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Question 36#

Giant cell arteritis

A. Tends to occur in white men older than 50 years
B. Has low remission rates
C. Is associated with ischemic optic neuritis in the majority of patients
D. Is diagnosed by temporal artery biopsy

Correct Answer is D

Comment:

Giant cell arteritis is also known as temporal arteritis, which is a systemic chronic inflammatory vascular disease with many characteristics similar to those of Takayasu disease. The histologic and pathologic changes and laboratory findings are similar. Patients tend to be white women older than 50 years, with a high incidence in Scandinavia and women of Northern European descent. Genetic factors may play a role in disease pathogenesis, with a human leukocyte antigen (HLA) variant having been identified. Differences exist between Takayasu and giant cell arteritis in terms of presentation, disease location, and therapeutic efficacy. The inflammatory process typically involves the aorta and its extracranial branches, of which the superficial temporal artery is specifically affected. The clinical syndrome begins with a prodromal phase of constitutional symptoms, including headache, fever, malaise, and myalgias. The patients may be initially diagnosed with coexisting polymyalgia rheumatica; an HLA-related association may exist between the two diseases. As a result of vascular narrowing and end-organ ischemia, complications may occur such as visual alterations, including blindness and mural weakness, resulting in acute aortic dissection that may be devastating. Ischemic optic neuritis resulting in partial or complete blindness occurs in up to 40% of patients and is considered a medical emergency. Cerebral symptoms occur when the disease process extends to the carotid arteries. Jaw claudication and temporal artery tenderness may be experienced. Aortic lesions are usually asymptomatic until later stages and consist of thoracic aneurysms and aortic dissections. The diagnostic gold standard is a temporal artery biopsy, which will show the classic histologic findings of multinucleated giant cells with a dense perivascular inflammatory infiltrate. Treatment regimens are centered on corticosteroids, and giant cell arteritis tends to rapidly respond. Remission rates are high, and treatment tends to have a beneficial and preventative effect on the development of subsequent vascular complications.