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      Management of autoimmune retinopathies with immunosuppression.

      Archives of ophthalmology (Chicago, Ill. : 1960)
      Adolescent, Adult, Aged, Aged, 80 and over, Autoantibodies, blood, Autoantigens, immunology, Autoimmune Diseases, drug therapy, Azathioprine, therapeutic use, Blotting, Western, Child, Cyclosporine, Drug Therapy, Combination, Electrophoresis, Polyacrylamide Gel, Female, Humans, Immunosuppressive Agents, Macular Edema, Male, Middle Aged, Paraneoplastic Syndromes, Prednisone, Retinal Diseases, Retrospective Studies, Treatment Outcome

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          Abstract

          To report the results of treating autoimmune retinopathy (AIR) with immunosuppression therapy. Retrospective review of 30 consecutive patients with AIR followed for 3 to 89 months (median, 17 months) who were treated with immunosuppression (systemic or local). Subgroups were cancer-associated retinopathy (CAR), nonparaneoplastic AIR (npAIR), and npAIR with cystoid macular edema (npAIR/CME). Outcome measures were improvement of Snellen visual acuity by at least 2 lines, expansion of the visual field area by more than 25%, and resolution of CME. Overall, 21 of 30 patients (70%) showed improvement. All 6 CAR patients, 7 of 13 (54%) with npAIR, and 8 of 11 (73%) with npAIR/CME showed improvement. Five of 21 patients (24%) had improvement in visual acuity, 15 of 21 (71%) had expansion of visual field area, and 6 of 11 (55%) had resolution of CME. Twenty-six of 30 patients exhibited diffuse retinal atrophy without pigment deposits. An autoimmune family history was common in all the groups: npAIR, 69% (9 of 13); npAIR/CME, 64% (7 of 11); and CAR, 50% (3 of 6). Long-term treatment with immunosuppression resulted in clinical improvement in all subgroups of AIR. The most responsive subgroup was CAR; the least was npAIR. These results challenge the commonly held belief that AIR is untreatable.

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