1. Takayasu’s Disease Arteritis affecting primarily the aorta and its main branches –Leads to segmental stenosis, occlusion, dilatation, and aneurysm formation –Pathology shows intimal proliferation and fibrotic contraction of media and adventitia as well as granulomatous vasculitis Initially thought to affect only young Asian women in arch vessels First described in 1908 with regard to retinal central vessel changes Occurs more frequently in the Far East –1/3000 autopsy cases in Japan –2.6 cases/1 million incidence in U.S. –6.4 cases/1 million incidence in Sweden

2. Etiology UNKNOWN! –Known association with microorganisms and aortitis Reports with Tb (60% of autopsy cases) –HLA associations –Tendency to affect women of reproductive age –Autoimmune influences Common association with IBD, SLE, PMR, AS, RA Elevated gamma globulins, +RF

3. Clinical Features Females affected 7-8x more frequently Occurs in patients younger than 40 “Great Imitator” Phases of disease process –Early (systemic inflammatory) Fever, myalgias, arthralgias, weight loss, carotidynia HTN, vascular bruits, asymmetric arm BP, early ischemic symptoms –Late (occlusive) Ocular signs HTN (renal artery stenosis or aortic coarctation) Aortic insufficiency CHF/CAD Associated with cutaneous changes –Erythema nodosum & pyoderma gangrenosum

4. Classification Divided into subtypes based on location Type V most common (60-70%)

5. Diagnosis Based on clinical features and imaging studies obtained at routine intervals Duplex imaging –Screen for renal, mesenteric, carotid, subclavian, and axillary abnormalities Angiography –Narrowing of aorta or other major branches Can be short and segmental, or long and diffuse –Fusiform or saccular aneurysms

9. Therapy Relieve systemic manifestations and treat inflammatory process on vessels –Prednisone 1mg/kg/day for 1-3 mo, with a 6-12mo taper –Cyclophosphamide –methotrexate Identify and treat complications of the vascular disease –PTA of renal and iliac vessels Timing is controversial, as is use of stents Restenosis rates 15-20%

10. Surgical Therapy Cerebrovascular disease –Stroke, TIA, amarosis occur in 8-35% –Bypass recommended from ascending aorta Renovascular disease –HTN present in 20-72% –PTA, then consider renovascular reconstruction Mesenteric disease –Incidence of involvement ranges from 5-66% –Bypass for symptomatic patients Aneurysms –Incidence of aneurysm in Takayasu’s ranges from 22-32% Can be multiple, saccular or fusiform, associated with stenotic lesions Most commonly found in ascending aorta, thoracic, or abdominal aorta –Incidence of aneurysm rupture is low

11. Outcomes 50% later experience relapse of initial symptoms 94% 5 year survival Survival after surgery can be up to 20-30 years –Low likelihood of anastamotic problems Death usually related to vascular complications from HTN, AI, stroke