RWCS Fellow Cases A Save

Hi, this is Doctor. Arti Kavanagh at RWCS twenty nineteen. We have a great conference and one of the highlights of the conference are some of the case presentations by the fellows. So very happy to, have a presentation from the University of Colorado. Doctor.

Mosa is gonna tell us about a very interesting case with some interesting therapeutic options.

Doctor. Mosa. Alright. Thank you. Today, I'm going to talk about this very interesting lady.

I know most of our patients in rheumatology are interesting, but this one is kind of like stood aside for me. She's a 35 years old lady who was initially diagnosed with antiphospholipid antibody syndrome based on three consecutive miscarriages and nonbacterial endocarditis. And while they started on anticoagulation and, like, shortly after she was discharged, she came in back with diffuse alveolar hemorrhage. We had to stop anticoagulation and on steroids. But as soon as we stopped anticoagulation, unfortunately, she developed MCA stroke.

So at that point, we had to decide to go aggressive as far as, like, immunosuppression, and we gave her three rounds of rituximab, Imuran maintenance, and four cycle of IVIG. However, her antifasolid was persistently positive. So after the third rituximab infusion, she was pretty much immunosuppressed. And unfortunately she developed bacterial endocarditis. And at that point, we kind of decide want to decide what is the next step because she bled with anticoagulation, she got infected with immunosuppression and we thought the IVIG was ineffective because she has persistently positive antiphospholipid.

That brought the question of the possibility of starting her nucilizumab which is a monoclonal antibody against c five, but we had to think about the possible side effect, especially with her recent endocarditis and not to mention the the very high cost of the As of now, we have back and forth discussion with our insurance to whether they can approve the eculizumab. So I kind of, like, took advantage of this opportunity to ask an open question whether should we proceed with acluzimab and giving her prophylactic antibiotics or should we just continue on the IVIG and the rituximab?

Well, it's interesting. One of the things we see in rheumatology lately, isn't it, is that we use autoantibody tests. We love autoantibody tests, but this case has two factors that could influence how you interpret the test. One is she's getting rituximab, which could conceivably decrease autoantibodies with or without concomitant clinical improvement but also IVIG which contains autoantibodies so whose antibodies are we measuring but it sounds like the eculizumab we certainly think about the meningococcal infection but it sounds it might be a reasonable way to go, a very difficult case.

Especially if you look at the pathogenesis of the antiphospholipid antibody syndrome, has this is where the eculizumab will act by blocking the conversion of c five to c five a and c five b, which will develop the membrane attack complex that will participate in the inflammations from both the fetal disc.

Alright. Thank you very much. Excellent presentation. Interesting case. And that's all for now from RWCS twenty nineteen.

Hi, I'm Doctor. Rachel Tate coming to you from RWCS twenty nineteen in beautiful Maui, Hawaii. We wish you all were here. I have the distinct pleasure of interviewing Doctor. Annie Wu today.

Annie is from Munster, Indiana and she has a really good poster about something that our patients ask us about that I think is really, really important and she has some really good salient points for us. Annie, thank you for being here. Tell me about your poster. Tell me about your case.

Thank you. This is a case I saw in my first year of fellowship. It's actually my first consult in the hospital. This is regarding a 48 year old African American female who had a known history of sarcoidosis. She was admitted for weakness.

She had right arm weakness as well as she couldn't walk for about a month. So we got on the case and of course she was put on the stroke pathway. Multiple imaging were done, including MRI brain right here. As you can see, there are some atypical lesions that brought our attention. Because of that, we had to expand our differential.

Given her history of sarcoidosis, neurosarcoidosis is certainly in a diagnostic differential, but given that she was also receiving immunosuppressive meds by the time she came in, we had to consider PML. So she was able to get an LP done very early on in the admission. And as you can see right here, JC virus by PCR is negative. And that's one of my points I wanted to point out is that sensitivity of PCR for JC virus can vary from test to test depending on the lab laboratory that you send a test to. In her case, this this this being negative really had to make us, talk to her about pursuing, the diagnosis.

Ultimately, we were able to convince her about doing a brain biopsy, and, obviously, this is not something that anybody would want to do. It took us a long time to convince her to do so, but we got very fortunate with a neurosurgeon who was really helpful as well. So the brain biopsy was actually done for her. As you can see from the biopsy, there were pathognomonic lesions that were consistent with PML, including right here. That's an astrocyte.

It's called a actually a plum purple astrocyte, and that is very typical. Along with right here, all the black dots are the JC virus in her brain. So in our case, ultimately, the treatment was to not treat her along for the immunosuppressant meds. We had to take most of it away, and she was kept on prednisone fifteen milligrams a day. As of now, it's nineteen months out, and she is still alive.

And that's what I wanted to really talk about is she received her brain biopsy on the ninth day of admission, and that ultimately contributed to her survival. In the study in 2014, an average of four months actually passed for most people to receive a PML diagnosis, and a lot of people actually died shortly after. So in her her case, we're very happy that she was able to do so well. In her last MRI imaging, she actually improved in some of her lesions.

Annie, that's really good news. I think what's important about this is treating the patient. And what you've shown us is that even though the JC virus PCR was negative, which it can be, absolutely, as you pointed out, I think that because you were really on top of the case, the patient had a better survival because you did the brain biopsy. And because once you know what's going on, you can actually treat the human. And so I really, I commend you guys, the whole team for that.

It's a really good, it's a good plan to treat the human, I think, and you know that. So I'm really proud of you. You've done a great job. Thank you for coming to Maui. Oh my gosh.

Of course. So thank you, doctor Wu. I appreciate you. And check us out on roomnow.com. And we hope that we will see you at RWCS twenty twenty, also in Maui.

Hi, I'm Doctor. Rachel Tate coming to you from Rheumatology Winter Clinical Symposium twenty nineteen in Maui and today I have the privilege of interviewing Doctor. Brian Abe regarding his poster. Brian, thank you for being here. And tell me a little bit about what brought you here.

What's your poster about?

Sure. So my poster, it's about a case of dermatomyositis with rapidly progressive interstitial lung disease. It was a patient, a 48 year old male who presented to our hospital with a sub acute presentation of the typical rash associated with dermatomyositis with the heliotrope rash. The V neck sign, the Scholl's sign, the Holster sign, Gottron's papules. But, he had this rapidly progressive shortness of breath and so we worked him up for infectious etiologies which were all negative.

The key things about his case however was that in addition to all the findings of dermatomyositis, he had other findings that were suggestive of the MDA five subtype including palmar papules as well as mucocutaneous ulcers especially in his mouth. We recognized this fairly early on and because his oxygen requirements increased very rapidly within twenty four hours, we decided to treat urgently and immediately with high dose immunosuppression. So, if you can look at the figure here, you'll see pictures of his heliotrope rash including the eyelids as well as above the eyebrows there. His Gotron's papules here but also the palmar papules on the palmar aspects of his hands which kind of led us to the MDA five subtype. Initial CT when we saw it showed these peripheral kind of ground glass opacities that actually within twenty four hours when we repeated the CT rapidly progressed.

He was actually intubated on hospital day two and we started a gram of Solu Medrol for three days. We started tacrolimus, something that the Japanese groups have shown to be somewhat effective for rapidly progressive ILD MDA five subtype. As well as IVIG and IV cyclophosphamide. Now, on hospital day three, day four, he rapidly deteriorated. He had to be intubated and he was actually placed on, extracorporeal membrane oxygenation.

And while he was in the ICU and I was trying to think of other treatment for him, my attending suggested, what about tocilizumab? What about the JAK STAT inhibitors? So, I did a little bit of research on this and there's actually a few case reports with successful use of JAK STAT inhibitors, specifically tofacitinib in treatment of rapidly progressive ILD associated with the MDA-five subtype. And so that's really what my poster is about is kind of reviewing the literature on tofacitinib or at least JAK STAT inhibitors in dermatomyositis and suggesting that this may be a potential role in the future.

Well, Brian, how did the patient do?

Unfortunately, on hospital day about twelve, he had multiple complications because of the ECMO and he was on heparin. Because of his oral ulcers, we decided to place a tracheostomy. However, he started bleeding from that. He started having GI bleeding on the heparin and ultimately the family decided to withdraw care.

I'm really sorry to hear that, but it seems now we have an additional case study about JaxStat and how it may be effective in these types of patients. You so much for sharing your expertise I and your really look forward to hearing more about this in the future. Thank you. And check us out on roomnow.com and coming to you live from Maui. Hi, I'm Doctor.

Rachel Tate coming to you from Maui at RWCS twenty nineteen. Today, I love interviewing the fellows about their posters. They're the experts in what they're talking to us about. Today, have Doctor. Erin Hammett from Scripps and she's going to tell us a little bit about a really interesting case of a rash and congenital hip dysplasia.

Erin, tell me

about poster. So this is a patient she's 43 years old and she came to us as a consultation for a positive ANA and a rash. So she had this new erythematous maculopapular rash that you can see here. And the question to us was does she have lupus? So we got her specific lupus antibodies and they were negative.

Her inflammatory markers were negative. The key part to the history was that she had had a metal on metal hip implant ten years earlier with cobalt chromium alloy. So we got a hip x-ray and it showed some increased metal parts around the head of the hip. So we checked her cobalt and her chromium levels and they were both elevated. So what we did was we explanted the hip and her rash went away.

So the key points to this case are that metal on metal hip implants were very popular from the years 2003 to 2010. About a million were placed placed and now they're recalling many of them. Some side effects to metal on metal hip implants are systemic toxicity and hip pain. So if you have a patient that has a metal on metal hip implant and they have any of those symptoms, consider checking and chromium level and if they're elevated, you may want to take the hip out.

Thank you so much Doctor. Hammett. I really appreciate it. I think this is a really good identification of something that would affect potentially a lot of our patients, especially given the time frame, especially given what happened and the dramatic improvement that we've seen. Thank you so much.

I'm so glad you're here. And more from RWCS twenty nineteen to come and check us out on roomnow.com.

Hi, this is Doctor. Arti Cavanaugh at RWCS twenty nineteen. Real highlight of the conference is the participation of the fellows that we have come and been fortunate to have a great group of fellows again this year. And the fellows are charged with bringing an interesting case and they have definitely not disappointed this year. I'm very pleased to have doctor Massouda is gonna talk to us about a case they saw at the University of Arizona on a very important and very topical issue.

Doctor. Masood.

Thank you very much. So actually I'm presenting to you a study that we've done with a compilation of patients, not just one, about examining ethnic differences in OA patients who and their attitudes about prescription NSAIDs. So this is a very important topic because in Arizona we have a huge Hispanic And, what we did was we submitted questionnaires to basically anyone who had, knee or hip away without, any history of surgery. And the questionnaires had a lot of questions, but one of the things that we ended up pooling was, you know, just, getting their opinions and perceptions about prescription NSAIDs, both topical and oral, and seeing, you know, where their, how their opinions different are difference make a difference and then when so that we can be informed on how to educate our patients. So, I wanted to just basically go over the results.

What we ended up finding out with that was that Hispanics are less likely to have even heard about prescription NSAIDs. They've heard about the over the counter medications, but the prescription NSAIDs, they didn't realize that that was even a thing or if that was stronger or anything like that. So compared to non Hispanic whites, that was a difference. Hispanics were also less likely to feel that the oral NSAIDs were beneficial, and they were also less likely to feel that they were risky or harmful. So those are both misconceptions that we were like, Oh, we need to do a better job in educating our Hispanic because, yeah, oral NSAIDs can be very beneficial, you know, at their prescription doses, but they can be harmful as well.

So we need to be on top of that part of our education. So the other thing was Hispanics were less likely to believe n topical NSAIDs were harmful. And, yeah, they're not very systematically disabsorbed, but they're still there's some harms in that too. So overall, we've, just learned a lot about our patient population that we're dealing with. And, all of these graphs basically are answer are like how they distribute for different questions, like helpful, helpful for themselves, pain relief for themselves, risky, dangerous questions.

But overall, you can see that, you know, there's a pretty clear delineation. And some of the things that, can confound that we also presented was the, overall, the sociodemographic and clinical characteristics between the two populations. The few things I did want to point out was that the Hispanic population was actually younger, overall than the, our non Hispanic white population, and also that they had an, you can see it in education, but also they had a, less likely to have an annual income of greater than $40,000. So those are some things to keep in mind as well.

So, very nice, study. It sounds like it lends itself toward thinking of an educational intervention. Are you all working on that?

Yes. I mean, we haven't come up with anything standardized yet, but I've made a point to, with my Hispanic patients, to spend more time and be able to allot more time to their visits so that we can, you know, get these, misconceptions clear.

Well, you very much, doctor Masood, and thank you for watching. And that's it for RWCS twenty nineteen.

Hi, I'm Doctor. Rachel Tate coming to you from RWCS twenty nineteen and I'm with Doctor. Lukas Lachon, who's gonna tell us about it one of his interesting cases and I think there are a lot of salient points to this and I'm really glad you brought this case for us Lucas.

Thank you. So my case I wanted to highlight because although there's not super interesting features necessarily that differentiate it, it's a really classic, case that we should always kind of think about on the differential, when we see a patient with sclerodactyly or indurated skin. So this is a 55 year old gentleman that initially came in the emergency department over the summer. That time was complaining of some neuropathic pain, nerve pain shooting down into his hands and feet, some chest pain. The team then checked a CK, they did a cardiac workup.

He was essentially discharged home. When we actually got called was about three months later, so this is in October. At that time, again still having the nerve pain described as shooting down from kind of forearms into the hands and from backs of the legs down into the feet. But then interestingly, he asked me specifically, doctor, why is my skin so thick? And so when we were looking at the skin, you could have you could see the pathology very firm and indurated.

But he also sort of held up his arm and said, why does my arm do this demonstrating his what you see in the pictures is his very classic Groove sign and Pau de Arrange changes. Notably, no Raynaud's, no rashes, no weakness, normal nail full capillaroscopy and then if you looked at his labs actually going back to the summer he had a peripheral eosinophilia that had previously been missed. So we were very concerned at that point even without the MRI sort of our leading diagnosis with the classic skin changes and even his neuropathy for eosinophilic fasciitis. And even before biopsy the MRI actually kind of clinched the diagnosis. Very classic findings of essentially fascial enhancement that actually extend in into the inter muscular planes but without any surrounding myositis And so I know at some centers actually, because one of our attendings did a lot the scleroderma research at UCLA and one of his partners in that field on the East Coast actually just used the skin findings and an MRI to make the diagnosis.

But since it was such an interesting case for us and we don't see that many come through, we decided to pursue the full thickness biopsy.

What I think is really interesting about this is that sometimes we miss the diagnosis. And to be fair, the team that saw him initially thought it was a cardiovascular issue, and of course in the emergency room we have to focus on the things that can kill a patient. But, what's interesting, and you pointed this out Lucas, is that he had a peripheral eosinophilia then. So, the question really becomes, had he been more apt to follow-up, maybe, you know, some other conditions I'm sure played a factor, then maybe we would have had a diagnosis sooner. But, you told me an interesting thing about this case too, that the patient now doing well.

What did you do for him? Tell me about that.

So, again not much literature in terms of directing us for treatment, but the larger case series, 10 or 15 patients, when I say large, Essentially first line is prednisone and from that data as well it kind of suggests again the number of patients is quite small that that most patients are not going to have a complete response to prednisone alone and so typically sort of the first line again as in most, of our diseases is to add methotrexate as a steroid sparing agent. The thing to keep in mind is the literature for patients who do respond may not see a response for eighteen, twenty four months. And so your goal with treatment is not so much in the short term to get the skin better, but it's to prevent it from getting worse. And so a big part of that is preventing joint contractures. The patients historically who did poorly, actually ended up with a lot of morbidity related to the joint contractures and so the steroids and the methotrexate, as well as help from our physical therapists and occupational therapists, is really to prevent, that morbidity even if we can't get a complete response in the skin.

Well, think this is a great case. It's one more for the books, especially since we don't have a huge patient population. Thank you for coming, Doctor. Lashan. I really appreciate it.

Thank you. And, more from roomnow.com, and continue to check us out at RWCS twenty nineteen.