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Welcome to the Quick Consult podcast brought to you by Metal. Before starting this podcast, please review the faculty information, disclosure statements, and learning objectives using the link in the episode description. To claim your CME credit, complete the evaluation using the link in the episode description. This podcast is a continuing education activity managed and accredited by Current Concepts Institute in collaboration with Medall. This activity is supported by an independent medical education grant from Bristol-Myers Squibb. Hello and welcome to the pulmonary fibrosis pathway series of the podcast. I'm your host, Doctor Phil McIlnay, and today we're focusing on strategies to optimize antifibrotic therapy, improve patient adherence and confidently evaluate emerging evidence in pulmonary fibrosis care. We're joined by Doctor Corey Kershaw, who is a professor of internal medicine and a specialist in. Interstitial lung diseases at UT Southwestern Medical Center. Doctor Kershaw, thank you so much for being with us here today. Happy to be here, thank you. To start, accurate diagnosis is foundational to management. Can you walk us through the current approach to differentiating idiopathic pulmonary fibrosis, IPF, and progressive pulmonary fibrosis, PPF from other interstitial lung diseases and the crucial role of high resolution CT and multidisciplinary assessment. I think the first thing that we should um keep in mind when viewing these two entities is that IPF is a diagnosis by itself. Progressive pony fibrosis is more of a, a clinical scenario is how to think about it. And number one, PPF progressive pony fibrosis, implies a non-IPF diagnosis. So really any any of these, any of the other fibrosing interstitial lung diseases can fall into this clinical entity definition of progressive pulmonary fibrosis. But what's important is, is that once someone with a non-IPF diagnosis, which usually is good news, that, that's something you see someone in clinic, they have something besides IPF, that, that's, that's a good day. Patients are pleased, we're slapping each other on the back. This is gonna be good news for you. We, we're gonna do some things to treat you, but this won't progress like IPF. But then it does. What if it does? Then patients that have a progressive pulmonary fibrosis entity, they begin to behave like someone with IPF. Their progression. can be at the same rate as untreated IPF patients and have a similar mortality. So, even though I have delivered good news to a patient that they have something besides IPF, I have to continue paying attention because if they do begin to progress, we have to address that right away because there's significant prognostic implications once they meet that definition for PPF. Using the high resolution CT scan for this assessment, it's very important initially because in addition to the history, the HRCT is, you know, it's, it's the history and HRCT are number 1 and #1A in the pieces of data that I collect to determine a diagnosis. And I can, I'm saying I, but I, I as in the world of interstitial lung disease, we can make a diagnosis for the patient often with those two pieces of data. Um, and the HRCT, the technology is tremendous. We can see the, the phenotype of the patient's ILD pattern. And because of that, using it in conjunction with the history, I can often make the diagnosis there. It doesn't require tissue acquisition, doesn't require going to the operating room, putting the patient under anesthesia, which, you know, the, the rule about anesthesia is that no anesthesia is always better than going to anesthesia. Going to the operating room is never the ideal, ideal scenario if we can get our information without it. And If I can make the diagnosis before then, great. Um, but what if I can't? What if the HRCT as powerful as it is, there are those rare scenarios where I cannot determine the patient's diagnosis. What do I do then? Well, I ask for help. We bring in, um, other specialties and we do what's called a multidisciplinary discussion conference or the MDD and The MDD is most powerful in the scenarios where it's not obvious. So, um, the MDD is helpful when I have a non-UIP pattern. I have a patient who is Their progression is at some unusual rate. It doesn't make sense what's happened here. There's some unusual testing that's involved. It's, it has to do, it, it really is helpful when You don't have the most obvious information to make a diagnosis and treat the patient. Um, I can speak for my own, um, institution, RMDD in, it has, uh, multiple pulmonologists. We have one thoracic radiologist. We have a couple of pulmonary histopathologists. We occasionally will bring a rheumatologist into the discussion and together, we review the data and make a consensus diagnosis and treatment plan. And that could be different at another institution, but it just shows you how challenging sometimes these one-off scenarios are when it doesn't make any sense. Often we can figure out what's going on before, but sometimes we can't, and that's where the NDD is most helpful. Once an IPF or PPF diagnosis is made, what are the key considerations when initiating antifibrotic therapy, and what should clinicians convey to patients regarding the expected benefit? Um, I first of all believe that all patients should be offered therapy with an IPF diagnosis and a PPF diagnosis because things, you know, progress and as I said, that is, that is a bad scenario. Um, and Most, you know, all the treat, there are 3 FDA approved treatments for idiopathic pulmonary fibrosis, neuronommalat, ninteninib, and perphenidone. All 3 of them have very similar outcomes, slowing progression of disease, and patients will often ask, well, which one is the best? And a, a better way to answer that question rather than saying one or the other is better, it's I need to decide what's best for you because I can have 5 patients come to my clinic and I may pick a different treatment for all 5 of them, and they, the reasons are patient-specific. What are the patients, what's their lifestyle like? Are they someone who spends a lot of time in the sun? Are they a patient who spent a lot of time in the car and therefore can't maybe get to a bathroom quick enough? Um, are they someone who will not take a pill 3 times per day no matter what? Um, so I, I, my, my considerations have to do with the individual patients. And so when I present and really all any of us present these treatments. I think it's important that we present them a little agnostically, that, you know, look, the outcomes are very similar and I don't necessarily think one is, and I'm, I'm gonna speak personally here. I don't necessarily think one is better than the other in terms of the disease itself. However, there may be one better for you than the others and it has to do with these potential side effects. And I tell patients that yes, I think any of these will slow down the progression of your disease if you tolerate them. I, I firmly believe that 100% and I will believe that forever and ever. However, I don't know that you're going to feel better and I have to, we have to be very transparent with patients about that because there's nothing worse than We make this grand presentation in clinic about how the world of IPF and fibrosing lung diseases is so different now in the last 10 years, and we've got these treatments and you're gonna get better cause often they come in very despondent that, you know, I've given them something that sounds terminal, and they come back in 3 months and like, I feel terrible. Why do I not feel any better? Well, then I messed up somewhere. I need to be upfront about that. So I think it's important we tell them what to expect and there's probably gonna be some other work that needs to be done to make them feel better. And then treating, treating comorbidities, perhaps a referral to pulmonary rehab, um, perhaps a support group. It's, there's multiple things that we have to do to help patients feel better beyond just giving them a prescription for an antifibrotic. There's more to it than that. So we've touched on side effects which can be a major hurdle for that long-term adherence. Can you summarize the most notable side effects for the currently available antifibrotic agents and share practical strategies for their mitigation? I'll talk about these one by one. So I'll start with ninteninib. Nintenib is a pill that you take uh twice per day, one pill twice per day. The most common side effect for ninteninib is diarrhea, and it is, it is common. It's, it's 6. 50% or higher um in all clinical trials. Nintendo has been studied now in 3 published clinical trials for different um fibrosing lung disease scenarios and that 60+% number is there in every single one of the trials. So, um, it's very important that you counsel patients to, you know, I would, I don't want to say expect diarrhea, but maybe not be surprised if they have diarrhea. And For an inteninib, the advantage is that it's only twice a day. Patients are often very attracted to the idea of one pill twice per day. So we really must work hard to ensure that they can tolerate it. So the first thing you, you must insist upon is they have to take it with food, and it has to be a real meal.