Transcription:
Treatment Options for Interstitial Lung Disease

Melanie Cole (Host):  Interstitial lung disease causes scarring of the lungs and can be a lifelong chronic health concern. My guest today is Dr. Borna Mehrad. He is a board certified physician in pulmonary medicine and critical care medicine whose specialties include interstitial lung disease. Welcome to the show, Dr. Mehrad. What is interstitial lung disease?

Dr. Borna Mehrad (Guest):  Hello. Interstitial lung disease is really not one disease. The best way to describe it is as a category of illnesses. There are perhaps 200 illnesses that fall under this umbrella term interstitial lung disease. All of these, what they have in common is varies degrees of fibrosis, scarring and inflammation in the lungs. Because there are a large number of illnesses that fall into this category, it is very important to identify which specific one a patient has because the treatment and outcome and so on of the illnesses are quite different.

Melanie:  What are some of the risk factors and symptoms and potential causes of these diseases?

Dr. Mehrad:  The most common, or the one that has the most press, is an illness called “idiopathic pulmonary fibrosis”. IPF is an illness of unknown cause that results in scarring of the lungs. Smokers are overrepresented in patients with idiopathic pulmonary fibrosis. There is often a remote history of smoking. In addition, there are also increasing numbers of genetic conditions that are being discovered with patients with idiopathic pulmonary fibrosis. However, many patients that are labeled as idiopathic pulmonary fibrosis--in other words, they are labeled as this person has pulmonary fibrosis and we don’t know the cause--once you look into the illness in a lot of detail and assess it, you find an identifiable cause for the interstitial lung disease.  I’ll give you some examples of that. There is a condition called “hypersensitivity pneumonitis”. That’s a mouthful. This is a condition where the body’s immune system that is meant to be fighting off infection makes an error and attacks inhaled substances that it should be ignoring. As a byproduct of this attack, it results in inflammation and scarring of the lung. This is very common in our practice. We see people who have had exposures – even low level exposures to things like mold in their environment or agricultural environments or pet birds – and, as a result of this, they develop lung inflammation and scarring that is often mistaken for idiopathic pulmonary fibrosis. This is a really important distinction, though, because both the outcome and the treatment for hypersensitivity pneumonitis and IPF are very different. Another example of an interstitial lung disease that can be mistaken for IPF are autoimmune diseases. The most famous of these are illnesses like lupus and rheumatoid arthritis. These are illnesses where the immune system actually attacks parts of the body by mistake. These illnesses can affect many organs in the body but almost all of them affect the lungs. In our practice, we often see patients whose lung symptoms predominate over their other problems. They may or may not have skin problems or joint problems but when they are really short of breath and progressively short of breath, that’s the thing that brings them to the doctor. When we look at it in detail, we find that, in fact, instead of having IPF, they have one of these autoimmune diseases. Again, the illnesses, treatment and prognosis are very different and really quite better than IPF.

Melanie:  What symptoms would someone notice? People cough or they think it’s bronchitis. These symptoms can be nonspecific. What would send them to see you?

Dr. Mehrad:  The lung only has one of a few ways in letting you know that something is wrong. The most common complaint is progressive shortness of breath. The person has shortness of breath. They notice that in carrying the groceries in from the car or going up a flight of stairs, they get more short of breath then they used to be. This is something that slowly progresses over time. Now, compared to six months ago compared to a year ago, they’re more short of breath. As you said, cough is also a symptom, although this shortness of breath is the most common thing that we see. Most patients who have shortness of breath in this way, initially go to their general doctor and often they get a chest x-ray and so on. Often patients with interstitial lung disease are initially thought to have something else. It’s not rare for our patients to have initially been treated for other more common lung diseases, such as asthma or COPD. After some period of time, when their symptoms don’t resolve, often months pass and then, eventually, they get referred on when the diagnosis is made.

Melanie:  What are some treatment options that are available - medication, oxygen, therapy? Do we do pulmonary rehab using spirometer? What do they do for interstitial lung disease?

Dr. Mehrad:  The first thing when we see a patient with interstitial lung disease is, we want to really work hard to identify the cause. Part of identifying the cause we do, as you said, spirometry depending on the severity of the illness, CT scan, and a bunch of blood tests. Depending on what we find, that guides further workup as to the cause, as to the underlying etiology. Your question is about treatment. A couple of things in pulmonary medicine have been clearly shown to prolong life in people with significant lung disease regardless of the actual cause of the lung disease. The first is, anybody who smokes has to stop smoking. Stopping smoking definitely prolongs life. The second is that we want to make sure that people are up to date on their vaccinations because people who have severe lung disease are more predisposed to more severe respiratory infections and if they get them they will do badly. We want to make sure that we reduce the likelihood to ensure that they have had their flu and pneumonia shots. The third issue, which is a very important issue is oxygen. In people, basically the way we measure oxygen is using a machine called a “pulse oximeter”. This measures oxygen as a percent saturation of hemoglobin. A normal saturation is about 95%. Expert data shows the longer a person spends below the threshold of 88%, the shorter the time they are going to live. So, we want to make sure that we give patients that have below 88%, however much oxygen they need to make them stay above 88% the whole time. These are generic treatments not specific to interstitial lung disease but they are extremely important treatments because they prolong life. Depending on the specific cause of the illness – what is the cause of their interstitial lung disease – we want to attack the underlying process. That depends, as I say, very much on what they have. For many of these illnesses, the high percentage of pneumonitis that I mentioned, for example, identifying the environmental exposure that caused the illness is very important. So, you want to avoid that. For these immunological illnesses such as autoimmune diseases and hypersensitivity pneumonitis, immune weakening medicines often have a role. And, most importantly, in the past year, two new drugs were approved by the FDA for the treatment of idiopathic pulmonary fibrosis. These drugs really transformed the landscape of how these patients are treated. University of Virginia was the center for testing one of these drugs – Pirfenidone. In the appropriate person--in the person who has idiopathic pulmonary fibrosis and not one of these other etiologies--treating the underlying disease with one of these drugs is an excellent choice because it slows the progression of the illness.

Melanie:  In just the last minute, Dr. Mehrad, why should someone come to UVA Pulmonary and Respiratory for their treatment and your best advice for someone who is suffering from lung disease?

Dr. Mehrad:  What we offer is real world experience. These illnesses are not a common part of the practice of a general primary doctor, even an excellent primary doctor.  We have three physicians that all we do is see people with interstitial lung disease. Our volume of patients is very large and, necessarily, our experience in dealing with them is very large. The second reason is we have a multidisciplinary team of lung doctors, radiologists, pathologists, and a number of ancillary services that we put our heads together and provide the best information about what the person has to try to help them. Lastly, research. We are a center for research in these diseases trying to find new treatments to try to help patients. Patients have the opportunity to get enrolled in clinic trials contributing new knowledge about their illness.

Melanie:  Thank you. It’s such great and very important information. You’re listening to UVA Health Systems Radio. For more information you can go to UVAHealth.com. That’s UVAHealth.Com. This is Melanie Cole. Thanks so much for listening.