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).
Tabs Content Clinical Overview Diagnosis
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+
Proteinase 3 (PR3) 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
Infectious
Malignancy (eg, multiple myeloma)
Connective tissue disease
Fibromyalgia
Statin therapy myopathy
Osteoarthritis
Other vasculitic disease
Background
Epidemiology
Prevalence – ~20/million in U.S.; most common primary vasculitis in older patients
Age – peak age 70-80 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
ARUP Lab Tests
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/PR3) with reflex to ANCA titer
Preferred test to detect acute phase inflammation (eg, autoimmune diseases, connective tissue disease, rheumatoid arthritis, infection, or sepsis)
Nonspecific test used to detect inflammation associated with infections, cancers, and autoimmune diseases
May help in ruling out infectious process
Initial screening for hepatobiliary inflammation
Screens for various metabolic and kidney disorders
References Choosing Wisely . An initiative of the ABIM Foundation. [Accessed: Aug 2019]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 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 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
Testing Algorithms Related Topics Last Update: November 2019
