Giant cell arteritis
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Giant cell arteritis (GCA) is a common granulomatous vasculitis affecting largemedium- to medium sizedlarge-sized arteries. It may also be known as temporal arteritis or cranial arteritis, given its propensity to involve the extra-cranial carotid artery branches such as the temporal artery.
Epidemiology
GCA is the most common primary systemic vasculitis. It has an incidence of 200 per million population per year 6. Typically affects older individuals (usuallywith patient usually being older than 50 years of age, with a peak age of 70-80 years 3). There is also a recognised female predilection.
Clinical features
There are many possible clinical features that present in a subacute fashion 10:
- 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 ischaemic optic neuropathy, central retinal artery occlusion, ischaemic stroke, etc.)
- in such cases there may be accompanying Charles-Bonnet syndrome
- reduced pulse over affected arteries
- bruits on auscultation over affected arteries
- clinical features of an aortic aneurysm or dissection if aorta is involved
Pathology
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.
Location
Can potentially affect any large-to-medium sizedmedium- to -large-sized vessels, affecting the aorta (~20% of cases 7) and its major branches, particularly the extracranial branches of the carotid artery 6.
Markers
- serum erythrocyte sedimentation rate (ESR): markedly raised
- serum C-reactive protein (CRP): often markedly raised
Associations
- polymyalgia rheumatica: seen in ~50% of cases
Complications
- thoracic aortic aneurysms (more commonly ascending aorta) 7
- aortic dissection (more commonly ascending aorta) 7
Radiographic features
Ultrasound
- increased diameter of the TA and hypoechoic wall thickening (halo sign)
- with duplex ultrasound, sensitivity of 87% and specificity of 96% 9
- more specific for GCA if bilateral 8
- reversible under corticosteroid treatment
- stenosis may be present but is not a specific sign for GCA 8
CT angiography
- may be useful for assessing luminal abnormalities, such as stenoses, occlusions, dilations, and aneurysm formation
- may also show wall thickening of affected segments, calcification and mural thrombi
MRI
-
T1 C+ (Gd)
- the best sequence for assessment, and is reported to show mural inflammation very well 2,4
- mean wall thickness increased in the affected region
- luminal diameter decreased in the affected region
- reported approximate sensitivity and specificity is at 80 and 97% respectively 2
Treatment and prognosis
Often treatedTreatment is with corticosteroid therapy and aspirin 11.
Differential diagnosis
Imaging differential considerations include
- Takayasu arteritis: affects younger patients (<50 years) and affects more proximal vessels
- atherosclerotic disease
-<p><strong>Giant cell arteritis (GCA)</strong> is a common granulomatous <a href="/articles/vasculitis">vasculitis</a> affecting large to medium sized arteries. It may also be known as <strong>temporal arteritis</strong> or <strong>cranial arteritis,</strong> given its propensity to involve the extra-cranial carotid artery branches such as the <a href="/articles/superficial-temporal-artery">temporal artery</a>. </p><h4>Epidemiology</h4><p>GCA is the most common primary systemic vasculitis. It has an incidence of 200 per million population per year <sup>6</sup>. Typically affects older individuals (usually older than 50 years of age, with a peak age of 70-80 years <sup>3</sup>). There is a recognised female predilection.</p><h4>Pathology</h4><p>It is histologically similar to other large vessel vasculitides (such as <a href="/articles/takayasu-arteritis">Takayasu arteritis</a>) 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 <sup>1</sup>.</p><h5>Location</h5><p>Can potentially affect any large-to-medium sized vessels, affecting the aorta (~20% of cases <sup>7</sup>) and its major branches, particularly the extracranial branches of the carotid artery <sup>6</sup>.</p><h5>Markers</h5><ul>- +<p><strong>Giant cell arteritis (GCA)</strong> is a common granulomatous <a href="/articles/vasculitis">vasculitis</a> affecting medium- to large-sized arteries. It may also be known as <strong>temporal arteritis</strong> or <strong>cranial arteritis,</strong> given its propensity to involve the extra-cranial <a href="/articles/external-carotid-artery-1">carotid artery</a> branches such as the <a href="/articles/superficial-temporal-artery">temporal artery</a>. </p><h4>Epidemiology</h4><p>GCA is the most common primary systemic vasculitis. It has an incidence of 200 per million population per year <sup>6</sup>. Typically affects older individuals with patient usually being older than 50 years of age, with a peak age of 70-80 years <sup>3</sup>. There is also a recognised female predilection.</p><h4>Clinical features</h4><p>There are many possible clinical features that present in a subacute fashion <sup>10</sup>:</p><ul>
- +<li>headache (most common) with or without scalp tenderness</li>
- +<li>systemic symptoms (e.g. fever, fatigue, weight loss)</li>
- +<li>jaw claudication</li>
- +<li>transient vision loss (<a href="/articles/amaurosis-fugax">amaurosis fugax</a>)</li>
- +<li>permanent vision loss (e.g. due to <a href="/articles/anterior-ischaemic-optic-neuropathy">anterior ischaemic optic neuropathy</a>, <a href="/articles/central-retinal-artery-occlusion">central retinal artery occlusion</a>, <a href="/articles/ischaemic-stroke">ischaemic stroke</a>, etc.)<ul><li>in such cases there may be accompanying <a href="/articles/charles-bonnet-syndrome-2">Charles-Bonnet syndrome</a>
- +</li></ul>
- +</li>
- +<li>reduced pulse over affected arteries</li>
- +<li>bruits on auscultation over affected arteries</li>
- +<li>clinical features of an <a href="/articles/aortic-aneurysms">aortic aneurysm</a> or <a href="/articles/aortic-dissection">dissection</a> if <a href="/articles/aorta">aorta</a> is involved</li>
- +</ul><h4>Pathology</h4><p>It is histologically similar to other large vessel vasculitides (such as <a href="/articles/takayasu-arteritis">Takayasu arteritis</a>) 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 <sup>1</sup>.</p><h5>Location</h5><p>Can potentially affect any medium- to -large-sized vessels, affecting the <a href="/articles/aorta">aorta</a> (~20% of cases <sup>7</sup>) and its major branches, particularly the extracranial branches of the carotid artery <sup>6</sup>.</p><h5>Markers</h5><ul>
-<a href="/articles/thoracic-aortic-aneurysm">thoracic aortic aneurysms</a> <sup>7</sup>- +<a href="/articles/thoracic-aortic-aneurysm">thoracic aortic aneurysms</a> (more commonly ascending aorta) <sup>7</sup>
-<a href="/articles/aortic-dissection">aortic dissection</a> <sup>7</sup>- +<a href="/articles/aortic-dissection">aortic dissection</a> (more commonly ascending aorta) <sup>7</sup>
-<li>increased diameter of the TA and hypoechoic wall thickening (halo)<ul>- +<li>increased diameter of the TA and hypoechoic wall thickening (halo sign)<ul>
- +<li>with duplex ultrasound, sensitivity of 87% and specificity of 96% <sup>9</sup>
- +</li>
-</li></ul><h4>Treatment and prognosis</h4><p>Often treated with corticosteroid therapy.</p><h4>Differential diagnosis</h4><p>Imaging differential considerations include</p><ul>- +</li></ul><h4>Treatment and prognosis</h4><p>Treatment is with corticosteroid therapy and aspirin <sup>11</sup>.</p><h4>Differential diagnosis</h4><p>Imaging differential considerations include</p><ul>
References changed:
- 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. <a href="https://www.ncbi.nlm.nih.gov/pubmed/16148727">Pubmed</a> <span class="ref_v4"></span>
- 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. <a href="https://doi.org/10.1002/art.20171">doi:10.1002/art.20171</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/15077317">Pubmed</a> <span class="ref_v4"></span>
- 9. Schmidt WA. Role of ultrasound in the understanding and management of vasculitis. Therapeutic advances in musculoskeletal disease. 6 (2): 39-47. <a href="https://doi.org/10.1177/1759720X13512256">doi:10.1177/1759720X13512256</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/24688604">Pubmed</a> <span class="ref_v4"></span>
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