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Henry A. Ferreyra, MD; Thiran Jayasundera, MD; Naheed W. Khan, PhD; Shirley He, PhD; Ying Lu, PhD; John R. Heckenlively, MD

Arch Ophthalmol. 2009;127(4):390-397.

Objective  To report the results of treating autoimmune retinopathy (AIR) with immunosuppression therapy.

Methods  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.

Results  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).

Conclusions  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.

Author Affiliations: Department of Ophthalmology, Kellogg Eye Center, University of Michigan, Ann Arbor (Drs Ferreyra, Jayasundera, Khan, He, Lu, and Heckenlively); and Department of Ophthalmology, Shiley Eye Center, University of California, San Diego (Dr Ferreyra).

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