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Takayasus Disease

Arteritis affecting primarily the aorta and its mainbranches Leads to segmental stenosis, occlusion, dilatation, and

aneurysm formation

Pathology shows intimal proliferation and fibrotic contraction of

media and adventitia as well as granulomatous vasculitisInitially thought to affect only young Asian women in archvessels

First described in 1908 with regard to retinal centralvessel 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

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Etiology

UNKNOWN!

Known association with microorganisms andaortitis

Reports with Tb (60% of autopsy cases) HLA associations

Tendency to affect women of reproductiveage

Autoimmune influencesCommon association with IBD, SLE, PMR, AS, RA

Elevated gamma globulins, +RF

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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

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Classification

Divided into subtypes based on location

Type V most common (60-70%)

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Diagnosis

Based on clinical features and imagingstudies obtained at routine intervals

Duplex imaging

Screen for renal, mesenteric, carotid,subclavian, and axillary abnormalities

Angiography

Narrowing of aorta or other major branchesCan be short and segmental, or long and diffuse

Fusiform or saccular aneurysms

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Therapy

Relieve systemic manifestations and treatinflammatory process on vessels Prednisone 1mg/kg/day for 1-3 mo, with a 6-12mo

taper

Cyclophosphamide

methotrexate

Identify and treat complications of the vasculardisease PTA of renal and iliac vessels

Timing is controversial, as is use of stents

Restenosis rates 15-20%

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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 reconstructionMesenteric disease Incidence of involvement ranges from 5-66%

Bypass for symptomatic patients

Aneurysms

Incidence of aneurysm in Takayasus ranges from 22-32%Can be multiple, saccular or fusiform, associated with stenoticlesions

Most commonly found in ascending aorta, thoracic, or abdominalaorta

Incidence of aneurysm rupture is low

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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