 Next, we have one of our home University of Utah students. He's been working with the retina group some, and he's Amit Subhash, and he's going to be presenting on autoimmune retinopathy in a herologiae. Thank you, Julia. My name is Amit Subhash, as she mentioned. I would like to point out that Taylor and I did not work together despite our backgrounds being identical. We're going to be talking about autoimmune retinopathy today. We'll start out with what prompted this discussion with a case, Mr. B, 51-year-old male who presented initially in 2014 for evaluation of scatoma. At that time, he presented stating that he noticed about five months ago that he just had his blind spot in the center of his vision of his left eye. It was gradual non-set and he stated it was just gradually harder to read. He described it as a gray circle around the very center of his vision. He stated that color seemed to be duller in the left when compared to the right. However, his right eye felt totally normal. At that time, his visual acuity was 2016 the left, 2020 in the right. His exam was otherwise normal and he was eventually referred to retina given his persistent complaints. With the retina team, this was later in 2014, he had an OCT, auto fluorescence, visual fields, everything was normal. He did have an ERG that came back abnormal, was followed up with an FF ERG that was stated to be abnormal as well. The patient was thought to have a cone rod dystrophy and six months later, he came back with subjective worsening of his vision. His visual acuity continued to correct in 2020. However, Dr. Bernstein noted atypical progressive cone degeneration with the normal fundus. Autoimmune retinopathy popped up on his differential and he happened to send blood samples to Oregon, to OHSU to detect the presence of antiretinal antibodies. So we'll start out with definitionally what is autoimmune retinopathy. It's an inflammatory mediated retinopathy. It's characterized by vision loss, scatomas, visual field deficits, photoreceptor dysfunction, and the presence of circulating antiretinal antibodies. It generally can be broken down into two categories, perineoplastic and non-perineoplastic. The perineoplastic category can be further broken down into cancer-associated retinopathy, or CAR and melanoma, oh, that's a type of melanoma, associated retinopathy. This phenomenon was actually first described in 1976 by Sawyer at all. They described vision loss and photoreceptor dysfunction associated with cancer. For the purposes of this talk, I focused a little bit more on non-perineoplastic retinopathy, so just to give you that kind of preface. As far as epidemiology, the prevalence of the disease is actually currently unknown. Some groups estimate that it constitutes less than 1% of all ocular immunology cases. However, as with many things, I think we often find that these estimates are under the true values just because we lack the diagnostic capabilities or diagnostic standards to make the call. And so, due to that lack of standardization, I suspect that the prevalence is probably higher. And beyond that, clinical features of disease kind of overlap with a lot of other diseases, so it's difficult to tease out. Like many other autoimmune diseases, there's a predominance of females affected by autoimmune retinopathy. As far as signs and symptoms, patients will often present with things like subacute vision loss, scutomas, photopsia, nyctalopia, photoeversion, dyschromotopsia. Visual acuity generally remains good in early stages of the disease. On exam, the funnest is generally unremarkable. Some literature has shown that patients can have a portfolio reflex. The disease is usually bilateral, but it can be asymmetric. A typical patient presentation would be a woman in her 50s or 60s. No history of visual problems prior to onset of photopsia. Presence of scutoma and no family history of things like RP. And here, so on the left here, this figure just shows what I was mentioning previously about a portfolio reflex. Other groups have described funnest autofluorescence in patients, revealing the sort of outer ring of hyperpigmentation or hyperautofluorescence. And you can clearly see that with arrow sign over here. And additionally, there's been some suggestion that OCTs may have loss of inner segment, outer segment, loss of the inner segment, outer segment, junction with preservation of the phobia. And once again, that's demonstrated with the arrows here. As far as the pathophysiology, so autoimmune retinopathy, it's thought to be triggered by molecular mimicry between tumor antigens and retinal proteins. There's two major antigenic retinal proteins that are currently described, well described in literature, recovering, which is a calcium binding protein in photoreceptor cells and alpha enolase, which is a glycolytic enzyme that's present in many non-retinal tissues, as well as retinal tissues that's a magnesium binding protein and is a transcription factor in DNA synthesis and whatnot. It's possible that the non-perineoplastic forms of the disease may also be triggered by a cross reaction between retinal proteins and presumed viral or bacterial proteins. Recoverant, although it's most commonly described as being associated with a cancer-associated retinopathy, it can also be present in non-perineoplastic forms of autoimmune retinopathy as well. And alpha enolase has been shown to have the same kind of properties as recoverant as far as being present in both perineoplastic and non-perineoplastic forms. As far as how to make the diagnosis, there's actually no, as I was mentioning earlier, there's no current gold standard. However, a paper in 2014 kind of suggested that we have these proposed essential for major criteria, no evidence of malignancy after a thorough workup, no evidence of degenerative eye disease such as RP, a positive screen for serum antiretinal antibodies and abnormalities in ERG findings with or without visual field abnormalities. Some additional supportive criteria are just kind of those signs that patients or symptoms that patients may describe, things that I had already mentioned for topsy, scatoma, and myctolopia, et cetera. Currently, the only commercial testing is available at OHSU, which is why we had sent our patients blood work to OHSU. Dr. Bernstein and Dr. Shakur have joked on many occasion that we should be receiving some sort of financial compensation for keeping their institution afloat with the number of tests we've been sending to them. As far as treatment, for the perineoplastic autoimmune retinopathy, essentially identifying a primary malignancy with CT scans, and then following that up with whatever the indicated cancer treatment would be, whether it be chemo, radiation, et cetera. For non-perineoplastic forms, generally speaking, we're looking at immunosuppressive therapies at this point in time, so systemic or local steroids, IVIG, plasma pharesis. Therapy's not been shown to be very useful once widespread retinal degeneration has occurred, and there's some literature that suggests that cytoreductive surgery, or just a debulking, may have some benefit in specifically the melanoma associated retinopathy. As for our patient, he is currently on a, so he had CT scans that were performed of his abdomen and chest, everything came back negative. He's presumed to have a, I should say that his blood work came back positive. Western blot was positive, and immunohistochemical staining was positive. With regard to the Western blot testing, given the nature of looking for bands at specific kill adultins, we know that the testing itself is not very specific, but kind of backed up by IHC staining. We're pretty confident that the patient does have a non-perineoplastic form of autoimmune retinopathy, and he's been initiated on a prolonged prednisone taper that's supposed to be followed up with a celseph and later with cyclosporin, so essentially going the immunosuppressive route. The current research for autoimmune retinopathy is focused around establishing better diagnostic standards. There is some suggestion that eyes with autoimmune retinopathy have loss of retinal tissue when compared to eyes without known ocular pathology, specifically in the RPE, so it's possible that therapeutic options could be targeted towards that in the future. However, one of the biggest challenges at the moment is identifying biomarkers for what patients may or may not respond well to, for example, our immunosuppressive regimen, and here are my references, that's all I have for you today. I'd like to say thank you to Dr. Bernstein, Dr. Shakur in any questions. Does not, do they do not? Around $700, is that right? 850. So as far as our patient, I know that we were looking, it's basically whole body CT scans is what I gathered, essentially looking for a primary malignancy. We did, from what I gathered from our patient, it was essentially chest CT, abdomen, pelvis, CT, those all came back negative, skin.