Giant cell arteritis, 446.5
Systemic lupus erythematosus, 710.0
I. Definition
A. Systemic panarteritis of the medium-sized and large arteries, usually the temporal artery or the aorta
B. Believed to represent the extreme spectrum of polymyalgia rheumatica
1. Headache, jaw claudication, scalp tenderness, and throat pain are classic symptoms.
2. Polymyalgia rheumatica present in half of cases—clinical diagnosis based on pain and stiffness of the shoulders and pelvis of longer than 1 month’s duration
3. Associated with weight loss, fever, and malaise
C. Considered a medical emergency
1. Untreated temporal arteritis may result in irreversible blindness.
2. Untreated aortic arteritis may result in the serious or life-threatening sequela of aortic occlusion.
II. Etiology/incidence/predisposing factors
A. Vascular endothelial cells become “activated” and
1. Act as antigen-presenting cells and sources of cytokine production
2. Interact with immune-competent cells
3. Express adhesion molecules, promoting leukocyte binding and aggregation
B. Occurs most commonly in adults older than age 50
C. Slightly more common in women than in men
D. Approximately 50% of patients with giant cell arteritis also have polymyalgia rheumatica.
IV. Physical examination findings
A. Difficulty talking
B. Fever
C. Temporal artery may be large, nodular, or pulseless.
D. Blindness
E. Carotid/supraclavicular bruits
F. Pallor
G. Edema of otic disc
V. Laboratory/diagnostic findings
A. CT/MRI
B. Normal WBC count between 6000 and 10,000/ml
C. Elevated erythrocyte sedimentation rate (ESR) (median 65 mm/hour)
D. C-reactive protein elevation
E. Interleukin-6 most sensitive but not widely used
F. Biopsy of the affected artery is the gold standard.
1. Positive biopsy result confirms the diagnosis.
2. False-negative biopsy result can occur; if clinical suspicion is strong, the patient should be treated, regardless of the negative result.
G. Aortogram as indicated by clinical presentation of aortic aneurysm or occlusion
H. Reactive thrombocytosis with normochromic anemia
VI. Management
A. Should begin immediately upon clinical diagnosis
1. Do not wait for biopsy.
2. Biopsies obtained 1 to 2 weeks after initiation of prednisone are reliable.
B. Prednisone, 1-2 mg/kg/day (typically 60 mg) for 6 weeks to 2 months
C. Prednisone may be tapered after 6 weeks to 2 months if symptoms have subsided.
E. Educate patient regarding signs and symptoms of recurrence; patients are at greater risk for recurrence.
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