All right, just similar to Dr. Faux, except I don't have prizes. Everybody needs to participate. That will help us get through everything. So I have nothing to disclose. We're briefly just going to talk about a 61-year-old gentleman who presented with one week of increasing yellow skin. He was having significant itching, very fatigued, has noted unintentional weight loss up to 15 pounds. He denied any abdominal pain, nausea, or vomiting, no significant past medical history. As you can see here, nothing has run in his family, no new medication, supplements, any recent antibiotics. So briefly going through, he went to his primary care physician with these complaints. As you can see here, the labs do show a pretty significant hyperbilirubinemia, mainly direct. He was going to have an ALKFOS of 349, his albumin was 3.7, and he also was found to be COVID-19 positive. With these labs, similar story. Most PCPs just say go to the emergency room. So briefly, I just wanted to see, you know, what kind of things are sparking into your mind about differential diagnosis. So anybody? Malignancy? So I think malignancy is kind of the top of everyone's list. So when he got to the ER, because you go to the ER, you get a CT scan. So here are just some findings. Does anybody want to comment on a few of the things that you see here on these two representative images? It's a very thick wall of common bile ducts. Right. So you can see that in the far one, you can see that there's concentric thickening of the bile duct. On this image here, you can see that the gallbladder wall itself also looks pretty thick. The other thing that I don't show you entirely, but you can kind of get a general sense of what the pancreas looks like. So I don't see any marked pancreatic duct dilation. The pancreatic parenchyma is present, even though you can't see really the head of the pancreas here. So does this change anything that you may do or may not do? The sooner you get lunch, the sooner you speak up. Anybody have enough information to just warrant endoscopy? Maybe colangeo and not a pancreatic head cancer. So maybe wouldn't go straight for EUS, would go directly to an ERCP maybe at this point. I think that's fair. I think that's what a lot of us would probably do. Think that maybe you could target a sample with the ERCP itself. In this situation, they did happen to get an MRCP. I think we've already been through our differential next steps. So here you can just see a representative image of the MRI. And so what are you basically seeing here? I think two main things stick out. So there's abrupt cutoff at the level of probably the common hepatic duct with marked intrahepatic dilation above. Another thing you note here is that I see a nice looking, relatively nice looking pancreatic duct. So I think shifting your differential, moving maybe more away from a pancreatic cancer to is this just colangeo? So with this information, we felt that it was reasonable to go to ERCP. So I go and do ERCP. And here are just some images. And at first I thought I blinked and missed something because I don't feel that this really represents the same thing that the MRI represented. So you kind of have a moment where I think this was a late add-on. Am I even looking at the right patient's chart? You know, you have a couple of those freak outs. But what do you see here that's a little bit different than what the MRI showed? Yeah. And so I know you might say there's not a lot of contrast in the intrahepatics. But once I got these two representative images, I thought, do I really need to over-inject them? So how would your differential in this situation potentially change? What do you think you may be dealing with? Is this classic for colangeo? Are you still going to try to do sampling? How is in the field of biliary, does this change your decision process of how you might manage it or what your mind's thinking? What does the intrahepatic colangeogram look like? Do you think that's normal intrahepatics? No. Those look like contracted. Is it racist, this patient? He's white. OK. IGG4-related disease, but... PSC. PSC. Yeah. So I think initially, we know we all think, you know, PSC. You kind of see the intrahepatics are kind of what I always say is ratty. You see a few little segments of dilation, maybe a beating appearance. You can kind of convince yourself that's what you might see. At this step, what I can tell you is when I had a balloon inflated, the balloon there was mild holdup at the site of the suspected stricture area. So I did make the decision to go ahead and sample that, get brushings, see how, you know, ensure, because even if this is PSC, that's a significant risk factor for having colangeo. So would any... So that's important to note. So the thing that I probably regret doing that I don't have images... I did take brushings. I did do a dilation across that relative narrowing. I did end up putting a stent in, which I think after the fact, I thought, why did I do that? But it was one of the things where I did put a stent in. So after a patient was initially feeling better, but over the next week he actually had progressive jaundice and fatigue, still denied pain, still denied fevers. My brushings returned rare atypical cells favoring reactive. He called. He still wasn't feeling well. Repeat labs now show that the bilirubin is up to 27.7. Only a very, very mild transaminitis. So at this point, remember, I'm a little bit angry at myself for having that stent in. But at least I know he's not febrile. So what would you do here? The other thing on the scans, they did comment on a few surrounding lymph nodes. So are you still convinced that this could be cancer? Does anybody want to do an EUS to look at the nodes? Does anybody say, you just got to get that stent out and, you know, help this man? Okay. So I mean spyglass does help us investigate when things are more indeterminate structures. So you can always consider that. But I don't know if his duct was large and we're seeing some kind of progressive changing. So now I told you the span of kind of a week, maybe I think five days went by. Does this change your differential at all? This guy is not getting better. There was an abrupt change from the MRI to the ERCP. Anything raise suspicion? Yeah. So I think, you know, one of the things, this guy had COVID but he wasn't very, very sick with COVID. Do we have to start considering does he have COVID cholangiopathy? We've seen that. Typically those people tend to be very sick in the ICU. But again, with COVID, we're still learning day to day of what changes people can have. So this was a day I didn't have access to endoscopy. So I relied on my colleague, Dr. Pho. And we did a repeat ERCP. So now these are her images, which you can still see here. There's not really a pronounced stricture in that area. The enteropatics still look very ratty. I wouldn't argue to say this might look worse than the initial one even five days ago. So now, again, we're convinced we have a rapidly progressing form of, you know, bioduct disease. So I think we've talked about PSC. We've talked about COVID. People have thrown out the idea of autoimmune. Does anybody think anything else needs to be done? Diagnostically, what do we tell this patient? Your bioducts look terrible. We can't stent anything. What are we going to do? How are we going to get to the diagnosis? I mean, this whole thing doesn't make sense. Because on the initial CAT scan, I know it was only two slides. There was no biliary dilation. Right. Then you show this MRCP with marked biliary dilation. And now you show what appears to be non-dilated. Yeah. Irregular. I mean, Dr. Pho and I stood in the room and just stared at this. Because it was a series of five days that everything has happened. This is... So... Well, I'm re-imaging at this point. Yeah. Transsectional imaging of some kind. Mm-hmm. To see what that... Yeah. ...biliary dilation is looking like. I don't think we know. I don't think we do. I don't think we do. So we did... IgG4 was negative? So at that point, we looked back, and we did want to go through, you know, additional workup. We saw that the CA19-9 was elevated, but at the time, you know, his bilirubin's 27. We got an acute hepatitis panel, all negative, looking for, is there a subsequent liver process going on? ANA was positive, and at this time, we did find out that the IgG level was through the roof with an IgG4 level of 382. So what was... The CBC, did it show any eosinophilia? I actually don't recall offhand, but good question. I thought IgG4 has to be on the... So this kind of steered our diagnosis and kind of confirmed the fact that we were dealing with an IgG4-related cholangiopathy. So this, again, falls into the spectrum of systemic inflammatory disorders with the hallmark of elevated IgG4 in plasma cells. Oftentimes these can manifest as pseudotumors. With autoimmune pancreatitis and IgG4 cholangiopathy, oftentimes you see them together, but you can get an absence of pancreatic disease, as was the case in this patient. Another hallmark is that it can be pretty rapidly deteriorating, and that's why, you know, sometimes we check these labs even sooner on presentation. As somebody had mentioned when they were coming up with the differential and asked about race, this typically does appear in men, slightly more in the white population. Higher than 75% do present with CBD obstruction and jaundice, but the intrapancreatic CBD is actually the most involved. Biliary tract changes, like we thought, are very similar to that in PSC. The hallmark is tissue infiltration greater than 10 per high-powered field of IgG4 plasma cells. Getting that can sometimes be difficult. You know, when we were dealing with a higher-up structure, you don't really want to take an EUS needle and just start coring through it. So a lot of times getting that is actually difficult. If you were to do a liver biopsy, you may see more portal and lobular inflammation than that in PSC. So with that, the patient was started on corticosteroids, 40 milligrams of prednisone. We do know that the longer-standing the disease, the less responsive they can be. Relapse can occur in greater than 40% of people, and monitoring IgG4 levels can actually predict which patients may relapse. Rarely is this self-resolved, and often, in some situations, people do need maintenance immunomodulatory therapy. So this was actually quite recent, but I will show you our lovely EMR system of how labs are reported. It actually starts in this direction, but if you follow the Billy Rubin, you can see it was 15 when he came in, and the span, and it reached 27. I guess there was... I did the ear speed, I think, on the 7th, so you can see that there was maybe two weeks it was up high. He was initiated on steroids, and then even in a month of steroid therapy, his Billy Rubin is down to two. So marked response. I still don't have great long-term follow-up for you about if he is starting just now to wean down on his steroids with taper. So it'll be interesting to see if he is one of those patients that kind of has a relapse or needs additional immunomodulatory therapy. Has anybody described his association with COVID being a triggering event for him? And that's what we were actually talking about in the room, is how does COVID potentially play a role in this? Because we do know, of what's out there about the associated cholangiopathy, it has kind of this rapidly progressive, and so was that enough to kind of trigger him to be more severe or more frequent? I don't think we know. It's a case to keep you thinking. Make sure you always look at the same MRI for the patient, right? Patient, I thought, oh gosh, what did I do? MRIs and ERCPs, just they're different. I don't know why.

yellow skin

itching

fatigue

hyperbilirubinemia

COVID-19