II. Epidemiology

  1. Age: 30-40 years old
  2. Gender: Women account for up to 90% of cases
  3. Race: More common in asian descent

III. Pathophysiology

  1. Large vessel Vasculitis
  2. Granulomatous inflammation of the aorta and its branches

IV. Symptoms

  1. Early in course
    1. Fatigue
    2. Weight loss
    3. Low-grade fever
  2. Later in course
    1. Finger coldness and numbness
    2. Arthralgias
    3. Myalgias
  3. Focal symptoms
    1. Related to specific arterial involvement
    2. Example: Carotid Artery involvement with CNS symptoms

V. Signs

  1. Hypotension
  2. Upper extremities with diminished Arterial Pulses
  3. Visual disturbances
  4. Neurologic deficit

VI. Labs

  1. Complete Blood Count (CBC)
    1. Normochromic Normocytic Anemia
  2. Increased acute phase reactants
    1. Increased C-Reactive Protein (C-RP)
    2. Increased Erythrocyte Sedimentation Rate (ESR)
    3. Pentraxin-3

VII. Imaging: CT angiogram, MRA Angiogram or Standard Angiography of arterial tree

  1. Smoothly tapering vessel lumens or Occlusion
  2. Vessel wall thickening

VIII. Differential Diagnosis

  1. See Vasculitis
  2. Fibromuscular dysplasia
  3. Ergotamine toxicity
  4. Ehlers-Danlos Syndrome
  5. Temporal Arteritis

IX. Management

  1. High dose Corticosteroids (e.g. Prednisone 40-60 mg orally daily)
  2. Monitoring for response with acute phase reactants and imaging

X. References

  1. Hunder (2011) in UpToDate, Clinical features and diagnosis of Takayasu Arteritis, Accessed 11/30/11

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