Journal of The Indian Academy of Echocardiography & Cardiovascular Imaging

INTERESTING IMAGES
Year
: 2019  |  Volume : 3  |  Issue : 3  |  Page : 195--197

Case of Takayasu Arteritis


Rajesh Krishnachandra Shah 
 Shrikrishna Hospital, Aurangabad, Maharashtra, India

Correspondence Address:
Rajesh Krishnachandra Shah
Shrikrishna Hospital, 223, Samarthnagar, Aurangabad - 431 001, Maharashtra
India




How to cite this article:
Shah RK. Case of Takayasu Arteritis.J Indian Acad Echocardiogr Cardiovasc Imaging 2019;3:195-197


How to cite this URL:
Shah RK. Case of Takayasu Arteritis. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2019 [cited 2020 Feb 21 ];3:195-197
Available from: http://www.jiaecho.org/text.asp?2019/3/3/195/273308


Full Text

A 23-year-old female was referred for hypertension. She complained of occasional uneasiness and restlessness. She had a 2-year-old child with normal delivery. No history of preeclampsia or any history of hypertension was reported.

General examination revealed a high blood pressure of 210/110 mmHg in both the upper extremities and a feeble pulse in the lower extremities. She had no signs of heart failure. Her cardiovascular examination showed a systolic murmur all over the precordium, which was not conducted to the carotids. The carotids were well palpated. There was a bruit over the epigastrium. All other systems were normal.

Her X-ray showed mild cardiomegaly [Figure 1].{Figure 1}

2D echocardiography showed left ventricular hypertrophy, normal other chambers [Videos 1 and 2]. The aorta was normal, with aortic root being 30 mm and the ascending aorta being 32 mm [Figure 2]. The aortic valve was tricuspid with normal leaflets showing normal coaptation [Video 3].{Figure 2}

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Color Doppler showed no regurgitation or stenosis of any valves. The aortic root and arch and arch vessels appeared normal [Video 4]. In the subcostal examination thoracoabdominal aortic flow was blunted and not pulsatile as routinely seen [Videos 5 and 6], with a diastolic gradient of 6 mmHg [Figure 3], giving a clue to a proximal obstruction. Hence, computed tomography (CT) angiogram was recommended.{Figure 3}

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CT angiogram was done which showed mildly dilated aortic root, and ascending aorta, with multiple stenotic lesions all along the thoracic and abdominal aorta [Figure 4] and [Figure 5], particularly proximal to the renal arteries [Figure 6], explaining the cause of severe hypertension. The echocardiography is known to underestimate the aortic measurements as much as 20 mm, as compared to CT measurements explaining the lower aortic measurements obtained here.[1] This is also because in echo the measurements are in one orthogonal plane. The aorta was irregularly thickened at multiple places with normal arch arteries. There was significant luminal narrowing postrenal artery before the bifurcation.{Figure 4}{Figure 5}{Figure 6}

 Introduction



The disease gets its name after Professor Takayasu, an ophthalmologist at Kanazawa University, Japan. Takayasu arteritis is a well-known yet rare form of large-vessel vasculitis.[2] Takayasu arteritis is also known as pulseless disease, occlusive thromboaortopathy, and Martorell syndrome, wherein there is a chronic inflammatory arteritis affecting large vessels, predominantly the aorta and its main branches. The vessel inflammation leads to wall thickening, fibrosis, stenosis, and thrombus formation.[3] It has now been recognized worldwide in both sexes. The disease commonly presents in the 2nd or 3rd decade of life, often with a delay in diagnosis from the onset of first symptom, of months to years. Manifestations range from asymptomatic disease found as a result of impalpable pulses or bruits to catastrophic neurological impairment. A two-stage process has been suggested with a “prepulseless” phase characterized by nonspecific inflammatory features, followed by a chronic phase with the development of vascular insufficiency, in some cases accompanied by intermittent flares, although not all patients conform to this pattern.[4]

 Histopathology



Macroscopically, in the chronic phase, the aorta is thickened secondary to fibrosis of all three vessel layers. The lumen is narrowed in a patchy distribution, often affecting multiple areas. If disease progression is rapid, fibrosis can be inadequate with subsequent aneurysm formation. The intima may be ridged, with a “tree bark” appearance, a feature common to many aortitides.[5]

 Diagnosis



From the more typical features of Takayasu arteritis, the American College of Rheumatology defined specific diagnostic criteria for this disorder in 1990.[6] The different criteria considered were:

Age at the disease onset <40 yearsClaudication of extremitiesDecreased brachial artery pulseBlood pressure difference >10 mmHgBruit over subclavian arteries of aortaArteriogram abnormalities.

If at least three out of the six criteria were present, it was enough for the diagnosis of Takayasu arteritis.[6]

The differential diagnoses include other causes of large-vessel vasculitis as follows:

(1) Inflammatory aortitis (syphilis, tuberculosis, lupus, rheumatoid arthritis, spondyloarthropathies, Behcet's disease, Kawasaki disease, and giant-cell arteritis); (2) developmental abnormalities (coarctation of the aorta and Marfan's syndrome), and (3) other aortic pathologies, such as ergotism and neurofibromatosis.

Most of these have specific features that enable diagnosis, but tuberculosis has remained an important differential and possible etiological factor.[2]

 Classification



Angiographic

An attempt has been made to classify the disease on the basis of angiographic findings.[6] These findings are from Type 1 to Type I as follows:

Branches from the aortic archIIa Ascending aorta, aortic arch and its branchesIIb Ascending aorta, aortic arch and its branches, thoracic descending aortaType III Thoracic descending aorta, abdominal aorta and/or renal arteriesType IV Abdominal aorta and/or renal arteriesType V Combined features of types IIb and IV.

They have even extended the classification to coronary and pulmonary arteries, and if they are involved, they are represented as C(+) or P(+).

Type V is the most common finding, and Type IV is observed in India and Thailand but is very rare in the United States and Japan.[7] Hence, our case falls in the Type III variety, involving the descending thoracic and abdominal aorta with the renal arteries.

Clinical

Ishikawa defined clinical groups based on the natural history and complications of the disease. Four grades of disease are described [Table 1].[8] The four most important complications were defined as Takayasu retinopathy, secondary hypertension, aortic regurgitation, and aneurysm formation, each being graded as mild/moderate or severe at the time of diagnosis. The experience from India supports this classification for prognostic assessment.[9]{Table 1}

Treatment

Currently, the best evidence-based treatments include steroids, to which 50% respond, and methotrexate to which a further 50% respond.[2]

 Conclusion



The reason to present this case is that, in every case, the abdominal aorta should be examined with color flow mapping and interrogated by pulsed wave Doppler, so as not to miss such an important proximal obstructive condition, which may not be seen in other transthoracic views.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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