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6.1 Systemic Immune-Mediated Disease-Associated Scleritis: Vasculitides

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involvement. Patients with UC or CD with other manifestations, such as skin lesions (erythema nodosum and pyoderma gangrenosum), Þnger clubbing, mouth ulceration, or uveitis, are more likely to develop arthritis. Proctocolectomy may lead to remission of arthritis in many patients with UC but in only a small number of patients with CD [268].

Sacroiliitis with or without spondylitis, indistinguishable from AS, usually precedes the intestinal involvement and progresses independently of the bowel disease or proctocolectomy [268].

6.1.6.3 Ocular Manifestations

The reported incidence of ocular manifestations in patients with IBD ranges from 1.9 to 11.8% [269Ð272]. The most common eye Þndings in IBD patients include episcleritis, anterior uveitis, keratitis, and scleritis; conjunctivitis, macular edema, serous retinal detachment, choroidal inÞltrates, orbital pseudotumor, extraocular muscle paresis, retrobulbar neuritis, papillitis, orbital cellulitis, and myositis are seen less frequently [273, 274]. Ocular involvement in CD or UC patients is more common in those with arthritis [269, 270, 274]; other extraintestinal manifestations, such as anemia, skin lesions, liver disease, and oral ulcers, also are frequently associated with ocular disease. Ocular involvement in CD patients is more likely in those with colitis or ileocolitis than in patients with small bowel involvement alone [274]. Although eye lesions may precede the bowel disease, they often occur with exacerbation of colitis [269, 272Ð275]. After proctocolectomy, the ocular prognosis is variable [269]. Because effective treatment of the bowel disease may improve the ocular and systemic prognoses, patients with ocular manifestations and gastrointestinal symptoms must be studied to deÞne the nature of their gut involvement. The ophthalmologist may be the Þrst to diagnose an IBD.

Anterior Uveitis

Anterior uveitis is usually nongranulomatous and recurrent, with Þne white keratic precipitates, moderate cells, and ßare. It may occur before, during, or after the initial bowel attack, and often is associated with the presence of arthritis, particularly

spondylitis [270, 271, 274]. IBD must always be considered in the differential diagnosis of anterior uveitis.

Scleritis

The reported incidence of IBD in patients with scleritis ranges from 2.06 to 9.67% [121, 123, 124]. It is more common in patients with arthritis, anemia, skin manifestations, oral ulcers, or liver disease than in patients without extraintestinal manifestations [271, 274]. Although scleritis may precede bowel disease, it usually occurs some years after the onset of gut symptoms, particularly during active episodes [272, 274, 276]. Scleritis associated with IBD is recurrent and may take the form of almost any type of scleritis, including necrotizing anterior scleritis [204, 274, 277]. Treatment of the bowel manifestations may control the ocular condition. Knox et al. [271] reported that the presence of scleritis or episcleritis in a patient with IBD is useful in differentiating CD and UC because, in their experience, these ocular lesions are not associated with UC.

In our own series of 500 patients with scleritis, 11 patients had IBD (2.2%), nine had CD, and two had UC. The patients were eight women and three men with a mean age of 47 years (range, 33Ð67 years). All nine patients with CD had arthritis. Other extraintestinal manifestations included anemia, sclerosing colangitis, and oral ulcers. In two patients, scleritis was the initial manifestation whose study led to the diagnosis of CD. Six patients had diffuse scleritis, one patient had nodular scleritis, one patient had necrotizing scleritis, and one patient had posterior scleritis. The scleritis was persistent or recurrent, and often related to episodes of active bowel disease. There were anterior uveitis in two patients, peripheral keratitis in three patients, and glaucoma in one patient, but Þnal visual acuity was not affected. Scleritis occurred after several episodes of anterior uveitis in two patients. Any patient who develops scleritis after recurrent anterior uveitis should be examined for CD. CrohnÕs disease must always be included in the differential diagnosis of a patient with scleritis.

The two patients with UC had diffuse scleritis with anterior uveitis. There were no corneal

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