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Welcome to UAB Med Cast, a continuing education podcast for medical professionals. Bringing knowledge to your world. Here's Melanie Cole.

Melanie: Welcome to UAB Med Cast. I'm Melanie Cole, and I invite you to listen in as we discuss lung transplantation for patients with interstitial lung disease. Joining me is Dr. Victoria Rusanov. She's a lung transplant pulmonologist, and she is a Clinical Assistant Professor at UAB Medicine. Dr. Rusanov, it's a pleasure to have you join us today. Tell us a little bit about interstitial lung disease terms, the most frequently seen ILDs, and what's the role of lung transplant in their management.

Dr Victoria Rusanov: Hi, Melanie. It's a pleasure to talk today. Interstitial lung disease is a big umbrella term used for more than 200 lung diseases causing inflammation and fibrosis of the lungs. It can be divided into two big groups of known and unknown etiology. The most frequently diagnosed ILD today is idiopathic pulmonary fibrosis, which is responsible for about 25% to 30% of all ILD cases, followed by sarcoidosis and connective tissue disease-related interstitial lung disease.

Of those ILDs with known etiology, the most commonly diagnosed are drug-related, environmental, occupational and, again, connective tissue disease ILD. Idiopathic pulmonary fibrosis is a progressive disease with limited survival of less than four years. Even though we have two antifibrotic medications available now for treatment, which are pirfenidone and nintedanib, they can slow the rate of lung function progression or decline, but despite of all the treatment, the usual course is progressive to the end-stage respiratory failure, secondary pulmonary hypertension and finally death. When all medical options have been exhausted, the only treatment with the potential to improve quality of life and prolong patient's survival is lung transplantation.

Melanie: Wow. So interesting, Dr. Rusanov. So what patients with interstitial lung disease or pulmonary fibrosis should be referred to a transplant center? And why is early referral so important for these patients?

Dr Victoria Rusanov: Yeah, this is a very important topic, to talk about the timing of referral. The International Society of Heart and Lung Transplantation recommends referring to transplant center patients with diagnosed interstitial lung disease where they just have evidence either pathological or radiographic evidence of UAP or any type of fibrosing interstitial lung disease, regardless of their lung function. Or if you have patient on any amount of supplemental oxygen, even if it's about a liter or less on exertion.

And this is why it's so important. We have to make two different clinical decisions. The first is when to refer the patient to transplant center and then when to place the patient on the waiting list because the course of pulmonary fibrosis or other type of fibrosing ILD is very variable. And some patients may do well for prolonged periods of time while the other patient can rapidly deteriorate or have what we call the flare and have very limited survival. We know that majority of the patients with idiopathic pulmonary fibrosis diagnosed relatively late in their life at the age 60 and older, many of them have different comorbidities which can complicate lung transplantation.

Referring the patient to the transplant center early will provide us with sufficient time to diagnose and treat the co-morbidity and hopefully make transplant successful. For instance, you will be surprised by the prevalence of obstructive coronary artery disease in patients with IPF is even higher than in patient with COPD. And this may be related to the pro-inflammatory state. Some patients may successfully undergo revascularization prior to transplant while a multivessel disease or impaired left ventricular function will be considered contraindication for transplant.

Pulmonary hypertension, this is evident in 80 to 90 or even higher percentage of the patients, especially at the late course of the disease and may be underestimated by a transthoracic echo. Elevated pulmonary pressure has linear correlation with mortality and requires special attention and management during the anesthesia in surgery.

Gastroesophageal reflux, this occurs almost in 90 or even high percentage of the IPF patient and have been linked to worsen lung function. We have this patient on the proton pump inhibitors, but sometimes non-acid reflux doesn't respond to this treatment and keeps driving fibrosis through microaspiration. Laparoscopic fundoplication or antireflux surgery can be done prior to transplant. It's safe and well-tolerated and can improve native lung function prior to transplant and help prevent lung damage after the transplantation.

Nutritional status, both malnutrition and obesity have important prognostic value in our patients. It can increase the risk of mortality, prolong ICU stay and need for mechanical ventilation. We know, for example, that BMI greater than 30 is an independent risk factor for early post-transplant mortality.

And the very important, not the least, is the functional status. A patient who is deconditioned, they will have much higher risk of complication and their survival is lower than the patient who is more functional prior to transplant. This is why the early referral to transplant center is very important for the diagnosis and management of this complication.

Melanie: Well, thank you, Dr. Rusanov, for that comprehensive answer and letting other providers know about the importance of objectively assessing those comorbid conditions and possibly clinical decline. So tell us a little bit about what happens for a patient once they're placed on the waiting list. You can first tell us a little bit about what the evaluation process looks like, but you have just touched on some of that, but then tell us a little bit about the waitlist experience, and this is important I think, how you would like other providers to know about managing some of those comorbid conditions while their patient is on the waitlist, because I imagine this is very multidisciplinary, many providers involved all at the same time. So what would you like other providers to do while that patient is on the waitlist?

Dr Victoria Rusanov: You're absolutely right. So talking about the evaluation, most of the patients have their evaluation process done as outpatient while we can do it in special circumstances as inpatient. Usually, it takes about five to seven busy days of getting through multiple labs, images, consults, and minimally invasive procedures like heart catheterization.

After this thorough evaluation, the patient will be discussed on their selection transplant multidisciplinary meeting. And if we don't see any major complication for transplant, we will proceed with the listing. For example, the patient with interstitial lung disease or IPF, our trigger to list the patient would be progressive decline in lung function, especially vital capacity and DLCO, development of the pulmonary hypertension, hospitalization because of the pneumothorax or an exacerbation.

A patient will not be eligible for transplant if they meet absolute contraindications like dysfunction of another major organ like heart, kidney, liver, recent history of malignancy, coronary disease, morbid obesity, active substances abuse, resistant infection or poor functional status.

We work closely together with the other providers, especially with the cardiology, sometimes heart transplant team, gastroenterology, rheumatology, trying to manage together these co-morbidities. The functional status is very important. And the cardiopulmonary rehab is the main goal before and after a transplant.

Melanie: Well then, do you use the similar approach if it's non-idiopathic pulmonary fibrosis? Tell us a little bit about the difference that you might use.

Dr Victoria Rusanov: Fibrotic NSIP or fibrotic interstitial lung disease of other etiology refractory to treatment have similar to idiopathic pulmonary fibrosis prognosis and indication for transplant. Some cases of familial interstitial lung disease or interstitial lung disease at young age can be caused by a relatively rare syndrome called a short telomere syndrome. This syndrome is associated with the extrapulmonary manifestations, such as bone marrow failure, liver cirrhosis. And this may compromise evolution after the transplant. Here at UAB, we've transplanted several patients with short telomere syndrome with good outcome.

Connective tissue disease-related ILD, they possess special challenges for transplant. These challenges can be related to GI complication such as the GERD, esophageal motility, gastric motility, small intestinal bacterial overgrowth, malabsorption. renal complication, cardiac or neuromuscular involvement. Sometimes patients with connective tissue disease may have elevated anti-human leukocyte antigen antibody, and this will significantly limit the potential donor pool.

Here in UAB, we have experience of many years transplanting this complex patient, especially with connective tissue disease and working in collaboration with the rheumatologists and gastroenterologists.

Melanie: Doctor, this is really such an important topic we're discussing today. So as we wrap up, showcase for us how UAB is the have-it-all destination for end-stage lung disease, with access to subject experts and clinical trials. And tell us a little bit why a multidisciplinary approach is so important for these patients and what you would like referring providers to take away from this episode.

Dr Victoria Rusanov: This is a very important point you just touched it right now. UAB has a very well-known interstitial lung disease program led by experts in the field, participating in multiple clinical trials. Referring your patients with interstitial lung disease to UAB, will provide these patients with an early access to the clinical trial, to the early diagnosis of the interstitial lung disease.

Working together, interstitial lung disease pulmonologist, lung transplant pulmonologist, gastroenterologists, rheumatologists, we have this multi-disciplinary approach, which helps us to make the right diagnosis, find the best treatment including the clinical trials or lung transplantation is needed. We also have extracorporeal life support program, one of biggest programs here. We can manage and sometimes bridge these patients to lung transplantation. This is why UAB is a good place where the patient with interstitial lung disease can meet their diagnosis and treatment.

Melanie: What an informative episode. Thank you so much, Dr. Rusanov, for joining us and sharing your expertise today. And a physician can refer a patient to UAB Medicine by calling the MIST line at 1-800-UAB-MIST. That concludes this episode of UAB Med Cast.

For more information on resources available at UAB Medicine, please visit our website at UABMedicine.org/physician. Please also remember to subscribe, rate and review this podcast and all the other UAB Medicine podcasts. I'm Melanie Cole.