Giant Cell Arteritis - Temporal Arteritis

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

Indications for Testing

  • Patient >50 years with visual changes or new onset headache

Criteria for Diagnosis

  • American College of Rheumatology (1990); 3 of 5 required (may have limited clinical use dependent upon patient population)
    • Age – >50 years
    • New localized headache
    • Temporal artery tenderness or decreased pulsation
    • ESR – >50 mm/hr
    • Abnormal arterial artery biopsy

Laboratory Testing

  • Nonspecific testing – helpful in excluding other diagnoses
    • CBC –  thrombocytosis, normochromic normocytic anemia
    • C-reactive protein (CRP)
    • Liver function studies – often abnormal
  •  Vasculitis evaluation
    • ANCA – high percentage have pANCA with MPO+
      • PR3 rarely positive

Histology

  • Temporal artery biopsy – demonstrates medium- and large-vessel vasculitis with giant cell infiltration
    • May have skip lesions; therefore, 1.5-2 cm long biopsy is recommended
  • 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

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).

Epidemiology

  • Incidence – ~20/million in U.S. (Weyand, 2014); 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

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.

C-Reactive Protein 0050180
Method: Quantitative Immunoturbidimetry

Recommended Use 

Preferred test to detect acute phase inflammation (eg, autoimmune diseases, connective tissue disease, rheumatoid arthritis, infection, or sepsis)

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

Recommended Use 

Preferred reflex panel for managing patients with a known diagnosis of vasculitis; may be assistive in evaluating suspected vasculitis

For the workup of suspected vasculitis, the preferred reflex panel is ANCA-associated vasculitis profile (ANCA/MPO/PR-3) with reflex to ANCA titer

Panel includes ANCA, IgG; myeloperoxidase antibody and serine protease 3 antibody

Reflex pattern – if screen is positive, titer and MPO/PR-3 antibodies testing will be added to aid in antibody determination

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]

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

Tebo, Anne E., PhD, D(ABMLI), Medical Director, Immunology at ARUP Laboratories; Associate Professor of Clinical Pathology, University of Utah

Last Update: August 2016

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