Key ILD Topics at Mount Sinai’s Advances in Pulmonary Medicine 2024

In an interview with The American Journal of Managed Care^® (AJMC^®), Maria L. Padilla, MD, professor of medicine and director of the advanced lung/interstitial lung disease (ILD) program at Icahn School of Medicine at Mount Sinai, previews Mount Sinai Respiratory Institute's Advances in Pulmonary Medicine 2024, a 2-day event in New York City to be held on November 7 and 8; Padilla will be involved in multiple ILD discussions during the event.

She discusses major event objectives and agenda items related to ILD and idiopathic pulmonary fibrosis (IPF), including recommended diagnostic and management strategies. Padilla also emphasizes the importance of a multidisciplinary, collaborative approach in IPF care, highlighting the teamwork between specialists and community physicians.

At Advances in Pulmonary Medicine 2024, experts will discuss critical management strategies for interstitial lung disease and idiopathic pulmonary fibrosis, including the latest diagnosis and treatment protocol advancements. | Image Credit: Mount Sinai

This transcript has been lightly edited for clarity.

Transcript

What key complications associated with IPF require vigilant management?

The most important factor in the management of IPF, a progressive lung disease with a very poor long-term prognosis, is early and accurate diagnosis. We have to have diagnostic accuracy to the best of our ability.

Once the diagnosis has been established by excluding any other potential etiologies and utilizing a multidisciplinary discussion, our attention then turns to strategies to relieve patient symptoms and slow disease progression by instituting treatments, assessing decline in function, and maintaining vigilance for possible complications.

For key complications, and if I could do 3, it would be the progression of the disease; the monitoring and addressing of acute exacerbations, which is one of the significant complications that we see; and the development of other comorbidities, such as pulmonary hypertension and lung cancer in our patients with pulmonary fibrosis.

Could you elaborate on the recommended management strategies for patients with IPF?

There are many strategies that we all follow, but in general, it begins with the education of our patients, their relatives, and their providers about the disease; understanding the disease is critical. Avoiding those risks that can aggravate the disease goes hand in hand with any specific strategies that we could use, which include providing antifibrotic therapy in a timely fashion, monitoring and managing potential adverse effects of these medications, and periodic evaluation of pulmonary function and the 6-minute walk test to assess whether they are declining or in need of requiring additional therapy, such as oxygen, which can then be provided to the patient.

We also anticipate potential complications, such as the development of pulmonary hypertension, that will affect a significant portion of our patients. Some with diseases that have connective tissue underpinning or immunological underpinnings may experience a higher risk of this, so it's always very important to monitor for the potential development of pulmonary hypertension in our patients.

Also, our patients are at risk of developing lung cancer, so we follow them closely for that possibility. Although we try to minimize their radiation exposure, we feel that it's very important that they get a CT scan at periodic intervals. That interval has not been well established, but we use a guideline of about every year, looking for areas that may be the beginning of lung cancer.

In addition, we refer patients for pulmonary rehabilitation. As I mentioned, we'll prescribe them oxygen supplementation if needed. We also refer patients for transplantation. Although we talk to all our patients with fibrotic lung disease about the potential for lung transplantation, deciding when to pursue this becomes a call that involves both the patient and the progression of their disease. So, we must keep this in mind so the window of opportunity is not lost for these patients.

We provide emotional support. This is a disease that has a lot of burden—emotional, physical, and even financial—with the loss of their ability to work and do the things that they enjoy. If necessary, we'll refer our patients for psychiatric support or to any other kind of social group that may help deal with these symptoms.

We also want to make sure that when we have exhausted all potential therapies, and the patient is not a candidate for transplantation, we are there to support them through that difficult time when their function is continuing to decline and for which there are disciplines, such as palliative care, that are invaluable to our patients.

All of this is with the express goal that we have to arrest the disease, to slow it down. Our dream is to reverse it, but that is a dream that I don't know will happen in my lifetime because it has to be a selective reversal of fibrosis in the lungs only.

What protocols do you follow for managing critically ill patients experiencing acute exacerbations of ILD?

This is a dreaded complication, and it happens in a good number of our patients. Although we monitor to try to anticipate it, sometimes the patients present amid an acute exacerbation and require intensive care unit support. At that time, we utilize corticosteroids to a large extent.

No proven therapy exists to treat acute exacerbations of IPF, particularly, but corticosteroids have been the backbone of therapy. Some centers also utilize intravenous immunoglobin as one of their therapies. Predominantly, this comes from studies conducted in Japan that showed a slight benefit to the patient.

How will these topics be explored at Advances in Pulmonary Medicine 2024?

Ganesh Raghu, MD, our keynote speaker, will be talking about acute exacerbations. I am looking forward to hearing from him and seeing what new knowledge he brings to us about acute exacerbations, not only in IPF but also in other progressive fibrotic lung diseases.

He is a professor of medicine at the University of Washington and director of their Center for Interstitial Lung Diseases. He is a luminary in the field of ILD. He has been an ILD educator and influencer who has brought us a lot of information, guidelines, and studies that have looked at the disease as it has been developing.

There are a lot of developing advances in the ILD field. We have moved into a different era now with the ability to look at biomarkers that may help diagnose the disease, prognosticate the disease, and alert us to an upcoming acute exacerbation, for instance. I am looking forward to that in his discussion of IPF and the advances that we have had in the status of the disease at this time. He will also be addressing hypersensitivity pneumonitis, which is another very important topic, and he will participate in our panel discussion.

Could you provide a preview of the ILD panel discussion and small group discussions you are involved in?

The panel discussion, which is geared toward interaction with the audience, is designed to give a brief overview of 3 particular topics.

One is the genetics of ILD, which is something new, I think, for many of us. The other one is to take a panoramic view of the connective tissue diseases associated with ILD. The ILDs with immunologic underpinnings often present even before you get the rheumatologic manifestation. They can present with a pulmonary manifestation of the disease, so we must always keep that in mind and think about connective tissue disease as a potential cause of ILD, particularly fibrotic lung diseases. The last part of that panel discussion is acute exacerbation, which he will address.

Then we have the small group sessions, which are also interactive. This is a problem-based learning model that we utilize to engage the audience to decide on the evaluation of a patient and arrive at a diagnosis with the tools that we can present. It's meant to simulate the encounter with a patient in their own office. This allows us to emphasize important aspects of each case and provide guidance while also learning from the audience on how they might approach different patient scenarios.

What are you most looking forward to at the event?

With any of these seminars and things that we work on, I look forward to interacting with our colleagues, not only in pulmonary but in the other disciplines that intercept the care of a patient with pulmonary fibrosis, for instance. We look forward to interacting with rheumatologists, cardiologists, pathologists, radiologists, and oncologists so we can better take care of our patients who have IPF or a progressive pulmonary fibrotic disease.

In no place else is the remark "It takes a village" more fitting than caring for a patient with pulmonary fibrosis. That is so apt to what we do because it’s not only about the multidisciplinary discussions within the center but also the essential interactions and collaboration with the physicians and colleagues caring for our patients within the community.