Takayasu Arteritis

  • Diagnosis
  • Algorithms
  • Background
  • Lab Tests
  • References
  • Related Topics

Indications for Testing

  • Clinical symptoms suggestive of Takayasu arteritis
    • Asymmetric blood pressure
    • Decrease of brachial artery pulse
    • Bruit over subclavian arteries or aorta
    • Claudication of extremities
    • Blood pressure difference between arms

Criteria for Diagnosis

  • Ishikawa's criteria for Takayasu arteritis (modified according to Sharma, 1995)
    • Proposed modifications to criteria include
      • Removal of criterion of age <40 years
      • Inclusion of characteristic signs and symptoms as a major criterion
      • Removal of age in defining abdominal aortic lesion
      • Addition of coronary artery lesion in absence of risk factors

Laboratory Testing

  • Nonspecific testing –helpful in excluding other diagnoses or identifying organ dysfunction
    • Urinalysis – negative for hematuria
    • CBC – may show elevated WBC
    • C-reactive protein (CRP)
    • ANCA –  rule out ANCA-associated vasculitis

Histology

  • Inflammation, granulomas, and giant cells in large artery walls
  • Often indistinguishable from giant cell arteritis

Imaging Studies

  • MRA/CT angiography – narrowings, occlusions, or aneurysms of the aorta and its branches
    • US not as well established as diagnostic tool

Differential Diagnosis

  • Mycotic aneurysm
  • Atherosclerosis
  • Congenital – aortic coarctation
  • Hereditary disorders
    • Ehlers-Danlos syndrome
    • Marfan syndrome
    • Loeys-Dietz syndrome
  • Fibromuscular dysplasia
  • Postradiation syndrome
  • Chronic aortic aneurysm
  • Chronic infection

Takayasu arteritis is a form of granulomatous arteritis that affects primarily the aorta and its major branches. It is most prevalent in adolescent girls and young women and is characterized by ocular disturbances and marked weakening of the pulses in the upper extremities.  It is categorized as a large vessel vasculitis. (Chapel Hill 2012)

Epidemiology

  • Incidence – rare
  • Age – mean 20s
  • Sex – F>M, 29:1 in U.S.
  • Ethnicity – predominantly Asian

Pathophysiology

  • Large-vessel vasculitis characterized by adventitial thickening and cellular infiltration of the tunica media with local destruction of vascular smooth muscle cells and elastin
    • Strong predilection for aorta, its branches, and pulmonary arteries
  • Pathogenic T-lymphocyte response

Clinical Presentation

  • Constitutional – fever, fatigue, malaise, weight loss
  • Cardiovascular – hypertension, asymmetric blood pressure between limbs, diminished pulses, bruits (carotid most common)
  • Musculoskeletal – arthralgias, myalgias, limb claudication
  • Neurologic – syncope, lightheadedness, headache, visual disturbances
Tests generally appear in the order most useful for common clinical situations. Click on number for test-specific information in the ARUP Laboratory Test Directory.

C-Reactive Protein 0050180
Method: Quantitative Immunoturbidimetry

Anti-Neutrophil Cytoplasmic Antibody with Reflex to Titer and MPO/PR-3 Antibodies 2002068
Method: Semi-Quantitative Indirect Fluorescent Antibody/Semi-Quantitative Multiplex Bead Assay

Limitations 

Not specific for Takayasu arteritis

CBC with Platelet Count and Automated Differential 0040003
Method: Automated Cell Count/Differential

Urinalysis, Complete 0020350
Method: Reflectance Spectrophotometry/Microscopy

Guidelines

American Society for Clinical Pathology. Choosing Wisely - Five Things Physicians and Patients Should Question. An initiative of the ABIM Foundation. [Last revision Feb 2015; Accessed: Jan 2016]

Arend WP, Michel BA, Bloch DA, Hunder GG, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY, Lie JT, Lightfoot RW. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum. 1990; 33(8): 1129-34. PubMed

Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, Flores-Suarez LF, Gross WL, Guillevin L, Hagen EC, Hoffman GS, Jayne DR, Kallenberg CG M, Lamprecht P, Langford CA, Luqmani RA, Mahr AD, Matteson EL, Merkel PA, Ozen S, Pusey CD, Rasmussen N, Rees AJ, Scott DG I, Specks U, Stone JH, Takahashi K, Watts RA. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013; 65(1): 1-11. PubMed

General References

Alibaz-Oner F, Aydin SZehra, Direskeneli H. Advances in the diagnosis, assessment and outcome of Takayasu's arteritis. Clin Rheumatol. 2013; 32(5): 541-6. PubMed

Cakar N, Yalcinkaya F, Duzova A, Caliskan S, Sirin A, Oner A, Baskin E, Bek K, Soylu A, Fitoz S, Bayazit AKarabay, Bircan Z, Ozen S, Uncu N, Ekim M. Takayasu arteritis in children. J Rheumatol. 2008; 35(5): 913-9. PubMed

Chatterjee S, Flamm SD, Tan CD, Rodriguez R. Clinical diagnosis and management of large vessel vasculitis: Takayasu arteritis. Curr Cardiol Rep. 2014; 16(7): 499. PubMed

de Souza AWagner Sil, de Carvalho JFreire. Diagnostic and classification criteria of Takayasu arteritis. J Autoimmun. 2014; 48-49: 79-83. PubMed

Seko Y. Giant cell and Takayasu arteritis. Curr Opin Rheumatol. 2007; 19(1): 39-43. PubMed

Sharma BK, Siveski-Iliskovic N, Singal PK. Takayasu arteritis may be underdiagnosed in North America. Can J Cardiol. 1995; 11(4): 311-6. PubMed

Medical Reviewers

Last Update: August 2016