So, thank you for participating in this session entitled ILD, Choose Your Own Adventure, where if you will get your app open, and in the very first question, there's a QR code for that. This QR code on the screen now takes you to the full course that's in the e-learning library of CHEST. But this is a portion of a larger program where you are going to guide kind of where we go. I'm Dr. Sandra Adams from UT Health San Antonio, and I also work at the South Texas Veterans Healthcare System. I'm joined by Dr. Zulma Yount, who is Associate Professor at National Jewish Health, and also Dr. Kevin Chan, who's a professor in medicine in the Pulmonary Diseases and Critical Care Medicine Division, and from the University of Michigan. And you can see our disclosures as they went up on the slides. We plan to share with you the evaluation, diagnosis, and really the interprofessional multidisciplinary management of patients with various forms of ILD. So as I mentioned, in order to use the ARS session, ARS, which is going to be really important for this session, you can go into this session on your app and scroll to the bottom and use, hopefully you've been able to do this for those of you who were here yesterday. And if not, when the first question comes up, there's a QR code. I understand you only have to scan it once for the session, and then you can just answer the questions after that. We'd like, if possible, as many people to vote as possible, as many as we can, just because I think that you'll get a lot more out of it, because we'll go mostly with the majority, but sometimes if there are two that are pretty close, then we may talk and go some other routes. So let's start our first case. This is Susie, and she's a 61-year-old woman who comes to see you with the following responses to your questions. What brings you in today? My doctor referred me to you because my cough and my breathing is getting worse. When and how did your symptoms start? So like one year and a half, I start with very dry cough. And then like one month later, I have short of breath, so it's getting worse. When your symptoms didn't improve, did you get them evaluated? I went to see my regular doctor, and he thought I have asthma, so he gave me some inhaler to see if that improved. My doctor referred me to a pulmonary, so they check and they say I have some scars, something they don't know what is coming from. Okay. And do you have a cough? So I start coughing like one and a half year ago, and it's very dry, so I don't, it's just coughing. Okay. And are you still working? Yes. I'm a school bus driver. At home, do you have any visible mold, flooding, or water damage? I have no mold and no water damage in my house. Okay. What about down or feather pillows? Yes, I love the pillows, and I have it for like five years. As far as you know, do you have any heart, kidney, or autoimmune disease? No, not that I know of. How about any joint pain, swelling, or rashes? No, not that I notice. Okay. Well, let's look at some of Susie's information here. She was given the albuterol MDI as needed. She's got bilateral upper lobe inspiratory squeaks with bivascular crackles. She has some moderate restriction with diffusion capacity that's also reduced at 54%. She was only able to walk 600 feet on her six-minute walk test, but she did not desaturate. So let's look at her high-res CT. Give you just a second to look at it yourself there. So you see prone imaging on the left, supine on the right. And if we can basically shows moderate diffuse. There's some upper lobe significant subplural reticulation, some patchy ground glass. See there are areas of ground glass in there with some mild bivascular traction, bronchiectasis. But I don't see any honeycombing on there at all. There's a little bit of mosaicism, especially on the like supine top right image. You can see that laterally on the left. There's some air trapping there associated and around that ground glass. And then especially on expiratory imaging, you can see some air trapping as well. That leads us to our first question. So this is where when this comes up, get your phones ready if you haven't already gotten into the ARS because you're going to scan the QR code and answer this question. Based on the information you got from Susie's history and everything else, what do you think her most likely diagnosis is? Is it HP? Is it RA associated ILD? Is it IPF? Is it SSC ILD for scleroderma associated? Or is it combined pulmonary fibrosis and emphysema CPFE? Go ahead and vote now. Okay, so we have 22% that said maybe IPF, one with CPFE, and 74% with hypersensitivity pneumonitis. So the majority rules on this one. We actually, this is hypersensitivity pneumonitis. And there was one by the way question. So let's ask Susie if she has any pets. So I was raised with, my family have chickens, and then I don't have dog or pets. But my daughter give me a parakeet, and his name is George. So I just clean and feed him every day. It's in my living room. Okay, so that was a by the way deal. So let's, the down pillows were enough to make your suspicion go up that this was hypersensitivity. The high-pitched squeaks are one of the things. And the big deal with hypersensitivity pneumonitis, which you probably know, is that we really need to get the inciting agent out. And so whatever we do, as far as, you know, treatment, the biggest thing is getting whatever it is out of the house. And so George needs to find him a really good home if that's possible at all. And at a minimum, to move him to an area far away from where she stays and, you know, get others to clean the cage. There are industrial hygienists that go in and look for mold. And then the HP panels vary a lot. So they're not really sensitive. They're not really specific. But sometimes we can find them a little bit helpful. So with that, I'm gonna turn it over to Dr. Yount. Good morning, everybody. Okay, so we're gonna move on to our next patient, Ginger. This is Ginger. So Ginger's a 45-year-old woman who comes in to see you with the following responses to your questions. Ginger, what brings you in today? So my primary care physician referred me to you because my breathing just keeps getting worse. When and how did your symptoms start? About a year ago, I started getting short of breath on my morning walks with my husband. And do you feel short of breath at rest or only with activity? So I'm actually okay at rest. I don't have any trouble breathing then. But I am able to go on walks, but mainly just about a mile or so. And I'll do that about three or four days a week, just kind of depending on whether or not the weather is warm outside. Do you have a cough? You know, now that you mention it, I do have a dry cough. It started about six months ago or so, but it doesn't really bother me. And do you have joint pain, swelling, or skin rashes? You know, sometimes in the morning, I'll get some pain in my wrists and in my hands, and in my joints in my hands. And then I notice sometimes my hands are cold a lot, actually, and they'll look kind of gray. Do you have any heartburn? Actually, yes, I do. I kind of eat antacids almost like candy sometimes. Have you ever smoked cigarettes? Oh, absolutely not, I've never smoked. At home, do you use down or feather pillows, or have any visible mold or water damage? No, none at all. And does anyone in your family have any heart, lung, or autoimmune disease? So my mom had rheumatoid arthritis. I have a cousin with lupus. Other than that, there's no lung disease or anything like that in my family. Okay, so next let's look at some of Ginger's medications and data. So medications, she's taking famotidine, as she mentioned, for her significant heartburn. On exam, note that when her saturations are checked on her fingertips, it looks like she's adding 86%, but when the probe is put on her earlobe, it's 96%. Vitals are stable. On exam, she has some perioral skin tightening. Chest exam, she has dry crackles at the bases. And on her skin and extremities, she has no rashes, but mild skin tightening and puffiness on the fingers bilaterally. And now we're gonna look at some of her lab data. So the sort of basic labs are normal. An ANA, however, is positive with a one to 320 titer in a nucleolar pattern with a positive SCL70. She has otherwise negative autoimmune serologies. On her PFTs, she has a restrictive physiology with a reduced FEC and total lung capacity. DLCOs are also notably reduced. And on her six minute walk test, she walked 1,400 feet on room air and did not desaturate during that test on her using that earlobe pulse, pulse oximeter. So on the screen now are some images from her HRCT. Let you guys kind of take a look there. Note primarily that as you get towards the bases of the lungs, there are areas of some ground glass, primarily, it's always hard to tell if there's not a little bit of reticulation there. You can often tell if you look for a basal or bronchiectasis or bronchiolactasis, like in that bottom right one, you can kind of see a little bit of it. But there's otherwise no significant mosaicism, nodules, masses, or effusions. And notably on this exam, especially that top left one, you can see that the esophagus looks quite dilated there, almost looking like a smooshed second trachea there. So that's a notable finding on this. Okay, so then this leads us to our first question on Ginger. So you can get your phones out ready to vote. So based on this information, what is Ginger's most likely diagnosis? So the options are hypersensitivity pneumonitis, RA-associated ILD, idiopathic pulmonary fibrosis, systemic sclerosis ILD, or combined pulmonary fibrosis and emphysema. All right, good. So I think everyone was kind of clued in on the autoimmune here. So rheumatoid arthritis, we have 23% of the vote and 77% with systemic sclerosis, which fits with this overall patient with an elevated ANA, nucleolar pattern, her age, some of her skin findings, re-nodes. Just a little information about systemic sclerosis. So, yes, it's an autoimmune condition. It's characterized by immune dysregulation and scarring and fibrosis that develops and deposits in various organs. Here on the right are some sort of the characteristic findings. There's two predominant forms now recognized, limited and diffuse cutaneous, and a lot of us grew up learning about CREST, which isn't really used so much anymore, but that goes more with the limited cutaneous. And the diffuse cutaneous can affect all parts of the body in the way of skin, hence the name, and can be the more severe form in the lungs. Interstitial lung disease and pulmonary arterial hypertension are the predominant manifestations that we get concerned about, and both can carry a very high mortality in this disease. I'm including this slide here just to inform everyone that there is more to the antibodies than just SCL70 and Centromere. There are entire panels now that can be sent. This is, on the right there, lists the ones that we have over at National Jewish that we send. And this Venn diagram is courtesy of Virginia Steen at Georgetown, who is certainly a leader and an expert in this field. And as you can see that these antibodies kind of lean one way or the other, and with the way of diffuse and limited, can be helpful to rheumatologists, but all of them can have some significance, so worth noting. In terms of, as a pulmonologist, you know, what do you need to know? I think what's really critical when you have a patient with scleroderma ILD, you know, when is it gonna be progressive? When is this something that you need to be really clinically concerned? And one of the key, key things is early in the diagnosis. So within the first five years of the scleroderma diagnosis is a time that needs to be monitored quite heavily. Diffuse scleroderma is a bit more likely than limited, but both can be progressive. In the way of the antibodies, the SCL70 and a nucleolar pattern of clinical significance as our inflammatory markers, and then in terms of physiology, it reduced FEC in just physiology, greater than 20% extent of fibrosis on HRCT. So these are all kind of things to be aware of. It's kind of unique to scleroderma in that there are some things that we know are significant in the way of risk of progression. Okay, so let's watch as Ginger's clinician talks to her about her diagnosis. Well, Ginger, I've now had an opportunity to look at all of the CAT scans and X-rays that you brought. Thank you very much. And taking those in the context of the shortness of breath, et cetera, that you were telling me about earlier today, I think you have something called scleroderma interstitial lung disease. It's also just abbreviated S-S-C-I-L-D. That's a big alphabet soup, so let me break that down a little bit. The scleroderma refers to that the disease can affect many portions of the body. And so, for example, in addition to your shortness of breath, you were also telling me about your reflux and needing all those antacids. Can also affect the joints. And you were telling me, yeah, the soreness and then the white-gray discoloration when they get really cold. So taking care of this disease not only includes seeing a pulmonologist, but also seeing a stomach doctor, gastroenterologist, and also a joint specialist, a rheumatologist. And so, since you're in my pulmonary office, the ILD, interstitial lung disease. And what that means is that there's a lot of inflammation in the lungs. And the interstitial just describes part of the lung tissue that gets really inflamed. And then lung is your lungs and the disease. And so, what we need to do is to make sure that we have a multidisciplinary team, meaning the stomach doctor and the joint doctor, gastroenterologist, rheumatologist, working with me so that we take care of all of these pieces together. From my end, it will be making sure that we have all of your vaccines up to date. All the lung things that the CDC, Center for Disease Control, recommends for folks who have lung disease. And then we will also keep monitoring how well your lungs are functioning. And also, if you need oxygen, making sure that that gets taken care of. So, I'll have you see a few other physicians, and then we'll go from there. Okay, so now let's take a look at our next ARS question. Which of the following would be a recommended initial treatment approach for a ginger? So, start oral cyclophosphamide. B is prednisone, 60 milligrams daily for three months with a taper. C, profenadone. D, nintedanib. E, don't start any therapy at this time and monitor for clinical deterioration. F, tocilizumab. And G, mycophenolate. And this is where we really, where we go is based on what you choose. So, we have a sprinkling here on various answers, but it looks like the majority went with mycophenolate, which is one of two correct answers, of two potential correct answers on this one. I wanted to just take a moment to say that for those that chose prednisone. So, prednisone in general should be avoided in this setting. The 60 milligrams daily is quite a bit anyway, but there are treatments that are known to be effective and prednisone has a lot of negative side effects. There's also still, there's the concern about scleroderma renal crisis too with steroids. The profenadone has not been approved outside of IPF and nintendinib, we'll get to, but it does have approval for this indication. However, given various disease manifestations that ginger has, there are probably anti-inflammatory autoimmune therapies that would be sort of a better start. Hence, the bottom two answers, the tocilizumab and mycophenolate having, would probably be the right way to go. And mycophenolate in particular probably has sort of the best dedicated sort of RCT data for this. So, the scleroderma lung study two was a study that looked at mycophenolate versus cyclophosphamide and basically was, showed that they were equally efficacious, so non-inferiority in this case, looking at 24 months, but with better tolerated, so fewer side effects. So in general, this has historically been kind of the first line for immune suppressive therapy for several years in this disease. Okay, so let's see how ginger's doing on mycophenolate after three months. Ginger, it's lovely to see you this afternoon. I think it's been about three months since we first met and I started you on that new drug. How have you been feeling since you've been taking it? Well, I actually am doing a little bit better. I'm not having such a hard time when I'm gardening. I'm able to take some walks with my husband, but I do have the dry cough still, just a little bit. But, you know, things were okay. I think it could be better, but it's okay for now. I'm glad to hear that. That's absolutely fantastic. I think at this point, I'd like to continue you on your medication and we're gonna need to check in with each other about every three months, making sure that those blow tests, those pulmonary function tests are staying stable, checking in and making sure that your shortness of breath, your cough are not getting worse, and making sure that you're not having any other, or having any other concerning symptoms. So at this point, what other questions do you have for me? I think that's it. Okay. Thank you. Well, let me know, certainly in the interim, if your cough is getting worse, your shortness of breath is getting worse, and then we'll meet sooner than three months. Okay. Okay, so now we're gonna jump ahead a little bit to two years in the future. You know, in the meantime, as the clinician there said, you know, close follow-up every three months, kind of making sure that no new symptoms evolve, that you get the multidisciplinary team involved if needed with other referrals. But now, kind of returning now to the sort of her pulmonary presentation two years later. So now, she comes back in, and her oxygen saturations on the fingertip are now 74%. She does have that rain node that's been identified. So on the earlobe, she's 92% at rest on room air. On her six-minute walk test, remember previously she actually walked around 1400 feet without desaturating. Now she's at 85% using the earlobe pulse oximeter after five minutes on room air, and so she's only walking 900 feet now. On her PFTs, you can see going from left to right how her FEC has dropped initially from 69%, now down to 58%. DLCO is now down as well, down to 48%. And under HRCT, she has significantly worsened new fibrosis, more subplural reticulations, still no honeycombing, some stable ground glass changes. On echo, she has normal right and left chambers, so no evidence of pulmonary hypertension in this interim. Okay, so now let's watch to see Ginger and her clinician now discussing her new symptoms. Ginger, it is always so lovely to see you. It's been, golly, two years now. Well, how have you been feeling over the past three months? Well, you know, so I saw you three months ago, but about a month before that, I started going downhill, and I wasn't sure exactly if I should say anything or not, so I just, I left it alone just to see, you know, if I would continue to take the drugs, if it would improve, and it just didn't. It actually kind of went downhill over the last three months, and maybe four months, really. So my breathing's been harder. I'm not able to walk as far, so it's kind of. And your gardening. My gardening's just a lot worse. I still have this dry cough. It's gotten worse. It just, everything has just kind of gone downhill. Well, truthfully, I'm not surprised to hear you say that. Looking at your blow tests, those pulmonary function tests, those are not as strong as they were the last time I saw you, and your CT scan, actually, in addition to having more inflammation on it, is now starting to show some scarring. And, you know, we had you walk around the office and check your oxygen, and it's looking like you need some oxygen when you walk around, and I'm fairly convinced you'll probably need it while you're sleeping, as well. Okay. Yeah. Is there anything else we need to do from here? Well, we'll have to talk more about that. Okay. Okay, so just to remind you of some of the data here before our next question. Okay, which is question number four now. Which of the following would be the best approach for Ginger at this point? And you can go ahead and vote. Would you start oral prednisone now, 60 milligrams daily for a month with a taper, start perfenadone along with clinical trial referral and lung transplant referral, start nitetinib with these same referrals, or continue to monitor and no further therapy at this time? All right, so 90% said start Nintedanib with referrals to lung transplant program and possible clinical trials. That is the correct answer, as the majority of you said. The data here, the next slide here is the census trial, which was initially what led to approval for Nintedanib in scleroderma ILD, and as you can see, this is a graph showing sort of the take-home point here with the reduction in FVC being markedly reduced in the Nintedanib group relative to the placebo group in this study. And that wraps up Ginger's cases. Y'all are doing great with these questions and stuff. So next, we're gonna move to Dr. Chan. All right, moving on to the next case. So John is coming to see you. He's a 72-year-old man, coming with the following responses to these questions. So I'm gonna start with, hey, John, what brings you in today? My primary care doctor referred me here today because I've been having trouble breathing and it's been getting worse lately. When and how did your symptoms start? Well, you know, it was about a year and a half ago, I think I was on a family ski trip to Colorado, and I noticed I was getting winded very easy when I was skiing and when I was in the higher altitude. But when I got back to Miami, when I got back home, the symptoms went away. It seemed like they were fine in Miami. But then about nine months ago or so, I started noticing that I would get winded easy when I would carry things, like I was carrying lumber into the garage and I noticed I was getting winded very easy. And then it kept getting worse and I noticed even going up and down stairs, I was getting winded very easy and breathing was becoming more difficult and even carrying groceries in and out from the house became harder and harder. Do you feel short of breath at rest or only when you're active? When I'm resting, I really don't feel short of breath. It's mainly with activity. It's mainly when I notice it on the staircase or when I'm trying to move around and it's been getting worse. Do you have a cough? I do have a frequent cough. It is not productive. It's a dry cough. I don't notice that I'm coughing anything up. Do you have any known heart, kidney or autoimmune disease? No, not that I know of. How about problems with joint pain or swelling, skin rashes or any new lumps or bumps? I do have some problems with my right knee that I date back to an old high school injury. But other than that, I don't have any of those problems. All right, let's look at John's medicine exam. He's on a Meprizol, a torvastatin for hyperlipidemia. His oxygen levels are good. He has bi-basal dry crackles without wheezing. And he's got clubbing of his digits. His labs are relatively normal and he has mild restrictive ventilatory defect present with diffusion impairment. And he walked a pretty good distance, 1,400 feet on room air without significant desaturation. Now let's look at John's HRCT. And as you can see, these are prone images and they are, this is the upper lobes and you can see some reticulation. A little bit of ground glass, just a very, very small amount, but these reticular changes are in the subpleural distribution. Next slide. And here at the bases, it's more prominent. We have some areas of traction, bronchiectasis and honeycombing at the bases and again, in a subpleural distribution. So it leads us to the next question. Based on this information, what is John's most likely diagnosis? Hypersensitivity pneumonitis, rheumatoid arthritis associated ILD, IPF, systemic sclerosis associated ILD or CPFE. Next slide. All right, I think the majority got the right answer of idiopathic pulmonary fibrosis. In fact, John, in this particular instance, has all of the characteristics of IPF. And I just wanted to point out that there was a new consensus statement put out by the conjoined ATS, ERS, Japanese and the Latino or South America thoracic societies. And this was an update of the 2011 guidelines. That was in 2015, but more recently, they updated again. It just came out this year that added progressive pulmonary fibrosis to the consensus statement. These are very good guidelines that can assist us. And CHESS just, in fact, published a guideline on the practical integration of these guidelines for us. And that is also available for review and can help you understand and determine diagnosis between hypersensitivity pneumonitis and idiopathic pulmonary fibrosis. And in that guideline, and just as we had presented in this patient, with your clinical and history physical and with the addition of the HRCT scan, and you can follow this algorithm going down and using the chest HRCT pattern. In fact, this patient's HRCT is definite UIP. And in that particular way, the definitive, the confidence that the patient has histopathologic diagnosis of IPF is very high because he has no alternative diagnosis as a cause for the changes of UIP. His diagnosis of IPF is definite without the need for biopsy or tissue. Next slide. So based on this information, let's look at another question. How many of the following interventions would you recommend for John next as his physician? And just note that we just read the, we'll go through the possible options and we'll determine how many of those options that are available are the best answer. So would you just follow up with the patient in one year and repeat full PFTs and a CAT scan? Would you give him vaccinations as recommended by the CDC? Return the patient to the PCP for follow-up care, referral to hospice, pulmonary rehabilitation, referral to a pulmonary fibrosis foundation or other support group, or prescribe and or prescribe prescript supplemental oxygen therapy? Great, some had four, but three is the correct answer. And we'll go over that. Obviously, vaccinations by the CDC is important, pulmonary rehab and referral for the support group. Let's go on to the next video with John, discussing these questions and answers. So, Mr. Jacobson, I've had the opportunity to look at the different studies that you've had, your x-ray, your CT scan, and putting those in context with your history about your shortness of breath and how long that's been going on and how it's been progressing, leads me to feel that you have something called idiopathic pulmonary fibrosis. We shorten that for IPF. What's that? Right, what does that mean in English? Idiopathic means doctors are idiots, we don't know what causes it. Pulmonary refers to the lungs, and fibrosis means scarring. So what it is, it's a condition in which your lungs continue to scar more and more and more over time. And as they become more scarred, folks feel that their breath is more short over time because they have less lung that's doing the right thing. Wow. Yeah. That sounds awful. Is there anything we can do for that? You know, there are a lot of things that we can do. We can't cure it yet, but there are a lot of things we can do to help your body work with it as best it can. Well, what can we do? Well, the most important thing is actually what's called pulmonary rehab. And what that means is it's like physical therapy for your lungs. Helping your body learn how to use the oxygen you bring to it as best it can. So, physical exercise. And they have programs that are designed specifically for folks who have lung disease. You can tell me where to go and how to get that? Absolutely, yes. Yes, we're gonna help you with all of this. Thanks. Oh, yeah. The next piece is making sure that your immune system is as strong as it can be. And what I mean by that is having you receive all of the vaccines that are recommended by the CDC or the Center for Disease Control. Oh, that's good. Yeah. The last piece I think is really important. And it's kind of twofold. The first is, this is a hard road to walk. And so, if you'd like to join a support group, meaning getting together and talking with folks who also have IPF and their families, it can be really helpful because the challenges that you're going to be facing and your family's gonna be facing will be similar. And it's often nice to be able to share those circumstances that are the same. And you can hook me up with those people? Absolutely. That's good. Yeah. The next piece, and I recommend this for all of my patients, regardless of why they are ill, is having a medical proxy, a power of attorney. Because sometimes we all get sick and we can't make decisions for ourselves. So having somebody who knows what you want and then you've also discussed this with your family, that way, if you're not able to speak for yourself, folks know what it is that you would want to have happen if you become really ill. Is that something I should have done and talked to my wife and kids about? Absolutely. That sounds like a good idea to me, too. I don't want them to be surprised. I'd certainly need their help through this. Without a doubt. All right, based on this information, what would you recommend for John? Would you prescribe a trial of prednisone, 60 milligrams for four weeks, followed by a slow taper? Prescribe a trial of triple therapy with prednisone, azathioprine, and acetylcysteine? Prescribe a trial of an acetylcysteine alone? Start an antifibrotic agent, such as perfenadone or nantenatib? Or refer the patient to ILD Specialty and Lung Transplant Center? And this is for best answer. So, this is, I'm really, really happy that nobody picked the top three. I was, in fact, this is just funny, off topic, but I was talking to Sandra about that, and I said, there's just no way anyone's going to pick those anymore. But we're sometimes surprised, so congratulations. I think the bottom two are outstanding answers. We now have the antifibrotic agents, and as pulmonologists, we're very much aware of that. But also, to refer to an ILD specialty lung and transplant center. This is, to me, a mixed answer. As long as you have the ability to get prior authorizations and will monitor and have experience with the antifibrotic agents, I think us as pulmonologists can manage that. But at the same time, I think that the accessibility and referral to an ILD specialty and lung transplant center is important. Part of that is that the chance for a lung transplant, there was recently a paper that was just published that I just reviewed, indicating that if you refer to a patient to an ILD specialty center with a transplant center, there's a much, there's four times greater chance that the patient will get a transplant when they need it. That's just something to consider. All right. Next slide. This just updates the information. Again, I refer back to the official guidelines. And in 2015, the questions about IPF therapy with nintenidib or profenadone were both recommended by the consensus group. Next slide. So now that we've moved on to that point, which, I'm sorry. So let's watch as John's clinician sees him in three-month follow-up after his referral to ILD Center of Excellence. Mr. Jacobson, it's wonderful to see you. I think it's been about three months since we first started to talk about your IPF. And I've got great notes from your attendance with pulmonary rehab. Thank you for getting all of your vaccines. That's absolutely wonderful. And it seems like the support group is, you know, been really helpful for you and your family. The support group, the rehab has really been helpful. Fantastic. So I had also asked that you make an appointment with one of the IPF specialty centers. Tell me about that visit. You know, that visit worked out quite well. I went and they put me on a drug. I forget the name of it. It starts with a P. Profenadone. Yes. And that seems to have helped quite a bit. And they also gave me something to help with the diarrhea and the nausea and the stomach cramping. So they really worked with me very well on that, making sure that that wasn't as big a problem. So you were able to work through a lot of those GI side effects. Yeah, we did. We worked through those. And so I'm really feeling better. Thank you so much. Absolutely. So we're going to move forward in time. And we're going to look at new data. You've been following the patient every three to six months. And 15 months after his initial visit, he gets new lung tests. And you can see that the FEC has fallen a very small amount, from 71% to 68% predicted. TLC is at 70% predicted. But the diffusing capacity has really fallen. He gets an echocardiogram, and it shows that there's a mildly enlarged right atrium and right ventricle. And they could not measure the tricuspid jet, but they determined and measured the TAPSE, which is 13 millimeters and low. So then he gets a right heart catheterization because of suspicion for pulmonary hypertension. And you can see here that his mean pulmonary pressure is 61 millimeters of mercury with a PVR of 7.3 wood units. Normal is less than two wood units. And he gets a new CAT scan that really doesn't show any change. So there's no new ground glass or worsening reticulation. So let's look at the next question. Which of the following would you now recommend for John at this point? Do nothing. Progression is inevitable. Next switch antifibrotic agents. Next start oral ambrosentin. Next start sildenafil or triprostanil with pH center of excellence referral. Excellent, so again, we were looking at this, oral ambrosentin, it definitely is not the answer. Switching an antifibrotic agent is unlikely, there's no data to support that, it may be helpful. And obviously, in a person that's progressing, we don't want to just watch, especially since we've definitively defined that he has pulmonary hypertension related to the group three pulmonary hypertension. This just shows that the ambrosentin has been studied and it has a negative effect, in fact, increased hospitalizations and mortality. And sildenafil, interestingly enough, it did not, in the STEP-IPF trial, did not meet criteria, the primary endpoint of six minute walk distance, but there were some small benefits of quality of life and dyspnea and exertion. And in a subgroup with right ventricular systolic dysfunction, there was actually improvement in six minute walk distance. Nevertheless, since those were not primary endpoints, the consensus group did not recommend utilization of these medicines, but sometimes it is utilized by our colleagues and some of the pulmonary pulmonologists. But I think that the, next slide, the most important aspect in a patient now that has group three pulmonary hypertension related to idiopathic pulmonary fibrosis, that the recent study by Waxman and colleagues had shown a significant improvement and difference from baseline six minute walk distance, which was the primary endpoint of inhaled triprostanil, that there was a 31 meter, six minute walk distance improvement. And so this medication has been approved for use for treatment of pulmonary hypertension related IPF as of April 21st, 2021. The other thing is that, very interestingly enough, post hoc analysis revealed that the FEC was protected. And now that is also being studied in a 52 week trial, because this was only a 16 week trial. Next slide. Now what's interesting is that this initial, this treatment is nebulized and it was more difficult. You had to do 12 puffs, 10 to 12 puffs a day, or four times a day, I should say. And what's more important now, and next slide, is that this medication is now available in dry powder inhaler that was just approved. In fact, I did not even realize it was present until very recently, which really changes how this medication could be administered as well as compliance. And so I do recommend, this is a diagram from, again, the most recent updated IPF, Progressive Pulmonary Fibrosis Guideline, that just takes you through this algorithm of pharmacologic, non-pharmacologic therapy and monitoring for disease progression and referring patients for lung transplant. Thank you so much. We really appreciate y'all joining and participating. Just wanna say the QR code that's up there, we had it up there at the very beginning, is the full program of this. So if you wanna go through and answer something wrong just to see what we say, then you're great with doing that. And then there are some extra cases that are in there as well. Please don't forget to evaluate the session. And thank you so much for joining us. We really appreciate it. Thank you.

ILD

Interstitial Lung Disease

Hypersensitivity Pneumonitis

autoimmune antibodies

restrictive lung disease

idiopathic pulmonary fibrosis

pulmonary rehabilitation

multidisciplinary care

Idiopathic Lung Disease