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Takayasu Arteritis

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Takayasu Arteritis, Young Female Arteritis, Occlusive Thromboarteriopathy, Martorell Syndrome, Raeder-Harbitz Syndrome, Reverse Coarctation, Middle Aortic Syndrome, Idiopathic Medial Aortopathy and Arteriopathy

  • Epidemiology
  1. Age: 30-40 years old
  2. Gender: Women account for up to 90% of cases
  3. Race: More common in asian descent
  • Pathophysiology
  1. Large vessel Vasculitis
  2. Granulomatous inflammation of the aorta and its branches
  • 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
    4. Skin lesions similar to Erythema Nodosum or Pyoderma Gangrenosum
  3. Focal symptoms
    1. Related to specific arterial involvement
    2. Example: Carotid Artery involvement with CNS symptoms
  • Signs
  1. Hypotension
  2. Upper extremities with diminished Arterial Pulses
  3. Visual disturbances
  4. Neurologic deficit
  • 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
  3. Biopsy of vascular tissue
    1. Cytotoxic Lymphocyte infiltration (esp. gamma delta T Lymphocytes)
  • Imaging
  • CT angiogram, MRA Angiogram or Standard Angiography of arterial tree
  1. Smoothly tapering vessel lumens or Occlusion
  2. Vessel wall thickening
  • Differential Diagnosis
  1. See Vasculitis
  2. Fibromuscular dysplasia
  3. Ergotamine toxicity
  4. Ehlers-Danlos Syndrome
  5. Temporal Arteritis
  • Management
  1. High dose Corticosteroids (e.g. Prednisone 40-60 mg orally daily)
  2. Monitoring for response with acute phase reactants and imaging
  • References
  1. Hunder (2011) in UpToDate, Clinical features and diagnosis of Takayasu Arteritis, Accessed 11/30/11
  2. Serra (2016) Ann Vasc Surg 35:210-25 +PMID:27238990 [PubMed]