A Case of Idiopathic Sclerosing Orbital Inflammation with Exudative Retinal Detachment

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  • 滲出性網膜剥離を伴ったidiopathic sclerosing orbital inflammationの1症例

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Abstract

Idiopathic sclerosing orbital inflammation (ISOI) is a rare inflammatory condition of the orbit, which is distinct from idiopathic orbital inflammation. It is characterized by an orbital mass lesion caused by immune-related cellular infiltrate and tissue fibrosis. We report a woman with ISOI who presented with complaints of hyperemia and pain in the right eye, associated with diplopia. Swelling and ptosis of the right eye lid were present, and exophthalmos and limited eye movements were observed upon examination. In addition, scleritis, uveitis, and exudative retinal detachment were noted. Topical steroid therapy was ineffective. An MRI revealed an intra-orbital mass lesion; positron emission tomography (PET) revealed a hot spot in the same location. The orbital mass was biopsied; lymphocytic infiltration and sclerosis were observed in the tissue on histological examination. Malignant lymphoma, IgG4 related eye disease, and other systemic disorders were ruled out. These observations led us to diagnose the condition as ISOI. Systemic steroids (oral prednisolone, 80 mg/day) were started, followed by a slow tapering over one year. The treatment successfully ameliorated the symptoms with a decrease in the size of the mass. However, the symptoms recurred 2 months later, after cessation of steroid therapy. Systemic steroids were restarted but were ineffective. Thus, physicians should be aware that ISOI can be steroid-refractory in nature and supplemental immunosuppressive therapies or radiation may be required in some cases.

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