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A 53-year-old Caucasian woman with long-standing, well controlled, severe rheumatoid arthritis, treated with methotrexate, salazopyrin, naprosyn, prednisone and plaquenil, presented with progressive visual loss in each eye. She had a past history of non-necrotizing anterior scleritis that was treated with increased doses of prednisone. She developed left then right central scotomas, reduced vision and optic atrophy. Eventually a diagnosis of methotrexate-induced optic atrophy was made.

Purpose: To compare the long-term efficacy of different systemic therapeutic regimens for patients with noninfectious anterior scleritis to establish guidelines for institution of therapy. Methods: Therapeutic failure of systemic nonsteroidal anti-inflammatory drugs (NSAIDs), systemic steroidal anti-inflammatory drugs, and systemic nonsteroidal immunosuppressive drugs was evaluated in 132 patients with noninfectious anterior scleritis (diffuse, nodular, or necrotizing types). Results: In patients with diffuse scleritis, therapeutic failure for initial regimens occurred in 7% of patients treated with NSAIDs, in 16% of patients treated with steroids, and in 27% of patients treated with immunosuppressive drugs. In patients with nodular scleritis, therapeutic failure for initial regimens occurred in 9% of patients treated with NSAIDs, in 28% of patients treated with steroids, and in 25% of patients treated with immunosuppressive drugs. Addition or substitution of steroids or immunosuppressive drugs as second- or third-line therapies helped control the scleritis. In patients with necrotizing scleritis, therapeutic failure for initial regimens occurred in 100% of patients treated with NSAIDs, in 91% of patients treated with steroids, and in 26% of patients treated with immunosuppressive drugs. Conclusions: In patients with diffuse and nodular scleritis, NSAIDs should be the initial choice; in case of therapeutic failure, steroids should be added or substituted as second-line therapy, tapering and discontinuing them as soon as possible while maintaining remission with continued NSAIDs; in case of therapeutic failure, immunosuppressive drugs should be added or substituted as third-line therapy. In patients with necrotizing scleritis, immunosuppressive drugs should be the initial choice.

The ocular manifestations of rheumatoid arthritis (RA) are common and they can vary from patient to patient. However, necrotizing anterior scleritis without inflammation (scleromalacia perforans) is a rare and serious opthalmic complication, and it is typically associated with long-standing RA. Although the etiology and pathogenesis of scleromalacia perforans are diverse and they are not completely understood, ophthalmic surgery is one of the well known causes of scleromalacia perforans. Patients with systemic autoimmune disease such as RA have an especially higher risk of scleromalacia perforans after opthalmic surgery. Because scleromalacia perforans is a potential threat not just to eyesight, but to life as well, early diagnosis and prompt treatment are required for its successful management. We experienced a case of scleromalacia perforans that developed after scleral excision of pterygium in a 58 year old woman who had a 7 year history of RA, and this was well treated with an early screral graft. We report here on this case along with a review of the relevant literature.