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).

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+
  - 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

Infectious
- Virus
- Hepatitis A, B, C
- HIV
- Endocarditis
Malignancy (eg, multiple myeloma)
Connective tissue disease
Fibromyalgia
Statin therapy myopathy
Osteoarthritis
Other vasculitic disease
- Granulomatosis with polyangiitis (formerly Wegener granulomatosis)
- Eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome)
- Microscopic polyangiitis
- Polyarteritis nodosa

Background

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

ARUP Lab Tests

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

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

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)

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

Recommended Use

Nonspecific test used to detect inflammation associated with infections, cancers, and autoimmune diseases

Medical Reviewers

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

References

Guidelines

Choosing Wisely. An initiative of the ABIM Foundation. [Accessed: Jun 2018]
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

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

Testing Algorithms

Vasculitis in Adults Testing Algorithm

Read more about Vasculitis in Adults Testing Algorithm

Last Update: July 2018


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	Giant Cell Arteritis - Temporal Arteritis. ARUP Consult^©. Retrieved October 15, 2018, from: https://arupconsult.com/content/giant-cell-arteritis