Heart Vessels (1992) Suppl. 7:97-i01

Heart andVesse~ s ©Springer-Verlag 1992

Angiographic characteristics of Takayasu arteritis Y o u n g D. C h o 1 a n d K y u T. L e e 2 1Department of Diagnostic Radiology, Kosin Medical Center and 2Departmcnt of Pathology, Kosin Medical Coll•ge, Pusan, Korea

Introduction Takayasu arteritis is a chronic inflammatory arteriopathy that involves primarily brachiocephalic vessels but may also affect the aorta and any of its primary branches. Clinically, the disease is subdivided into an early systemic and a late stenotic phase. And in the latter phase, variable ischemic signs secondary to arterial stenoses develop [1-4]. The diagnosis is confirmed radiologically and the combination of the arteriographic pattern and the location of the lesion is usually diagnostic. The disease has been known as a form of obstructive arteritis confined to the aortic arch since the term "pulseless disease" was suggested in 1952 by Shimizu and Sano who reported a detailed description of the disease [5]. In the following decades, however, it was shown that the disease is not confined to the aortic arch alone but also may extend to the descending thoracic and abdominal aorta as well [6-8].

Basic angiographic manifestation Takayasu arteritis may cause either arterial stenosis or dilatation. The disease process, however, predominantly results in arterial stenosis and/or obstruction. Based on angiographic findings in 70 patients in our series at the Kosin Medical Center, the narrowing or stenotic forms account for 91% (n = 64), and the dilatation in 9%. The earliest detectable changes at arteriography may be a localized narrowing or an irregularity of the aortic wall [9-11]. The appearance of each type of lesion, occlusion, stenosis, irregularity and dilatation or aneurysm, is not specific for any particular disease; rather, the combination of these lesions orten involving multiple proximal branches of Address correspondence to: Y.D. Cho

both the thoracic and abdominal aorta provides the pattern necessary for a specific diagnosis of Takayasu arteritis. The steno-obstructive forms of aortoarteritis are often manifested by even or uneven concentric narrowing of the thoracic (Fig. 1) or abdominal aorta (Fig. 2) with long extensive or segmental involvement and orten associated with stenosis or obstruction of main branches. Multiple vessel involvement is common. In our series of 70 patients, in more than two-thirds of the patients, two or more vessels were involved while single vessel involvement was seen in only nine patients (Table 1). All arteries arising from the aorta may be involved with stenoses and most commonly affected branch of the thoracic aorta is the left subclavian artery (45%). Occlusion is the second most common finding and often a characteristic "flame shaped" appearance of occluded vessel is seen (Fig. 3). Obstruction of the main branches of aortic arch is usually associated with abundant collateral cireulation (Fig. 4). Marked dilatation and aneurysm formation of either thoracic or abdominal aorta are uncommon, although mild luminal ectasia and cylindrical dilatations are orten noted associated with proximal segmental stenosis or occlusion of major branch (Fig. 5). Dilatation (more than 4 cm in diameter in the ascending aorta and over 3cm in the descending and abdominal aorta) is most commonly noted in the ascending aorta [12]. The dilatation may involve a long, continuous segment of the aorta [13, 14] and, in some instances, the entire aorta may be irregularly dilated [2].

Classification Ueno et al. [15] classified the disease into three varieties according to its extent. In type I, the involvement is localized to the aortic arch and its

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Y.D. Cho et al.: Angiographic characteristics of Takayasu arteritis

Fig. 1. A classic steno-obstructive aortoarteritis of an 11-year-old female with hypertension. The thoracic aortogram shows complete occlusions at the origin of the left subclavian (long arrow) and left common carotid artery and a long segment narrowing (arrow heads) with eventual occlusion of the right common carotid artery. Note dilated right vertebral artery and prominent collateral vessels (open arrow) in the neck supplied by enlarged right thyrocervical trunk. (Courtesy of Suk Kil Zeon, MD, Keimyung Medical College, Taegu, Korea)

Fig. 3. Digital subtraction angiography (DSA) assessment in a 43-year-old female. Left anterior oblique view (magnified) of thoracic aortogram shows occlusion of all major branches of the aorta and characteristic "flame shaped" occlusion of the innominate artery (open arrow)

Fig. 2. Abdominal aortogram in a 30-year-old female with renovascular hypertension. A long segment narrowing of the infrarenal abdominal aorta is seen (open arrows). Proximal portion of the right renal artery shows marked stenosis (arrowhead). Prominent collateral anastomotic arcade of Riolan and a large gastroduodenal artery (long arrow) are seen t o revascularize the superior mesenteric territory. (Courtesy of Jae H. Park, MD, Seoul National University, Seoul, Korea)

branches. In type II, the disease is confined only to the descending thoracic (Fig. 6) and abdominal aorta and Their branches. Type III is the combination of both types. Lupi-Herrera et al. [16] suggested the addition of type IV, in which the pulmonary artery is involved. Nasu's classification [9] is as follows: involvement of the branches of the aortic arch (type I), involvement of the thoracic aorta and its branches (type II), involvement of the abdominal aorta and its branches (type III), and extensive involvement of the whole length

Y.D. Cho et al.: Angiographic characteristics of Takayasu arteritis

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Table 1. Summary of angiographic findings in 70 cases Artery Thoracic aorta Innominate Subclavian, R/L Axillary R/L Carotid, R/L Vertebral, R/L Abdominal aorta Celiac Superior Mesenteric Renal, R/L Lower abdominal aorta Iliac, R/L

Stenosis

Occlusion

Irregularity

Dilatation

31 6 10/18 0 5/9 3/1 31 3 3 18/30 10 4/4

0 0 1/0 0/1 1/7 0 0 2 2 5/8 0 0

8 0 1/0 0 0 0 9 0 0 0 3 1/1

4 0 0 0 1/1 0 3 0 0 1/0 0 0/1

Fig. 4. Multiple cervical collateral vessels and patent vertebral arteries are well visualized with intra-arterial DSA in 19-year-old female. All supra-aortic branches are involved and a long segment of diffuse narrowing and irregularities is seen in the left common carotid artery. (Courtesy of Jae H. Park, MD, Seoul National University, Seoul, Korea)

Fig. 5. A dilated type of aortitis in a 29--year-old hypertensive female. Abdominal aortogram demonstrates a segmental dilatation (open arrow) and irregularity involving inter-renal portion of abdominal aorta and complete occlusion of the right renal artery

of the aorta and its branches (type IV). Salto [17] reported the pulmonary arteries are commonly involved because of abundant elastic tissues in their walls. The pulmonary artery involvement (Fig. 7) in Takayasu arteritis has been reported to occur in 5 0 % 86% of cases [18-20, 26].

Evaluation of angiographic diagnosis Because the clinical features are determined by the extent and severity of the specific artery involved in the occlusive process, it is important to perform comprehensive angiography whenever arteritis is

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Y.D. Cho et al. : Angiographic characteristics of Takayasu arteritis diagnosis of Takayasu arteritis. Using DSA, not only a smaller amount of contrast medium required, but there is also better visualization of collaterals in patients with severe involvement. DSA is particularly useful in the follow up of the patients with an established diagnosis of Takayasu arteritis. Pulmonary arterial involvement could also be demonstrated with intravenous DSA [27]. Excellent images can be obtained with intraarterial DSA using small amount of contrast material, particularly for the patients who have extensive vascular abnormalities and impaired renal function. Intra-arterial DSA also can be performed on an out patient basis as catheters of small diameter are used [28].

Angiographic clinical correlation

Fig. 6. Arch aortogram with intravenous DSA of 21-yearold hypertensive male. Multiple constrictive narrowing (ùbeaded appearance") of the descending aorta (open arrow) distal to patent left subclavian artery (arrow) is seen

suspected. Thoracic and abdominal aortography must be done in all patients to visualize the entire aorta and all of its branches [21-26]. Views of the brachiocephalic arteries should include the proximal brachial arteries and the carotids at least to the proximal internal and external divisions. In the abdominal area the proximal celiac, superior mesenteric, and renal arteries should be studied. The importance of total aortography had been emphasized by Lande et al. [21-24]. The advent of digital subtraction arteriography (DSA) has proved an important tool in the early

In general, angiographic findings are very closely correlated with clinical mainfestations. The renal artery is the most frequently involved branch of the abdominal aorta in patients with Takayasu arteritis and the presence of hypertension strongly suggests renal artery stenosis. Repair of renal artery stenosis with angioplasty usually yields good results when the blood pressure is difficult to control [29]. Coronary artery involvement was first described by Young et al. [30] and tater by others [31, 32] with most of the coronary artery involvement reported to be osteal or proximal in the artery and manageable with angioplasty [29].

Differential diagnosis Since this disease usually affects young patients, confusion with arteriosclerosis is seldom a problem. The

Fig. 7. Pulmonary arteriogram in 31-yearold female. Note extensive obstruction of the left pulmonary artery branches

Y.D. Cho et al.: Angiographic characteristics of Takayasu arteritis main disease to be considered in differential diagnosis vary with the regions involved. T e m p o r a l arteritis causes similar changes in the subclavian, axillary, brachial, and femoral arteries [33, 34]. Neurofibromatosis and congenital coarctation may involve the abdominal aorta, renal, superior mesenteric, celiac, and iliac arteries, causing diagnostic confusion. Both ergotism and Buerger's disease may affect the femoral, brachial and axillary arteries [35]. Radiation fibrosis may affect any arterial bed in the radiation field including the aorta. Clinical correlation is therefore important in correct diagnosis.

17. 18. 19. 20. 21.

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Angiographic characteristics of Takayasu arteritis.

Heart Vessels (1992) Suppl. 7:97-i01 Heart andVesse~ s ©Springer-Verlag 1992 Angiographic characteristics of Takayasu arteritis Y o u n g D. C h o 1...
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