Both hemifield contain point(s) 15 dB within 5 of fixation;22 or smooth or near flat 30-Hz flicker amplitude and/or combined a-wave and b-wave amplitudes

Both hemifield contain point(s) 15 dB within 5 of fixation;22 or smooth or near flat 30-Hz flicker amplitude and/or combined a-wave and b-wave amplitudes. We looked for stability or improvement of visual acuity, HVF patterns and parameters (total deviation, pattern deviation, mean deviation, and pattern standard deviation), and electroretinography parameters (scotopic rod response amplitude, maximal RSV604 combined a-wave and b-wave amplitudes, 30-Hz flicker amplitude, and 30-Hz flicker implicit time) at the most recent visit. (50%) eyes and improved in two (16.7%) eyes. Electroretinography was stable or improved in eight (66.7%) eyes. The average number of anti-retinal and anti-optic nerve antibody bands was reduced. Conclusion Stabilization and/or improvement of visual acuity, visual field parameters, and electroretinography parameters were observed in a high number of patients (75%) on rituximab, as a monotherapy (one patient) or in combination therapy. strong class=”kwd-title” Keywords: birdshot retinochoroidopathy, bortezomib, CD20, cyclophosphamide, HLA-A29, HLA-B27, immunomodulatory therapy, systemic lupus erythematosus Introduction Autoimmune retinopathy (AIR) is an immune-mediated retinopathy that can cause acute, subacute and chronic vision loss, visual field defects, photopsias, nyctalopia, and color vision defects.1,2 AIR is characterized by the presence of circulating anti-retinal antibodies that are believed to damage photoreceptors in the retina; however, the exact mechanism is not entirely comprehended.3,4 AIR is subdivided into paraneoplastic (cancer-associated retinopathy and melanoma-associated retinopathy) and non-paraneoplastic categories. Non-paraneoplastic AIR is composed of a large group of autoimmune retinopathies with features similar to those of carcinoma-associated retinopathy, but without any known underlying malignancy. Non-paraneoplastic AIR is usually diagnosed at a younger age,4 in people with a known personal and/or familial history of autoimmune diseases.2,5,6 Long-term immunosuppressive therapy is considered the mainstay of treatment for AIR.7 Different regimens of immunomodulatory therapy, such as corticosteroid,8 plasmaphoresis,9 intravenous immunoglobulin,10 cyclosporine,6 and mycophenolate mofetil,6 have been used in the treatment of paraneoplastic and non-paraneoplastic AIR. However, the rarity of this entity combined with difficulty in confirming the diagnosis and monitoring the response to treatment makes treatment challenging.5 Rituximab, a cluster of differentiation 20 molecule inhibitor (CD20), has been used as a treatment option for carcinoma-associated retinopathy in limited studies.11,12 There is also a case report using rituximab in the treatment of non-paraneoplastic AIR.13 RSV604 The aim of this study is to examine the efficacy of rituximab as a monotherapy or in combination therapy in preserving or improving visual function parameters in patients with non-paraneoplastic AIR. Methods This was a single-center, retrospective interventional case series of patients with non-paraneoplastic AIR who were treated at the Massachusetts Vision Research and Surgery Institution (MERSI) between September 2010 and January 2015. Each patient had at least 6 months of follow-up from when the diagnosis of non-paraneoplastic AIR was made. Ethical approval for this study was obtained through the New England Institutional Review Board, which issued a waiver of written informed consent based on standard operating procedures for retrospective Bglap chart reviews. This study was performed in accordance with the Declaration of Helsinki and was Health Insurance Portability and Accountability Act compliant. Diagnosis of AIR was made based on four essential criteria with subjective symptoms as supportive criteria.5 Essential criteria included no evidence of systemic malignancies or infections after a complete history was taken and a physical examination and appropriate testing were performed; no evidence of a preexisting degenerative vision disease; confirmed progressive abnormality on repeated electroretinography and/or visual field; and the presence of anti-retinal and/or anti-optic nerve antibodies. Supportive criteria were the acute or subacute presence of symptoms, such as decreased vision, visual field defects, nyctalopia, photoaversion, photopsia, and color vision deficits in patients without active anterior or posterior uveitis. Blood samples and pertinent medical history of each patient were collected at MERSI and were sent to the commercially available testing center at Oregon Health Sciences University. Western blots were used to evaluate the presence of RSV604 the anti-retinal and anti-optic nerve antibodies. These tests were repeated every 6 months. All patients underwent fluorescein angiography, indo-cyanine green (ICG) angiography, full-field electroretinography, and automated perimetry. Optical coherence tomography was obtained as part of the initial examination or follow-up examinations in all patients with clinical evidence of decreased central visual acuity at any time or angiographic suspicion of macula edema. Patient demographics, RSV604 including personal and family histories of autoimmune diseases, were collected. Best corrected visual acuity and related logarithm of the minimum angle of resolution (LogMAR) were recorded for every patient at the initial.