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Melanie Cole (Host): Welcome to UAB MedCast. I'm Melanie Cole. Today we're discussing treatment options available for patients with autoimmune associated interstitial lung disease. Joining me is Dr. Pilar Acosta. She's a pulmonologist in critical care medicine and an assistant professor at UAB Medicine. Dr. Acosta, thank you so much for joining us today. Can you start by telling us a little bit about the scope of the issue we're talking about here today? The interstitial lung diseases and the prevalence of them.

Dr Pilar Acosta: Yes, thank you. So interstitial lung diseases is, one of the most common and clinically important manifestation of connective tissue diseases. Although ILD, which is the short, term for interstitial lung disease, often occurs in patients that have a known autoimmune disease. It can also be the first and only manifestation of previously unrecognized autoimmune disease process. Some of the common autoimmune diseases that can manifest as an interstitial lung disease include rheumatoid arthritis. So talking a little bit about rheumatoid arthritis affects about, a half to 1% of the US population.

And ILD is the most common pulmonary manifestation in this patients than occurs about 10 to 20% of patients. So in that proportion, probably have more patients to have, ILD related to the rheumatoid arthritis. Another autoimmune disease that is also, highly prevalent to. create interstitial lung disease is systemic sclerosis. It's not as common as rheumatoid arthritis. There is a less proportion of patients that have it. However, in patients that have scleroderma or systemic sclerosis, lung involvement is most common and almost universal.

It has been described that most of patients that have sclero, they may have some type of lung involvement when, seen, in the 70% of patients. Another type of, autoimmune disease that can also manifest for patients, with interstitial lung disease is Shagra disease, less common than the other ones that I talk about, but it's also common to have some type of lung involvement as interstitial lung disease. So the most common ones are rheumatoid arthritis, sceraderma and shagran..

Melanie Cole (Host): Well thank you for that. So what are some issues for other providers to consider as patients with autoimmune associated interstitial lung diseases and CTD ILD as they often present with a poorer quality of life if this is present and therapy can also be associated with severe. Effects and adverse effects. So when is treatment generally initiated? Are you looking for when symptoms become clinically significant or progressive? Tell other providers what you're looking for at that time?

Dr Pilar Acosta: Not all patients that have an autoimmune disease will develop interstitial lung disease, but having a high index of suspicion of can my patient develop an ILD down the road? So that requires monitoring. And that requires you kind of have that high index of suspicion, make sure the patient gets a pulmonary function test to assess for the lung function. Also, any kind of lung imaging can be a plain x-ray, but we typically do a high resolution CT scan and that will let us know if there is any type of lung involvement such an interstitial lung disease in those patients. These some patients that have unknown autoimmune disease process.

Melanie Cole (Host): Well then speak a little bit about the mainstay of therapies. What the medications that you might look to immunosuppression, either by steroids or steroids sparing agents. Tell us a little bit about that. And Dr. Acosta the role of managing the comorbidities in patients with autoimmune associated interstitial lung disease.

Dr Pilar Acosta: So, yes, in the progression of the interstitial lung disease in these patients that have an autoimmune disease process is different than you compare to. Patients having other type of ILDs like pulmonary fibrosis, idiopathic pulmonary fibrosis. Typically we see a more, subtle progression or they don't progress as fast as patients that have IPF do, but they do progress. The other difference between this type of patients, with interstitial lung disease, secondary and autoimmune disease has to do with treatment. So treatment is different in the sense that in many cases the main stand of treatment includes, immunosuppression medicines that will bring down that inflammatory drive that is accelerated in patients with an autoimmune process.

Trying to, slow down the inflammation and the progression of the, fibrosis that can ensue in patients that have this autoimmune disease process. So medications that we, prescribe. It's called Mycophenolate. This is a, one of the medicines that we use more commonly. It's a medication that is given by mouth patients, take it, start with a low dose and we increase it as the patient is able to tolerate it. Overall, medicine, it's well tolerated, but can have some side effects, most of them gastrointestinal problems. So we very closely counsel patients about having any side effects from the medicine.

There is also this medicines require monitoring. So our patients require blood work, done on a quarterly basis to make sure all their blood counts and deliver enzymes, are stable and have not progressed. most recently, that's a new insight in the treatment of CTD ILD or the autoimmune disease process. We have data from two trials that showed that we using one of our anti-fibrotic, medicines that we use in patients that have idiopathic pulmonary fibrosis has been used. Patients who have an autoimmune disease such as scleroderma with positive results. So, we had the inbuilt trial, which this was a double blind, randomized placebo control trial using the medicine called nintedanib.

Which is an antifibrotic agent, has been used, for almost 10 years in patients idiopathic pulmonary fibrosis. And the results from the trial were very, very, encouraging because what we saw is that the disease progression was also, attenuated in patients taking this medication. So, very interesting trial. This has also, changed our practice management. So now we see a combination of treatments using immunosuppressive drugs as well as antifibrotics.

Melanie Cole (Host): And Dr. Acosta, this is so interesting and as a result that there are autoimmune conditions related. What other specialist consultations are indicated in the treatment of autoimmune associated interstitial lung disease? And please showcase for us how UAB is a destination for lung diseases in general with access to subject experts and clinical trials. Tell us about this multidisciplinary approach?

Dr Pilar Acosta: So, in patients that have CTD ILD, we extrapolate what we know works for patients with all their ILDs, like idiopathic pulmonary fibrosis. So we need to screen for the use of supplemental oxygen. So a six minute walk test to assess for hypoxemia either rest or with exertion and patients that have hypoxemia. Then we will provide them with supplemental oxygen. The other comorbidity that are at risk is gastroesophageal reflux disease. Many patients that have an autoimmune disease, they may also have esophageal dismotility or bad acid reflux. And we do know that uncontrolled acid reflux can make any kind of lung disease worse.

So we are very proactive in managing and diagnosing, gastroesophageal reflux disease that may be overt or maybe silent. The other big comorbidity that we screen for is something called pulmonary hypertension. This is very prevalent, especially on patients that have scleroderma. So we screen patients yearly with an echocardiogram or a Ry heart cad depending on the situation to screen for these comorbidity that is difficult to treat, and it just adds complexity to the overall condition of the patient.

They need routine follow up with specialists, that know, and they're familiar with patients that have an autoimmune disease, interstitial lung disease. We work very closely with our rheumatologists here at UAB to trying to find the right combination, for the patient to treat their condition. It's not one size fits all, but it's tailored to what the patient needs. And what they're requiring at the moment. As UAB is part of the care, network center and is part of the Pulmonary Fibrosis Center of Excellence. So we are a designated center to see patients with interstitial lung disease, including those.

That are related to an autoimmune process. We see referrals from all over the state and also out of state, from Florida to Mississippi, Tennessee. So we get patients from all over this area and we're happy to see them. We have a group of several physicians that are trained, and this is what we do every single day, interstitial lung diseases, and one of them's autoimmune disease process.

Melanie Cole (Host): Thank you so much, Dr. Acosta. That was so informative. Thank you for joining us and for more information about autoimmune associated interstitial lung diseases or to a refer a patient to UAB Medicine, you can call the MIST line at 1-800-UAB-MIST, or you can visit our website at uabmedicine.org/physician. That concludes today's episode of UAB Med Cast. I'm Melanie Cole.