Giant cell arteritis: a review

Figures & data

Figure 1 Schematic representation of posterior retinal circulation. Parts of the blood supply to the optic disk, and the segmentation of the choroidal circulation are illustrated. The short posterior ciliary arteries (a–i) supply segments of the choroid (A–I) posterior to the equator of the globe.

©2006 Nature Publishing Group. Reproduced with permission from Aristodemou P, Stanford M. Therapy insight: the recognition and treatment of retinal manifestations of systemic vasculitis. Nat Clin Pract Rheumatol. 2006;2(8):443–451.²³

Table 1 Features of arteritic and nonarteritic anterior ischemic optic neuropathy

Features	Arteritic anterior ischemic optic neuropathy	Nonarteritic anterior ischemic optic neuropathy
Incidence	Relatively less common	Most common form of anterior ischemic optic neuropathy
Age	>60 years	Can affect a patient at any age, about 10% of patients under 45 years
Gender	Three times more common in females	Males and females are affected equally
Race	More common in whites	No racial difference
Vision loss	Rapid	Gradual
Preceding symptoms	Amaurosis fugax, diplopia, jaw/tongue claudication	Amaurosis fugax is extremely rare
Systemic symptoms	Anorexia, weight loss, headache, neck pain, myalgia, malaise and anemia	Patients with nonarteritic anterior ischemic optic neuropathy have no systemic symptoms
Visual field defect	Visual defects are much more extensive and severe	Loss of vision towards the nasal field and less commonly altitudinal loss
Severity	Severe visual loss in range of counting fingers or worse	Relatively less severe
Recurrence	High risk of second eye involvement in 2 weeks	Relatively low risk
Association	Giant cell arteritis/polymyalgia rheumatica	Diabetes mellitus, hypertension
Pathogenesis	Inflammatory occlusion of the short posterior ciliary arteries	Transient nonperfusion/hypoperfusion of the optic nerve circulation below the critical autoregulatory level, causing optic disc ischemia without choroidal ischemia
Inflammatory markers	Raised	Normal
Fundoscopy	Chalky white disc swelling ± central retinal artery occlusion or cotton wool spots	Diffuse or segmental disc edema
Temporal artery biopsy	Vasculitic changes	Not involved
Treatment	Blindness preventable with steroids	No proven therapy available
Prognosis	Variable	Better outcome

Figure 2 “Halo sign” of giant cell arteritis. Hypoechoic area (asterisk) around the temporal artery in longitudinal view (A) and transverse view (B) in a 70-year-old lady who presented with jaw pain and high inflammatory markers.

Figure 3 Fluorodeoxyglucose-positron emission tomographic scan showing florid uptake in large vessels including the aorta in a 74-year-old man who presented with polymyalgic symptoms and high inflammatory markers.

AristodemouPStanfordMTherapy insight: the recognition and treatment of retinal manifestations of systemic vasculitisNat Clin Pract Rheumatol20062844345116932736

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