Giant Cell Arteritis - Temporal Arteritis

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

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

Vasculitis in Adults Testing Algorithm

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

Indications for Laboratory Testing

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

Additional Tests Available

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

Comments

May help in ruling out infectious process

Urinalysis, Complete 0020350
Method: Reflectance Spectrophotometry/Microscopy

Comments

Screens for various metabolic and kidney disorders

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

Comments

Initial evaluation for suspected vasculitis

Hepatic Function Panel 0020416
Method: Quantitative Enzymatic/Quantitative Spectrophotometry

Comments

Initial screening for hepatobiliary inflammation

Panel includes albumin; ALP; AST; ALT; bilirubin, direct; protein, total; and bilirubin, total

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 G, Bloch D, Michel B, Stevens M, Arend W, Calabrese L, Edworthy S, Fauci A, Leavitt R, Lie J. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum. 1990; 33(8): 1122-8. PubMed

Jennette J, Falk R, Bacon P, Basu N, Cid M, Ferrario F, Flores-Suarez L, Gross W, Guillevin L, Hagen E, Hoffman G, Jayne D, Kallenberg C, Lamprecht P, Langford C, Luqmani R, Mahr A, Matteson E, Merkel P, Ozen S, Pusey C, Rasmussen N, Rees A, Scott D, Specks U, Stone J, Takahashi K, Watts R. 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 R, Dhadly M. Giant cell arteritis: diagnosis, management, and cardiovascular implications. Cardiol Rev. 2007; 15(2): 55-61. PubMed

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

Nusser J, 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 C, Waldman S, Waldman R. Giant cell arteritis. Med Clin North Am. 2013; 97(2): 329-35. PubMed

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

Medical Reviewers

Last Update: December 2015