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4.2 Scleritis

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Uveal effusion syndrome and posterior scleritis may both present with annular ciliochoroidal detachments, bullous serous retinal detachments, and/or serous macular detachments; [136, 137] however, in uveal effusion syndrome, there is minimal or no pain, there may be dilated episcleral vessels but no scleritis, and there is usually bilaterality. Furthermore, there is minimal or no uveitis (some vitreous cells); there are hyperpigmented spots in the retinal pigmented epithelium (Òleopard spotsÓ), and there is a clear subretinal ßuid. Fluorescein angiography in uveal effusion syndrome shows slow perfusion of the choroid and prolonged choroidal hyperßuorescence [138]. Occasionally, there are some focal leaks in the pigment epithelium, but this Þnding is much less common than in posterior scleritis [137]. Ultrasonography shows choroidal thickening and serous ciliochoroid and/or retinal detachments in both entities; however, the detection in some cases of an eye smaller than normal (nanophthalmos) or the Þnding of retrobulbar edema may be helpful for establishing the diagnosis of uveal effusion syndrome or posterior scleritis, respectively. Unlike posterior scleritis, the response to steroids in uveal effusion syndrome is poor.

A history of recent intraocular surgery is helpful in differentiating a postoperative serous ciliochoroidal detachment from one appearing in posterior scleritis.

The Þnding of a retinal break with folds in a retinal detachment with serous ciliochoroidal detachment is characteristic of a rhegmatogenous retinal detachment.

VogtÐKoyanagiÐHarada syndrome should also be considered in the differential diagnosis of posterior scleritis because both may present with bullous serous retinal detachments, serous macular detachments, and, although infrequently in the former, serous ciliochoroidal detachments [139Ð 142]. In both, ßuorescein angiography may show multifocal subretinal leaks. Furthermore, in both, there may be anterior and/or posterior uveitis, disk edema, and cloudy subretinal ßuid. However, VogtÐKoyanagiÐHarada syndrome also presents with signs of integumentary (vitiligo, poliosis, and alopecia), auditory (dysacusis and tinnitus),

and neurological (meningeal inßammation) involvement. Patients with VogtÐKoyanagiÐ Harada syndrome are often orientals or have dark skin pigmentation, and they have bilateral involvement. As in posterior scleritis, ultrasonography in VogtÐKoyanagiÐHarada syndrome shows choroidal thickening and serous retinal detachment; however, unlike in posterior scleritis, the choroidal thickening shows a low internal reßectivity, and there is no retrobulbar edema.

Idiopathic central serous chorioretinopathy and posterior scleritis may have serous macular detachments and/or bullous serous retinal detachments, but in the former there are neither the ocular Þndings of uveitis, anterior scleritis, and disk edema, nor the ultrasonographic Þndings of sclerochoroidal thickening and retrobulbar edema [137].

Disk and Macular Edema

Disk and macular edema may occur in posterior scleritis, in many inßammatory conditions of the uveal tract, and after intraocular surgery [8]. Past and present history, review of systems, ocular examination, laboratory tests, and imaging studies may be helpful in distinguishing these conditions from posterior scleritis.

4.2.5Associated Diseases

Connective tissue diseases, vasculitic diseases, herpes, tuberculosis, and rosacea are the diseases most commonly associated with scleritis [2, 5, 17]. In our series, an associated systemic disease was found in 36% of the patients with scleritis (Table 4.4), including 25% with connective tissue or vasculitic diseases, 10% with infectious diseases, and 1% with miscellaneous diseases (rosacea, foreign body) (Table 4.5). Within the connective tissue diseases and vasculitic diseases, rheumatoid arthritis was the most common entity, followed by HLA-B27+ (without spondyloar- thropathy)-associated scleritis, granulomatosis with polyangiitis (Wegener), relapsing polychondritis, arthritis and inßammatory bowel disease, and systemic lupus erythematosus. Scleritis may

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