Takayasu arteritis: Diagnosis of a rare clinical entity: A case report

Takayasu arteritis is a rare, autoimmune, granulomatous inflammatory vascular disease of the aorta and its main branches. Delayed diagnosis was attributed to vague early clinical features, lack of specific diagnostic marker, rarity of disease, and lack of awareness by physicians of the disease condition.

A 28-year-old woman presented with sudden onset right sided body weakness associated with transient loss of consciousness, left-sided facial deviation, and slurring of speech. She had history of limb claudication. She was told to have rheumatic regurgitant aortic valve disease 5 years back. Physical examination revealed elevated blood pressure, reduced left radial artery pulse, faintly palpable right dorsalis pedis and posterior tibial arteries pulse, blood pressure asymmetry in arms, early diastolic murmur at erb's area, and right-sided hemiparesis with ipsilateral supra-nuclear facial palsy. Laboratory markers revealed high erythrocyte sedimentation rate. Two dimensional-transthoracic echocardiography and neck Doppler ultrasound revealed aortic regurgitation, and stenosed and thrombosed left common carotid artery, respectively. Post-contrast computed tomography showed thickened, occluded and thrombosed left common carotid artery, stenosed right common carotid artery, and thickened, stenosed and calcified descending thoracic aorta and abdominal aorta. Brain magnetic resonance imaging showed left basal ganglia infarction. Diagnosis of thromboembolic stroke secondary to Takayasu arteritis was made.

Diagnosis of Takayasu arteritis was settled using modified Ishikawa Diagnostic Criteria and American College of Rheumatology Classification Criteria.

She was started on glucocorticoids 50 mg po daily for 3 months and tapered by 5 mg po weekly over 3 months, azathioprine 100 mg po daily, aspirin 81 mg po daily, atorvastatin 40 mg po daily, amlodipine 10 mg po daily, and cotrimoxazole 960 mg po trice weekly. Physical therapy was continued.

The patient showed marked symptomatic improvement after 3 months of treatment. She was referred to higher health institution for evaluation of vascular intervention.

High index of clinical suspicion is required for early diagnosis of rare diseases to prevent adverse outcomes.