Takayasu’s DiseaseTakayasu’s DiseaseArteritis affecting primarily the aorta and its main Arteritis affecting primarily the aorta and its main branchesbranches– Leads to segmental stenosis, occlusion, dilatation, and Leads to segmental stenosis, occlusion, dilatation, and

aneurysm formationaneurysm formation– Pathology shows intimal proliferation and fibrotic contraction of Pathology shows intimal proliferation and fibrotic contraction of

media and adventitia as well as granulomatous vasculitismedia and adventitia as well as granulomatous vasculitis

Initially thought to affect only young Asian women in arch Initially thought to affect only young Asian women in arch vesselsvesselsFirst described in 1908 with regard to retinal central First described in 1908 with regard to retinal central vessel changesvessel changesOccurs more frequently in the Far EastOccurs more frequently in the Far East– 1/3000 autopsy cases in Japan1/3000 autopsy cases in Japan– 2.6 cases/1 million incidence in U.S.2.6 cases/1 million incidence in U.S.– 6.4 cases/1 million incidence in Sweden6.4 cases/1 million incidence in Sweden

EtiologyEtiology

UNKNOWN!UNKNOWN!– Known association with microorganisms and Known association with microorganisms and

aortitisaortitisReports with Tb (60% of autopsy cases)Reports with Tb (60% of autopsy cases)

– HLA associationsHLA associations– Tendency to affect women of reproductive Tendency to affect women of reproductive

ageage– Autoimmune influencesAutoimmune influences

Common association with IBD, SLE, PMR, AS, RACommon association with IBD, SLE, PMR, AS, RAElevated gamma globulins, +RFElevated gamma globulins, +RF

Clinical FeaturesClinical Features

Females affected 7-8x more frequentlyFemales affected 7-8x more frequentlyOccurs in patients younger than 40Occurs in patients younger than 40““Great Imitator”Great Imitator”Phases of disease processPhases of disease process– Early (systemic inflammatory)Early (systemic inflammatory)

Fever, myalgias, arthralgias, weight loss, carotidyniaFever, myalgias, arthralgias, weight loss, carotidyniaHTN, vascular bruits, asymmetric arm BP, early ischemic symptomsHTN, vascular bruits, asymmetric arm BP, early ischemic symptoms

– Late (occlusive)Late (occlusive)Ocular signsOcular signsHTN (renal artery stenosis or aortic coarctation)HTN (renal artery stenosis or aortic coarctation)Aortic insufficiencyAortic insufficiencyCHF/CADCHF/CAD

Associated with cutaneous changesAssociated with cutaneous changes– Erythema nodosum & pyoderma gangrenosumErythema nodosum & pyoderma gangrenosum

ClassificationClassification

Divided into subtypes based on locationDivided into subtypes based on location

Type V most common (60-70%)Type V most common (60-70%)

DiagnosisDiagnosis

Based on clinical features and imaging Based on clinical features and imaging studies obtained at routine intervalsstudies obtained at routine intervalsDuplex imagingDuplex imaging– Screen for renal, mesenteric, carotid, Screen for renal, mesenteric, carotid,

subclavian, and axillary abnormalitiessubclavian, and axillary abnormalities

AngiographyAngiography– Narrowing of aorta or other major branchesNarrowing of aorta or other major branches

Can be short and segmental, or long and diffuseCan be short and segmental, or long and diffuse

– Fusiform or saccular aneurysmsFusiform or saccular aneurysms

TherapyTherapy

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

tapertaper– CyclophosphamideCyclophosphamide– methotrexatemethotrexate

Identify and treat complications of the vascular Identify and treat complications of the vascular diseasedisease– PTA of renal and iliac vesselsPTA of renal and iliac vessels

Timing is controversial, as is use of stentsTiming is controversial, as is use of stentsRestenosis rates 15-20%Restenosis rates 15-20%

Surgical TherapySurgical TherapyCerebrovascular diseaseCerebrovascular disease– Stroke, TIA, amarosis occur in 8-35%Stroke, TIA, amarosis occur in 8-35%– Bypass recommended from ascending aortaBypass recommended from ascending aorta

Renovascular diseaseRenovascular disease– HTN present in 20-72%HTN present in 20-72%– PTA, then consider renovascular reconstructionPTA, then consider renovascular reconstruction

Mesenteric diseaseMesenteric disease– Incidence of involvement ranges from 5-66%Incidence of involvement ranges from 5-66%– Bypass for symptomatic patientsBypass for symptomatic patients

AneurysmsAneurysms– Incidence of aneurysm in Takayasu’s ranges from 22-32%Incidence of aneurysm in Takayasu’s ranges from 22-32%

Can be multiple, saccular or fusiform, associated with stenotic Can be multiple, saccular or fusiform, associated with stenotic lesionslesionsMost commonly found in ascending aorta, thoracic, or abdominal Most commonly found in ascending aorta, thoracic, or abdominal aortaaorta

– Incidence of aneurysm rupture is lowIncidence of aneurysm rupture is low

OutcomesOutcomes

50% later experience relapse of initial 50% later experience relapse of initial symptomssymptoms

94% 5 year survival94% 5 year survival

Survival after surgery can be up to 20-30 Survival after surgery can be up to 20-30 yearsyears– Low likelihood of anastamotic problemsLow likelihood of anastamotic problems

Death usually related to vascular Death usually related to vascular complications from HTN, AI, strokecomplications from HTN, AI, stroke