Giant Cell Arteritis (Gca)
Basics
Basics
Basics
Description
Description
- Chronic vasculitis of large- and medium-sized vessels that occurs among individuals over 50 yr of age
- Often referred to as temporal arteritis (TA)
- Median age of onset is 72 yr
- Most commonly causes inflammation of arteries originating from the arch of the aorta
- Although usually clinically silent, involvement of the thoracic aorta occurs in a significant minority of patients, and aortic aneurysm or dissection may result
- Thoracic aortic aneurysm is a late manifestation with an incidence 17 times those without TA
- Abdominal aortic aneurysm is about twice as common in those with giant cell arteritis (GCA)
- Pathologic specimens feature patchy mononuclear granulomatous inflammation resulting in a markedly thickened intima and occlusion of the vessel lumen
- Occlusive arteritis may involve thrombosis of the ophthalmic artery resulting in anterior ischemic optic neuropathy (AION) and acute visual loss:
- Visual symptoms are an ophthalmic emergency
- Inflammation of arteries supplying the muscles of mastication results in jaw claudication and tongue discomfort
- Age is the greatest risk factor:
- Rare in patients <50 yr old
- >90% are >60 yr old
- Prevalence in individuals >50 yr is estimated at 1:500
- Increased prevalence in Northern latitude with highest incidence in those of Scandinavian descent
- 2–4 times more common in women
- Rare in African American, Latino, and Asian patients
- There is a strong association with polymyalgia rheumatica (PMR) ∼50%
Genetics
Genetic predisposition is linked to HLA-DR4 – 60% prevalenceEtiology
Etiology
- Unknown
- Genetic, environmental, and autoimmune factors have been identified
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