Maggie McKay (Host): Welcome to UK HealthCast, a podcast presented by UK HealthCare. I'm your host, Maggie McKay. When you hear the diagnosis sarcoidosis, do you know what it means and what it involves? Today, we'll discuss both pulmonary and cardiac sarcoidosis with pulmonologist, Dr. Parijat Sen and cardiologist, Dr. Sonu Abraham. Thank you both for being here today.

Sonu Abraham, MD: Thank you for having us. It's an exciting time to talk about this disease.

Parijat Sen, MD: It's an absolute pleasure, Maggie, to be here.

Host: For me too. Thank you. Dr. Sen, let's start with you. Let's begin by talking about sarcoidosis, what it is, and how it affects the body and its organs.

Parijat Sen, MD: Sarcoidosis is a chronic inflammatory condition where you have this ongoing low-grade inflammation, which could happen in different organs. The commonest organ affected is the lungs, which is probably how a lot of lung doctors like me end up becoming sarcoid doctors, followed closely by the skin, the heart, the eyes, the nervous system, the joints. So, you can see that pretty much the disease can affect almost every organ system in the body.

Host: Also, who's most affected by sarcoidosis? How common is it? Are there known risk factors? Is it genetic?

Parijat Sen, MD: So, sarcoidosis is what's considered a relatively rare disease. But that being said, we probably have more than 200,000, 250,000 patients with sarcoidosis in the United States alone, the number being much higher globally. There is definitely a genetic component. There are certain specific genes that have been found to be associated with sarcoidosis. Particularly, this disorder does involve the immune system, so genes that are linked to the immune system of the body, some of them have been linked to development of sarcoidosis.

But as a lot of our patients ask, "Well, if this is genetic, does this mean that my children or subsequent generations can get this?" There is a possibility, but then again the genetic association isn't the only factor that leads to development of sarcoidosis. We feel that there's some sort of an external trigger, which could be an infection, which could be something in the environment, is needed to trigger off this abnormal chronic inflammation in the body. So yes, there's a genetic link, but there has to be some sort of an external trigger as well. And those external triggers are probably the risk factors that we worry about. Once again, there's not a single one that has been identified, but it could be an infection, like bacterial infections, it could be molds in the environment, it could be concrete or dust. Certain medications, especially some of the newer medications used to treat different cancers, have also been found to trigger the development of sarcoidosis. So, the list is pretty long.

Host: Wow, that would be discouraging to be taking a medicine for, say, cancer, for example, and then to get sarcoidosis. Dr. Abraham, sarcoidosis can have a profound impact on patient's hearts. So, how common is it that sarcoidosis affects the heart, and what can that impact? What does it look like, the symptoms?

Sonu Abraham, MD: Yes, that is very true. It can affect the heart and cardiac sarcoidosis occurs when this clusters of white blood cells called granulomas go and deposit in the heart muscle. The data about how common it is and how in terms of affecting the heart is very variable. If you look at the patients who have symptomatic cardiac involvement, it comes to about 5%. But if you look at autopsy data, it goes up to 25%. So, the variability is profound in terms of incidence in this disease. And if you also look at patients who have other kinds of sarcoid and just screen all of them for cardiac, the incidence goes from 4% to 55%. So, this highlights the importance of talking to patients about their symptoms at every visit. The main cardiac symptoms these patients might have include lightheadedness, dizziness, passing out spells of fatigue. They can have palpitations like their heart is racing or skipping beats. They can have breathing problems like shortness of breath or chest tightness. And what happens is these granulomas go and deposit in the electrical system of the heart and cause something called heart block. And they can also cause abnormal, dangerous rhythms that come from the lower part of the heart. So if you have a heart block, you might be a candidate for a pacemaker. If you have abnormal rhythms that are dangerous, you might be a candidate for a defibrillator. And so, there's a lot of nuance into how we take care of these patients. And therefore, it's very important to identify if they have cardiac involvement.

Host: Also Dr. Abraham, how do the symptoms of sarcoidosis compare to other heart conditions that people might be more familiar with, like arrhythmia or heart failure?

Sonu Abraham, MD: So just from what I just said, you can see that many of the symptoms of sarcoidosis overlap with arrhythmias and heart failure. And so, there lies a big challenge in diagnosing sarcoidosis. You know, the subtle progression of cardiac sarcoid can delay, diagnosis which is why it's very important to have high degree of suspicion.

An example would be like, suppose a patient comes into the emergency room, a very young man, 38-year-old, for example, comes in with lightheadedness and dizziness and has heart block. There's no real reason why a 38-year-old should have heart block. And so, this should trigger in your mind, "Okay, I should look for cardiac sarcoid and screen for that in this particular patient." And similarly, this holds true for patients who might have ventricular arrhythmias, like abnormal rhythms coming from the lower part of the heart, new onset of heart failure without any clear cause, and that should trigger you to think of cardiac sarcoid in your differentials.

Host: How is sarcoidosis diagnosed?

Parijat Sen, MD: A lot of it depends on which organ system is affected. I'm going to let Dr. Abraham talk about the heart, after me. But when it's involves the lung, one of the things that commonly triggers a suspicion is a scan of the lungs that either shows really enlarged lymph nodes in the chest or little spots in the lungs, which we call nodules. That's often what triggers off the suspicion.

Most of the time this would lead to needing a biopsy, either from the lung or the lymph nodes, which shows the specific kind of inflammation that Dr. Abraham just mentioned, little granulomas or clusters of white cells. These biopsies are usually done through a bronchoscopy, so you'll undergo a bronchoscopy. And then, the bronchoscopist would take some biopsy from the lymph nodes and the lungs as well. Sarcoidosis can cause other manifestations, such as eye inflammation, so you could have a sudden painful red eye or some loss of vision in the eye. And an eye doctor on exam can maybe see signs of sarcoidosis in the eye.

Similarly, it can cause some abnormalities in the blood as well, like really high calcium levels, or if it affects the liver, abnormal liver enzymes. So, those could be other things that trigger off the suspicion that this is a person who could have sarcoidosis.

Sonu Abraham, MD: There are ways that we can identify how it impacts the heart before patients develop symptoms. When patients have sarcoidosis in other parts of the body, like the lung, at minimum, we should get an EKG for screening for any clues for cardiac involvement. And we can also do longer durations of monitoring of patients for determining what their heart rhythm looks like. So, it's something called a Holter monitor where, based on the degree of suspicion, we can look at whether they have abnormal rhythms or heart block that's happening in their heart over a longer duration of time.

We can also do an echocardiogram, which is an ultrasound of the heart and look at structural abnormalities in the heart. So, things we look for, a low heart function, certain parts of the heart not moving like the rest of the heart, certain parts of the heart being thinner or thicker than the rest of the heart. These are all clues that we look for to see if the patient might have cardiac involvement when they have other involvement in the rest of their body.

If we find that patients do have cardiac symptoms or ECG findings or they have echo findings, we then proceed with further testing. So, we can do cardiac MRI, we can do a cardiac PET scan and these help us to narrow down the diagnosis and see for sure if it's affecting the heart.

Host: Dr. Sen, what kind of treatment options are available for people living with sarcoidosis?

Parijat Sen, MD: Unfortunately, the treatment options that we have for sarcoidosis are fairly limited. One of the medications that's very commonly used and is first line in sarcoidosis is steroids. But with steroids, I always talk of it as a canon. It controls the inflammation broadly, can often help with symptoms of sarcoidosis, but the collateral damage is really, really high because being on steroids for long term can lead to a lot of other problems, such as development of diabetes, high blood pressure, weight gain, as well as developing brittle bones that which we call osteoporosis.

So, for patients who may need to be on treatment for the long term, as physicians taking care of these patients, we often try to get them on other medications besides steroids, which can help control the inflammation. But then again, these medications have their own side effects as well, because a lot of them would, to control the inflammation, affect the immune system and lower the overall immunity of the individual.

And as you know, once your overall immunity is lowered, they would be at higher risk for developing infections as well. So, one of the things that we keep talking about in the sarcoidosis community is the need for developing really targeted, focused therapies, which can just affect the kind of inflammation that we see with sarcoid without causing a lot of these collateral damages and side effects that come from the treatment.

Host: Dr. Abraham, let's talk about somebody who is living their life with cardiac sarcoidosis. What's the impact of that day to day? What can they do to improve their quality of life?

Sonu Abraham, MD: That's an excellent question. The impact on our patient's day-to-day life varies between patients actually, and it really depends on the severity of the disease and the extent of cardiac involvement. So if the patients have mild asymptomatic disease, they can usually lead a relatively normal life. While those with more advanced disease may experience significant limitations. So, my patients have expressed symptoms like fatigue, inability to exercise as one of their major concerns.

If they have a defibrillator, many of them tell me they are anxious and fearful of being shocked by their device, and that does impact their day to day lives because they're worried whether they would get shocked. And if patients are on immunosuppressive therapy, like Dr. Sen just mentioned, like steroids, side effects, like weight gain, mood changes, uncontrolled sugars, brittle bones, all of that do affect their overall wellbeing.

To help them improve their quality of life, we encourage our patients to follow the medication regimen that we've prescribed to help control the symptoms and the progression of the disease to a large extent, which we can do with the medications. I also tell my patients to continue to follow a healthy diet and do moderate exercise because that's good for them on a general wellbeing standpoint. Coping with a chronic disease like this is challenging. And so, we provide resources like mental health support, which can help manage their stress and anxiety that's related to the disease by itself.

Host: Dr. Sen, what is UK HealthCare doing to advance the treatment and the understanding of sarcoidosis and also cardiac sarcoidosis, which we'll talk with Dr. Abraham about.

Parijat Sen, MD: So, I think the first step that UK HealthCare took was to address the need that there need to be specialists who take care of sarcoid patients in a regular basis, given how relatively rare this disease is and the understanding of the disease is.

So, about couple of years back, we started a sarcoidosis clinic at UK HealthCare, which involves a lot of specialists, like lung doctors like me, heart doctors like Dr. Abraham, rheumatology doctors, as well as specialists in GI Health and eye doctors and neurologists as well. With this, we can ensure that patients who may have involvement of different organ system gets care from the different specialists under the same roof as well.

The other thing that we are working on right now and in the process of development is having a patient support group. Because as Dr. Abraham mentioned, sometimes coping with a chronic disease like sarcoidosis can be really challenging, and it could be a lonely journey for those affected with it. So, having a patient support group where you can talk to other people who either suffer from the disease or have taken care of a loved one who suffers from the disease can help the journey be a little less lonely, as well as share experiences and stories as well.

Also at UK, we are actively involved in research related to sarcoidosis. We work very closely with the scientists, the immunologist. At UK HealthCare, we are actively trying to bring in clinical trials that focus on patients with sarcoidosis because we want to get the cutting edge science and research on sarcoidosis to our patients here in the state of Kentucky.

Sonu Abraham, MD: As you've heard from the cardiac perspective too, there needs to be a multidisciplinary team, and we have that here at uk. So, we have heart failure cardiologist as myself, and we have a few more heart failure cardiologists on the team. We have electrophysiology cardiologists who are the heart rhythm doctors. ,Then we have cardiac imaging cardiologists who actually do the cardiac MRIs and the cardiac PETs. And so, all of these doctors help manage these patients in a collaborative fashion, and that helps the patient in the end. And it's very important to follow these patients closely as a small fraction of these patients do not improve with medical therapy and they may actually need a heart transplant.

And so, that's where it becomes important for doctors like me, like heart failure doctors, who are available to these patients at UK. And we have the ability to offer heart transplantation to these patients, and we have successfully transplanted several such patients here at UK.

Host: That's amazing. It sounds like UK HealthCare has all the bases covered when it comes to sarcoidosis, whether it's pulmonary or cardiac. So, good to hear from both of you and have you here today. Thank you both so much for sharing your expertise on this. 

Again, that's Dr. Sen and Dr. Abraham. To find out more, please visit ukhealthcare.uky.edu. That's ukhealthcare.uky.edu. And if you found this podcast helpful, please share it on your social channels and check out our entire podcast library for topics of interest to you. Thank you for listening to UK HealthCast, a podcast from UK HealthCare.