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6.1 Systemic Immune-Mediated Disease-Associated Scleritis: Vasculitides

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incidence in adult RA than in the general population [140]. In our series of 32 patients with rheumatoid scleritis, seven patients (22%) had associated uveitis at some point during the course of the disease. The incidence of anterior uveitis in our series of 468 patients with nonrheumatoid scleritis was similar (27%), indicating that there is no correlation between anterior uveitis and RA per se.

Glaucoma

Increased intraocular pressure may be caused by the adjacent rheumatoid scleral inßammation with or without uveitis. The reported incidence of glaucoma in patients with rheumatoid scleritis is approximately 19% [114], although a histological study of enucleated eyes increases the percentage to 45% [127]. The mechanisms implicated in the development of glaucoma in patients with rheumatoid scleritis are the same as the ones implicated in patients with nonrheumatoid scleritis (see Sect. 4.2.6.3).

Cataract

Long-term systemic steroid treatment in patients with RA without scleritis may lead to the development of posterior subcapsular cataract. The combination of long-term systemic steroid treatment with long-standing rheumatoid scleritis with or without uveitis increases the risk of development of posterior subcapsular cataract by threefold [114].

Retinal, Choroidal, and Optic Nerve Changes

Funduscopic changes in patients with RA can be seen in association with posterior scleritis, including choroidal folds, retinal striae, subretinal mass, annular ciliochoroidal detachment, serous retinal detachment, disk edema, and macular edema [131].

Funduscopic changes in patients with RA, although uncommon, also can be seen in the absence of scleritis. Cotton-wool spots have been correlated with exacerbation and improvement of RA [141, 142]. Ischemic optic neuropathy and posterior ciliary arteritis have been reported in a patient with rheumatoid vasculitis [143].

Motility Disturbances

Motility disturbances have been found to occur in 12.9% of patients with rheumatoid scleritis, although this incidence is not signiÞcantly different when compared with patients with nonrheumatoid scleritis [125].

Some ocular motility disturbances in rheumatoid scleritis may be the result of the extension of scleral inßammation, particularly posterior scleritis, to the extraocular muscles [114]. Symptoms and signs of orbital myositis in posterior scleritis are pain, diplopia, visual loss, chemosis, lid edema, and limitation of ocular movements. In our series of patients with rheumatoid scleritis, the only patient who had posterior scleritis also had restriction of the medial rectus muscle.

Whether or not associated with rheumatoid scleritis, formation of rheumatoid nodules on the posterior tendon of the superior oblique muscle may account for the occurrence of BrownÕs syndrome, which consists of inability to raise the adducted eye above the mid-horizontal plane, with a smaller elevation deÞciency when the eye is in abduction [144, 145]. Slight downshoot of the adducting involved eye is often present. Diplopia, particularly noted in upgaze, and a clicking feeling as the tendon of the superior oblique passes through the trochlea, may be the presenting symptoms. One of our patients with rheumatoid scleritis had BrownÕs syndrome. Rheumatoid vasculitis with or without rheumatoid scleritis may involve the nervous system, leading to pupillary abnormalities and oculomotor palsies [146Ð148].

Episcleritis

There is no evidence of any signiÞcant relationship between episcleritis and RA [107]. The reported incidence of RA in patients with episcleritis ranges from 4.4 to 5.7% [114, 121Ð123]. Conversely, the incidence of episcleritis in patients with RA, as reported in one study [114], is 0.17% (7 of 4,210 RA patients). Rheumatoid episcleritis affects women more frequently than men, is most common in the sixth decade of life, and is unilateral as commonly as it is bilateral [114]. Episcleritis in RA may be simple or nodular [114, 122]. In our own series of 85 patients

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