Jean M. Elwing, MD - phaware® interview 521

May 20, 2025

Dr. Jean Elwing, a leading expert in pulmonary hypertension, discusses groundbreaking advancements in treating pulmonary hypertension associated with interstitial lung disease (PH-ILD). For years, patients with this condition had limited treatment options beyond oxygen therapy and symptom management. However, recent studies have introduced new hope, showing improved patient outcomes and quality of life. Dr. Elwing emphasizes the importance of early diagnosis, ongoing research, and clinical trial participation in pushing the field forward.

This Special Edition episode is sponsored by Gossamer Bio and Pulmovant.

My name is Dr. Jean Elwing. I'm a Professor of Medicine and I'm the Director of the Pulmonary Hypertension Program at the University of Cincinnati. I've been there about two decades now, really focused the entire time on the care of patients with pulmonary hypertension and related illnesses. Over that time, we've built a large practice of pulmonary vascular disease related conditions and are trying to do our best to improve the lives of people we take care of.

Today, I'd like to talk to you about interstitial lung disease and pulmonary hypertension. The type of pulmonary hypertension that develops in the setting of lung disease is called hypoxic pulmonary hypertension or Group 3 disease. This can happen in different types of lung diseases, including interstitial lung disease and COPD to name a few. But we're going to concentrate today on interstitial lung disease and its associated pulmonary hypertension.

It's a very exciting time for patients with pulmonary hypertension associated with interstitial lung disease, because we now are making progress in this area. Five years ago, we knew that pulmonary hypertension was associated with this condition, but we didn't have a lot we could do to focus on the pulmonary hypertension except watching, giving oxygen, monitoring right heart failure symptoms and treating them and referral to transplant in appropriate patients.

But recently, there's been more of an interest in assessing medications for the treatment of the pulmonary hypertension that occurs in the setting of interstitial lung disease. We've learned over time that this is not easy to treat. We've tried multiple medications without successful outcomes. In small studies, case series, but more recently we studied inhaled treprostinil for the treatment of interstitial lung disease and pulmonary hypertension and found that this was an intervention, a therapy that could improve outcomes.

So for the first time, we have hope that we can change the outcome in this disease, slow it down, pump the brakes, give patients a little more time, and hopefully feel better, do more during that time. We would love to have a cure for it. We would love to be able to say we are turning the dial back to zero. We're not there yet, but there's a lot of interest now. There's new studies coming, new therapies being looked at. I have a lot of hope that we're going to be able to make progress here.

Interstitial lung disease is a condition where you have inflammation and scarring in the lungs. It can happen from multiple different reasons. It could be related to exposure, it could be related to underlying autoimmune disease or multiple other factors can contribute. But at some point in patients' lives, they may develop a new problem associated with that, and that's pulmonary vascular disease and pulmonary hypertension.

At one point, we really thought that that only happened when the disease was quite advanced, but we've learned over time, it can happen at any time in the course of a patient with interstitial lung disease. Once it develops, it's associated with a lot of different things, including more oxygen requirement, less exercise tolerance, more exacerbations, and a shorter life expectancy. It is a trigger for us to think about, “Hey, what can we do to try to slow this down?”

We try to optimize the interstitial lung disease, manage everything that's contributing, take away any potential triggers for worsening, manage infections quickly, so that doesn't cause a worsening event requiring hospitalization and additional intervention. But the pulmonary hypertension piece is the part that, as I mentioned, did not have an intervention previously. Now, when we see patients that have signs that their pulmonary vascular disease may be progressing, like more oxygen, lower exercise tolerance, more fatigue, right heart failure symptoms, we look, we obsess with an echocardiogram. We proceed to right heart catheterization to evaluate for the possibility of true elevated pulmonary pressures or pulmonary hypertension. Then, we offer treatment to the appropriate patients.

That treatment's not easy, it's an inhaler, four times a day, but as I mentioned, we've learned that it can be very helpful to patients based on the INCREASE trial, where we saw that patients were able to ambulate further. It reduced stress on the heart based on a lab marker called BNP, and it reduced worsening. So this is just the tip of the iceberg. We're just starting this journey with pulmonary hypertension in the setting of interstitial lung disease, but at least now we have a starting point.

As I mentioned, there is a therapy available and approved for patients with interstitial lung disease and pulmonary hypertension, and we're working on others through research. What I have to remind everyone is to say thank you to all those individuals who participate in clinical trials. They give us options. Without their efforts and all the work they do to participate in trials, we wouldn't have new medications.

So I just encourage you, if you are the right person that meets criteria for a trial to consider it. Look at the options. See if that might be something you would like to explore. Not only could it be something that would benefit you, potentially, it also has the opportunity to impact many other individuals affected by this condition.

Pulmonary hypertension has so many faces, and for so long we've really concentrated on pulmonary arterial hypertension Group 1 disease. But now, I'm so happy to say that we are getting options for therapies that are impacting outcomes in many groups of pulmonary hypertension, including patients with lung disease. We have a long way to go. We've only touched the tip of the iceberg on this. ILD individuals have an option for treatment. We have many other conditions that of course, over time we hope to better understand and hopefully with that understanding be able to impact the development of pulmonary hypertension and control of the condition to allow patients to have longer, fuller, and more active lives.

Thank you so much for listening. My name is Dr. Jean Elwing, and I'm aware that my patients are rare.

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