Giant Cell Arteritis - Temporal Arteritis

Giant cell arteritis (temporal arteritis) is a systemic vasculitis that can produce permanent blindness if not treated in a timely manner. It is categorized as a large-vessel vasculitis (Chapel Hill 2012).

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

Indications for Testing

  • Patient >50 years with
    • Visual changes or new onset localized headache
    • Elevated inflammatory marker(s)

Criteria for Diagnosis

  • Histologic diagnosis – temporal artery biopsy showing segmental inflammation

Laboratory Testing

  • Nonspecific testing – helpful in excluding other diagnoses
    • CBC –  thrombocytosis, normochromic normocytic anemia
    • C-reactive protein (CRP)
    • Erythrocyte sedimentation rate (ESR) – has been used traditionally with threshold of >50 mm/hr
    • Liver function studies – often abnormal
  •  Vasculitis evaluation
    • Antineutrophil cytoplasmic antibodies (ANCA) – high percentage have perinuclear ANCA (pANCA) with MPO+
      • PR-3 rarely positive

Histology

  • Temporal artery biopsy – demonstrates medium- and large-vessel vasculitis with giant cell infiltration
    • 1.5-2 cm long biopsy is recommended due to possibility of skip lesions
  • Biopsy should be performed before day 4 if steroid therapy initiated
  • If initial biopsy is negative, perform biopsy on other side

Imaging Studies

  • Duplex ultrasonography
    • Halo sign is often present
      • Defined as hypoechoic region around the lumen of the artery
    • Sensitivity – <70%

Differential Diagnosis

Epidemiology

  • Prevalence – ~20/million in U.S.; most common primary vasculitis in older patients
  • Age – peak age 70-80 years
    • Rare – <50 years
  • Sex – M<F, 1:2-6
  • Ethnicity – more common in Caucasians than African or Asian Americans; rare in Hispanic population (Weyand, 2014)

Pathophysiology

  • Medium- and large-vessel vasculitis – tends to affect aorta and extracranial branches of the carotid artery
  • Vasculitis causes ischemic disease – most common in the optic nerve
    • Can lead to vision loss, caused primarily by occlusive vasculopathy
  • Granulomas form in arterial media
  • Often associated with polymyalgia rheumatica

Clinical Presentation

  • Constitutional – fever, fatigue, malaise, weight loss
  • Otorhinologic – jaw claudication
  • Ophthalmologic – transient visual changes, diplopia, visual field cuts, permanent vision loss
  • Cardiovascular – arm claudication, thoracic artery aneurysm
  • Neurologic – headache, temporal artery pain, mononeuropathy
  • Polymyalgia rheumatica – may occur in up to 50% of patients
    • Manifests with severe stiffness and pain in shoulders, thighs, and buttocks
  • Dermatologic, renal, pulmonary – rarely involved
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.

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

C-Reactive Protein 0050180
Method: Quantitative Immunoturbidimetry

Sedimentation Rate, Westergren (ESR) 0040325
Method: Visual Identification

Guidelines

Choosing Wisely. An initiative of the ABIM Foundation. [Accessed: Sep 2017]

Hunder GG, Bloch DA, Michel BA, Stevens MB, Arend WP, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY, Lie JT. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum. 1990; 33(8): 1122-8. 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, Lamprecht P, Langford CA, Luqmani RA, Mahr AD, Matteson EL, Merkel PA, Ozen S, Pusey CD, Rasmussen N, Rees AJ, Scott DG, 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

Dasgupta B, Hassan N, British Society for Rheumatology Guidelines Group. Giant cell arteritis: recent advances and guidelines for management. Clin Exp Rheumatol. 2007; 25(1 Suppl 44): S62-5. PubMed

Eberhardt RT, Dhadly M. Giant cell arteritis: diagnosis, management, and cardiovascular implications. Cardiol Rev. 2007; 15(2): 55-61. PubMed

Jennette JC, Falk RJ. The role of pathology in the diagnosis of systemic vasculitis. Clin Exp Rheumatol. 2007; 25(1 Suppl 44): S52-6. PubMed

Nusser JA, Howard E, Wright D. Clinical inquiries. Which clinical features and lab findings increase the likelihood of temporal arteritis? J Fam Pract. 2008; 57(2): 119-20. PubMed

Villa-Forte A. Giant cell arteritis: suspect it, treat it promptly. Cleve Clin J Med. 2011; 78(4): 265-70. PubMed

Waldman CW, Waldman SD, Waldman RA. Giant cell arteritis. Med Clin North Am. 2013; 97(2): 329-35. PubMed

Weyand CM, Goronzy JJ. Clinical practice. Giant-cell arteritis and polymyalgia rheumatica. N Engl J Med. 2014; 371(1): 50-7. PubMed

Medical Reviewers

Last Update: October 2017