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Aortic and extracranial large vessel giant cell arteritis: a review of 72 cases with histopathologic documentation
1Department of Pathology, University of California Davis School of Medicine, USA.
Insights
Giant cell arteritis (GCA) can affect large vessels beyond the head, leading to severe complications. Early diagnosis and intervention are crucial for managing this systemic vasculitis in the elderly.
Area of Science:
- Rheumatology
- Vascular Medicine
- Pathology
Background:
- Giant cell arteritis (GCA) is typically associated with temporal arteritis and polymyalgia rheumatica in the elderly.
- However, GCA is a systemic disease with potentially life-threatening manifestations.
- Extracranial involvement can present initially with severe vascular events.
Purpose of the Study:
- To review cases of aortic and extracranial GCA to understand its systemic impact.
- To highlight the spectrum of GCA beyond cranial arteries.
- To emphasize the importance of considering GCA in elderly patients with large-vessel disease.
Main Methods:
- Retrospective review of 72 histopathologically verified cases of aortic and extracranial GCA.
- Analysis of clinical presentations, affected arterial segments, and outcomes.
- Correlation of extracranial GCA with temporal arteritis and systemic complications.
Main Results:
- Up to 15% of temporal arteritis patients show extracranial GCA.
- Common initial manifestations include aortic insufficiency, aneurysm, dissection, stroke, and myocardial infarction.
- The ascending aorta and aortic arch were most frequently involved (39%), followed by subclavian/axillary (26%) and femoropopliteal arteries (18%).
- 12.5% of patients required limb amputation, and 18 deaths were directly attributed to extracranial GCA (aneurysm rupture, dissection, stroke, MI).
- 25% of patients with aortic/extracranial GCA had asymptomatic temporal arteritis.
Conclusions:
- GCA is a systemic vasculitis that frequently involves extracranial large vessels, including the aorta.
- Clinical presentation can be diverse, with severe vascular events as the initial sign.
- It is crucial to differentiate GCA from atherosclerosis in elderly patients with large-vessel disease.
- Timely diagnosis and surgical intervention are vital for limb salvage and improving survival in GCA patients.
Abstract:
Giant cell arteritis (GCA) is closely identified with the temporal arteritis-polymyalgia rheumatica syndrome of the elderly. It is also a systemic disease that can cripple and kill. Up to 15% of patients with temporal arteritis may have angiographic evidence of extracranial GCA, and aortic insufficiency, ruptured aortic aneurysm, aortic dissection, stroke, or myocardial infarction may be the initial manifestation of systemic GCA. A review of 72 cases of aortic and extracranial GCA, all with histopathologic verification of the disease, revealed that 25% of patients with aortic and extracranial large-vessel GCA had asymptomatic temporal arteritis; the ascending aorta and aortic arch were most frequently involved (39%), followed by the subclavian and axillary arteries (26%), and the femoropopliteal arteries (18%). Nine patients (12.5%) underwent an upper or lower limb amputation. Of the 18 patients whose death was directly attributable to extracranial GCA the causes were ruptured aortic aneurysm (6), aortic dissection (6), stroke (3), and myocardial infarction (3). The findings of these 72 cases caution against attributing all aortic and large-vessel arterial disease in the elderly to atherosclerosis and emphasize that timely surgical intervention may be necessary for life-saving and limb-salvage in patients with aortic and extracranial GCA.