Pattern-Based Differential Diagnosis in Pulmonary Vasculitis Using Volumetric CT
Katharina Marten1,
Pierre Schnyder2,
Eckart Schirg3,
Mathias Prokop4,
Ernst J. Rummeny1 and
Christoph Engelke1
1 Department of Radiology, Klinikum rechts der Isar, Technical University
Munich, Ismaningerstrasse 22, Munich 81675, Germany.
2 Department of Radiology, Centre Hospitalier Universitaire Vadois, Rue du
Bugnon 46, Lausanne 1011, Switzerland.
3 Department of Radiology, Hanover Medical School, Carl Neuberg Strasse 1,
Hanover 30625, Germany.
4 Department of Radiology, Universitair Medisch Centrum Utrecht, Postbus 85500,
Utrecht 3508 GA, The Netherlands.
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Fig. 1. —32-year-old woman with Takayasu's arteritis. 4-MDCT angiogram shows
inflammatory wall thickening of right main and left lower lobe arteries
(arrowheads) with evidence of pulmonary artery trunk dilatation
(asterisk) due to pulmonary hypertension.
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Fig. 2A. —54-year-old man with Hughes-Stovin syndrome. Single-detector CT
angiograms show right second segmental and lower lobe artery aneurysms
(black asterisks) with infarction (white asterisk, A)
of right posterior upper lobe segment. (Courtesy of Paul L. Molina, University
of North Carolina, Chapel Hill, NC)
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Fig. 2B. —54-year-old man with Hughes-Stovin syndrome. Single-detector CT
angiograms show right second segmental and lower lobe artery aneurysms
(black asterisks) with infarction (white asterisk, A)
of right posterior upper lobe segment. (Courtesy of Paul L. Molina, University
of North Carolina, Chapel Hill, NC)
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Fig. 3A. —8-MDCT images of 35-year-old man with Wegener's disease and frank
hemoptysis. Image obtained with mediastinal window settings displays extensive
middle lobe infiltrate in massive diffuse alveolar hemorrhage.
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Fig. 3B. —8-MDCT images of 35-year-old man with Wegener's disease and frank
hemoptysis. Images show large inhomogeneous right middle lobe and left
anterior upper lobe, lingular air-space consolidations with lobular sparing
(arrows), and intralobular (black arrowheads, B) and
interlobular interstitial thickening, suggestive of recurrent episodes of
pulmonary hemorrhage.
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Fig. 3C. —8-MDCT images of 35-year-old man with Wegener's disease and frank
hemoptysis. Images show large inhomogeneous right middle lobe and left
anterior upper lobe, lingular air-space consolidations with lobular sparing
(arrows), and intralobular (black arrowheads, B) and
interlobular interstitial thickening, suggestive of recurrent episodes of
pulmonary hemorrhage.
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Fig. 4A. —Two cases of Churg-Strauss syndrome. 4-MDCT image of 68-year-old man
with Churg-Strauss syndrome shows peribronchovascular and subpleural, almost
geographic, air-space consolidations and discrete centrilobular nodules within
areas of ground-glass opacity (arrows). There is additional evidence
of smooth interlobular septal thickening (arrowheads).
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Fig. 4B. —Two cases of Churg-Strauss syndrome. Single-detector high-resolution
CT image of 43-year-old woman with Churg-Strauss syndrome shows apical
ill-defined subpleural air-space consolidations surrounded by coalescing
nodular opacities with peripheral ground-glass opacification
(asterisks). Note smooth interlobular septal thickening
(arrowheads). Patterns suggestive of pulmonary hemorrhage are not
present.
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Fig. 5A. —8-MDCT images of 63-year-old man with rheumatoid arthritis. CT was
performed for suspected interstitial lung disease. Diagnosis of pulmonary
hypertension was previously unknown. Images show pulmonary hypertension with
dilatation of right heart and main pulmonary arteries.
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Fig. 5B. —8-MDCT images of 63-year-old man with rheumatoid arthritis. CT was
performed for suspected interstitial lung disease. Diagnosis of pulmonary
hypertension was previously unknown. Images show pulmonary hypertension with
dilatation of right heart and main pulmonary arteries.
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Fig. 5C. —8-MDCT images of 63-year-old man with rheumatoid arthritis. CT was
performed for suspected interstitial lung disease. Diagnosis of pulmonary
hypertension was previously unknown. Images show centrilobular micronodules
(arrowheads, C), peripheral branching linear densities
(white arrow, D), discrete subpleural ground-glass opacity
(asterisks), and moderate peribronchial thickening (black
arrows). Findings are consistent with follicular bronchiolitis with mild
degree of nonspecific interstitial pneumonia.
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Fig. 5D. —8-MDCT images of 63-year-old man with rheumatoid arthritis. CT was
performed for suspected interstitial lung disease. Diagnosis of pulmonary
hypertension was previously unknown. Images show centrilobular micronodules
(arrowheads, C), peripheral branching linear densities
(white arrow, D), discrete subpleural ground-glass opacity
(asterisks), and moderate peribronchial thickening (black
arrows). Findings are consistent with follicular bronchiolitis with mild
degree of nonspecific interstitial pneumonia.
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Fig. 6A. —4-MDCT angiograms of 35-year-old woman with chronic Takayasu's
arteritis. Images show dilated arteries in areas of relatively high density of
right upper lobe and lingula corresponding to hyperfused lung. There are
various segmental branch stenoses (arrows) that are visible on left
side.
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Fig. 6B. —4-MDCT angiograms of 35-year-old woman with chronic Takayasu's
arteritis. Images show dilated arteries in areas of relatively high density of
right upper lobe and lingula corresponding to hyperfused lung. There are
various segmental branch stenoses (arrows) that are visible on left
side.
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Fig. 7A. —45-year-old woman with giant cell arteritis. 4-MDCT images show
inflammatory thickening and enhancement of pulmonary arterial
(arrowheads, A) and ascending aortic l (asterisks,
A) wall, high-grade stenosis of right main pulmonary artery, and
obliteration of right lower lobe artery (asterisk, B). No
aneurysms were found. Patient had normal pulmonary artery pressures.
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Fig. 7B. —45-year-old woman with giant cell arteritis. 4-MDCT images show
inflammatory thickening and enhancement of pulmonary arterial
(arrowheads, A) and ascending aortic l (asterisks,
A) wall, high-grade stenosis of right main pulmonary artery, and
obliteration of right lower lobe artery (asterisk, B). No
aneurysms were found. Patient had normal pulmonary artery pressures.
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Fig. 7C. —45-year-old woman with giant cell arteritis. Pulmonary arterial
digital subtraction angiogram shows subtotal occlusion of right main pulmonary
artery. Narrowed lumen can be seen as thin line of contrast material
(arrowheads). No lobar or segmental pulmonary artery branch is
visible.
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Fig. 7D. —45-year-old woman with giant cell arteritis. Axial 4-MDCT angiogram
(D) and anterior coronal thick-slab maximum-intensity-projection image
(E) show right pulmonary oligemia due to high-grade stenosis of right
main and occlusion of lower lobe arteries. Normal-sized vessels notable in
right lung are pulmonary veins, whereas arteries appear of small caliber.
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Fig. 7E. —45-year-old woman with giant cell arteritis. Axial 4-MDCT angiogram
(D) and anterior coronal thick-slab maximum-intensity-projection image
(E) show right pulmonary oligemia due to high-grade stenosis of right
main and occlusion of lower lobe arteries. Normal-sized vessels notable in
right lung are pulmonary veins, whereas arteries appear of small caliber.
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Fig. 8A. —35-year-old man with Wegener's disease and frank hemoptysis (same
patient as in Figs. 3A,
3B, and
3C). Chest radiographs obtained
during 24-hr intensive care observation period show bilateral perihilar and
lower lobe consolidations that have increased significantly, as shown on
B, compared with control image (A). Patient was diagnosed as
having diffuse alveolar damage with pulmonary hemorrhage.
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Fig. 8B. —35-year-old man with Wegener's disease and frank hemoptysis (same
patient as in Figs. 3A,
3B, and
3C). Chest radiographs obtained
during 24-hr intensive care observation period show bilateral perihilar and
lower lobe consolidations that have increased significantly, as shown on
B, compared with control image (A). Patient was diagnosed as
having diffuse alveolar damage with pulmonary hemorrhage.
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Fig. 8C. —35-year-old man with Wegener's disease and frank hemoptysis (same
patient as in Figs. 3A,
3B, and
3C). Coronal
minimum-intensity-projection 8-MDCT image obtained at time of second chest
radiograph (B) shows fixed gravity-dependent air-space consolidations,
predominantly at bases of upper and lower lobes, and ground-glass infiltrates,
predominantly in lower lobes with intralobular and interlobular interstitial
thickening.
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Fig. 9. —33-year-old woman with Wegener's disease and minor hemoptysis.
Single-detector high-resolution CT image depicts distribution of ground-glass
opacification, respecting interlobular septa and resulting in patchwork-like
pattern in left posterior upper lobe segment. There is impression of
gravity-dependent density gradient toward dorsal lung periphery and
pronunciation of posterior subsegmental upper lobe bronchus ("dark
bronchus" sign).
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Fig. 10A. —36-year-old woman with history of asthma and acute hemoptysis in
Churg-Strauss syndrome. 16-MDCT images show bilateral peripheral lobular areas
of ground-glass opacity in basal lower lobes. On lung biopsy, there was
evidence of diffuse alveolar hemorrhage, eosinophilic alveolar septal
infiltration, and eosinophilic capillaritis.
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Fig. 10B. —36-year-old woman with history of asthma and acute hemoptysis in
Churg-Strauss syndrome. 16-MDCT images show bilateral peripheral lobular areas
of ground-glass opacity in basal lower lobes. On lung biopsy, there was
evidence of diffuse alveolar hemorrhage, eosinophilic alveolar septal
infiltration, and eosinophilic capillaritis.
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Fig. 11A. —33-year-old woman with acute lupus pneumonitis in systemic lupus
erythematosus. 4-MDCT images show widespread centrilobular
(arrowheads, B) and diffuse ground-glass opacity, confluent
lower lobe air-space consolidation, and minimal pleural effusion. Appearances
are nonspecific; diagnosis in patients without clinical diffuse alveolar
hemorrhage is by lung biopsy or by exclusion.
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Fig. 11B. —33-year-old woman with acute lupus pneumonitis in systemic lupus
erythematosus. 4-MDCT images show widespread centrilobular
(arrowheads, B) and diffuse ground-glass opacity, confluent
lower lobe air-space consolidation, and minimal pleural effusion. Appearances
are nonspecific; diagnosis in patients without clinical diffuse alveolar
hemorrhage is by lung biopsy or by exclusion.
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Fig. 12. —39-year-old man with Wegener's disease. 4-MDCT image shows bilateral
diffusely distributed centrilobular ground-glass nodules
(arrowheads), which are consistent with granulomas or inflammatory
infiltrates, that coalesce to larger nodular opacities such as at oblique
fissure in right upper lobe (arrows).
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Fig. 13A. —Two cases of microscopic polyangiitis. 4-MDCT image of 43-year-old
man with microscopic polyangiitis that was obtained during intermediate phase
of disease activity (A) and magnified view (B) of A show
bilateral diffuse angiocentric ground-glass nodules (arrowheads),
reflecting capillaritis or accumulation of alveolar macrophages due to minimal
diffuse alveolar hemorrhage. Neither interlobular nor intralobular
interstitial thickening is present.
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Fig. 13B. —Two cases of microscopic polyangiitis. 4-MDCT image of 43-year-old
man with microscopic polyangiitis that was obtained during intermediate phase
of disease activity (A) and magnified view (B) of A show
bilateral diffuse angiocentric ground-glass nodules (arrowheads),
reflecting capillaritis or accumulation of alveolar macrophages due to minimal
diffuse alveolar hemorrhage. Neither interlobular nor intralobular
interstitial thickening is present.
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Fig. 13C. —Two cases of microscopic polyangiitis. Single-detector CT image of
62-year-old man with active pulmonary microscopic polyangiitis and hemoptysis
shows two lobular areas of ground-glass opacity (arrows) in upper
lobes that are consistent with foci of pulmonary hemorrhage.
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Fig. 14A. —15-year-old boy with polyarteritis nodosa who presented with
recurrent episodes of perirenal hemorrhage, gastrointestinal hemorrhage, and
dyspnea. CT image shows bilateral lobular and arcade-like ground-glass
infiltrates located at branching vessels within central perihilar and
peripheral lung regions. Also, note absence of segmental or lobar
consolidation pattern, such as in pulmonary hemorrhage. Open lung biopsy
showed evidence of focal nonhemorrhagic pneumonitis with vasculitis involving
small to medium-sized vessel and sparing of arterioles and capillaries.
Digital subtraction angiography (not shown) of pulmonary arteries was negative
for aneurysmal disease.
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Fig. 14B. —15-year-old boy with polyarteritis nodosa who presented with
recurrent episodes of perirenal hemorrhage, gastrointestinal hemorrhage, and
dyspnea. CT image obtained 2 weeks after initiation of steroid and
immunosuppressive therapy shows that infiltrates have almost cleared.
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Copyright © 2005 by the American Roentgen Ray Society.
