Management of autoimmune retinopathies with immunosuppression.
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.
Ferreyra
Henry A
HA
Department of Ophthalmology, Kellogg Eye Center, University of Michigan, Ann Arbor, USA.
Jayasundera
Thiran
T
Khan
Naheed W
NW
He
Shirley
S
Lu
Ying
Y
Heckenlively
John R
JR
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood; D001324:Autoantigens / Q000276:immunology; D001327:Autoimmune Diseases / Q000188:drug therapy* / Q000276:immunology; D001379:Azathioprine / Q000627:therapeutic use*; D015153:Blotting, Western; D002648:Child; D016572:Cyclosporine / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D004591:Electrophoresis, Polyacrylamide Gel; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008269:Macular Edema / Q000188:drug therapy / Q000276:immunology; D008297:Male; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000188:drug therapy / Q000276:immunology; D011241:Prednisone / Q000627:therapeutic use*; D012164:Retinal Diseases / Q000188:drug therapy* / Q000276:immunology; D012189:Retrospective Studies; D016896:Treatment Outcome
D001323:Autoantibodies; D001324:Autoantigens; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D001379:Azathioprine; D011241:Prednisone