Page 365 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Ophthalmologic and Neuro-Ophthalmologic Aspects of Vasculitides 339

The ACR 1990 criteria for the classification of GCA [10], designed for use in investigative
studies to help distinguish GCA from other types of vasculitides, are not useful for making
the diagnosis in individual patients [11]. Five criteria were selected by the ACR [10] from
which three or more in a given patient was associated with a sensitivity of 93.5% and a
specificity of 91.2%, from among the following: age equal to or greater than 50 years at
disease onset, new localized headache, temporal artery tenderness or decreased temporal
artery pulse, elevated erythrocyte sedimentation rate (ESR) to 50 mm/hour or more, and
vascular tissue biopsy sample showing necrotizing arteritis with predominance of
mononuclear cells infiltration or granulomatous multinucleated giant cell inflammation. The
histopathology of biopsy-positive GCA is typified by vessel wall infiltration by mononuclear
cells, CD4+ T-cells, activated macrophages and multinucleated giant cells, that latter of
which form granulomas close to the internal elastic lamina of involved vessels in up to one-
half of specimens [12].

Visual loss due to anterior ischemic optic neuropathy (AION), which results from
occlusion of short posterior ciliary arteries that supply the optic nerve head, is the commonest
ocular symptom in GCA with a frequency that varies from 10% to 60%. Other causes of
visual loss include vasculitic involvement of the choroid, posterior optic nerve and retina
[13]. Visual loss may be partial, complete, or permanent, and is preceded by amaurosis fugax
in 44% of patients [9]. A prospective study of 170 patients with biopsy-confirmed GCA [14]
noted ocular involvement in 85 (50%) patients, including visual loss present in 83 (97.7%),
amaurosis fugax in 26 (30.6%), eye pain in seven (8.2%), and diplopia in 5 (5.9%). Ocular
ischemic lesions consisted of arteritic AION in 69 (81.2%), central retinal artery and
cilioretinal occlusions each in 12 (14.1%), the latter after satisfactory fundus fluorescein
angiography (FFA); as well as, posterior ischemic optic neuropathy in 6 (7.1%), and ocular
ischemia in 1(1.2%) patient. Those with ocular involvement were significantly older than the
patients without ocular involvement, and surprisingly less likely to have prominent
constitutional and systemic symptomatology. Among 42 other patients from Olmsted County
Minnesota studied by Huston and coworkers [15], visual symptoms preceded the clinical and
histopathologic diagnosis of GCA in 15 (40%) patient, of which blurred vision was most
common so noted in 6 (19%) of patients, followed by diplopia in 5 (12%), transient vision
loss in 5 (12%), permanent partial loss in 4 (10%), and permanent complete loss in 3 (10%)
patients. A followup Olmsted County cohort twenty-five years later totaling 168 patients with
GCA by Nuenninghoff and colleagues (16) found visual disturbances overall at presentation
in 16 (9.5%) patients, and at the time of diagnosis in 37 (22%) patients, of whom 14 (8.3%)
patients had transient vision loss, 18 (10.7%) had permanent vision loss, and 14 (8.3%) had
diplopia.

Of 18 patients with varying visual loss and occult GCA [17], amaurosis fugax was noted
in 6 (33.3%), diplopia in 2 (11.1%), and eye pain in 1 (5.6%) patient. Ocular ischemic lesions
included AION in 17 (94.4%) patients, and central retinal artery occlusion and cilioretinal
artery occlusions each in 2 (11.1%) after FFA. The authors [17] concluded a high index of
suspicion for GCA for patients older than 50 who develop amaurosis fugax, visual loss, or
AION in the absence of constitutional and systemic symptoms (Figure 1).

Ocular involvement was more common in patients with GCA without large vessel
vasculitic involvement according to Prieto-González and colleagues [18] who studied 40
patients with newly diagnosed biopsy-proven GCA by computed tomographic (CT) imaging
to define LVV involvement of the aorta and its tributaries. Ocular involvement was noted

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