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D. Rajguru, J.R. Smith, L.K. Gordon, J.T. Rosenbaum; Scleritis and Optic Neuropathy . Invest. Ophthalmol. Vis. Sci. 2005;46(13):2848.
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Purpose: Although optic nerve involvement is a recognized complication of posterior scleritis, the association is rare and not previously described in a series of patients to our knowledge. This report seeks to characterize the association. Methods: A retrospective chart review of patients seen either at the Inflammatory Eye Disease Clinic at the Casey Eye Institute or in a neuro–ophthalmology clinic at UCLA was performed. Results: Six patients (3 male, 3 female) aged 11–79 (mean 49) with scleritis and optic neuropathy were identified. Their diagnoses included idiopathic (n=3), Waldenstrom's macroglobulinemia (n=1), reactive arthritis (n=1), and pernicious anemia with an indeterminate cANCA and ANA positive autoimmune disorder (n=1). Treatment regimens included: initial oral and topical corticosteroids replaced by steroid–sparing therapy (n=4); prednisone and cyclophosphamide with eventual cessation of both (n=1); and indomethacin followed by a limited course of prednisone (n=1). Two patients were also treated for elevated intraocular pressure, which was considered a major factor in the neuropathy of one. In all patients, it was possible to control symptoms with therapy, but visual outcomes varied. Three patients remained stable with good final visual acuity (20/25 or better, Snellen acuity). One patient experienced a two–line decrease in vision. Another patient progressed to a three–line decrease in vision. One patient suffered severe vision loss to counting fingers visual acuity. All patients followed a chronic course, and only two have discontinued therapy. Conclusions: Optic neuropathy and scleritis rarely occur in combination. However, the recognition of an association had a marked impact on the therapeutic decisions. In this small cohort of six individuals with varied diagnoses, we noted chronic and diverse clinical courses. Although most patients responded well to therapy symptomatically, visual loss was observed in three cases.
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