AJR 2005; 184:1945-1950
© American Roentgen Ray Society
Imaging Findings in Takayasu's Arteritis
Michael B. Gotway1,2,
Philip A. Araoz3,
Thanila A. Macedo3,
Anthony W. Stanson3,
Charles B. Higgins1,
Ernest J. Ring1,2,
Samuel K. Dawn1,2,
W. Richard Webb1,
Jessica W. T. Leung1 and
Gautham P. Reddy1
1 Department of Radiology, University of California, San Francisco, 505
Parnassus Ave., San Francisco, CA 94143.
2 Department of Radiology, San Francisco General Hospital, 1001 Potrero Ave.,
Rm. 1X 55A, Box 1325, San Francisco, CA 94110.
3 Department of Radiology, Mayo Clinic, 200 First St. SW, Rochester, MN
55905.
Received June 28, 2004;
accepted after revision September 27, 2004.
Address correspondence to M. B. Gotway.
Abstract
OBJECTIVE. The objective of our study was to evaluate the clinical
usefulness of cross-sectional imaging for establishing the diagnosis of
Takayasu's arteritis (TA), an inflammatory vascular disorder that produces
arterial stenoses and aneurysms primarily involving the thoracoabdominal aorta
and its branches and the pulmonary arteries.
CONCLUSION. CT and MRI findings of TA include vascular wall
thickening and enhancement early in the disease, and arterial stenoses,
occlusions, and aneurysms later in the disease. Cross-sectional imaging is
useful for establishing the diagnosis of TA and for showing response to
nonsurgical therapy or for planning a surgical intervention.
Introduction
Takayasu's arteritis (TA) is an idiopathic inflammatory vascular
disorder that may involve the thoracoabdominal aorta and its branches and the
pulmonary arteries. TA has a much higher incidence in women than in men and is
most frequently found in Asian patients, although the condition may occur in
North American, European, African, and Middle Eastern patients
[1].
Pathophysiology
TA causes arterial media destruction, leading to aneurysm formation and,
uncommonly, rupture of involved arteries. Histopathologically, early TA
changes consist of an adventitial mononuclear infiltrate with perivascular
cuffing of the vasa vasorum, followed by medullary mononuclear inflammation,
occasionally accompanied by granulomatous changes
[2].
Because histopathologic specimens are seldom available as a result of the
large vessels commonly affected and the fact that the histopathologic
appearance of TA can mimic other arteritides, the diagnosis of TA is largely
based on the combination of clinical information, laboratory evaluation, and
diagnostic imaging. Therefore, knowledge of the radiologic features of TA is
essential for accurate diagnosis and early treatment. Although angiography has
been widely used for the diagnosis of TA, increasingly CT and MRI are used as
the diagnostic techniques of choice.
Clinical Presentation
TA has traditionally been divided into an early, "prepulseless"
systemic phase, and a late, occlusive phase. In the early systemic phase,
diagnosis is difficult and symptoms are usually nonspecific and
constitutional, including fever, myalgias, weight loss, and arthralgias. In
the occlusive phase, ischemic symptoms dominate, including angina,
claudication, syncope, and visual impairment
[3]. Late-phase TA may be
further subclassified as classic pulseless disease (type 1), a mixed type
(type 2), an atypical coarctation type (type 3), and a dilated type (type 4)
[1]. Most patients present with
a form of late-phase disease.
Treatment
High-dose corticosteroids are the mainstay of TA therapy. Treatment of
symptomatic fibrotic lesions (stenoses or occlusions) requires either
interventional or surgical therapy. This can be achieved by angioplasty with
or without stenting or, in severe cases, by vascular resection and surgical
placement of composite grafts
[4,
5] (Figs.
1A, and
1B).
View larger version (118K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1A. —45-year-old-woman with Takayasu's arteritis and graft
placement for abdominal aortic occlusion. Coronal maximum-intensity-projection
(MIP) (A) and volume-rendered (B) images show supraceliac
aorta-to-right iliac artery bypass graft (single large arrow) and
supraceliac aorta-to-right renal artery bypass graft (double small
arrows) with occlusion (single small arrow) of midportion
abdominal aorta below superior mesenteric artery origin, best shown on MIP
image (A). Left renal artery is supplied via bypass graft (single
arrowhead). Superior mesenteric artery is supplied from left iliac artery
bypass graft (double arrowheads).
|
|
View larger version (57K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1B. —45-year-old-woman with Takayasu's arteritis and graft
placement for abdominal aortic occlusion. Coronal maximum-intensity-projection
(MIP) (A) and volume-rendered (B) images show supraceliac
aorta-to-right iliac artery bypass graft (single large arrow) and
supraceliac aorta-to-right renal artery bypass graft (double small
arrows) with occlusion (single small arrow) of midportion
abdominal aorta below superior mesenteric artery origin, best shown on MIP
image (A). Left renal artery is supplied via bypass graft (single
arrowhead). Superior mesenteric artery is supplied from left iliac artery
bypass graft (double arrowheads).
|
|
View larger version (137K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 2. —50-year-old woman with Takayasu's arteritis (TA). Catheter
angiography shows mild infrarenal abdominal aortic stenosis (arrow).
Although atherosclerosis commonly affects infrarenal abdominal aorta,
atherosclerosis usually produces abrupt caliber changes just beyond stenosis
sites; the smooth tapered nature of this stenosis favors TA.
|
|
View larger version (139K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 4. —45-year-old woman with Takayasu's arteritis. Oblique
projection catheter angiogram shows segmental stenosis of origin of right
upper lobe pulmonary artery (single arrow). Other stenoses are also
visible (double arrows).
|
|
Imaging Findings
Angiography
Angiography, particularly digital subtraction angiography, has
traditionally been the procedure of choice for the diagnostic evaluation of TA
[6,
7]. Angiography often shows
long, smooth, tapered stenoses ranging from mild
(Fig. 2) to severe (Figs.
3 and
4) or frank occlusions
(Fig. 5); collateral vessels
(Fig. 6), or the subclavian
steal phenomenon [4], are also
well shown. Angiography is useful in guiding interventional procedures such as
angioplasty or stent placement. However, angiography is invasive, carries a
substantial radiation dose, may require a large amount of iodinated contrast
material, and can be difficult to perform in patients with long-segment
stenoses or heavy arterial calcification. Furthermore, angiography does not
depict wall architecture changes as effectively as cross-sectional techniques
and cannot differentiate vascular narrowing due to acute mural inflammation
from stenoses due to chronic transmural fibrosis. Finally, the frequency of
ischemic complications resulting from angiography in patients with TA has been
shown to be high, and cross-sectional techniques are therefore attractive for
diagnosis and disease monitoring
[8].
View larger version (95K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 5. —52-year-old woman with Takayasu's arteritis. Catheter
angiogram shows severe infrarenal abdominal aortic stenosis, ending in aortic
occlusion (arrow), with extensive collateral vessel formation.
|
|
View larger version (146K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 6. —38-year-old woman with Takayasu's arteritis. Catheter
angiogram shows severe infrarenal abdominal aortic stenosis
(arrowhead) with extensive collateral vessel formation by enlarged
pancreaticoduodenal artery between superior mesenteric and celiac arteries
(arrows).
|
|
Sonography
Sonography reveals homogeneous circumferential thickening of affected
vessels (indistinguishable from atherosclerotic plaque), vascular occlusions
and dilation, and flow velocity elevations beyond stenotic lesions in patients
with TA. Limitations of sonography include lack of a consistent acoustic
window to allow visualization of the root of the great vessels, obscuration of
abdominal vasculature by overlying bowel gas, and technically limited studies
in obese patients. Invasive imaging with transesophageal echocardiography and
intravascular sonography provides extremely high spatial resolution and allows
recognition of subtle wall changes in aortic segments that appear normal with
other imaging techniques.
CT
Because the basic pathologic feature of early-phase TA is great vessel wall
thickening, CT is useful for early diagnosis because it allows evaluation of
wall thickness rather than merely the luminal diameter
[6], which is especially
important because early diagnosis and treatment are associated with improved
prognosis. The spectrum of findings on CT angiography includes stenoses;
occlusions; aneurysms (Figs.
7A,
7B,
7C,
8,
9A, and
9B); and concentric arterial
wall thickening affecting the aorta and its branches, the pulmonary arteries,
and occasionally the coronary arteries
[1-3,
5-8]
(Figs. 9A, and
9B). In the later stage of
disease, extensive vascular calcification may occur
(Fig. 10). Limitations of CT
include the need for iodinated contrast material and ionizing radiation; the
latter may limit the utility of CT for following up patients undergoing
treatment.
View larger version (137K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 7B. —40-year-old woman with aneurysmal form of Takayasu's
arteritis. Thoracic CT angiogram obtained caudal to A shows large
aneurysm (arrow) of brachiocephalic artery and proximal right
subclavian artery.
|
|
View larger version (91K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 8. —45-year-old woman with aneurysmal form of Takayasu's
arteritis. Axial CT image shows aneurysm of proximal descending thoracic aorta
(arrow). Differential diagnosis should include atherosclerosis,
mycotic aneurysm, and Behçet's syndrome.
|
|
MRI
MRI advantages include the lack of need for ionizing radiation and
iodinated contrast material; therefore, MRI is ideal for serial evaluation of
patients with TA who are undergoing treatment. Furthermore, MRI provides the
ability to view vessels in any desired plane, and techniques like cine MRI can
detect cardiovascular functional and hemodynamic changes, such as aortic
regurgitation, in patients with TA
[2]. As with CT, MRI is useful
for early diagnosis because of its ability to evaluate wall thickness rather
than just the luminal narrowing
[1,
2,
8].
Findings of TA on MRI include mural thrombi, signal alterations within and
surrounding inflamed vessels (Figs.
11A,
11B,
12A, and
12B), fusiform vascular
dilation, thickened aortic valvular cusps, multifocal stenoses (Figs.
13 and
14), and concentric thickening
of the aortic wall [1,
2,
8] (Figs.
12A, and
12B). MRI may also reveal
pericardial effusions and signal alterations within the pericardial sac,
representing fluid and granulation tissue.
View larger version (86K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 11A. —19-year-old man with early Takayasu's arteritis. Axial
T1-weighted MR image (TR/TE, 500/20) obtained through superior mediastinum
shows thickening of right brachiocephalic artery wall
(arrowheads).
|
|
View larger version (108K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 11B. —19-year-old man with early Takayasu's arteritis. Axial
T1-weighted gadolinium-enhanced MR image (500/20) shows extensive enhancement
of abnormally thickened right brachiocephalic artery wall
(arrowheads).
|
|
View larger version (115K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 12B. —32-year-old woman with Takayasu's arteritis. Axial
T1-weighted MR image (500/20) with fat saturation after gadolinium
administration shows extensive enhancement of thickened abdominal aorta
(arrow).
|
|
View larger version (84K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 13. —50-year-old woman with Takayasu's arteritis. Coronal MR
angiogram shows short-segment occlusion of infrarenal abdominal aorta
(long arrow). Occlusion of right common femoral artery (short
arrows) is also evident.
|
|
View larger version (128K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 14. —59-year-old woman with multifocal great vessel stenoses due
to Takayasu's arteritis (TA). Maximum-intensity-projection coronal MR
angiogram shows occlusion of right brachiocephalic artery (arrow),
severe stenosis of right subclavian artery (single arrowhead), and
occlusion of proximal left subclavian artery (double arrowheads).
Subclavian arteries are reconstituted by collateral vessel formation
bilaterally. Note relatively proximal great vessel involvement; whereas giant
cell arteritis may have imaging appearance similar to that of TA, lesions are
usually located more distally in latter disorder.
|
|
Disadvantages of MRI include difficulty in visualizing small branch vessels
and poor visualization of vascular calcification. MRI is also expensive and is
often less available in regions where TA is most prevalent.
MR angiography provides detailed vascular information, including the
location, degree, extent of stenoses and dilation, and patency of collateral
vessels and surgical bypass grafts (Fig.
15). Limitations of MR angiography include the possibility that
vascular branch points may be improperly interpreted as occlusions
(breath-hold techniques have lessened this problem) and that
maximum-intensity-projection images may falsely accentuate the degree of
vascular stenoses. Regarding the latter, we believe it is advisable to assess
the degree of vascular stenoses from the MR angiography source images.
View larger version (104K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 15. —55-year-old woman with Takayasu's arteritis, infrarenal
abdominal aortic occlusion, and surgical arterial bypass graft. Coronal
maximum-intensity-projection MR angiogram shows occluded infrarenal abdominal
native aorta (arrow) and surgical bypass graft (arrowheads)
with reimplanted abdominal vasculature.
|
|
Conclusion
Angiographic and cross-sectional imaging findings of TA include vascular
stenoses, occlusions, and aneurysm formation. Familiarity with the demographic
and imaging features of TA will facilitate accurate diagnosis and allow early
treatment, improving patient outcome.
References
- Matsunaga N, Hayashi K, Sakamoto I, Ogawa Y, Matsumoto T. Takayasu
arteritis: protean radiologic manifestations and diagnosis.
RadioGraphics1997; 17:579
-594[Abstract]
- Matsunaga N, Hayashi K, Sakamoto I, et al. Takayasu arteritis: MR
manifestations and diagnosis of acute and chronic phase. J Magn
Reson Imaging 1998;8:406
-414[Medline]
- Kerr GS, Hallahan CW, Giordano J, et al. Takayasu arteritis.
Ann Intern Med1994; 120:919
-929[Abstract/Free Full Text]
- Park JH, Han MC, Kim SH, Oh BH, Park YB, Seo JD. Takayasu
arteritis: angiographic findings and results of angioplasty.
AJR 1989;153:1069
-1074[Abstract/Free Full Text]
- Miyata T, Sato O, Koyama H, Shigematsu H, Tada Y. Long-term
survival after surgical treatment of patients with Takayasu's arteritis.
Circulation2003; 108:1474
-1480[Abstract/Free Full Text]
- Park JH, Chung JW, Lee KW, Park YB, Han MC. CT angiography of
Takayasu arteritis: comparison with conventional angiography. J
Vasc Interv Radiol 1997;8:393
-400[Medline]
- Paul JF, Fiessinger JN, Sapoval M, et al. Follow-up electron beam
CT for the management of early phase Takayasu arteritis. J Comput
Assist Tomogr 2001;25:924
-931[Medline]
- Yamada I, Numano F, Suzuki S. Takayasu arteritis: evaluation with
MR imaging. Radiology1993; 188:89
-94[Abstract/Free Full Text]
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
C. J. Francois, D. Tuite, V. Deshpande, R. Jerecic, P. Weale, and J. C. Carr
Unenhanced MR Angiography of the Thoracic Aorta: Initial Clinical Evaluation
Am. J. Roentgenol.,
April 1, 2008;
190(4):
902 - 906.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
N. Pipitone, A. Versari, and C. Salvarani
Role of imaging studies in the diagnosis and follow-up of large-vessel vasculitis: an update
Rheumatology,
April 1, 2008;
47(4):
403 - 408.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. Gothi and J. M. Joshi
A 16-Year-Old Girl With Hemoptysis, Intermittent Loss of Vision, and a Carotid Bruit
Chest,
January 1, 2008;
133(1):
300 - 304.
[Full Text]
[PDF]
|
|
|
|
|
|
|
F. Stepansky, E. M. Hecht, R. Rivera, L. E. Hirsh, B. Taouli, M. Kaur, and V. S. Lee
Dynamic MR Angiography of Upper Extremity Vascular Disease: Pictorial Review
RadioGraphics,
January 1, 2008;
28(1):
e28 - e28.
[Abstract]
[Full Text]
|
|
|
|
|
|
|
E. Sueyoshi, I. Sakamoto, and M. Uetani
MRI of Takayasu's Arteritis: Typical Appearances and Complications.
Am. J. Roentgenol.,
December 1, 2006;
187(6):
W569 - W575.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
T. A. Bley, M. Markl, and O. Wieben
Inflammatory hyperenhancement persists in delayed high-resolution MRI in giant cell arteritis.
Am. J. Roentgenol.,
April 1, 2006;
186(4):
1197 - 1198.
[Full Text]
[PDF]
|
|
|
Top
Abstract
Introduction
