Navigating the Burden of Interstitial Lung Disease

For part 1 of this video series, Ross Summer, MD, professor of medicine, director of interstitial lung disease program, section chief of allergy, pulmonary and critical care medicine at Thomas Jefferson University, explains the incidence of interstitial lung disease and its burden on patients.

This interview is a part of the video series “Inside Interstitial Lung Disease: Overall Burden, Therapy Options, & Looking Ahead.”

Read the full transcript:

My name is Ross Summer, and I am a Physician Scientist here at Thomas Jefferson University. I'm also the Section Chief of Pulmonary and Critical Care and the Director of the in Interstitial Lung Disease Program at Thomas Jefferson University Hospital in Philadelphia.

Can you describe the incidence and burden of interstitial lung disease (ILD) and other fibrotic lung diseases? What are some notable differences between the varying classifications?

That's a very tough question and it's tough for several reasons, but first before I answer the question, I think it's important to define terms. The term ILD or interstitial lung disease is somewhat confusing to patients so I want to talk a little bit about that terminology. ILD stands for interstitial lung disease. I think most patients and people will understand the lung and the disease part, but it's the term interstitial that confuses people and is one that requires a little bit more details.

Interstitial refers to the interstitial space of the lung, which is the space that surrounds the air sacs or where all the gas exchange happens in the lung. Now, we all know that there are more than just two balloons in the lung. There's millions and millions of balloons, and they're surrounded by tiny little blood vessels that allow oxygen in and out of the body.

That space, where there's normally just blood vessels, is the interstitial space. It is between the air sacs. And sometimes that space gets thickened and that is when it leads to interstitial lung disease.

It's important to know because interstitial lung disease is not one disease, but many diseases. In fact, some people think there's over 200 different types of interstitial lung diseases. And so, it's really important when you go to see a doctor to understand that having interstitial lung disease does not mean you have a specific diagnosis, but you're in the category of people that have this problem in their interstitial space. It is very important to then get a specific diagnosis, which usually requires a specialist who focuses on this.

What makes treating this patient population so challenging?

There are probably 3 big reasons why taking care of interstitial lung disease is so challenging. Number one is that the presentation is often nonspecific. And what I mean by that is that people can have very vague symptoms like “I'm tired” or “I'm fatigued, or a little short of breath,” or even just a cough. These symptoms can also track with many other diseases and that's why many patients take a very long time to get diagnosed.

In fact, for some diseases like idiopathic pulmonary fibrosis, which is one form, the average time to the diagnosis is about 1 to 1.5 years in the community because people often get one, two or even three diagnoses before the physician reaches the final diagnosis. I think the vagueness of symptoms plays a big role in that.

The second reason why this is a tough disease to treat is because of what I mentioned earlier is that there's many different types of interstitial lung disease and getting at the right diagnosis can sometimes require a lot of specialists like a rheumatologist, a pulmonologist, a good radiologist, and even sometimes a pathologist. That can make it very challenging.

And then the third reason why it's difficult is because the natural history of every interstitial lung disease is different. Even if you have one of the 200 different types of interstitial lung diseases, your clinical course could be very different than someone else's clinical course. And so, patients sometimes find that very unsettling to not know what their clinical course is going to be like and we won't know it until we just follow you over time. I think dealing with that stress and helping people to understand that we will not know the clinical course over time can be a challenge.

This transcript has been edited for clarity.