Page 368 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

342 Sarah Conway and David S. Younger

The commonest ischemic ocular manifestation of TAK is retinopathy, first described by
Takayasu [41] who noted peculiar fundus changes consisting of dilated vessels around the
optic disc due to arteriovenous (AV) anastomoses in female patients without a palpable radial
pulse. Less common ocular ischemic manifestations include anterior ischemic optic
neuropathy (AION), central retinal artery occlusion, and ocular ischemic syndrome [29, 42].
The retinal changes in TAK vary in symptomatology and severity depending on the location
of carotid artery involvement, the rate and duration of ocular vascular hypoperfusion, and the
presence of collateral blood supply to the eye [38].

Uyama and Asayama [30] studied 80 patients with TAK noting that 38 (48%) of patients
had involvement of the aortic arch and stenosis or occlusion of its proximal branches denoting
type I TAK; 25 (31%) patients had type II TAK characterized by involvement of the whole
aorta and its branches, and 16 (20%) had type III TAK characterized by involvement of the
descending thoracic and abdominal aorta and its branches. Altogether, 38 (100%) of patients
with type I TAK had retinopathy. Uyama and Asayama (30) classified retinopathy into stages
1 to 4 wherein 20 (53%) patients with stage 1 had dilations of small vessels; 12 (32%)
patients with stage 2 disease had microaneurysm formation; 3 (8%) patients each with either
stage 3 or 4 disease, respectively manifested AV anastomoses or ocular complications of
vitreous hemorrhages, proliferative retinopathy and vision loss . The authors attributed the
retinal vascular changes to decreasing blood flow in the retinal vasculature noting decreased
intraocular pressure with disease progression. In contrast to type I TAK, hypertensive fundal
changes predominated in patients with type III disease with 12 (86%) demonstrating
hypertensive changes occurring in the fundus, and 2 (14%) manifesting hypertensive
retinopathy.

Chun and colleagues [38], who reviewed medical records and FFA of 156 eyes from 78
patients with TAK, noted that the commonest ocular symptoms were subjective decrease of
vision in 23 (29.5%) patients; amaurosis fugax in 20 (25.6%) patients; ocular pain in 9
(11.5%) patients; and metamorphopsia in 3 (4%) patients. Among 13 cases of retinopathy,
69% occurred in patients with TAK involving primarily the aortic arch and its branches, and
among 25 patients with hypertensive retinopathy, 18 (72%) patients had involvement of the
thoraco-abdominal aorta, its branches, and the aortic arch. Thus, patients with retinopathy
were more likely to have carotid artery or aortic arch involvement, while those with
hypertensive retinopathy were more likely to have involvement of the descending aorta.

The importance of staging retinopathy was highlighted by Chun and coworkers [38] who
demonstrated that visual acuity (VA) dramatically worsened as patients progressed from stage
3 to stage 4 retinopathy. The best corrected VA in patients with Stage 1 to Stage 3 retinopathy
ranged from 20/15 to 20/30, while those with stage 4 disease had VA ranging from 20/200 to
hand motion alone. Causes of impaired VA in such patients included total cataract in 4
patients, neovascular glaucoma in 2 patients, vitreous hemorrhages in 1 patient, tractional
retinal detachment in 3 patients, optic atrophy in 3 patients, and AION in 1 patient. Peter and
coworkers [29] demonstrated an association between VA and retinopathy, noting a causal
relation between uncorrected refractive error among 18 patients that was due to a cataract in 6
(33%) patients, grade 4 retinopathy in 4 (22%) patients, optic atrophy in 2 (11%) patients,
hypertension with retinal detachment, longstanding papilledema, and retinal pigment changes
of the fovea, and a posterior capsular opacity each in 1 (6%) patient. Although there was no
correlation of patient age, duration of symptoms, history of limb claudication, presence of a
carotid bruit, hemiplegia, or increased ESR or C-reactive protein (CRP) levels with the

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