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Table of Contents    
Year : 2015  |  Volume : 63  |  Issue : 5  |  Page : 652-653

Giant cell arteritis (temporal arteritis) revisited

Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India

Date of Web Publication6-Oct-2015

Correspondence Address:
Sanjeev V Thomas
Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.166571

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How to cite this article:
Sai SK, Thomas SV. Giant cell arteritis (temporal arteritis) revisited. Neurol India 2015;63:652-3

How to cite this URL:
Sai SK, Thomas SV. Giant cell arteritis (temporal arteritis) revisited. Neurol India [serial online] 2015 [cited 2020 Mar 31];63:652-3. Available from:

Giant cell arteritis (GCA) is an inflammatory vasculopathy involving medium and large arteries with a predilection to affect the temporal arteries and other branches of the external carotid artery.[1] The detection of varicella zoster antigens in the temporal artery biopsy (TAB) had brought a paradigm shift in the understanding of the disease and its management. This issue of Neurology India includes a series of GCA from a teaching hospital in the North India.[2] GCA is an often missed diagnosis until some of the catastrophic complications such as visual loss or double vision have occurred. Irreversible visual loss, stroke, and aortitis leading to dissection and aneurysms are other complications of GCA. This has serious implications, given the prompt response of GCA to steroid therapy, and the risk of serious complications if left untreated. Clinician need to maintain a high index of suspicion in any person older than 50 years presenting with abrupt onset headache, scalp pain/tenderness, jaw claudication, visual symptoms, abnormal temporal arteries, and an elevated erythrocytic sedimentation rate or C-reactive protein. The American College of Rheumatology classification requires three of the following five criteria to diagnose GCA: (1) Age over 50 years, (2) new onset headache, (3) abnormal temporal artery (tenderness or decreased pulsation), (4) erythrocyte sedimentation rate ≤50 mm in the 1st h, and (5) an abnormal TAB.[3] Histopathology of the temporal artery is the gold standard for the diagnosis of GCA.[4] Disruption of the internal elastic membrane, mononuclear cell infiltration, and granulomas with multinucleated giant cells located close to the intima-media junction are the characteristic findings in the biopsy. Considering the segmental nature of involvement of the temporal artery, TAB is normal in approximately 10–30% of cases.[5]

Color duplex sonography (CDS) of the temporal artery in GCA shows a luminal narrowing and concentric hypoechoic vessel wall thickening ("halo") corresponding to the inflammatory edema. Unilateral halo sign has a sensitivity of 68% and a specificity of 91% for establishing the diagnosis of GCA.[6] However, CDS-guided TAB may not improve the sensitivity of the biopsy procedure.[7] Other imaging modalities such as angiography (computed tomography [CT] or magnetic resonance imaging) and 18-fluorodeoxyglucose (FDG)-positron emission tomography (PET) have proven to be useful in the detection of extracranial arteritis in the setting of GCA. While magnetic resonance and CT can detect established large vessel arteritis, 18 FDG PET can detect potentially reversible early inflammatory changes.[8]

What incites the inflammation in this idiopathic arteritis is currently unknown. Various factors, including genetic polymorphisms of genes involved in immune and inflammatory pathways and infective agents, have been investigated for a causal link with GCA. Possibly, only a limited number of genes (eNOS, MMP-9, and TLR4) and no infectious agent have been convincingly identified to be associated with GCA.[4]

In the past 15 years, several groups of researchers have demonstrated varicella zoster antigens in biopsy-proven cases of GCA[9],[10],[11] and in others negative on biopsy.[12] Varicella zoster virus antigen was present mostly in the adventitia, followed by the media and intima, raising the possibility that the virus enters the artery through the adventitia, presumably after transaxonal spread via ganglionic afferent fibers followed by transmural migration with the infection persisting in the thickened intima.[9] This is similar to the pathophysiology of GCA, wherein the inflammation begins in the adventitia and progressively involves media and intima of the vessel wall.[4]

The treatment of GCA involves the use of glucocorticoids in moderately high does (equivalent to 0.7–1 mg/kg body weight of prednisolone) until the clinical symptoms and signs subside. Most patients need to continue on a lower dose of glucocorticoids for several months to prevent catastrophic complications such as visual loss, ophthalmoplegia, or other neurological deficits. The role of antiviral drugs such as acyclovir needs to be established in the management of GCA.

Given the fact that up to 30% of patients having GCA have a negative yield on TAB, there is a need for biomarkers. Recently, antibodies against the N-terminal part of ferritin were demonstrated in 92% of the untreated patients, in 69% of the patients with a disease flare up, and in 13% of patients in remission. These antibodies were also present in 29% of lupus patients, but in only 3% of rheumatoid arthritis patients and 1% of blood donors.[13] The potential diagnostic yield of GCA can be improved upon by combining three human ferritin peptide antibodies.[14]

Corticosteroids are the mainstay of treatment of GCA, which need to be initiated immediately on clinical suspicion without waiting for the biopsy results especially in cases of acute visual loss.[15] A meta-analysis showed that the use of immunosuppressive agents in addition to glucocorticoids did not improve the therapeutic efficacy or safety when compared with the use of glucocorticoids alone.[16]

To conclude, our current understanding of the pathophysiology of GCA lies in bridging the gap between the infectious and the idiopathic inflammatory entities, thus paving the way for a tectonic shift in the way we treat this disorder.

  References Top

Nesher G. The diagnosis and classification of giant cell arteritis. J Autoimmun 2014;48-49:73-5.  Back to cited text no. 1
Sharma A, Sagar V, Prakash M, Gupta V, Khaire N, Pinto B, et al. Giant cell arteritis in India: Report from a tertiary care center along with total published experience from India. Neurol India 2015;63:681-86.  Back to cited text no. 2
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Hunder GG, Bloch DA, Michel BA, Stevens MB, Arend WP, Calabrese LH, et al. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum 1990;33:1122-8.  Back to cited text no. 3
Ly KH, Régent A, Tamby MC, Mouthon L. Pathogenesis of giant cell arteritis: More than just an inflammatory condition? Autoimmun Rev 2010;9:635-45.  Back to cited text no. 4
Gonzalez-Gay MA, Garcia-Porrua C, Llorca J, Gonzalez-Louzao C, Rodriguez-Ledo P. Biopsy-negative giant cell arteritis: Clinical spectrum and predictive factors for positive temporal artery biopsy. Semin Arthritis Rheum 2001;30:249-56.  Back to cited text no. 5
Arida A, Kyprianou M, Kanakis M, Sfikakis PP. The diagnostic value of ultrasonography-derived edema of the temporal artery wall in giant cell arteritis: A second meta-analysis. BMC Musculoskelet Disord 2010;11:44.  Back to cited text no. 6
Germanò G, Muratore F, Cimino L, Lo Gullo A, Possemato N, Macchioni P, et al. Is colour duplex sonography-guided temporal artery biopsy useful in the diagnosis of giant cell arteritis? A randomized study. Rheumatology (Oxford) 2015;54:400-4.  Back to cited text no. 7
Puppo C, Massollo M, Paparo F, Camellino D, Piccardo A, Shoushtari Zadeh Naseri M, et al. Giant cell arteritis: A systematic review of the qualitative and semiquantitative methods to assess vasculitis with 18F-fluorodeoxyglucose positron emission tomography. Biomed Res Int 2014;2014:574248.  Back to cited text no. 8
Salazar R, Russman AN, Nagel MA, Cohrs RJ, Mahalingam R, Schmid DS, et al. Varicella zoster virus ischemic optic neuropathy and subclinical temporal artery involvement. Arch Neurol 2011;68:517-20.  Back to cited text no. 9
Mathias M, Nagel MA, Khmeleva N, Boyer PJ, Choe A, Durairaj VD, et al. VZV multifocal vasculopathy with ischemic optic neuropathy, acute retinal necrosis and temporal artery infection in the absence of zoster rash. J Neurol Sci 2013;325:180-2.  Back to cited text no. 10
Gilden D, White T, Khmeleva N, Heintzman A, Choe A, Boyer PJ, et al. Prevalence and distribution of VZV in temporal arteries of patients with giant cell arteritis. Neurology 2015;84:1948-55.  Back to cited text no. 11
Nagel MA, Bennett JL, Khmeleva N, Choe A, Rempel A, Boyer PJ, et al. Multifocal VZV vasculopathy with temporal artery infection mimics giant cell arteritis. Neurology 2013;80:2017-21.  Back to cited text no. 12
Baerlecken NT, Linnemann A, Gross WL, Moosig F, Vazquez-Rodriguez TR, Gonzalez-Gay MA, et al. Association of ferritin autoantibodies with giant cell arteritis/polymyalgia rheumatica. Ann Rheum Dis 2012;71:943-7.  Back to cited text no. 13
Große K, Schmidt RE, Witte T, Baerlecken NT. Epitope mapping of antibodies against ferritin heavy chain in giant cell arteritis and polymyalgia rheumatica. Scand J Rheumatol 2013;42:215-9.  Back to cited text no. 14
Dasgupta B, Borg FA, Hassan N, Alexander L, Barraclough K, Bourke B, et al. BSR and BHPR guidelines for the management of giant cell arteritis. Rheumatology (Oxford) 2010;49:1594-7.  Back to cited text no. 15
Yates M, Loke YK, Watts RA, MacGregor AJ. Prednisolone combined with adjunctive immunosuppression is not superior to prednisolone alone in terms of efficacy and safety in giant cell arteritis: Meta-analysis. Clin Rheumatol 2014;33:227-36.  Back to cited text no. 16


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