Giant cell arteritis (GCA) is a common granulomatous vasculitis affecting medium- to large-sized arteries. It is also known as temporal arteritis or cranial arteritis, given its propensity to involve the extracranial external carotid artery branches such as the superficial temporal artery.
Giant cell arteritis is the most common primary systemic vasculitis. It has an incidence of 200 per million persons per year 6. Typically affects older individuals with patients usually being older than 50, with a peak incidence between the ages of 70 and 80 3. There is a recognized female predilection.
There are many possible clinical features that present in a subacute fashion 10:
- a headache (most common) with or without scalp tenderness
- systemic symptoms (e.g. fever, fatigue, weight loss)
- jaw claudication
- transient vision loss (amaurosis fugax)
- permanent vision loss (e.g. due to anterior ischemic optic neuropathy, central retinal artery occlusion, ischemic stroke, etc.)
- in such cases, there may be accompanying Charles-Bonnet syndrome
- weak pulse over the affected arteries
- bruits on auscultation over the affected arteries
- clinical features of an aortic aneurysm or dissection if the aorta is involved
It is histologically similar to other large vessel vasculitides (such as Takayasu arteritis) showing granulomatous inflammation of arteries with infiltration predominantly by histiocytes, lymphocytes, and multinucleated giant cells. The characteristic multinucleated giant cells are only found in ~50% of cases 1.
Areas of normal superficial temporal artery interspersed within inflamed sections of artery, known as skip lesions, results in false negatives in up to 8-28% of cases 12,13,15.
In a study of 285 patients with biopsy proven giant cell arteritis there were 4 main histological patterns 12:
- Adventitial pattern: inflammatory cells restricted to the adventitia.
- Adventitial invasive pattern: local invasion of the media with preservation of the intima.
- Concentric bilayer pattern: inflammatory infiltration of adventitia and intima with preservation of the media.
- Panarteritic pattern: inflammatory infiltrates in the three arterial layers.
Can potentially affect any medium to large-sized vessels, affecting the aorta (~20% of cases 7) and its major branches, particularly the extracranial branches of the carotid artery 6.
- serum erythrocyte sedimentation rate (ESR): markedly raised
- serum C-reactive protein (CRP): often markedly raised
- polymyalgia rheumatica: seen in ~50% of cases
- thoracic aortic aneurysms (more commonly ascending aorta) 7
- aortic dissection (more commonly ascending aorta) 7
- increased diameter of the superficial temporal artery and hypoechoic wall thickening (halo sign)
- with duplex ultrasound, sensitivity is 87% and specificity is 96% 9
- more specific for giant cell arteritis if bilateral 8
- reversible under corticosteroid treatment; this is reflected in the normalization of the sonographic features
- stenosis may be present but is not a specific sign for giant cell arteritis 8
Findings on CT include:
- wall thickening of affected segments
- mural thrombi
Arterial phase CT (angiography) is useful for assessing luminal abnormalities:
- aneurysm formation
T1 C+ (Gd)
- the best sequence for assessment
- shows mural inflammation very well 2,4
- mean wall thickness increased in the affected region
- luminal diameter correspondingly decreased in the affected region
- reported approximate sensitivity and specificity is 80% and 97%, respectively 2
Treatment and prognosis
Treatment is with corticosteroid therapy and aspirin 11. Methotrexate may be used in combination with corticosteroid therapy initially, or as a corticosteroid-sparing drug 14.
Imaging differential considerations include:
- 1. Bau JL, Ly JQ, Borstad GC et-al. Giant cell arteritis. AJR Am J Roentgenol. 2003;181 (3): 742. AJR Am J Roentgenol (full text) - Pubmed citation
- 2. Bley TA, Uhl M, Carew J et-al. Diagnostic value of high-resolution MR imaging in giant cell arteritis. AJNR Am J Neuroradiol. 2007;28 (9): 1722-7. doi:10.3174/ajnr.A0638 - Pubmed citation
- 3. Bley TA, Wieben O, Uhl M et-al. High-resolution MRI in giant cell arteritis: imaging of the wall of the superficial temporal artery. AJR Am J Roentgenol. 2005;184 (1): 283-7. AJR Am J Roentgenol (full text) - Pubmed citation
- 4. Bley TA, Weiben O, Uhl M et-al. Assessment of the cranial involvement pattern of giant cell arteritis with 3T magnetic resonance imaging. Arthritis Rheum. 2005;52 (8): 2470-7. doi:10.1002/art.21226 - Pubmed citation
- 5. Markl M, Uhl M, Wieben O et-al. High resolution 3T MRI for the assessment of cervical and superficial cranial arteries in giant cell arteritis. J Magn Reson Imaging. 2006;24 (2): 423-7. doi:10.1002/jmri.20639 - Pubmed citation
- 6. Rubin GD, Rofsky NM. CT and MR angiography, comprehensive vascular assessment. Lippincott Williams & Wilkins. (2008) ISBN:078174525X. Read it at Google Books - Find it at Amazon
- 7. Martínez-Valle F, Solans-Laqué R, Bosch-Gil J et-al. Aortic involvement in giant cell arteritis. Autoimmun Rev. 2010;9 (7): 521-4. doi:10.1016/j.autrev.2010.02.014 - Pubmed citation
- 8. Karahaliou M, Vaiopoulos G, Papaspyrou S, Kanakis MA, Revenas K, Sfikakis PP. Colour duplex sonography of temporal arteries before decision for biopsy: a prospective study in 55 patients with suspected giant cell arteritis. Arthritis research & therapy. 8 (4): R116. doi:10.1186/ar2003 - Pubmed
- 9. Schmidt WA. Role of ultrasound in the understanding and management of vasculitis. Therapeutic advances in musculoskeletal disease. 6 (2): 39-47. doi:10.1177/1759720X13512256 - Pubmed
- 10. Gonzalez-Gay MA, Barros S, Lopez-Diaz MJ, Garcia-Porrua C, Sanchez-Andrade A, Llorca J. Giant cell arteritis: disease patterns of clinical presentation in a series of 240 patients. Medicine. 84 (5): 269-76. Pubmed
- 11. Nesher G, Berkun Y, Mates M, Baras M, Rubinow A, Sonnenblick M. Low-dose aspirin and prevention of cranial ischemic complications in giant cell arteritis. Arthritis and rheumatism. 50 (4): 1332-7. doi:10.1002/art.20171 - Pubmed
- 12. Hernández-Rodríguez J, Murgia G, Villar I, Campo E, Mackie SL, Chakrabarty A, Hensor EM, Morgan AW, Font C, Prieto-González S, Espígol-Frigolé G, Grau JM, Cid MC. Description and Validation of Histological Patterns and Proposal of a Dynamic Model of Inflammatory Infiltration in Giant-cell Arteritis. (2016) Medicine. 95 (8): e2368. doi:10.1097/MD.0000000000002368 - Pubmed
- 13. Stacy RC, Rizzo JF, Cestari DM. Subtleties in the histopathology of giant cell arteritis. (2011) Seminars in ophthalmology. 26 (4-5): 342-8. doi:10.3109/08820538.2011.588656 - Pubmed
- 14. Alfred D. Mahr, Juan A. Jover, Robert F. Spiera, César Hernández‐García, Benjamin Fernández‐Gutiérrez, Michael P. LaValley, Peter A. Merkel. Adjunctive methotrexate for treatment of giant cell arteritis: An individual patient data meta‐analysis. (2007) Arthritis & Rheumatism. 56 (8): 2789. doi:10.1002/art.22754 - Pubmed
- 15. Bowling K, Rait J, Atkinson J, Srinivas G. Temporal artery biopsy in the diagnosis of giant cell arteritis: Does the end justify the means?. (2017) Annals of medicine and surgery (2012). 20: 1-5. doi:10.1016/j.amsu.2017.06.020 - Pubmed