Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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September 6, 2018
Giant cell arteritis
- Background
- Giant cell arteritis (GCA) is a non-necrotizing granulomatous vasculitis that affects medium and large size vessels. Its diagnosis can be difficult when symptoms are nonspecific or the temporal artery biopsy (TAB) is negative. Recently, the use of 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) has gained attention for the diagnosis and activity assessment in large-vessel vasculitis (LVV), since it has been proven that macrophages and granulation tissue take up FDG avidly, especially during active stages.
- Clinical Presentation
- Patients are usually older than 50-years-old with a peak incidence between ages 70 and 80. They may present with systemic and/or neurological symptoms; including sudden headache, visual disturbances, and scalp tenderness. Some can manifest jaw, tongue, or limb claudication and about 20% of people develop blindness. Patients have an increased rate of thrombo-embolic disease and may experience multiple ischemic complications, such as stroke and/or cranial nerve palsy.
- Key Diagnostic Features
- Although TAB remains the diagnostic standard, ultrasound and vessel-wall MRI have been used to assess the vascular inflammation by evaluating arterial wall thickening and peripheral enhancement. More recently, FDG-PET has been included as a potential diagnostic method for patients with suspected large-vessel vasculitis. However, its practical utility as a follow-up tool is still to be determined because of the persistent FDG uptake (up to 6 months), despite the regression of clinical symptoms.
- Differential Diagnosis
- The main differential diagnosis among other LVV is Takayasu arteritis, which mostly affects younger patients and usually involves larger and proximal vessels. The findings of GCA may mimic atherosclerosis or arterial dissection of the cervical arteries.
- Treatment
- Prompt treatment initiation is recommended because of the significant complications that GCA can trigger. Glucocorticoids are considered the treatment of choice, but dosing and administration regimens remain controversial. The use of immunosuppressive agents like methotrexate is suggested to decrease both the relapse rate and the cumulative glucocorticoid dose.
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