Giant cell arteritis
Giant cell (temporal) arteritis, which affects older adults, is a potentially blinding granulomatous inflammatory disease involving the aorta and its branches. It is of particular concern to ophthalmologists and is discussed at length in BCSC Section 5, Neuro-Ophthalmology.
Like giant cell arteritis, Takayasu arteritis affects large arteries, particularly branches of the aorta; but, in contrast, it occurs primarily in children and young women. The disease is rare in Western countries but is more common in Asia, particularly Japan. Other names include aortic arch arteritis, aortitis syndrome, and pulseless disease.
This disease may involve the entire aorta or be localized to any segment of the aorta or its primary branches. The inflammatory process is characterized by panarteritis with granulomatous inflammation. The involved vessels may ultimately become narrowed or obliterated, resulting in ischemia of the supplied tissues. Areas of weakened vascular walls may develop dissections or aneurysms.
Systemic features such as fatigue, headache, weight loss, and low-grade fever are common. Evidence of vascular insufficiency due to large-artery narrowing leads to the characteristic pulseless phase. Angiography, including magnetic resonance angiography, is essential in confirming the diagnosis. Treatment is generally with systemic corticosteroids, which may successfully suppress the disease. Cyclophosphamide or methotrexate is added in resistant cases. Surgical reconstruction of stenotic vessels may be necessary.
Excerpted from BCSC 2020-2021 series: Section 1 - Update on General Medicine. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.