Takayasu arteritis presenting as renovascular hypertension with hypertensive encephalopathy

Takayasu Arteritis (TA) is a type of chronic granulomatous vasculitis of unknown cause (majority of authors agree on its autoimmue etiology). It has worldwide distribution, with the greatest prevalence among Asians. TA commonly manifest with various constitutional symptoms like fever, bodyache, weakness but sometimes it remains undiagnosed till late and manifest primarily with uncommon presentations like hypertensive encephalopathy. This report is representing such an uncommon presentation of a rare disease.


INTRODUCTION
Takayasu Arteritis (TA) is a type of chronic granulomatous vasculitis of unknown cause (majority of authors agree on its autoimmue etiology).It has worldwide distribution, with the greatest prevalence among Asians.TA commonly manifest with various constitutional symptoms like fever, bodyache, weakness but sometimes it remains undiagnosed till late and manifest primarily with uncommon presentations like hypertensive encephalopathy.This report is representing such an uncommon presentation of a rare disease.

CASE REPORT
A 42 years old lady presented in the emergency department with complaints of headache, dizziness, confusion, and blurring of vision for 5 days.There was history of nonspecific complaints like bodyache, generalized body weakness, fatigue, low energy and difficulty in doing daily routine activities for last 6 months.There was no history of loss of consciousness/ abnormal body movements/ joint pain/weight loss/fever/ anorexia/ cough with or without expectoration.Patient had never been detected as having high blood pressure before this.
On examination patient was found to have high blood pressure As patient presented with BP and pulse discrepancies in both upper limbs in the background of severe hypertension, so we initially thought that patient might have large vessel vasculitis possibly involving branches of aortic arch and renal arteries.

Takayasu arteritis presenting as renovascular hypertension with hypertensive encephalopathy
Vikram Singh Tanwar,1* Anjali Saini,2 Anurag Ambroz Singh, 1 Rakesh Tank, 1 page 36  In our patient aortic arch with its branches, abdominal aorta, renal artery and iliac arteries are involved which put her into type 5 TA.There was extensive involvement of medium to large vessels along with elevated acute phase reactant suggesting very high disease activity so we started medical therapy in term of prednisolone and methotrexate in combination with antihypertensive agents and the response comes out to be very good.Her symptoms were improved; BP was controlled and absent pulses was reappeared after one month of therapy.

CONCLUSION
Primarily the report shows the uncommon presentation of a rare disease like takayasu arteritis.Secondly, in the presence of new onset hypertension or hypertensive emergency with pulse and BP discrepancies one should always suspect any vasculitic condition.In the presence of active ongoing inflammatory process the timely started immunosuppressive therapy has very good result and better prognosis.
i.e. 210/130 mmHg (measured in right arm taken in supine position) but in left arm BP was not recordable.Pulse (radial and brachial) was not palpable in left upper limb.Pulse was well palpable in right upper limbs and both lower limbs.No other abnormal clinical finding was detected on cardiovascular, respiratory and neurological examination.In the emergency, first high BP was managed with antihypertensive agents which lowered down to 150/90 mmHg and then MRI brain was done that showed slight parenchymal enhancement in the occipital areas and both cerebral hemispheres suggestive of hypertensive changes.Patient was admitted with provisional diagnosis of hypertensive encephalopathy and further workup was planned to find out the secondary cause of hypertension.
So we went for CT angiography (CTA) for both thoracic aorta and abdominal aorta and their branches.CTA thoracic aorta revealed the multiple areas of intimal thickening of aortic arch and its branches; irregularity with stenosis at the origin of left common carotid arteries, and occlusion of the left subclavian artery.CTA of abdominal aorta and its branches revealed right renal artery stenosis, diffuse intimal thickening with dilation and narrowing of abdominal aorta.

Figure 1 :
Figure 1: CTA of thoracic aorta revealing the multiple areas of intimal thickening of aortic arch and its branches; irregularity with stenosis at the origin of left common carotid arteries, and occlusion of the left subclavian artery