A Case of a 48 year old female who presented with acute aortic occlusion in the setting of takayasu arteritis /
Lydeleth B. Pagdagdagan.
- Fairview, Quezon City Department of Medicine, FEU-NRMF, 2018
- illustrations and photos (black and white); (in folder)
Includes bibliographical references.
ABSTRACT: Takayasu arteritis is a rare, chronic large vessel vasculitis of known etiology which predominantly effects women younger than 40 years of age. Symptoms are highly variable based on the location and extent of the stenosis, arterial occlusion, aneurysm, and thrombosis. In patient with Takayasu's arteritis, platelet and coagulation activities are significantly increased, leading to hypercoagulable state and thrombus formation.. It causes extend narrowing of the aorta with symptoms of acute inflammation, aortic coarctation affecting the cardiovascular system. It is therefore considered to be important to perform aggressive immunosuppressive therapy and early revascularization to prevent symptoms and signs related to vascular stenosis and/ occlusion. Takayasu can be life-threatening by an occlusion of the ascending aorta and it's major branches, without any coronary arteries involvement. Acute Aortic Occlusion is rare, emergent vascular event which can arise secondary to the intimal fibrosis, thrombosis and calcifications of Takayasu Arteritis. Apart from constitutional complaints, nearly all patients with TA sooner or later present with symptoms and signs related to vascular stenosis and/or occlusion. They include hypertension with blood pressure differences in the extremities, pulse deficits, bruit, and upper and/or lower extremity claudications. Diagnosis is based on clinical presentation, relevant laboratory work-up, and imaging findings of wall thickening and stenosis of medium and large vessels. Management includes glucocorticoid therapy, frequently paired with adjunctive immunosuppressants, and sometimes surgical intervention in severe cases. Here, we present a unique case of Takayasu arteritis presenting with right upper extremity swelling, with severe narrowing of the brachiocephalic branch and left subclavian artery, near complete occlusion of the left common carotid artery, calcified proximal portions of the aortic arch branches and extensive calcification causing near complete narrowing seen from descending thoracic aortic down to the proximal abdominal aorta (before take off of celiac trunk. Based on our literature review, our article presents a very rare presentation of Acute aortic occlusion secondary from extensive aortic calcifications in Takayasu Arteritis patient .