original magnification, x20.) (Fig 17c courtesy of Dr F. Capron, Assistance Hôpitaux Publique de Paris, France.)

View larger version (143K): [in a new window]   Figure 17c. Langerhans cell histiocytosis. (a) Transverse thin-section CT image shows fine reticulonodular pattern in the upper lobes, with relative sparing of the lower lobes. (b) Transverse contrast-enhanced CT image shows marked dilatation of the main pulmonary artery (arrowheads) relative to the ascending aorta (a), indicating severe pulmonary hypertension. (c) Photomicrograph of biopsy specimen in another patient shows Langerhans cell histiocytosis nodule involving and partly obliterating a pulmonary artery (arrowheads). (Original magnification, x20.) (Fig 17c courtesy of Dr F. Capron, Assistance Hôpitaux Publique de Paris, France.)

View larger version (186K): [in a new window]   Figure 18. Pulmonary capillaritis. Photomicrograph shows intense neutrophilic infiltration within the wall (arrowheads) of a small pulmonary artery (neutrophilic vasculitis), with some extravasation of red blood cells into surrounding airspaces. (Original magnification, x400.)

View larger version (105K): [in a new window]   Figure 19. Transverse thin-section CT image through the lower lobes in a 31-year-old patient with Goodpasture syndrome shows combination of widespread areas of ground-glass opacity, with poorly defined nodular elements and more intense opacification within the right middle lobe (arrowheads), reflecting extravasation of blood into the airspaces.

View larger version (117K): [in a new window]   Figure 20. Transverse thin-section CT image through the upper lobes in a 45-year-old man with Wegener granulomatosis. This is an unusual manifestation of Wegener granulomatosis, with widespread areas of ground-glass opacity and intense opacification (there were no nodules or cavitating masses elsewhere). The pathologic correlate of this CT pattern is diffuse pulmonary hemorrhage as consequence of a pulmonary capillaritis.