A Case Report on Takayasu’s arteritis associated Aortic Regurgitation

 

Dhivya K*, Sonia B, Gulshan E Shabha, Leelavathi T

Department of Pharmacy Practice and Pharm-D, Sri Venkateswara College of Pharmacy, Chittoor- 517127

 

ABSTRACT:

Takayasu arteritis (TA), a rare large vessel vasculitis that affects aorta and its major branches. This report describes a 27 year-old woman who presented with dyspnoea on exertion and leg edema. Echocardiography revealed increased left ventricular volume and severe aortic regurgitation (AR).  Aortic valve replacement (AVR) was performed. Histological study and other postoperative examinations revealed Takayasu’s arteritis (TA). Patient was treated with steroid therapy.

 

 

 

INTRODUCTION:

Takayasu arteritis (TA), an auto-immune, idiopathic, large vessel vasculitis of unknown etiology that causes inflammation of the main blood vessel (aorta and its primary branches) which carries blood from the heart to the rest of the body that results in narrowing, stenosis, occlusion, dilatation, or aneurysm of the involved arteries. TA predominantly affects young females and has been reported to occur in all races and ethnicities ^{[1, 2]}. It is otherwise called as Pulse less disease, occlusive thromboaortopathy, and Martorell syndrome ^[3]. Clinical features range from asymptomatic disease found as a result of impalpable pulses or bruits to catastrophic neurological impairment^[4]. AR is a complication in 13-25% of TA patients and is considered as an important risk factor for mortality. The management of TA is challenging because treatment options varies depends on the stage of the disease at the time of its diagnosis. Steroids have formed the mainstay of treatment for TA ^[5]. Here, we report a case of 27-year old woman who was diagnosed with TA and aortic regurgitation.

 

CASE REPORT:

A 27- year old woman was brought to the hospital with complaints of dyspnoea on exertion and chest pain. Her blood pressure was 134/ 54 mmHg.

 

Laboratory findings showed an elevated erythrocyte sedimentation rate (ESR) of 65 mm/h (normal value <20mm/ h) and serum C-reactive protein level of 3.13 mg/dl (normal value <0.5 mg/dl). Results of different laboratory tests were normal. Echocardiography revealed increased left ventricular volume with mildly impaired wall motion and severe AR with aortic cusps thickening and motion limitation. AVR was planned and performed. Histological study showed an infiltration of lymphocyte, plasma cells and multinucleated giant cells with granulomatous change in the ascending aorta and fibrous thickening of aortic cusps. Gadolinium-enhanced magnetic resonance imaging (MRI) performed postoperatively revealed that ascending aorta was mildly dilated up to 31mm. Fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) depicted positive uptake in the ascending aorta. On the basis of these findings, the diagnosis of TA was made and treated her with oral prednisolone 0.5 mg/kg /day. Within 4 weeks after starting steroid therapy, her CRP and ESR values were normalized. Four months after AVR, echocardiography revealed normal left ventricular volume and ejection fraction without any valve complications.

 

DISCUSSION:

Takayasu arteritis is an uncommon disorder which affects multiple organs. Patients with TA may have no symptoms and often delays in detecting TA. This condition was first reported by a Japanese ophthalmologist, Mikito Takayasu in 1908^{[6, 7]}.  Criteria for the classification of TA were set by the American College of Rheumatology in 1990, which includes: (i) age at disease onset <40 years; (ii) claudication of extremities; (iii) decreased brachial artery pulse; (iv) systolic blood pressure difference >10 mmHg; (v) bruit over subclavian arteries or aorta; and (vi) arteriogram abnormality^[8]. Our patient revealed none of these criteria, demonstrated only clinical inflammatory signs. MRI and FDG PET/CT showed inflammatory change in the ascending aorta which is useful for the diagnosis of TA. AR is a potentially fatal complication of TA and has been reported in 25% of cases^[9]. Surgical intervention with aortic valve replacement may become necessary for severe AR patients but it is complicated by fragile and inflamed tissue, which may lead to valve detachment or anastomotic aneurysm formation. Hence, surgery is preferably delayed until reduction of vascular inflammation which is achieved with immunosuppressive therapy to avoid complications after AVR ^{[10, 11]}. TA often needs treatment to prevent further narrowing of affected arteries. Persisting damage to arteries sometimes needs a vascular procedure or surgical treatment which includes angioplasty (widening a narrowed or blocked blood vessel) with or without placement of a stent and other option is bypass grafting (a surgery to redirect blood flow around a blockage in a blood vessel). The disease can recur after treatment or can silently get worse. It is often very hard to know whether TA is active again^{[12, 13]}. A variety of clinical manifestations sometimes make diagnosis difficult, it is significant to give a correct diagnosis in TA patients with AR. So, patients need frequent doctor visits and angiograms. In addition, clinical pharmacy professionals could improve the quality of treatment both in terms of safety and efficacy by providing therapeutic interventions such as dose titration in case of steroids, safer alternatives and non-therapeutic interventions such as patient education and psychological support.

 

ACKNOWLEDGEMENT:

The authors are thankful to the management of Sri Venkateswara College of Pharmacy for providing excellent research guidance and support.

 

REFERENCES

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10.  Matsuura K, Ogino H, Kobayashi J, Ishibashi-Ueda H, Matsuda H, Minatoya K, et al. Surgical treatment of aortic regurgitation due to Takayasu arteritis: long-term morbidity and mortality. Circulation 2005; 112:3707–12.

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13.  Fukui S, Ichinose K, Tsuji S, Umeda M, Nishino A, Nakashima Y et al. Hypocholesterolemia predicts relapses in patients with Takayasu arteritis. Mod Rheumatol. 2016; 26(3):415-420.

 

 

 

Accepted on 20.03.2017           © RJPT All right reserved