Large Vessel Vasculitis

Takayasu Arteritis


  • Granulomatous inflammation of the aorta, its major branches, and the pulmonary arteries, leading to fibrosis and luminal stenosis or aneurysm formation. Triggering mechanism remains unknown (1)

Presentation (2)

  • Typically, women < 40 years old. Early, nonspecific symptoms include headaches, fever, weight loss, myalgias, and arthralgias (systemic phase)
  • Later symptoms include hypertension, discriminatory pulses in limbs or carotid arteries, pain with use of limb, and visual defects (obstructive phase)

Diagnosis (3)

  • ESR and CRP: Elevated
  • ANCA: Negative
  • MRA/CTA (Magnetic resonance angiography/computed tomography angiography): Vascular narrowings and/or occlusions
  • Biopsy: Granulomatous inflammation in large-vessel walls (histopathologically indistinguishable from Giant Cell Arteritis)

Treatment (4,5)

  • Remission induction: Glucocorticoids. Recommended initial treatment prednisone/prednisolone 1 mg/kg/day, tapered over a number of months once symptoms controlled
    • Conventional immunosuppression (Methotrexate, azathioprine, leflunomide, and MMF) can be started during initiation of steroids or while tapering
      • If ineffective: Biologic agents (Anti-TNF-α agents, tocilizumab) may be useful
  • Revascularization to address critical, symptomatic stenoses, during inactive disease if possible

Giant Cell Arteritis


  • Granulomatous inflammation of large- and medium-sized vessels, particularly the carotid, vertebral, and temporal arteries. Initial triggering mechanism has not been well-identified. Likely related to multiple factors, including genetics, environment, and age-related changes (6)

Presentation (7)

  • Typically, women > 50 years old
  • Headache, jaw/tongue claudication, loss of vision in one eye, as well as “B” symptoms (fever, myalgia, weight loss, anorexia, fatigue)
  • Polymyalgia rheumatica

Diagnosis (8)

  • American College of Rheumatology (ACR) diagnostic criteria: Patient should meet 3 of the following:
    • New, localized headache
    • Tenderness or decreased pulse in the temporal artery
    • ESR ≥ 50 mm/h
    • Temporal artery biopsy showing mononuclear cell infiltrates or a granulomatous process with multinucleate giant cells


  • Glucocorticoids should be started immediately (prednisolone 1 mg/kg body weight per day (max. 60 mg) has been suggested by EULAR), tapered over several months after control of disease (9)
  • Tocilizumab, abatacept are currently under investigation as steroid-sparing agents (10)


  1. Espinoza, J. L., Ai, S., & Matsumura, I. (2018, September 6). New insights on the pathogenesis of takayasu arteritis: Revisiting the microbial theory. Pathogens (Basel, Switzerland). Retrieved December 1, 2021, from 
  2. Takayasu’s arteritis. Johns Hopkins Vasculitis Center. (2012, September 18). Retrieved December 1, 2021, from 
  3. Genzen, J. R., & Tebo, A. E. (n.d.). Takayasu arteritis. Takayasu Arteritis | Choose the Right Test. Retrieved December 1, 2021, from 
  4. Keser, G., Direskeneli, H., & Aksu, K. (2013, October 4). Management of takayasu arteritis: A systematic review. OUP Academic. Retrieved December 1, 2021, from 
  5. Hellmich, B., Agueda, A., Monti, S., Buttgereit, F., Boysson, H. de, Brouwer, E., Cassie, R., Cid, M. C., Dasgupta, B., Dejaco, C., Hatemi, G., Hollinger, N., Mahr, A., Mollan, S. P., Mukhtyar, C., Ponte, C., Salvarani, C., Sivakumar, R., Tian, X., … Luqmani, R. A. (2020, January 1). 2018 update of the EULAR recommendations for the management of large vessel vasculitis. Annals of the Rheumatic Diseases. Retrieved December 1, 2021, from 
  6. Terrades-Garcia, N., & Cid, M. C. (2018, February 23). Pathogenesis of giant-cell arteritis: How targeted therapies are influencing our understanding of the mechanisms involved. OUP Academic. Retrieved December 3, 2021, from 
  7. Zwicker, J., Atkins, E. J., Lum, C., & Sharma, M. (2011, March 22). An atypical presentation of giant cell arteritis. CMAJ : Canadian Medical Association journal = journal de l’Association medicale canadienne. Retrieved December 3, 2021, from 
  8. Hunder GG;Bloch DA;Michel BA;Stevens MB;Arend WP;Calabrese LH;Edworthy SM;Fauci AS;Leavitt RY;Lie JT; (n.d.). The American College of Rheumatology 1990 criteria for the classification of Giant Cell Arteritis. Arthritis and rheumatism. Retrieved December 3, 2021, from 
  9. Ness, T., Bley, T. A., Schmidt, W. A., & Lamprecht, P. (2013, May). The diagnosis and treatment of giant cell arteritis. Deutsches Arzteblatt international. Retrieved December 3, 2021, from 
  10. Ponte, C., Rodrigues, A. F., O’Neill, L., & Luqmani, R. A. (2015, June 16). Giant cell arteritis: Current treatment and management. World journal of clinical cases. Retrieved December 3, 2021, from 

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