3. 938 J Ultrasound Med 23:937–944, 2004
Sonographic and Color Doppler Findings in Aortoarteritis
Figure 1. Long-standing aortoarteritis in a 22-year-old woman: gray scale and color Doppler sonograms. A, Aortic arch (AOA) involvement with thickening
of the wall. B, Almost complete occlusion of both the carotid arteries on B-mode transverse images showing wall thickening and a clot and longitudinal color
Doppler images of the carotid arteries showing low-velocity flow in the vasa vasorum. C, Filling of the left internal carotid artery (ICA; red) by reversal of flow
in the left external carotid artery (ECA; blue). D, Filling of the right ICA (red) by reversal of flow in the right ECA (blue). E, Right SCA wall thickening and
stenosis with high-velocity flow on Doppler imaging, left SCA proximal occlusion (arrowhead) with distal filling through the collateral (arrow), and retrograde
monophasic low flow velocity in the mid SCA from the collateral. (continues)
A C
D
E
B
4. and its major branches, usually at the points of
origin from the aorta, the most frequently affect-
ed arteries being the subclavian (90%), carotid
(45%), vertebral (25%), and renal (20%).1
The
most commonly affected artery in patients after
initial diagnosis was found to be the left subcla-
vian artery (SCA), followed by the right SCA and
the left common carotid artery (CCA).2
However,
in patients with aortoarteritis who had the dis-
ease for more than 2 years, the left and right SCAs
were found to be equally affected, followed by
the left CCA.
Pathologic Mechanism
Classically, the natural history ofTakayasu arteri-
tis has been described in 3 phases.The early pre-
pulseless phase is characterized by systemic
symptoms (ie, malaise, low-grade fever, weight
loss, and arthralgia). It is followed by the phase of
active vascular inflammation, when the clinical
picture is dominated by pain localized over the
affected area and the appearance of symptoms
and signs of vascular insufficiency. Finally, fibrot-
ic and stenotic lesions characterize the so-called
burnout disease. However, the triphasic pattern
of the disease can no longer be considered a rule,
and the absence of systemic clinical features
does not exclude ongoing vascular inflamma-
tion, nor does the presence of ischemic symp-
toms always indicate active inflammation of
vessels.7
Sonographic Findings
Sonography is often the primary modality of
investigation in a patient with signs and symp-
toms of aortoarteritis. Sonographic findings can
be divided into the following according to the
nature of the lesion.
Wall Thickening
This is the earliest finding in aortoarteritis and is
universally seen in all patients with aortoarteri-
tis. There is uniform thickening of the wall of the
vessels involved (Figure 2). The earliest wall
thickening is seen in the SCAs, most commonly
the left SCA.The arch of the aorta is also involved
early; however, because of difficulty in visualiza-
tion of the aortic arch, the presence of aortoar-
teritis is inferred from assessment of the major
aortic arch branches.8
It is easier to assess the
abdominal aorta and its branches. Measurement
of abdominal aortic wall thickness is difficult
because of the constant motion. In Takayasu
arteritis, long segments of diffuse, homoge-
neous, moderately echoic circumferential vessel
wall thickening are found. This is seen more
commonly in the CCA in Takayasu arteritis and
has been described as the “macaroni sign”
(Figure 3). It can be distinguished from arte-
riosclerosis, which is more inhomogeneous.9,10
An increase in wall thickness is associated with
secondary signs such as decreased pulsatility
and loss of a normal triphasic flow pattern. The
J Ultrasound Med 23:937–944, 2004 939
Chaubal et al
Figure 1. (continued) F, Monophasic low flow velocities in the left upper extremity arteries due to proximal SCA occlusion and filling through the collateral.
G, Loss of the triphasic pattern in the right upper extremity arteries due to involvement by aortoarteritis.
F G
5. involved vessels reveal loss of the triphasic pat-
tern, with a monophasic or biphasic parvus tar-
dus type of spectral flow pattern (Figure 1). This
type of pattern is also seen distal to an occlusion
when there is reformation of vessels by collater-
als, but wall thickening associated with damp-
ened flow suggests the diagnosis of aortoarteritis.
Luminal Narrowing or Stenosis
Luminal narrowing or stenosis is common in
aortoarteritis because of wall thickening, which
leads to a decrease in the luminal diameter. This
stenosisornarrowingiscommonlyseenasalong
segment (Figure 4) compared with atherosclero-
sis or fibromuscular dysplasia, in which the
stenoses are commonly short segments.
Luminal Dilatation and Aneurysms
Luminal dilatation and aneurysms are not as
common as narrowing. It is suggested to be due
to inadequate supportive fibrous tissue or focal
intima weakness.11
The aorta is most commonly
affected, especially the thoracic and abdominal
portions (Figure 5).
Calcification
Calcification is uncommon in aortoarteritis and
more commonly seen in atherosclerosis.
Occlusions
Occlusions are seen in the later stages of the dis-
ease (Figures 6 and 7). Smaller vessels such as the
carotid, subclavian, vertebral, and renal arteries
940 J Ultrasound Med 23:937–944, 2004
Sonographic and Color Doppler Findings in Aortoarteritis
Figure 2. Sonograms of the abdominal aorta (A; arrows) and brachio-
cephalic artery (BCA; B) showing uniform thickening of the wall.
A
B
Figure 3. Sonograms of the CCA in 2 patients (A and B) showing the “macaroni sign” (B, arrowhead).
A B
6. are commonly involved. Because of the chronic-
ity and slow progression of the disease, occlu-
sions are commonly associated with collateral
flow (Figure 8).12
Pulsatility
Pulsatility or compliance of the involved arteries
has been found to be decreased in the pulseless
stage of the disease in all cases of aortoarteritis.
Changes in compliance have been known to pre-
cede the angiographic changes in vessels affected
by aortoarteritis. Measurement of arterial compli-
ance may provide indices of early vascular
changes that predispose to the development of
major vascular disease. Measurement of arterial
stiffnesscouldbeusefulinidentifyingtheprolifer-
ative stage, the identification of which could be
critical in early institution of treatment, which
could prevent the further progression of the
disease. Arterial compliance can be studied by
various invasive and noninvasive methods.13,14
Similar methods could be applied to the study of
arterial compliance in patients with aortoarteritis.
Associated Organ Involvement
Heart
Involvement of the heart is usually secondary to
long-standing hypertension and ensuing con-
gestive heart failure or left ventricular hypertro-
phy.15
Congestive heart failure has been
postulated to be a result of hypertension, rapidly
developing pressure overload, and high levels of
aldosterone and angiotensin II.
J Ultrasound Med 23:937–944, 2004 941
Chaubal et al
Figure 4. Sonograms showing long segment narrowing of the ECA (A)
and aorta (B).
A
B
Figure 5. Sonograms showing luminal dilatation in the right
SCA (A) and aorta (B).
A
B
7. Lung and Pulmonary Artery
Various autopsy series have described involve-
ment of the pulmonary arteries in aortoarteri-
tis.15,16
The pulmonary trunk is found to be more
commonly involved compared with the intrapul-
monary arteries. Cases appearing primarily as
pulmonary hypertension without any involve-
ment of the aorta have been described.17
Miscellaneous
On autopsy, retroperitoneal fibrosis, with or with-
out the presence of mediastinal fibrosis, has been
found to be associated. Periaortic fibrosis has also
been described.18
Renal involvement, in the form
of nephrotic syndrome19
and glomerulonephri-
tis,20
has been described (Figure 2). Ulcerative col-
itisandCrohndiseasehavealsobeenassociated.21
Conclusions
Color-coded Doppler sonography can facilitate
the accurate diagnosis of Takayasu arteritis by
the characteristic appearance. Associated organ
involvement can also be assessed. The response
to treatment and progress of disease can be
assessed. Sonography is a noninvasive modality
and is useful for assessing these patients. Color-
coded Doppler sonography can identify the
arteries that are affected by the disease process
but not shown on angiography.
942 J Ultrasound Med 23:937–944, 2004
Sonographic and Color Doppler Findings in Aortoarteritis
Figure 6. Long-standing aortoarteritis in a 30-year-old woman. A, Occlusion of the superior mesenteric artery (SMA; arrowhead). CA indicates celiac
artery. B, Stenosis of the origin of the celiac artery shown by the high-velocity Doppler flow pattern. C, Stenosis at the origin of the right renal artery
shown by the high-velocity Doppler flow pattern and the parvus tardus pattern in the intrarenal arterial segment.
A B
C
8. J Ultrasound Med 23:937–944, 2004 943
Chaubal et al
Figure 7. Reversal of flow in the vertebral artery (flow direction, blue)
compared with the CCA (flow direction, red) suggestive of a steal due to
subclavian occlusion.
Figure 8. Sonogram from a patient with hypertension secondary to renal
artery (RA) stenosis due to aortoarteritis with corresponding high-velocity
flows at the origin of the right and left renal arteries. AO indicates aorta;
arrow, left RA; arrowhead, right RA; and IVC, inferior vena cava.
References
1. Eagle KA, De Sanctis RW. Diseases of the
aorta. In: Braunwald E (ed). Heart Disease: A
Textbook of Cardiovascular Medicine. 4th
ed. Philadelphia, PA: WB Saunders Co;
1992:1528–1553.
2. Kinare SG, Gandhi MS, Deshpande JR.
Nonspecific Aortoarteritis (Pathology and
Radiology). Mumbai, India: Quest
Publications; 1998.
3. Arrend WP, Michael BA, Block DA, et al. The
American College of Rheumatology 1990
criteria for the classification of Takayasu’s
arteritis. Arthritis Rheum 1990; 33:1129–
1134.
4. Padmavati S, Aurora AP, Kasliwal RR.
Aortoarteritis in India. J Assoc Physicians
India 1987; 35:442–444.
5. Lupi-Herrera E, Sanchez-Torres C,
Marcushamer J, et al. Takayasu’s arteritis:
clinical study of 107 cases. Am Heart J
1977; 103:93–94.
6. Shelmar JH, Volkman DJ, Parrillo JE, et al.
Takayasu’s arteritis. Ann Intern Med 1994;
120:919–929.
7. Nasu T. Takayasu’s truncoarteritis: pulseless
disease on aortitis syndrome. Acta Pathol
Jpn 1982; 32(suppl 1):117–131.
8. Matsunaga N, Hayashi K, Sakamoto I.
Takayasu’s arteritis: protean radiologic man-
ifestations and diagnosis. Radiographics
1997; 17:579–594.
9. Kerr G. Takayasu’s arteritis. Curr Opin
Rheumatol 1994; 6:32–38.
10. Maeda H, Handa N, Matsumoto M, et al.
Carotid lesions detected by B-mode ultra-
sonography in Takayasu’s arteritis: “maca-
roni sign” as an indicator of the disease.
Ultrasound Med Biol 1991; 17:695–701.
11. Kozuka T, Nosaki T, Sato K, Tachiiri H.
Aneurysm-associated aortitis syndrome.
Acta Radiol 1968; 7:314–320.
12. Sharma S, Rajani M. Aortic occlusion in
nonspecific aortoarteritis (Takayasu disease):
incidence and spectrum of involvement.
Australas Radiol 1993; 37:57–59.
9. 13. Gamble G, Zorn J, Sanders G, et al. Estimation of
arterial stiffness, compliance and distensibility from
M-mode ultrasound measurements of the common
carotid artery. Stroke 1994; 25:11–16.
14. Emoto M, Nishizawa Y, Kawagishi T, et al. Stiffness
index of the common carotid and femoral arteries
are associated with insulin resistance in NIDDM.
Diabetes Care 1998; 21:1178–1182.
15. Arora P, Shankar J, Sethi KK, et al. Congestive car-
diomyopathy in nonspecific aortoarteritis. J Assoc
Physicians India 1985 3:589–591.
16. Lupi-Herrera E, Sanchez-Torres G, Horwitz S.
Gutierrez FE. Pulmonary artery involvement in
Takayasu’s arteritis. Chest 1975; 67:69–74.
17. Tyagi S, Kaul UA, Gambhir DS, et al. Pulmonary
artery involvement in aortoarteritis. Indian Heart J
1987; 39:415–419.
18. Lance NJ, Levinson DJ. Aortitis and periaortic fibrosis.
J Rheumatol 1991; 18:1095–1097.
19. Misra SS, Prakash S, Agarwal PL. Pulseless disease
(Takayasu’s syndrome). Am Heart J 1959; 57:177–
180.
20. Hellmann DB, Hardy K, Lindenfeld S, Ring E.
Takayasu’s arteritis associated with crescentric
glomerulonephritis. Arthritis Rheum 1987; 30:
451–452.
21. Achar KN, Al-Nakib B. Takayasu’s arteritis and ulcer-
ative colitis. Am J Gastroenterol 1986; 81:1215–
1217.
944 J Ultrasound Med 23:937–944, 2004
Sonographic and Color Doppler Findings in Aortoarteritis
