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Melanie Cole (Host):  Welcome. This is UF Health MedEd Cast with UF Health Shands Hospital. I’m Melanie Cole and today we will identify the symptoms of cardiac sarcoidosis and what physicians do to screen patients for it. We’ll discuss the diagnostic options and share the latest medical therapies being used to treat it. We’ll also examine ICD placement should be considered. Joining me is Dr. Divya Patel. She’s a Clinical Assistant Professor in Pulmonology at the University of Florida and practices at UF Health Chands Hospital. Dr. Patel, it’s a pleasure to have you join us today. Tell us a little bit about the incidence and prevalence of cardiac sarcoidosis.

Divya Patel, DO (Guest):  Hi Melanie. So, cardiac sarcoidosis is rare but can be deadly and so, it’s important to think about cardiac sarcoidosis. According to the ACCESS study which was a NIH funded study from ten years ago; they showed that the prevalence of cardiac sarcoidosis in the United States is less than 5%. However, there has been more recent studies using advanced cardiac imaging in asymptomatic patients that suggests that cardiac involvement can be as high as 30%. The problem is, we are not sure if that cardiac involvement seen on imaging that’s asymptomatic is clinically relevant.

But overall, we think that clinically relevant cardiac sarcoidosis occurs in about 5% of patients with sarcoidosis.

Host:  What do we know about the cause of it Dr. Patel and help other providers to identify the clinical manifestations or the symptoms of it.

Dr. Patel:  So, the cause of sarcoidosis is unknown. What we know about sarcoidosis is that it occurs in a genetically susceptible patient who are exposed to particular antigens that their immune system is honed to respond to. Now which patients with sarcoidosis get cardiac sarcoidosis specifically, is unknown. So, the important thing to know about cardiac sarcoidosis is that patients who have sarcoidosis can develop cardiac sarcoidosis at any point in time while their disease is active. In fact, cardiac sarcoidosis can even suddenly occur in patients who have inactive disease.

So, it’s really important to be vigilant about signs and symptoms of cardiac sarcoidosis in patients with whom you have diagnosed sarcoidosis. Some of the signs and symptoms that you could look for would be if you have a patient with sarcoidosis who is complaining about palpitations, syncope, or presyncope, chest pain; those are all potential signs for cardiac sarcoidosis. One important thing to keep in mind though is that sarcoidosis can present in patients in an isolated setting. So, a patient could have cardiac sarcoidosis without it affecting any other organ and the first presentation for a patient could be cardiac sarcoidosis. So, other situations in which I would think about cardiac sarcoidosis would be patients who have unexplained, nonischemic cardiomyopathy, who also have maybe some lymphadenopathy or have sustained ventricular arrythmias without an explanation but are younger and have potentially infiltrative cardiomyopathy based on the testing they’ve had.

Host:  So interesting. Dr. Patel, so what are physicians doing to screen patients? Are there some established screening guidelines? Tell us a little bit about diagnostic criteria, the options for cardiac sarcoidosis and why it’s sometimes difficult to diagnose.

Dr. Patel:  So, those are very good questions. And I wish I could tell you that we have very clear answers to those questions but unfortunately, we don’t. There is research that is ongoing, but unfortunately, we don’t know the answers to all of those questions. What we know from statements and guidelines, so from the Heart Rhythm Society statement that was published in 2014; we know that screening should include questions to appropriate patients, you should ask about palpitations, and consider an EKG. So, for example, in my clinic, for every patient who has sarcoidosis that we see, at every visit we ask about palpitations, we ask about arrythmias and we also make sure that we have an EKG on every patient once a year. Some people, some experts argue that echocardiograms should be a part of screening for cardiac sarcoidosis. But unfortunately, there’s no studies that show that echocardiogram or cardiac MRI or even cardiac PET are good screening mechanisms. So, at this point in time, there’s no studies that show one particular modality for screening is better than another. But the recommendations from the Heart Rhythm Society as they stand currently right now recommend asking about symptoms and obtaining an EKG.

In terms of diagnosis. Diagnosis is very challenging in cardiac sarcoidosis. And especially when patients have isolated cardiac sarcoidosis. So, diagnosis, in terms of diagnosis, there’s three organizations that have diagnostic criteria for cardiac sarcoidosis including the Japanese Ministry of Health and Welfare, the Heart Rhythm Society and the World Association of Sarcoidosis and other Granulomatous Disorders. Each of these organizations have a set of diagnostic criteria. Essentially, what they state is that definite diagnosis requires an endomyocardial biopsy, but the organizations recognize that endomyocardial biopsies have low sensitivity and because of this, they have a provision to give probable and possible diagnoses using positive biopsy – extracardiac biopsy.

So, for example, if you have a patient who has biopsy proven pulmonary sarcoidosis, but they present in complaint of palpitations; you obtain an echocardiogram that shows a low ejection fraction or you obtain long term EKG monitoring that shows sustained PVCs or frequent non-sustained ventricular tachycardia, that could be enough to give a probable diagnosis of cardiac sarcoidosis. So, cardiac sarcoidosis is one of the most challenging forms of sarcoidosis to diagnose. And requires patients be evaluated. It requires patients be evaluated at expert centers.

So, at our center, at UF Health Shands Hospital; our pulmonary division has a sarcoidosis program in which we provide comprehensive evaluation for sarcoidosis and we start with evaluation for pulmonary sarcoidosis and then we offer assessment and evaluation for all organ involvement and manifestations of sarcoidosis.

Host:  Well thank you for that explanation and before we talk about some of the treatment options available; because that initial presentation can range from asymptomatic ECGs to sudden death; tell us whose an integral part of the multidisciplinary team taking care of these patients? What types of providers are involved?

Dr. Patel:  That’s a great question. So, multidisciplinary care is imperative for patients with sarcoidosis whether it involves the heart or not. So, the doctors that are involved in managing patients with sarcoidosis include physicians that work in areas in which the organ manifestations occur. So, at UF Health Shands Hospital for instance; us in the pulmonary division, we really act like the primary care physician for our patients with sarcoidosis and we evaluate and assess for their pulmonary involvement, but we also have colleagues in the divisions of rheumatology, cardiology, department of ophthalmology, division of gastroenterology, department of dermatology. So, we include and involve physicians from all different divisions and departments within our own institution.

We also have colleagues in neurology that we work closely with who have neuro-sarcoidosis. Within our own division, we work very closely with our pulmonary hypertension specialists and also our lung transplant doctors who evaluate patients with more advanced disease in cases in which they may need lung transplantation.

Host:  Then let’s speak about some of the latest medical therapies. Start with any medicational intervention you’d like to speak about and then since patients with cardiac sarcoidosis are at risk of sudden death Dr. Patel and there’s not much data to help with risk stratification; tell us a little bit about when you feel it’s important to consider an implantable cardioverter defibrillator. When should placement be considered? But if you would, start with any medicational intervention that you would typically look at.

Dr. Patel:  So, treatment of sarcoidosis – we think about treatment of sarcoidosis in two situations. One when the sarcoidosis is life threatening so for example, in end stage lung disease in patients with pulmonary involvement of sarcoidosis. And other situations for example patients with severe pulmonary hypertension. Those patients may have a life threatening presentation of sarcoidosis and then thirdly, in patients with severe cardiac sarcoidosis with low ejection fraction that is decompensated. So, once again, in patients with life threatening sarcoidosis and then the second category in whom we consider treatment are those who have severe disease that impacts quality of life.

So, that’s when we consider treatment. Treatment primarily involves immunosuppression and the backbone of that immunosuppression is corticosteroids. We start with prednisone when patients have acute symptomatic sarcoidosis. However, we try very hard to use less corticosteroids because of the significant side effects associated with them. And in those situations, in people whom we think will need long term immunosuppression; we try to use drugs that are in the cytotoxic category so for example, methotrexate, azathioprine, mycophenolate and leflunomide. So, those are medications that we use frequently to treat chronic long-term sarcoidosis. In the most severe cases, we consider medications like TNF alpha inhibitors such as infliximab and the specific treatment that we pick and when we use specific treatments are dependent on again, one, the severity of the symptoms and the severity of the disease and how life threatening the organ involvement is.

So, that’s how we decide about medication use in patients with sarcoidosis. It’s important to remember that not every patient with sarcoidosis needs treatment. There are many people that are treated for sarcoidosis in whom the treatment is more harmful than the disease itself. So, it’s very important to be careful about whom you decide to treat and who you don’t decide to treat.

In terms of the question about the implantable cardioverter devices. The Heart Rhythm Society guidelines state that Class I indications for ICD placement are spontaneous sustained ventricular dysrhythmias, prior cardiac arrest, or left ventricular ejection fraction less than or equal to 35% despite optimal medical therapy. So, those are the patients in whom they have Class I indications.

It sounds very simple, but this is an area that can be very complicated and challenging in patients with cardiac sarcoidosis. So, we see many patients who have ejection fractions that are 40%, 45%, they are having some PVCs, or they are having a few episodes on non-sustained ventricular tachycardia or they may have a cardiac PET that shows uptake and hypermetabolism. Those patients, it’s very challenging and in those situations, we ask our colleagues in the electrophysiology department to do EP studies. So, in a patient with an ejection fraction of 40% with a positive EP study; those are patients in whom I may also consider ICD placement, but they don’t meet those Class I indications that the Heart Rhythm Society guidelines state.

Host:  So, what’s the general prognosis for patients? What are you seeing? What have been your outcomes?

Dr. Patel:  So, the general prognosis for patients with cardiac sarcoidosis in whom the cardiac sarcoidosis is detected early and treated is good. However, we do know that in terms of morality in sarcoidosis; patients with cardiac sarcoidosis tend to have much higher mortality than patients who don’t have cardiac sarcoidosis. So, it’s really important when you’re seeing patients, evaluating patients who have sarcoidosis to look for and diagnose cardiac sarcoidosis as early as possible.

Host:  As we conclude, what do you want other providers to know about what you’re working on at UF Health Shands Hospital, when you feel it’s important that they refer and anything you’d like to tell them about cardiac sarcoidosis.

Dr. Patel:  So, at UF Health Shands Hospital, we have a multidisciplinary clinic that’s organized in a way to provide complete comprehensive care to patients with sarcoidosis with any type of organ involvement. We have multidisciplinary meetings to support or multidisciplinary care, so we meet with our cardiology colleagues, we meet with our neurology colleagues frequently. We have identified clinicians in multiple divisions and departments who are our point people to have our patients evaluated by who really care about patients with sarcoidosis and really are knowledgeable and experts at caring for patients with sarcoidosis.

Host:  Well thank you so much Dr. Patel. It is such a fascinating topic and such great information. Thank you for joining us and sharing your expertise. And that concludes today’s episode of UF Health MedEd Cast with UF Health Shands Hospital. To learn more about this and other healthcare topics at UF Health Shands Hospital, please visit www.ufhealth.org/medmatters to get connected with one of our providers. Please remember to subscribe, rate and review this podcast and all the other UF Health Shands Hospital podcasts. For more health tips and updates on the latest medical advancements and breakthroughs, please follow us on your social channels. I’m Melanie Cole.