Therapy Insight: The Recognition and Treatment of Retinal Manifestations of Systemic Vasculitis

Petros Aristodemou; Miles Stanford


Nat Clin Pract Rheumatol. 2006;2(8):443-451. 

In This Article

Retinal Findings in Large-vessel Vasculitis

Giant cell arteritis (also known as temporal arteritis) is the most common of the systemic vasculitides. This necrotizing vasculitis affects arteries with an internal elastic lamina.[9] The superficial temporal, ophthalmic and posterior choroidal arteries (as well as the vertebral arteries) are commonly involved, whereas the internal carotid arteries are affected less often. Arteries distal to the dura are never involved.[10]

AION is the most common cause of visual loss in giant cell arteritis.[6] In AION, the presence of cotton-wool spots separately from the optic disk is almost pathognomonic of arteritic AION. Other posterior-segment pathology is less common. Choroidal infarcts might also occur. Although the central retinal artery is not thought to be directly involved in giant cell arteritis, occlusion of this artery can occur owing to proximal vessel disease.[116] AION can occur in the absence of arteritis, and the differentiation of arteritic and nonarteritic AION can be a challenge ( Table 1 ). Fluorescein angiography can help to distinguish between arteritic and nonarteritic AION.

Systemic corticosteroids are effective in controlling the symptoms of giant cell arteritis. Patients with ophthalmic symptoms can present with unilateral, acute loss of vision—which is in most cases irreversible. Without treatment, more than 70% of patients will suffer contralateral vision loss within 1 week,[12] so immediate treatment with high-dose systemic steroids is warranted. Rarely, patients with giant cell arteritis experience symptoms of fluctuating monocular vision, which can herald imminent infarction of the optic nerve head. These patients require immediate treatment to preserve their vision.


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