Page 342 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

316 Vanessa Quick and John Kirwan

Constitutional Findings

With the widespread availability of glucocorticoids in the empiric treatment of GCA,
constitutional symptoms such as fever, fatigue, malaise, anorexia and weight loss are less
often encountered at the formal diagnosis of affected patients [55]. Fever which occurs in
about one-half of patients is usually low grade. A small number of TAB-proven patients with
GCA present with only symptoms of systemic inflammation without localizing vascular
symptoms [29, 51, 56].

Ophthalmologic Involvement

An estimated 15% of patients with GCA experience ophthalmologic complications [57],
notably ischemic optic neuropathy (AION) due to arteritic involvement of the short posterior
ciliary arteries supplying the optic nerve head [58, 59], with the remainder comprised mainly
of retinal blindness due to central retinal artery involvement [58, 59]. Visual loss is painless,
partial or complete, and unilateral or bilateral; and once established it is usually irreversible
[38]. It may be preceded by fleeting visual blurring with exercise, amaurosis fugax or
diplopia, but commonly occurs without warning and may be the presenting symptom [46, 60].
Ophthalmoplegia is usually due to a partial or complete oculomotor or abducens nerve palsy,
and is a recognized complication of ischemia affecting the extra-ocular muscles, cranial
nerves or brainstem [14].

Other ischemic complications of GCA include transient ischemic attack (TIA) and stroke
which may occur due to thrombosis, microembolism or a combination of intimal hyperplasia
and distal thrombosis [55]. Although the vertebral arteries are inflamed in the large majority
of patients at postmortem examination [49], clinically significant vertebrobasilar insufficiency
is uncommon [55]; scalp and tongue necrosis are rare [61].

Polymyalgia Rheumatica

Although up to one-half of patients with GCA develop inflammatory shoulder and hip
girdle pain and stiffness [62], only about 5% of patients with frank PMR ever develop GCA
[7].

Large Vessel Involvement

Aortic inflammation can be observed in surgical biopsies or at postmortem examination
in GCA [5, 63] however the true frequency is difficult to ascertain. Computed tomography
angiography (CTA) [6] and helical aortic computed tomodensitometry [64] discern aortic
involvement in 45 to 65% of patients with GCA, with the thoracic aorta most often affected.
The relationship between aortitis and subsequent aortic aneurysm remains unclear [65]. In the
first year of the disease, aortitis confers a small significant risk of dissection or rupture even
in the absence of aortic aneurysm with resultant high mortality. Long term dilatation or
aneurysm formation can occur with an increased risk of rupture [66, 67]. Retrospective

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