A Rare Variety of Takayasu Arteritis reported

Dr Souren Pal at District Hospital, Howrah, West Bengal and colleagues have reported a rare case of Takayasu Arteritis (TA) involving subclavian arteries, the aorta, superior mesenteric artery and renal arteries. The case has appeared in the Journal of Association of Physicians of India. The authors have reported this case as a rare combination of vascular lesions in a patient with a relatively rare variant of Takayasu Arteritis(TA).

Takayasu Arteritis(TA) is a chronic granulomatous panarteritis of large-sized arteries, classically involving the aortic arch, but one-third of the cases also affect the remainder of the aorta, its branches, and pulmonary arteries. We report a case of TA type V with extensive and varied involvement of the aorta and its branches that responded well to anti-tuberculosis drugs and steroids.

A 28-year-old unmarried non-diabetic, hypertensive lady, presented with the chief complaints of pain and numbness of both left limbs for four months and weakness of the same limbs along with low-grade fever for the last one month. She was on Amlodipine 5 mg daily past 2 months and had been exposed to TB in her family 8 years back.

The patient was cachectic (BMI 17.3 kg/m2) and febrile on admission. The pulse rate was 82/min and radial and brachial pulsations were well palpable on the right side alone. Similarly, femoral, popliteal and dorsalis pedis arteries were palpable on the right side but not on the left. However, both carotid arteries were well palpable. The BP was 160/90 mmHg over the right brachial artery and 176/100 mmHg over the right popliteal artery. A longitudinal tubular mass with expansile pulsations involving the umbilical and hypogastric areas was appreciated with audible bruit over it. The power of left upper and lower limb was 4/5 in both proximal and distal group of muscles with normal tone without sensory involvement and preserved deep tendon reflexes.

The clinical examination was otherwise unremarkable. A provisional diagnosis of large vessel vasculitis with abdominal aortic aneurysm was entertained and investigations sent accordingly.

CBC revealed normal WBC counts, Hb 8.8 gm/dL, a microcytic hypochromic picture and an ESR of 120 mm at the end of one hour. Chest X-ray suggested superior mediastinal widening. Abdominal USG showed abdominal aortic aneurysm involving the entire length of the abdominal aorta, extending through the left common iliac artery into the left external iliac artery. The maximum diameter of the aneurysm was 6cm and effective luminal diameter was 2cm. Kidney sizes were normal. The diameter of induration on Mantoux test was 40mm. Sputum was unavailable and the Echo-Doppler study documented LVH and grade I diastolic dysfunction. ANA was negative by the Hep2 method.

CT angiography of aorta and its branches showed diffuse mural thickening with wall irregularity in the left subclavian artery and left axillary artery, chronic total occlusion of 2nd and 3rd parts of the left subclavian artery and tight stenosis of the left axillary artery (Figure 1). Marked tortuosity was seen in whole abdominal and thoracic aorta. Fusiform aneurysmal dilatation with intramural haemorrhage and mural thrombus was seen in the lower descending thoracic and whole length of the abdominal aorta up to its bifurcation (Figures 2 and 3). There was total thrombotic occlusion of the left common iliac artery with reformation at its bifurcation (Figure 4). No dissection flap was seen. There was focal moderate stenosis near origin of coeliac axis and total occlusion at the origin of SMA but large good calibre IMA with collaterals. There was total occlusion of the left main renal artery at its origin and wall irregularity with a focal moderate stenosis in right main renal artery.

Courtesy Journal of Association of Physicians of India

Courtesy Journal of Association of Physicians of India

We entertained a diagnosis of Takayasu arteritis with extensive involvement of the aorta and its branches, with mural thrombus and aortic dissection, related to a Tuberculous etiology. Opinion from the Department of Cardio-thoracic and Vascular Surgery ruled out any operative intervention in such extensive vascular involvement. The patient was put on Category I anti-tubercular therapy along with oral prednisolone 40 mg daily for the initial 4 weeks, followed by tapering off over the next month. Fever subsided rapidly and the patient was put on physiotherapy and discharged after 3 weeks of hospitalisation. Six months later, there was substantial improvement of her left sided weakness with an appreciable increase in her claudication distance but no return of left sided pulses. The ESR at this time was 19 mm at the end of 1 hour.

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