Takayasu Arteritis

Diagnosis

Indications for Testing

• Clinical symptoms suggestive of Takayasu arteritis

Criteria for Diagnosis

• American College of Rheumatology and Ishikawa's criteria for Takayasu arteritis (modified according to Sharma et al)

Laboratory Testing

• Nonspecific testing – not helpful in diagnosis
  • Urinalysis – negative for hematuria
  • CBC – may show elevated WBC
  • ESR/CRP – elevated
  • ANCA –  rule out other vasculitides that are typically ANCA-positive

Histology

• Inflammation, granulomas, and giant cells in large artery walls
• Pathology indistinguishable from giant cell arteritis

Imaging Studies

• MRI/US/CT angiography – narrowings, occlusions, or aneurysms of the aorta and its branches

Differential Diagnosis 

• Mycotic aneurysm
• Atherosclerosis
• Congenital – aortic coarctation
• Hereditary disorders
  • Ehlers-Danlos syndrome
  • Marfan syndrome
  • Loeys-Dietz syndrome
• Fibromuscular dysplasia
• Postradiation syndrome
• Chronic aortic aneurysm
• Chronic infection
  • Mycobacterium tuberculosis
  • Treponema pallidum

Clinical Background

Takayasu arteritis is a form of granulomatous arteritis that affects large-sized arteries, primarily the aorta and its major branches. It is most prevalent in adolescent girls and young women and is characterized by ocular disturbances and marked weakening of the pulses in the upper extremities. For this reason, it is also referred to as pulseless disease.

Epidemiology

• Incidence – 2-3/100,000 in U.S.
• Age – mean 20s
• Sex – F>M, 29:1 in U.S.
• Ethnicity – predominantly Asian

Pathophysiology

• Systemic large-vessel vasculitis characterized by adventitial thickening and cellular infiltration of the tunica media with local destruction of vascular smooth muscle cells and elastin
• Strong predilection for aorta, its branches, and pulmonary arteries
• Pathogenic T-lymphocyte response

Clinical Presentation

• Constitutional – fever, fatigue, malaise, weight loss
• Cardiovascular – hypertension, asymmetric blood pressure between limbs, diminished pulses, bruits (carotid most common)
• Musculoskeletal – arthralgias, myalgias, limb claudication
• Neurologic – syncope, lightheadedness, headache, visual disturbances

Treatment

• Corticosteroids – first-line therapy
• Immunosuppressants
• Antihypertensives
• Vascular interventions (eg, bypass procedure)

Indications for Laboratory Testing

• Tests generally appear in the order most useful for common clinical situations
• Click on number for test-specific information in the ARUP Laboratory Test Directory