shoulder girdle is the Þrst to become symptomatic; in the remainder, hips and neck are involved at the onset. Morning stiffness and ÒgellingÓ after inactivity are common. PMR may be the initial symptom of GCA in 20Ð40% of patients. Conversely, 15% of patients with PMR may have GCA [397]. PMR is not associated with blindness, stroke, and death per se [395], but may be indirectly associated because of its relationship with GCA [398]. If patients with PMR, even those who are Òadequately treated,Ó [396] develop complaints suggestive of vasculitic involvement, an ESR should be obtained as soon as possible and, if elevated, treatment with high-dose oral steroids should be started immediately.

6.1.12.3 Ocular Manifestations

Ocular involvement usually occurs several weeks after the onset of systemic manifestations. The most frequent ocular symptom is vision loss. Rates of vision loss as high as 35Ð50% have been reported in early reports [399Ð401], but more recent series present a lower rate of vision loss (7Ð8%), probably reßecting earlier recognition and treatment [383, 393]. Vision loss in GCA occurs as a result of arteritic anterior ischemic optic neuropathy (AION) or central or branch retinal artery occlusion. Arteritic AION in GCA is due to occlusion of the two main posterior ciliary arteries that supply the optic nerve and choroid. Arteritic AION in GCA is characterized by transient monocular visual loss or amaurosis fugax, sometimes alternating between the two eyes, persisting for 2Ð3 min, and rarely lasting more than 5Ð30 min. Amaurosis fugax is one of the most important warning symptoms of impending blindness. If the diagnosis and treatment of GCA are missed, sudden profound vision loss, usually to less than 20/200, may occur in 40Ð50% of patients. It is often bilateral, either simultaneously or sequentially. Other manifestations are color vision deÞcit, relative afferent pupillary defect (Marcus Gunn pupil), and severe disk edema. Fundus examination may help to distinguish arteritic AION from nonarteritic AION by revealing the chalky appearance of an edematous disk, a cilioretinal artery occlusion, or cottonwool spots [402].

Diplopia is also a common complaint in GCA [391] and may be the presenting symptom of GCA. Diplopia is due to sixth nerve palsy in half of the patients; third nerve palsy accounts for the other half. However, ocular motility disturbances in GCA may be the result of muscle ischemia rather than nerve ischemia.

Typically, there is no conjunctival, corneal, scleral, episcleral, uveal, or retinal vasculitis in GCA because the arteritic process usually involves only the mediumand large-sized arteries, sparing the ocular tissues. Occasionally, however, small vessels, such as the anterior ciliary and long posterior ciliary vessels, may be involved, leading to ocular manifestations, such as scleritis [403].

Scleritis

Scleritis is rare in GCA with or without PMR [132]. However, the diagnosis of GCA must be considered in an elderly patient with a high ESR, particularly in cases of diplopia or vision loss as symptoms and AION, central retinal artery occlusion, or branch retinal artery occlusion as signs. The reported incidence of GCA with PMR in patients with scleritis ranges from 0.58 to 0.68% [123, 124]. Scleritis may correlate with the activity of the systemic disease [204]. In our series of 500 patients with scleritis, one patient had GCA associated with PMR (0.2%). The patient was an 85-year-old female diagnosed with GCA and PMR, with diffuse scleritis and anterior uveitis after choroidal folds and retinal deposits. There were no signs of disk edema. The ESR was 88 mm/h. Increased doses of steroids controlled the scleral inßammatory process as well as the ESR. There were no patients with GCA in our series of patients with episcleritis.

6.1.12.4 Laboratory Findings

An elevated ESR is characteristic of most cases with GCA, although 5Ð10% of patients have a normal sedimentation rate [391, 404]. Because a normal sedimentation rate does not exclude the diagnosis, elevations of other acute-phase reactants, such as CRP, Þbrinogen, and haptoglobin, may be helpful in making the diagnosis [405, 406]. Leukocytosis and anemia are variably present.