Takayasu Arteritis

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

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II. Epidemiology

  1. Age: 20 to 40 years old
  2. Gender: Women account for up to 90% of cases
  3. Race: More common in asian descent

III. Pathophysiology

  1. Rare, large vessel Vasculitis
  2. Granulomatous inflammation of the aorta and its main branches

IV. Symptoms

  1. Phase 1: Inflammatory Response (Early in course)
    1. Fatigue
    2. Weight loss
    3. Low-grade fever
    4. Night Sweats
    5. Arthralgias
    6. Myalgias
  2. Phase 2: Vascular Insufficiency (Later in course)
    1. Finger coldness and numbness
    2. Skin lesions similar to Erythema Nodosum or Pyoderma Gangrenosum
    3. Focal symptoms are related to specific arterial involvement
      1. Carotid Artery involvement with CNS symptoms
      2. Abdominal aorta with lower extremity Claudication

V. Signs

  1. Hypertension
  2. Diminished Arterial Pulses (may start in upper extremities)
  3. Visual disturbances
  4. Neurologic deficit
  5. Blood Pressure discrepancy (e.g. decreased in lower extremities)
  6. Vascular Bruits

VI. Diagnosis: ACR Criteria (three of six present for diagnosis)

  1. Age at disease onset <40 years old
  2. Extremity Claudication
  3. Decreased brachial artery pulse
  4. Blood Pressure difference between arms >10 mmHg
  5. Subclavian Artery or Aortic Bruit
  6. Abnormal arteriogram

VII. 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)

VIII. Imaging: Angiography

  1. CT angiogram or the chest AbdomenPelvis (aortic survey)
    1. Alternatively, MRA Angiogram or Standard Angiography of arterial tree may be performed instead
    2. Findings
      1. Smoothly tapering vessel lumens or Occlusion
      2. Vessel wall thickening
  2. Doppler Ultrasound
    1. May identify vessel wall inflammation

IX. Differential Diagnosis

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

X. Management

  1. Monitoring for response with acute phase reactants and imaging
  2. High dose Corticosteroids (e.g. Prednisone 40-60 mg orally daily)
    1. Response to Corticosteroids in only 50% of patients
  3. Immunosuppressants (e.g. Cyclophosphamide, Azathioprine, Methotrexate)
    1. Typically administered in combination with Corticosteroids
  4. Hypertension Management
  5. Surgery Indications (for disease refractory to Corticosteroids and Immunosuppressants)
    1. Hypertension with critical Renal Artery Stenosis
    2. Extremity Claudication limiting Activities of Daily Living
    3. Cerebrovascular ischemia or critical stenosis of three of more Cerebral Vessels
    4. Moderate Aortic Regurgitation
    5. Cardiac ischemia with confirmed Coronary Artery involvement

XI. Complications

  1. Cardiovascular and systemic Hypertension complications
    1. Arterial aneurysm or dissection
    2. Dilated Cardiomyopathy
    3. Aortic Valve Insufficiency
    4. Congestive Heart Failure
  2. Neurologic
    1. Cerebrovascular Accident
    2. Seizure Disorder
    3. Intracranial Aneurysm
    4. Encephalitis
  3. Gastrointestinal
    1. Mesenteric Iscehmia
  4. Ocular
    1. Retinal ischemia
    2. Retinal Detachment

XII. References

  1. Hunder (2011) in UpToDate, Clinical features and diagnosis of Takayasu Arteritis, Accessed 11/30/11
  2. Mezan (2022) Crit Dec Emerg Med 36(8): 10-2
  3. Natraj Setty (2017) J Rare Dis Res Treat 2(2): 63-8
    1. https://www.rarediseasesjournal.com/articles/takayasus-arteritis--a-comprehensive-review.pdf
  4. Russo (2018) Front Pediatr 6:265 +PMID: 30338248 [PubMed]
  5. Serra (2016) Ann Vasc Surg 35:210-25 +PMID:27238990 [PubMed]

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