Date of download: 7/2/2016 Copyright © 2016 American Medical Association. All rights reserved. From: Management of Autoimmune Retinopathies With Immunosuppression Arch Ophthalmol. 2009;127(4): doi: /archophthalmol Patient 30. Treatment effect in cancer-associated retinopathy. An 83-year-old woman had a 4-year history of central and peripheral vision loss, a medical history of breast cancer, and no family history of retinitis pigmentosa. She had antiretinal antibodies (Table 2). A, Her examination showed visual acuity of 20/300 OU, generalized retinal and retinal pigment epithelial atrophy with peripheral pigmentary deposits, and pale optic nerves. B, The electroretinogram showed rod and cone dysfunction, with a negative waveform. C, Goldmann visual fields showed constriction of all isopters. The patient was unable to tolerate azathioprine or mycophenolate mofetil therapy, and she stopped taking these drugs after 30 and 8 days, respectively; her condition has been maintained every 2 months with subtenon injections of methylprednisolone acetate (40 mg) in both eyes as an alternative to systemic immunosuppression therapy. D, She showed a better response (right eye [OD], 88%; left eye [OS], 163%; IV-4 isopter) on Goldmann visual fields from her base maintained by methylprednisolone acetate therapy after the brief period of treatment with mycophenolate mofetil. E, Pretreatment and posttreatment Western blots show less immunoreactivity after treatment. Samples were heated at 80°C for 10 minutes. BR indicates bovine retina extract; HR, human retina extract; and MR, mouse retina extract. Figure Legend:

Date of download: 7/2/2016 Copyright © 2016 American Medical Association. All rights reserved. From: Management of Autoimmune Retinopathies With Immunosuppression Arch Ophthalmol. 2009;127(4): doi: /archophthalmol Patient 23, with posterior pole cysts treated with immunosuppression. A, A 21-year-old man with nonparaneoplastic autoimmune retinopathy with cystoid macular edema was initially seen with diffuse cystic changes of the posterior pole. He had antiretinal antibodies (Table 2) and, on initial examination in 1999, his visual acuity was 20/200 OD and 20/300 OS, which improved to 20/25 OD and 20/30 OS by 2007 with immunosuppression therapy. His electroretinogram (ERG) showed barely recordable rod-isolated ERG, whereas the photopic b-wave amplitude was 50% of normal, with a suggestion of a negative wave in the right eye. B, The bright flash dark-adapted ERG had negative waveforms and was diminished. C, Optical coherence tomography showed reduction of the cystic changes in the posterior pole to a thinner, but near normal appearance during 4 years of immunosuppression therapy. Goldmann visual fields (not shown) showed clearing of central scotomata. His grandmother had retinitis pigmentosa, but he had no pigmentary changes in his retina and otherwise met the criteria for autoimmune retinopathy. Figure Legend:

Date of download: 7/2/2016 Copyright © 2016 American Medical Association. All rights reserved. From: Management of Autoimmune Retinopathies With Immunosuppression Arch Ophthalmol. 2009;127(4): doi: /archophthalmol Patient 11, with nonparaneoplastic autoimmune retinopathy. A 27-year-old man had a 3-year history of peripheral vision loss, nyctalopia, and a medical history of asthma but no family history of retinitis pigmentosa. His visual acuity was 20/20 OU. A, He had generalized retinal and retinal pigment epithelial atrophy, vascular attenuation, mild optic nerve pallor, and no pigment deposits. B, The electroretinogram was nonrecordable. C, Goldmann visual fields were severely contracted, but D, they were re-expanded moderately after treatment with systemic immunosuppressive agents: prednisone (20-40 mg/d), azathioprine (100 mg/d), and cyclosporine (100 mg/d). E, Comparison of baseline and treated sera shows a decrease in antiretinal antibodies on the Western blots. Both blots were run together (earlier serum was unfrozen, which has been shown to not affect activity) and then side by side on the same gel. Samples were heated at 80°C for 10 minutes. BR indicates bovine retina extract; HR, human donor retina extract; kDa, kilodaltons; MR, mouse retina extract; OD, right eye; and OS, left eye. Figure Legend: