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Takayasu’s Disease

Explore the etiology, clinical features, diagnosis, therapy, surgical options, outcomes, and much more related to Takayasu's Disease, a rare type of arteritis primarily impacting the aorta and its branches.

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Takayasu’s Disease

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  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

  6. 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%

  7. 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

  8. 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

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