 I'll shorten the interval that you, the period that you guys have to answer questions if I have any questions, just in the interest of time. So, there are two lectures in this particular series as opposed to your band UBI this and this is non infectious posterior and pan UBI is and there's another lecture, maybe I think after your O caps on infectious posterior and pan UBI is. So a server the case this is an interesting one, not necessarily pan UBI just to begin with but illustrative. So this is a 37 year old African American lady who was referred for months history of pandas loss of vision. She's 2200 in her left eye and has a really big APD in the left eye. And cool. This is an easy one for you would use here goes asleep. I was muted sorry. I have kind of fuzzy optic nerve margins but the most prominent thing looks like some like vascular dropout and vasculitis. But the optic nerve is basically as a sprint tablet. This is the visual field, you can see superior and pure accurate defect with a really large, just basically constricted fields. Some studies were negative specifically ace and I said we're negative PCR of the CSF for HSV and VZV were negative. Just, just CT was read as normal. Here's the MRI Marshall. Also, there looks like there's maybe some enhancement of that left optic nerve. I think that's correct. There is and thickening and what does the chiasm look like to you. Again on that left side it looks like there's some enhancement actually bilateral so that's actually a little scary so this is kind of just ascending optic nerve enhancement and and thickening of the entire chiasm. So IV solumetrol vision got better. The patient then disappeared for a year as often happens one year later, worsening vision and now in both eyes fluctuating vision in the right. Now she's NLP in the left eye and 2020 in the right when you're NLP and you have no idea what's going on. So she had this granulometous and two years in the left eye that we could see and also a lot of victory is so it's very hard to see if there's this is the band UBI is or posterior UBI is black superior accurate defect in the right didn't know what's going on MRI shows enhancement now of both pre guy has no optic nerves. So, as the patient was no light perception and we didn't know what was going on we decided to biopsy the optic nerve. This is a temporal chance conjunctible approach, and you can actually see the optic nerve here see how thick it is and see how vascular it is. So we basically just took a big chunk of it because patients and it'll be anyway and What do we see here. Granny wellness. Yep, what kind. non casey 80. And you also see a lot of little white cells so there's, you can see some of the axons you can see the non casey eating granuloma as you can see a beneficial tissue and you can see multiple little lymphocytes surrounding. The small blue cells are always emphasized they're surrounding the granuloma so this delay type high for sensitivity and also just direct inflammation. So in summary, this is extra pulmonary sarcoidosis with a granulomatous. I would say pan UBI this sarcoid optic neuropathy. Normal chest CT and lab testing so you can get sarcoidosis. With optic nerve and you feel involvement with normal imaging and testing. Sarcoid can present in all kinds of ways and this is this unfortunate 15 year old kid who showed up with the macular variant is macular placard variant of sarcoidosis and on steroids patient did relatively well was 2100 but always had these. And this is a lot of central atrophy. And this is actually a retinitis retinocoronitis that you can actually get with sarcoidosis. And it's, it's pretty uncommon though. Here's a patient with neurosensory retinal detachment with these little granulomas underneath the retina looks almost like sympathetic but this was sarcoidosis. Patient with Lydia what does this look like to you. The optic nerve margins are not clear. And it looks like there's some leg lesions wide adjacent to the optic nerve. So, so this is actually an optic nerve granuloma so this is one finding in sarcoidosis that's 100% specific, but almost 0% sensitive because rare, but it almost always means sarcoidosis so this is an optic nerve granuloma. This is a patient who had a Mayan eagle tattooed to her arm and didn't go very well because it's sarcoidosis often you get tattoo granulomas, and often what's been presented to you as tattoo associated UVA this this patient had both UVA this and this elevated tattoo is actually sarcoidosis so if you when you biopsies these these are often indistinguishable from tattoo associated granuloma and sarcoidosis you still have those non case eating granulomas. And this this is her fundus and you can see it almost looks like birdshot reticulopathy. So the clinical science suggestives as sarcoidosis are many includes granulomatous. Now do you via this large or small carotid precipitate these are cappy nodules, the little iris granulomas that peak behind the iris margin. Then you get these besaka nodules which are these iris granulomas that are on the surface of the of the iris. There are four lanes nodules which are nodules on the on the trabecular mesh work and then when they resolve they form these dent shaped BS. So they're related, you have snowballs in the periphery. They're associated with inflammation or periflebitis optic nerve granulomata is corridor nodules are the large or small corridor nodules in a very vascular distribution or periflebitis in this candle wax dripping fashion. And the French word is the touch the bougie or candle wax dripping. Very phlebitis. So what's the utility of ace and lice is I'm not very good. Often a just extra changes seen quite often about 70%. But remember that just extra changes are often temporarily discreet from ocular findings so patients who have pulmonary sarcoidosis may not have ocular sarcoidosis yet in patients with ocular sarcoidosis may have had previous just extra changes but in about 70% those changes will resolve. So why make a diagnosis in the first place. Well, it's useful for prognostication it's useful because patients with sarcoid ubi just a sarcoid optic neuropathy are very high risk for neuro sarcoidosis which can often have devastating consequences. Hello gentlemen with a history of endothelitis and a blind painful left eye that was in UK. Two months earlier, three days vision loss in the right eye visual acuity in the right eye the only is 2,500. Interestingly, he has dangling of his fingers and toes neck pain and stiffness headache to notice. And this is his fundus picture which may be a little too blurry to be able to tell what's going on. The red free picture and the OCT Rachel. Oh wait I can't ask the fellow sorry. Never mind alley again. What do you see here. It's like a, like large subretinal fluid. What else do you see that's interesting. There's some anterior vitreous schmutz, and the core it looks like it's probably thick too. That's right so you can see the stereo Coroidal junctions, and the Coroid is very as undulating contour. And then look at the angiogram starts off with this kind of this multi focal leakage. And then ends with, or in the middle you have some pooling where there's pooling of the dye from these multi focal leakage areas. And then the late late this is about seven minutes you see these little punctured starry sky type things. So, I'll just real quick what do you think this is. kind of sort of this is actually sympathetic so he lost the other eye. So, the only difference between VKH and sympathetic is sympathetic is VKH with trauma, because sympathetic is still a bilateral disease and the reason I brought this case up is the fact that this patient had all of the symptoms of Gizmos the ringing in the ears that patients with acute VKH have and you can have that in quite a few cases with with sympathetic if you're asked. This is a neurospanic slash German male with loss of vision pain headache tonight is for several months is treated by an optometrist for anterior uveitis his vision was like perception both eyes and his pressure was zero. And your segment examination you can see this vibrant membrane on the surface of the of the lens, since he was on Dersol every hour for his and so called anterior uveitis he is that your segment was quiet and escapees were gone but he did have these pretty wild looking cataracts in both eyes. And this is not an ultrasound with a poor aspect ratio this is actually what the ultrasound looked when I applied just a little bit of pressure to the eye because of how low the pressure was and you can see how squished this is the posterior surface of the lens. This is the anterior surface the retina the retina seems partially detached and there's this thick thick go right. It's as the corridor is is approximately three millimeters in thickness. Similarly in the other eye. So what is this Marshall. I'm not sure. So near sensory retinal attachment hypotenuse 10 thick coroid. Yeah, I could still be VKH headache tonight is this is this is like late late late stage three kids remember to always dynamic people. And this kid. We treated him with two grams per per kilogram per day of oral steroids you put bilateral red asserts and treated him with multiple rounds of immunosuppression, psychophosphamide. And now he is 2400 in both eyes. The college works in tech and leads a center for the blind. Unfortunately, or fortunately, doing well. So, here's a 45 year old. Was it gentlemen or gentlemen actually who lost the left eye. No, this is a lady sorry, who lost the left eye recording ulcer and had lost a vision in the right eye. So here, cool real quick what kind of retinal detachment on your sensory retinal detachment is this like the fast Larry. That's right. Yeah, so here you have the newer sensory detachment here you have the best so there is attachment with this is outer retinal remnant. And what do you see here cool. Early and mid. Yeah, early and mid phase FA and then in the early phase you can see these big placoid changes that stain late with a little bit of leakage. And what's this thing here. That's that's pulling so this is leakage and this is your correct this is pulling. And then you have these block early stain late time collisions as well so what do you think this is this. This was the corny ulcer and the other is this as well. Yeah, this is also sympathetic. I mean, and this is kind of more classic with with neurosensory retinal detachment blocking early standing late. This kind of this bachelary neurosensory retinal detachment. Let's get here's another patient with tonight just had a vision loss. And this is actually the reason I bring this case up because we've actually seen a lot of the gates now in this presentation but the reason I bring this up is this person had symptoms for several, several months before she appeared for help and, you know, now 12 years later, I'm still seeing her. And so this is after she was on. This is before she was on steroids you can see this multi separated retinal detachment. And on prednisone and cell sept after a lot of time had elapsed without treatment, you can see what happens in patients with with BKH, where there is a multi separated retinal detachment that is chronic, and you treat it and then you end up with all of this. So diffuse the depigmented sunset low fundus, but also kind of this sub retinal fibrosis and this is how she looks now, but you can see that this diffuse atrophy in the in the right eye when the left eye is this big disco form scar. And this tells you how important it is to treat VKH rapidly either with steroids only or steroids and immunosuppression. VKH is a multisystemic disorder characterized by granulometers that new vi just with exudative retinal detachment. It's associated with neurological and cutaneous manifestations, and it's basically an autoimmune response to neural crest tissue. Denitis why do you get denied as well the hair cells in the ears come from come from the neural crest the RPE comes from the neural crest the skin it in documentary. Manifestations are all neural crest. The meninges are derived from the neural crest and hence we get meningitis. And of course, as is much of the UVA as well. It's Asian Middle Eastern Hispanic Native American populations. There is this HLA association. It's loose. They may test it on your O caps but otherwise it's completely useless. Incomplete VKH where you have the systemic symptoms you have incomplete VKH otherwise known as Harada syndrome is probable. We will not go through this in the interest of time but the diffuse corridor thickening and after deep agmentation. And we've already gone through the floor scene is meningitis and CSF pleocytosis that's lymphocytic predominant as alopecia poliosis and vitiligo which are your integumentary science. And of course, there is that racial predilection. Along with silk root and also Native American so specifically indigenous American populations. What's the difference with VKH and sympathetic of the army is one thing. And that is trauma. Histologically, it's, it's indistinguishable. Except that it's been said that sympathetic spares the cordial capillaries. This is not necessarily true. I don't know if they changed it in the O caps or not. But the fact that the appearance that sympathetic spares the cordial capillaries is a histological artifact. Downfuchs nodules are those little modules that you see under the red knife to show you that with a subretinal with subretinal fluid think about sympathetic. As far as inucleation of the inciting I before one or two weeks after trauma elapsed that do is something that's probably not valid anymore because we're actually quite successful treating sympathetic with immediate suppression and steroids. We're pretty good at recognizing it as well. So not as valid as it used to be. And remember that the number one inciting factor for sympathetic ophthalmia is surgery, especially the rectal surgery. Okay. Here's a 45 year old woman with lots of vision in both eyes. We're a bit gone. Abigail, what do you see here issues with him. No, you have, you're like me, you have general volume issues. Yeah, for sure. So there is kind of the scarring that's emanating from the optic nerve, like in the It looks kind of like surpishness to me. It is very good. So now, actually, we saw a patient in the, in the, in the VA that we thought might be that might be separated. So it was mentioned as such. This is the, so this is what suppress really does look like you have kind of this early blockage of the very middle and then you get this late brush fire staining of the of the periphery of the lesion. That's an inactive, relatively inactive suppression station and the opposite by you see mostly inactive with this brush fire staining but this particular aspect see, see where my cursor is that kind of blocks early and stains late that's an active front in a patient with surpishness if you go back to unfortunately the pictures can get it cut it off but this is this is a this is what surpishness looks like. Whereas, you know just non inflammatory helicoid geographic atrophy emanating from the nerve would have this peripheral brush fire like staining. And of course you get all of this diffuse out of direct in the loss how do you treat it. Well, you have to be pretty aggressive you can use steroids and cells but this is a get one of those diseases where you pretty quickly advanced to Rituximab or even an alkylating agent such as Cloramdosyl or cyclophosphamide, because it's very aggressive. The fundus outer fluorescence is pretty useful in suppression is to tell whether or not there's activity. This is the same I at different time points where the fundus outer fluorescence is just dark. Whereas in the lower image with the arrow you can see that there's leak that this kind of this hyper outer fluorescent in this front and this implies a focus of activity. So it's a chronic progressive recurrent bilateral devastating disease, often not discovered to late because it's painless and does not have any floaters or anything but it's often discovered when patients have a lesion that crosses into the phobia. They're chiroidal and RPE but they're secondary retinal involvement by pathology it's rare. Middle age male more than female and largely Caucasian. As if centrifugal extents is as multiple crops as recurrent attacks is often marked asymmetry. And a lot of patients with or without treatment progressed to 2200 or worse vision. So it's a pretty devastating disease if not treated. And just know one thing about supervision is is that if you see it always think about tuberculosis especially if it's asymmetric but you can have bilateral to be as well. So the one thing that that they can resemble suppression is a lot is kind of this. So all this think about that. Okay, here's a 36 year old with scintillations and in large line spot this is a pretty easy one maybe I don't know. So, Lydia what do you see here. And just to help you out the right eye is completely normal. The right eye is completely normal but left eye, I see hypopigment at like the media is maybe a little bit more hazy. Optic nerve margins are clear and I see some brighter spots and one eye. I would I would be concerned for something like pick versus other like multifocal corridors. So, yeah, so, so these. There is white yellowish punctate lesions. Thank you for not using hypopigmented because that's not a good term. But here's the fundus outer fluorescence what do you see here. I see high pair out of fluorescence emanating from the optic nerve in all directions. And similarly, on the on the FA you see these a little bit of very phabitis these little pocket hyper fluorescent lesions, and then you see this little in the in the in the fovea of the of the affected left eye you see this little elevation with this little crew cut like appearance. And the ICG just in that I show is persistent hypo fluorescence. Anybody know what this is. This is muted school I did this patient for FA. You did. Okay, yeah, it's it's muted. So, get a unilateral reads like hyper fluorescence and FA. Similar finding on fundus outer fluorescence and then have this more numerous lesions seen on ICG with these little sub retinal crew cut. This is not a bad idea to sometimes consider steroids although typically for your O caps, no steroids self resolving. So this was first diagnosed. This is not a bad idea to sometimes consider steroids although typically for your O caps, no steroids self resolving. So this was first diagnosed. First discussed in 84 by in Chicago by Dr. Jampol, mostly females, mildly myopic often have these prodromo flu like illnesses. There's few victory cells but sometimes if you look hard enough you see them. And there's often hyperemia blurring of the optic disc with isolated bascalar sheathing. And this is, you often don't see this, but there is this granularity of the phobia and if, if ever presented with this appearance do think about it. Here's a. This is a 35 year old patient with 2050 25 vision presents with kind of blurry vision and simulations one plus AC cell, and it seemed to have pictures cell and haze and both in just the right eye. Here is the right eye. Sorry, this picture is so blurry I apologize. But you can see these peripheral kind of bunched out lesions with pigment and then these other ones that appear a little bit newer OCT shows diffuse vascular leakage leakage at the disk and leakage of some of these these nodules. CME. And this is a patient with multifocal corditis. I don't know why it's advancing I'm sorry, but this can be it can often be a unilateral disease in the beginning, it presents often you're not free and it progresses to become bilateral. It is an 80% of patients, ultimately a bilateral disease, usually female white 45 years of age. And like other white dots and drums, it predominates in people with with mild or moderate myopia. And often until UVA is big try this, the lesions are larger than those that you see in in mutes with the, you know, variable from 50 to 350 microns. The scars then become a trophic new lesions may arise so you see multiple crops of disease you'll see peripheral go to retinal streaks anybody know what those are called. It was a secret secret. Yeah, legal lines, shaggle lion's secret streaks, not secrets, you just shaggle lion's you see you see those are multifocal corditis and you see peripheral peripathic pigment. Pigmentary changes around the nerve structural complications such as cataract CME. And you know this is another disease that you really want to treat with immunosuppression. This is an ICG angiogram showing multiple lesions that you can see more off. Should you do it ICG than if you do an FA fundus auto fluorescence is often quite revealing where you see these lesions hypo auto fluorescent lesions that sometimes precede the appearance of new lesions. Just by by a microscopy. The fundus auto fluorescence lesions in the peripheral area are often more numerous than if you had just viewed the retina. You can see this little sub retinal hemorrhage just to remind you that it's pretty common about one third of patients with, or actually 15% of patients with multifocal corditis will develop corroidal neovascular membranes and then sub retinal sub macular fibrosis, such as in this patient. You can treat with steroids and immunosuppression with immunosuppression using an anti metabolite in particular there is a far lower risk of posterior pole complications because these are what cause vision loss CME and growing neovascular membranes, and there's a far far greater risk at lower risk of severe vision loss. You can treat CNVM laser either thermal laser or photodynamic therapy in tributaryl steroid can sometimes make these inflammatory CNVMs go away. Anti VegF and sub macular surgery had been popular in the late 90s but don't do it. It's a very high rate patients going LP or NLP. PIC is basically multifocal corditis without retritis. Now it was also described in 84. And there's often a unilateral acute onset of blurred vision. And just remember it's about the same healthy young white women, mean, mean age of 29, and also moderate myopia. PIC is just like multifocal corditis to some extent except that there's no vitritis or anterior uveitis although now when people look at script source images on OCT, you do see some posterior vitrious cell. Posterior pole lesions 100-200 microns that progress to atrophic scars and a very, very, very high rate of coronal neovascularization. I would say most patients with PIC present in fact with coronal neovascular membranes and then you notice the bug date lesions in the peripheral macular and recurrences of course are common. And geographically the first thing you see with PIC is early hyperfluorescence, late staining and coronal neovascularization and you see more lesions on ICG than you do on that. And geography, can you treat these patients with immunosuppression? Yes, if you have bilateral disease and one eye is severely affected and the other eye is threatened, then yes, you can consider it and it is in fact useful. But conventionally you treat these with anti-vegetary and intravascular steroids. This is a 32-year-old, two weeks after COVID-19 vaccination, loss of vision and simplifications, and you can see these little placoid changes in both eyes, bilateral, and you see vasillary retinal detachment in both eyes. Kind of this thumbprint like hypo outer fluorescence. And then blocks early stains late in this placoid fashion, persistent hypofluorescence on ICG. What is this? Amp. Amp. That's right. It's the worst acronym I've ever seen. So Amp is, so this COVID-19 vaccination may have been a, or the COVID-19 infection, or this actually was infection, not vaccination, sorry, was actually, could have been a red herring or could have been just a, you know, a precursor to immunogenicity causing this. We don't know, but we're actually, we're writing up a series of cases with COVID and inflammatory eye disease. But yeah, this gentleman went on to get worse and had more of these hypo, a hyper and hypo outer fluorescent lesions in this cockade like pattern. Now what? So he hasn't formed any, he hasn't created any scarring. Will this get better by itself? Probably, but he's pretty disturbed now. So put him on steroids. And three weeks later, prednisone continues to get better and resolves. Now, this is actually a controversial topic as to whether or not you would treat amputee with oral steroids. Most people say, yeah, it'll just get better by itself. My contention is that some patients in amputee go on to develop out of retinal loss. And then those patients will end up with permanent visual loss. It may still be 2020, but they will often have this condomata and a loss of contrast sensitivity. So I think for amputee, it's worth treating with steroids because it certainly does no harm once you've ruled out syphilis. So once again, something for your O-caps, you say it's self-limiting and it gets better by itself. But in real life, you probably want to treat these patients. So this was described way back in the late 60s by gas. It's young healthy adults, mostly. It could be male or female. One-third have a prodromal viral illness. This is a HLA predilection, but once again, it's completely useless. HLA B7 and DR2. There's this rapid onset of blurred vision. It's bilateral and asymmetric often. There's mild vitritis. And there is an association with cerebral vasculitis that you can have up to 10% of patients with amputee have cerebral vasculitis. If you have headaches, meninges, miscellaneous neurologic deficit, immediately get an MRI and consider a cerebral angiogram and treat with steroids. There is an association with Wagner's polypanstheritis, GIA sarcoidosis, and ulcerative colitis. Okay, so this is an 18-year-old with a lost vision and a right eye bilateral extremity numbness. So, Cole, what did you see here? Did you see any? Yeah, good. In the OCT kind of sub-phovial and paraphobia, you have some outer retinal loss and irregularity, and then kind of these big black-coid, deep-core retinal lesions in both eyes. Yeah. And this is what it looks like a lot of fluorescence. You see multiple lesions. She had no symptoms in her left eye and her right eye. No symptoms from these lesions, which are of indeterminate age, and this is the new lesion that brought her in. And what did this lesion do? The new one blocks early and stains late, and then you see these peripheral lesions out here, and out here in both eyes, in her asymptomatic left eye. So what's this goal? This is a young person thinking about like syphilis, but is this like the Ampigenus? This is relentless black-coid. So Ampigenus is basically multifocal amputee with garring. So this is relentless black-coid or Ampigenus retinopathy. This patient had a bit of a twist because her lower extremity weakness and tingling was actually because of bilateral fontine strokes, which in turn was secondary to cerebral vasculitis. So whereas it has been described with amputee has not been described with relentless black-coid, my guess is this because it's rare. My guess is because Ampigenus is rare, so therefore it's hard to form an association with anything, but this was the first time that cerebral angiography ever showed a relationship between relentless black-coid and cerebral vasculitis. And so she actually did really well on rituximab vis-à-vis her vasculitis, the cerebral vasculitis, but her lesions continued to get worse. And so she was treated with a T-cell inhibitor, an anti-metabolite, and an anti-TNF agent before she quietened out. Now she's doing really well of all her medication for one year and still 2020 in her left eye, unfortunately 2050 in her right eye. Here's this 33-year-old woman with scintillations. I won't ask you guys, but this is another example of relentless black-coid only this time it was far, far more severe. It is a disease continuum when it comes to Amp, there are numerous lesions. Amp with multiple lesions is multiple crops of disease. This may not be true anymore because we have identified two patients now with cerebral vasculitis. The best treatment is unknown. Often these patients need immunomodulation with cytotoxics such as alkylating agents often rituximab, often anti-TNF agents, often multiple agents. This is a 72-year-old lady who presented with persistent vitritis in both eyes after cataract surgery. A week later had a vitrectomy with intravitril antibiotics worsened and was noted to have whitening and vitritis in the left eye and was referred for possible retinitis. So, Marshall, what do you see here? So, looks like there might be a bit of a hazy media. The right eye, you can see some vasculitis, especially superiorly, and maybe a few hemorrhages there. The left eye, the nervous pre-pale, and there's a black-coid lesion or fear in some of the other hypopigmented spots, but they don't like. Over here, you see some retinal whitening, did you mention that? Yeah, just vasculitis, but yeah. Yeah, but the other retina seems to be baler. And now it's a little more evident, right? Yeah. So, what do you think this is? Well, with retinal whitening, I would worry about any infectious causes. Specifically? Like VZV, HSV, CMV. Right, so necrotizing retinitis. So, yeah, okay, so we thought that too. We said, okay, this is R. So, we did the usual, tapped, intravagal, Gensectovir, balutrex, two grams, three times a day. Started on prednisone, improved after this treatment, but then three days later, the whitening seems to have spread towards the necula, but have kind of dissipated out towards the periphery. And then we noticed that there's all these little peripheral white lesions as well in the inferior periphery. And there's vitreitis in the left eye with this, this old black white lesion. So, we start to rethink our diagnosis. Now, Marshal, what do you see here in the OCT? So, there's kind of loss of the architectural layers and some diffuse retinal thickening. Now, can you see that with R? And absolutely you can. You can see this loss of architecture, this homogenous kind of soul plague, loss of architecture, kind of infiltration almost. So, continue to get worse. So you ended up with this, with submacular fluid, which is, you can see with R, but not unusual, but not typical. What else could this be? Lymphoma. That's right. So we did a vitreous biopsy. And, sure enough, there were large B cells, my D88 positive. And, the D88 gene rearrangement anomalies and inverted IL6, IL10 ratio. So, this was a patient with a primary vitro retinal lymphoma, which is a subset of primary central nervous system lymphoma, both are high-grade, large cell, chronic and quite aggressive. 15 to 25% of patients with primary central nervous system lymphoma eventually have ocular involvement. But when the converse is looked at, 56 to 90% of patients with PVRL primary vitro retinal lymphoma end up with CNS disease. So, this is obviously made worse if it's bilateral disease. So, it's very, very important to screen these patients and treat them systemically should you, even if you do not find CNS foci. Now, this is controversial, but in, at OHSU, every single patient with bilateral lymphoma is treated systemically. Whereas at the Huntsman, I believe they're a little more conservative and bilateral lymphoma is often given it. The conditions are often given a choice as to whether or not to treat systemically or not. Conversely, goroidal lymphomas are small B cell lymphomas with kind of a low-grade tumor, and they have no association CNS disease, but they do have association with retroperitoneal and other systemic lymphoma. Often affects elderly patients, the median survival time for patients with PVRL is 58 months, so they usually go on to form CNS lesions. It's rare for this to be multifocal to the sense that it's systemically seen outside of the CNS. And the prognosis for each is worse than on Hodgkin's lymphoma. The diagnosis as you see in this particular patient is often delayed. Steroids may decrease inflammation, giving you a false sense of security that you're treating the right thing. Just remember that when you do a vitreous biopsy, take them off steroids for a couple of weeks because steroids can decrease your cell count and decrease the sensitivity of your vitreous biopsy. Often you'll see blurred vision and floaters, the AC is usually quiet. There's no posterior scenicia, so see a lot of evident inflammation, but no posterior scenicia, think about lymphoma. Your cells and haze may be in superior locations and they look weird. They look like they're in sheets and they're whiter than normal cells. The cells can grow along brooks. You have plaques. And you can also have these interesting PED like changes along the RPE. So the gold standard for diagnosis of obtaining lymphoma cells from the eye, phytopathology and histopathology are useful. A high ratio of more than one of IL-10 to IL-6 is indicative of PVRL, although you have patients without lymphoma who can have that inverted ratio as well. All patients should have a brain MRI and lumbar puncture. Local therapy does consist of intravitual methotrexate. Conventional treatment from the 1970s and 80s is twice weekly intravitual methotrexate. But you can actually do methotrexate and rituximab on a monthly basis. And the study that showed that this was effective had a lower rate of complications, but in my clinic I find that rituximab, which is a chimeric anti-CD20 antibody. So half murine, half human, you can end up with pretty bad inflammatory reactions with occlusive vasculitis. I've actually driven one patient into new vascular glaucoma from rituximab injections. We have a paper on that. So it's not the safest thing, but it is useful as an adjunct treatment of PVRL and can decrease your treatment burden. I currently do my best not to use rituximab. There are no large prospective studies to show whether or not systemic therapy is necessary for all PVRL, bilateral disease, of course, should point you in that direction. Regimens for when the CNS are involved often involve multi-drug treatment, rituximab-based. Our job is conventionally like the first thing that you use, a brutanib can be used as an adjunct and as maintenance therapy. Most regimens also involve methotrexate, intervitral methotrexate and systemic. So for primary central nervous system lymphoma median survival is two to three months. This is all data aggregated from over 30 years. Now with your rituximab-based therapy that that survival is much better with the median survival, five-year survival of about 50%. This is the last case. So the reason I showed you lymphoma is just because you have posterior uvea just doesn't mean you should forget about mascarades. This is another patient with a mascarade type syndrome, Abigail. What is this? Shout it out. Anybody? Yep, this is syphilis. So this is an unfortunate and famous patient who actually passed away from a combination of alcoholism and untreated neuro syphilis and peripheral neuropathy. But yeah, this is perhaps one of the worst cases of placoid syphilis that I've ever seen. It was bilateral, it was progressive and just to show you that even syphilis was actually a pretty common cause of death in the 16th and 1700s. So all the way up to before penicillin. No, this too as another mascarade syndrome looking at posterior uvea to these obvious check for neoplastic disease, think about neoplastic disease and think about infectious disease. All right. I think that's it. Any questions? One thing that I haven't included in this presentation because it's a rather large topic and I've covered in another lecture previously is birdshot. I know we covered birdshot in our OCAP review but do read about that because they do like to ask about it and it is one of two meaningful HLA associations in uvea. The other HLA829, the other one being HLAB27 and maybe B5 and B51 for bedchats. But do read about birdshot a little bit. Okay. All right. Well, enjoy your conference guys.