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Welcome to UAB MedCast, a continuing education podcast for medical professionals providing knowledge that is moving medicine forward. Here's Melanie Cole.

Melanie Cole (Host): Clinical practice guidelines for management of idiopathic pulmonary fibrosis were recently updated with some significant changes in optimal management. Today, we are highlighting the key points for both pharmacologic and non-pharmacologic management of idiopathic pulmonary fibrosis based on current guidelines. Welcome to UAB MedCast. I'm Melanie Cole. Joining me today is Dr. Tracy Luckhardt. She's a pulmonologist in critical care medicine and an Associate Professor at UAB Medicine.

Dr. Luckhardt, it's a pleasure to have you join us today. As we get into this topic, please tell us a little bit about idiopathic pulmonary fibrosis, the prevalence, what you see in the trends.

Dr Tracy Luckhardt: Sure. So, idiopathic pulmonary fibrosis is a fibrotic lung disease, meaning that there is progressive scarring in the lungs. This is a disease that mostly occurs in our elderly population, so after the age of 60. This is a disease that is progressive, so most patients have progressive shortness of breath and cough. And eventually, it will lead to respiratory failure. Most patients with this disease survive about four to five years after the diagnosis.

So, idiopathic pulmonary fibrosis incidence is increasing. This is likely due to increased aging of our population as well as increased recognition and diagnosis of the disease. It is a rare disease, only affecting about one in every 100,000 people across the country. We do not necessarily have higher rates of IPF in the south than other areas of the country.

Melanie Cole (Host): Well, thank you for that. Then, we understand that clinical practice guidelines for management were recently updated with some changes in optimal management. Can you speak about those?

Dr Tracy Luckhardt: Sure. There are new guidelines as of 2022 with the American Thoracic Society, the European Respiratory Society, Japanese Respiratory Society. And the Latin America Group as well have updated the clinical practice guidelines on IPF and progressive pulmonary fibrosis. So, the key findings that were updated in the 2022 guidelines were to solidify that a probable UIP pattern on a high-resolution CT scan can support the diagnosis of IPF without having to do a biopsy or tissue confirmation, especially in the appropriate clinical setting and having been discussed in a multidisciplinary discussion. So basically, we can now make the diagnosis of IPF without a biopsy when you do have a probable UIP pattern in addition to a definite UIP pattern on the high-resolution CT scan.

Also, in the diagnostic arena for IPF, the committee also gave guidance that a transbronchial cryobiopsy is an acceptable alternative to surgical lung biopsy in patients with unknown interstitial lung disease. So basically, you can either do transbronchial cryobiopsy or surgical lung biopsy to make a definitive diagnosis of ILD. This is reserved for centers who have significant expertise in transbronchial cryobiopsy, but they did give a nod to that as an appropriate method for obtaining a histological diagnosis.

And then finally, as far as diagnostic goes, they discussed the new genomic classifier that is clinically available, but did not give any recommendations about whether or not it should or should not be used in diagnosing interstitial lung disease. And then, they also touched on the treatment of IPF. They said that patients with IPF should not be treated with antacid medications in the setting of asymptomatic disease or without a history of reflux disease. This has not been shown to improve outcomes in IPF patients. And so, just empiric treatment with antacid medication should not be done.

And then, patients should not be referred for anti-reflux surgery with the aim of improving respiratory outcomes. So, obviously, if they're having significant symptoms from a GI standpoint and that could improve their quality of life, and it's not a significant risk from a respiratory standpoint, patients can undergo anti-reflux surgery, but it should not be done for the sole purpose of improving outcomes in IPF patients, as that has not been shown to be beneficial.

Then finally, they talked about the concept of progressive pulmonary fibrosis, and defined progressive pulmonary fibrosis as a definite phenotype and gave a definition of that disease. So, those were the main updates in the 2022 guidelines for idiopathic pulmonary fibrosis.

Melanie Cole (Host): Thank you so much for going over those and you took some of the questions right out of my mouth as far as if they have a history of reflux and GERD, are we treating them with those medications? Are we looking at some of the anti-reflux surgical options? And of course, you mentioned that if it would help them with symptoms, but not necessarily for respiratory outcomes. I'd like you to speak about the key points for pharmacologic interventions and what you're looking at today. What are you doing for these patients medicationally? And then, you can go right into the non-pharmacologic management and what role surgical treatment, advanced life support, any of those things have in this disease course.

Dr Tracy Luckhardt: Sure. So right now, we have two antifibrotic medications, nintedanib and pirfenidone, which have been approved for patients with idiopathic pulmonary fibrosis. Those were approved in 2014. And for the last eight years, they've been the only therapies and remain the only therapies that we have for idiopathic pulmonary fibrosis. We now know that patients should be started on these medications at the time of diagnosis, even with very early disease, that these medications do improve outcomes and mortality in patients with IPF. If patients do have symptomatic reflux, they should be treated for that. Also for pharmacological therapies, we do recommend whenever possible that patients participate in clinical trials. There are a lot of new medications that are in phase II and phase III clinical trials for idiopathic pulmonary fibrosis, and we need patients to participate in those trials so that we can find new therapies that either add to or work better than our current therapies, especially given that our current therapies do not stop the progression of this disease, they just slow it down.

And then, for nonpharmacologic therapies, it's really important to pay attention to comorbidities in idiopathic pulmonary fibrosis, so looking for obstructive sleep apnea and treating when it is there, looking for pulmonary hypertension, so echocardiograms and right heart caths. We do now have an FDA approved treatment for patients with IPF who have pulmonary hypertension, and that is inhaled treprostinil. And so, we do need to screen for pulmonary hypertension and get right heart cath confirmation whenever we suspect that. We need to make sure that patients are up-to-date with all of their routine health screenings, such as lung cancer screening CTs and other cancer screening through their primary care and making sure that patients are fully vaccinated against respiratory illnesses, so that we can try to avoid them getting sick.

And finally, for patients who are otherwise healthy, other than their idiopathic pulmonary fibrosis, we need to think about lung transplant referral. And patients should be referred early in the course of treatment for idiopathic pulmonary fibrosis, either at the time of diagnosis or especially when they first start needing supplemental oxygen, they should be referred for lung transplant evaluation.

Melanie Cole (Host): Thank you, Dr. Luckhardt, for telling us about the important role of managing those comorbidities during treatment. And I would like you to speak to other providers about the importance of objectively assessing clinical decline. You just mentioned referral for transplant and that early referral is so important. So, I'd like you to speak about the referral criteria and how you take care of a patient on the wait-list or management during a flareup, really what happens for those patients as you're assessing that clinical decline and they're waiting on transplant?

Dr Tracy Luckhardt: Yeah. So, idiopathic pulmonary fibrosis is wholly a progressive disease, which is why referral to transplant early as possible is really key. So in general, patients will be candidates for transplant if they are not smoking, they're not using any other substances like alcohol or narcotics. They need to be healthy, so no liver disease, kidneys disease, significant heart disease. They need to be near normal weight. So in general, patients need to have a BMI less than 35 to be evaluated for lung transplant. They need to be active, so they need to be able to walk on their own about 600 feet and still be pretty active in their daily lives to be a candidate for transplant. But in general, it's really important, if there's any questions about whether or not a patient might be a transplant candidate, to go ahead and refer them to a transplant transplant center and have them evaluated to see if that might be a potential option for them.

And again, early referral is very important because the course of IPF can be difficult to predict and patients can experience acute exacerbations at any point along their course of disease and despite treatment with antifibrotics. And so, you would, if they are a transplant candidate, want that process to already be in motion if they do unfortunately have an acute exacerbation, so that they could be transferred to the transplant center and potentially bridged with ECMO or get on the list in an expedited fashion if they are having an acute exacerbation.

Melanie Cole (Host): Dr. Luckhardt, this is such a good topic. And as we wrap up, I'd like you to summarize it for us. Speak about the importance of that multidisciplinary team for treating these progressive patients and any other interventions. As you're thinking about other providers that are involved in that multidisciplinary approach, how they're helping their patients during that time with weight loss, smoking cessation, any of those comorbid conditions that you discussed, I'd like you to kind of wrap it up and tell us about your team.

Dr Tracy Luckhardt: It is really important to have a multidisciplinary team when you're taking care of IPF patients. It's important from the get-go for diagnosis to have patients discussed at a multidisciplinary conference with experienced thoracic radiologists, pathologists and ILD providers so that an accurate diagnosis can be made. It is important to have a good relationship with a lung transplant center, so that early referral can be made and that, they can follow along with you with the patient's course; gastroenterology for management of reflux symptoms and sleep physicians for management of obstructive sleep apnea. Also, working with pulmonary hypertension experts so that we can diagnose pulmonary hypertension and get patients with IPF treated with inhaled treprostinil if appropriate. It is also important to have good palliative care and supportive care providers to help with patients as their disease progresses to help manage symptoms of dyspnea, cough, weight loss and depression and anxiety.

Melanie Cole (Host): Thank you so much, Dr. Luckhardt, for joining us today. And a physician can refer a patient to UAB Medicine by calling the MIST line at 1-800-UAB-MIST or by visiting our website at uabmedicine.org/physician. That concludes this episode of UAB MedCast. For updates on the latest medical advancements, breakthroughs, and research, please follow us on your social channels. I'm Melanie Cole.