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Review
. 2013 May;110(21):376-85; quiz 386.
doi: 10.3238/arztebl.2013.0376. Epub 2013 May 24.

The diagnosis and treatment of giant cell arteritis

Thomas Ness  1 , Thorsten A Bley, Wolfgang A Schmidt, Peter Lamprecht
Affiliations
Review

The diagnosis and treatment of giant cell arteritis

Thomas Ness et al. Dtsch Arztebl Int. 2013 May.

Abstract

Background: Giant cell arteritis (GCA) is the most common systemic vasculitis in persons aged 50 and above (incidence, 3.5 per 100,000 per year). It affects cranial arteries, the aorta, and arteries elsewhere in the body, e.g., in the limbs.

Methods: We selectively review the pertinent literature, including guidelines and recommendations from Germany and abroad.

Results: The typical symptoms of new-onset GCA are bitemporal headaches, jaw claudiacation, scalp tenderness, visual disturbances, systemic symptoms such as fever and weight loss, and polymyalgia. The diagnostic assessment comprises laboratory testing (erythrocyte sedimentation rate, C-reactive protein), imaging studies (duplex sonography, high-resolution magnetic resonance imaging, positron-emission tomography), and temporal artery biopsy. The standard treatment is with corticosteroids (adverse effects: diabetes mellitus, osteoporosis, cataract, arterial hypertension). A meta-analysis of three randomized controlled trials led to a recommendation for treatment with methotrexate to lower the recurrence rate and spare steroids. Patients for whom methotrexate is contraindicated or who cannot tolerate the drug can be treated with azathioprine instead.

Conclusion: Giant cell arteritis, if untreated, progresses to involve the aorta and its collateral branches, leading to various complications. Late diagnosis and treatment can have serious consequences, including irreversible loss of visual function.

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Figures

Figure 1
Figure 1
a) Anterior ischemic optic neuropathy (AION) with swelling of the optic disk b) Central artery occlusion
Figure 2
Figure 2
Color-coded duplex sonography of the temporal artery:
Figure 3
Figure 3
High-resolution 3-T MRI of the superficial cranial arteries of a 72-year-old man with giant cell arteritis. The frontal branch of the superficial temporal artery shows bilateral signs of wall inflammation (thickening, uptake of contrast agent) (solid arrow). The right superficial occipital artery likewise shows inflammatory changes (open arrow). Thanks to the high spatial resolution, details can be enlarged, giving a particularly good visualization of the wall changes (arrow in the inset detail of the frontal branch of the left temporal superficial artery)
Figure 4
Figure 4
Inflamed temporal artery with intimal edema, cellular infiltration, and fragmented internal elastic membrane; box: typical giant cell with infiltration at the transition between intima and muscularis media in a temporal artery in a patient with giant cell arteritis
See this image and copyright information in PMC

References

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