Takayasu arteritis

Background

  • Large-vessel vasculitis, leads to fibrosis and narrowing of the vessels [1]
  • Most often affects women of Asian descent during reproductive age
  • Diagnosis is often delayed due to the rarity of the disease and varied symptoms
  • The disease process chronically improves and relapses[2]

Clinical Features

Differential Diagnosis

Evaluation

An MRA showing occlusion of the left subclavian artery and significant narrowing of the left common carotid artery
  • Work up should be tailored to the patient's symptoms as this disease process affects multiple organ systems due to vascular damage

Laboratory

  • ESR, CRP, WBC may or may not be elevated
  • Anemia may be present

Imaging

  • Angiography revealing narrowing/occlusion of large vessels (e.g. Aorta) is the gold standard [3]
  • MRA or CTA can be used as well to appreciate vascular stenoses

Management

  • Oral Prednisone 1 mg/kg for the first month followed by a taper
    • Taper to approximately 10 mg/day
  • The addition of Methotrexate or Mycophenolate mofetil to Prednisone therapy may be more effective than monotherapy
  • Additional processes (e.g. Hypertension or Congestive Heart Failure) will require disease specific treatments
  • In advanced disease processes, Cardiac or Vascular Surgery or Interventional Radiology may be required for angioplasty, stenting, or valvular repair

Disposition

  • Disposition dependent on presentation and disease severity
  • Admit patients with severe aortic regurgitation, large aortic aneurysm, or other concerning features

References

  1. Hellmann, DB et al. Chapter 31. Takayasu Arteritis. In: Imboden JB, Hellmann DB, Stone JH. eds. CURRENT Diagnosis & Treatment: Rheumatology, 3e New York, NY: McGraw-Hill; 2013.
  2. Hellmann, DB et al. Rheumatologic, Immunologic, & Allergic Disorders. In: Papadakis MA, McPhee SJ, Rabow MW. eds. Current Medical Diagnosis & Treatment 2017 New York, NY: McGraw-Hill
  3. Lin, PH et al. Arterial Disease. In: Brunicardi F, Andersen DK, Billiar TR, Dunn DL, Hunter JG, Matthews JB, Pollock RE. eds. Schwartz's Principles of Surgery, 10e New York, NY: McGraw-Hill; 2015.

Authors:

Claire, Devin Smith