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J. R. Heckenlively, H. Ferreyra, N. W. Khan, P. Titus, S. He, Y. Lu; Retrospective Analysis of Treatment Results in Autoimmune Retinopathy. Invest. Ophthalmol. Vis. Sci. 2008;49(13):5828. doi: https://doi.org/.
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Autoimmune retinopathy (AIR) characteristically presents with photopsias, rapid vision loss, and few ophthalmoscopic signs initially, but the electroretinographic changes including negative waveforms and positive western blots for anti-retinal antibodies, family histories of autoimmune diseases, and in some cases history of carcinoma help make the diagnosis. Occasional RP patients may present with AIR exacerbation (RP/AIR). When diagnosed, AIR patients should be treated with immunosuppression to try to arrest the progress of the condition. We retrospectively evaluated the results of immunosuppression treatment in a group of AIR patients.
AIR patients were studied in whom a minimum of four months of treatment was given and where there was at least six months follow-up evaluation. Best corrected visual acuities (BCVA), kinetic visual fields (VF), OCT, and as indicated, repeat electroretinograms were performed. Serum was drawn for Western blots on most visits. Parameters followed were visual field size, size of scotomata, best corrected visual acuities (BCVA), thickness of retina and presence of macula cysts, and number of bands on the Western blot. Treatments included prednisone, subtenons depomedrol, azithioprine, oral cyclosporine, and mycophenalat.
Thirty one patients were identified who met study criteria; these included 6 with a diagnosis of CAR, 16 with a diagnosis of non-neoplastic AIR (npAIR), and 9 with a diagnosis of RP/AIR. The group was predominantly female [74% female (23/31) versus 26% male (8/31)] and had a median age of 51 years. Overall, the response to treatment was 65% (20/31). When examined by subgroups, the responses were 83% (5/6), 50% (8/16), and 78% (7/9), for CAR, npAIR, and RP/AIR, respectively. At least one systemic immunosuppressive medication was discontinued in 10/30 patients (33%) due to side effects.
One of the lessons to be learned from this limited experience is that AIR patients (CAR, npAIR, RP/AIR), need appropriate immunosuppression that has to be administered over an extended period of time. Short pulse doses of steroids do not usually work in these patients. We found that there was a correlation between improvement or stabilization of visual fields and the disappearance of activity on Western blots. The patients with autoimmune family histories tended to be more resistant to treatment.
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