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Pooja Bhat, Kanish Mirchia, Surendra Basti, Robert Feder, Dmitry Pyatetsky; Necrotizing Scleritis- A Case Series and Review of Literature. Invest. Ophthalmol. Vis. Sci. 2012;53(14):1253.
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Necrotizing scleritis is a rare and severe form of scleritis that can threaten both vision and integrity of the eye with limited published information on its presentation,course, treatment and outcome. The purpose of our study is to identify patients with necrotizing scleritis diagnosed and treated at the Northwestern University’s Ophthalmology Department and compare our experience with that reported in literature.
Retrospective case-series. Seven patients with necrotizing scleritis were identified between 2008-2011. Information regarding the duration of scleritis, underlying autoimmune conditions, prior ocular surgeries, and systemic immunomodulatory therapy administered was collected. Ocular complications including impending or actual perforation and its management were recorded. A review of literature was performed between 1990 and 2011 and thirty two articles were identified.
In our series, 6 patients had underlying autoimmune diseases which included rheumatoid arthritis (n=3), and Wegener’s granulomatosis (n=3). One patient had surgically induced necrotizing scleritis (SINS) after pterygium excision with mitomycin C. The average duration of scleritis prior to the onset of necrosis was 2.8 yrs. Three patients developed bilateral necrosis and 2 had painless necrosis. Perforation requiring emergent patch grafting occurred in 2 patients. At the onset of scleral necrosis, intravenous cyclophosphamide (n=2), rituximab (n=1), infliximab (n=1) and oral methotrexate (n=3) was used in combination with systemic corticosteroids in all patients. Maintenance was achieved using methotrexate (n=5), mycophenolate mofetil (n=1) and prednisone (n=1). Complications from systemic immunomodulation included pancytopenia, pneumonia, and hepatitis which resolved on cessation of the offending agent. None of the patients developed recurrent necrosis during their follow-up. Categories among the published reports were SINS (n=15), autoimmune (n=13), masquerade (n=3) and infection (n=1). Pterygium excision, rheumatoid arthritis and squamous cell carcinoma were the most common factors in the first 3 categories while the infectious agent was acanthamoeba. The largest case-series of 4 patients was described in the SINS group while the most common medication used in the autoimmune group was infliximab.
Necrotizing scleritis may be associated with life-threatening systemic conditions requiring potent immunomodulatory therapy and can occur after uncomplicated ocular surgery. All our patients were successfully managed with a systematic approach, newer biologic agents and patch grafting when necessary, with preservation of the eyeball in all cases.
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