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KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • A severe inflammatory vascular disorder involving the aorta, its major branches, and the pulmonary artery

  • Predominantly found in Asian women < 40 years old

  • Evidence of diffuse vascular disease (coronary, cerebral, peripheral), especially involving the upper extremities (pulseless disease)

  • Elevated erythrocyte sedimentation rate

GENERAL CONSIDERATIONS

  • An uncommon vasculitis of the thoracic aorta and its major branches

  • Aneurysms and occlusions found

  • Half of those affected have a systemic illness

  • More common in Asian women aged 25–50 years

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • Fever, lymphadenopathy, arthralgias, or arthritis

  • Weakness of the arms or, less commonly, the legs

  • Chest or abdominal pain

  • Many are asymptomatic

PHYSICAL EXAM FINDINGS

  • Absent or diminished pulses in the upper extremities

DIFFERENTIAL DIAGNOSIS

  • Diffuse atherosclerosis

  • Other vasculitides (eg, Buerger’s disease)

DIAGNOSTIC EVALUATION

LABORATORY TESTS

  • Elevated erythrocyte sedimentation rate

IMAGING STUDIES

  • Magnetic resonance or CT angiography is usually diagnostic

DIAGNOSTIC PROCEDURES

  • Invasive angiography is the reference standard

TREATMENT

CARDIOLOGY REFERRAL

  • Suspected cardiac disease

  • Severe hypertension

HOSPITALIZATION CRITERIA

  • Acute coronary syndromes

MEDICATIONS

  • Corticosteroid: prednisone 5–60 mg PO daily

THERAPEUTIC PROCEDURES

  • Percutaneous transluminal revascularization procedures

SURGERY

  • Vascular surgery for occlusion not amenable to angioplasty

MONITORING

  • ECG monitoring in hospital as appropriate

DIET AND ACTIVITY

  • Restricted activity until disease controlled

ONGOING MANAGEMENT

HOSPITAL DISCHARGE CRITERIA

  • Resolution of problem

  • Successful revascularization

FOLLOW-UP

  • Depends on the severity and extent of disease

COMPLICATIONS

  • Hypertension due to renal artery involvement

  • Myocardial infarction

  • Stroke

  • Intestinal ischemia

  • Critical limb ischemia

PROGNOSIS

  • Generally good with treatment

  • Over 10 years, half of patients will have a relapse or a vascular complication

  • Mortality rate is low

RESOURCES

PRACTICE GUIDELINES

  • The diagnosis of this disease is difficult because the clinical presentation depends on the location and severity of the aortic branch lesions

  • Once the diagnosis is made, many respond well to anti-inflammatory therapy or revascularization

REFERENCE

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Comarmond  C, Biard  L, Lambert  M,  et al. Long-term outcomes and prognostic factors of complications in Takayasu arteritis: a multicenter study of 318 patients. Circulation. 2017;136:1114–1122.  [PubMed: 28701469]

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