Scott Webb (Host): Lupus is an autoimmune disease. And there's a strong correlation between lupus and heart disease. And my guest today is here to tell us more and emphasize that there are many good reasons to be diagnosed and treated if you are suffering from lupus. And I'm joined today by Dr. Marcia Johnson. She's a Board Certified Rheumatologist practicing at Franciscan Health.

 This is the Franciscan Health Doc Pod. I'm Scott Webb.

Doctor, it's great to have you here today. We're going to talk about lupus and heart disease and what those things mean and how they go together and really benefit from your expertise. And I know this is the first of two podcasts you're going to do. So today it's lupus. So let's start there. What is lupus?

Can you describe lupus to us?

Marcia Johnson, MD, PhD: Sure lupus is an autoimmune disease, where the body, instead of using the immune system to fight off bacteria or viruses, it's actually attacks yourself and it can affect multiple organs, including the skin, joints, heart. It can affect the nervous system and kidneys, so it has multiple effects. It's seen in about one out of a thousand people.

It's seen more in women than men, about a 10 to one ratio, women to men. It can occur at any age. Definitely can occur in childhood, unfortunately. And it's usually diagnosed by a combination of blood tests and symptoms. So sometimes the diagnosis isn't clear cut, so you sort of look for classic symptoms.

You look for supporting blood tests and try to put the two together. And there are people certainly where things suggest lupus, but we are not sure if they have it yet or not. Other people, it's quite obvious that they do have lupus.

Host: Yeah. Right. So you as the expert and then maybe the team there at Franciscan Health try to connect the dots. What is that connection though, between lupus and heart disease? Maybe you can, we can just focus in on that.

Marcia Johnson, MD, PhD: Sure. Well, actually there are sort of two connections. First of all, having lupus increases your risk of coronary artery disease, just regular heart disease that you think about it. They think that might be related to damage to the blood vessels due to lupus and the chronic inflammation, but it's a really striking increase. Actually nowadays, the major cause of death if you have lupus, is heart disease and regular coronary artery disease. It used to be other things, but it's not lupus. It's actually heart disease.is the major cause of death. It's actually about 50 times higher for a woman aged 35 to 44 if they have lupus. So it's a striking increase and people say, well, what can you do to prevent that?

 The main thing is to get the lupus in remission if you can. And also there's a drug called Plaquenil, hydroxychloroquine, classic lupus drug, which actually in some studies it's shown it decreases the risk of heart disease by 50%. And the other things you can do are the regular things you do if you have heart disease or concerned about it, you know, diet, exercise, monitoring cholesterol, not smoking.

The usual risk factors that you try to, you know, improve things as much as possible. The other thing is there are some specific heart manifestations of lupus. The main one is pericarditis. Pericarditis is an inflammation of a lining around the heart and you could leak fluid from the inflammation. You can actually get, it's called a pericardial effusion or fluid around your heart. If that is too large, your heart can't pump well, and that's incredibly serious. You'd end up in ICU. They feel that's seen in about 25% of lupus patients is what the literature says. I'm not sure it's quite that high. I mean, based on my experience, but definitely pericarditis is associated with heart disease.

It tends to cause chest pain as you might expect, which actually improves by leaning forward. And, ECG can show some changes that are kind of specific for it too. So it's something where people, if they're you know, feeling awful enough, they go to the emergency room where they would diagnose it.

 Also lupus can cause what's called myocarditis. That's rare, thank goodness. It's actually where the heart muscle is inflamed. And obviously if that is severe, it can, you know, definitely impair the ability of your heart to pump. It can also affect the conducting system, the electrical system around the heart.

And again, fortunately that is rarer. A lot rarer because it could be very serious. And then another thing lupus can do, which usually again, rarer, is it can affect the heart valves where you can actually have growths on the valves, not infection. But if you have that, you're at high risk for getting an infection affecting that, 'cause you kind of changed the structure of the valve. It's called Libman-Sacks endocarditis. And also there's a higher risk of, of stroke if those little vegetations, as it were, break off. So, you know, certainly lupus could affect the heart in multiple different ways. And people say, how do you protect against that?

I mean, the thought now is that hydroxychloroquine, Plaquenil may protect somewhat. And then for protecting yourself from regular heart disease, all the usual things one would do with diet, checking cholesterol, exercise, obviously, you know, things that one can do to reduce the risk of heart disease.

Host: Right. Yeah. Wondering what are some of the early warning signs or symptoms? I'm assuming, you know, discomfort in the chest, pain in the chest, maybe. But from your perspective with the lupus patients, what should they be aware of as it, you know, might be an indication of a heart related problem?

Marcia Johnson, MD, PhD: Well, for a regular atherosclerotic or you know, coronary artery disease, you would look for problems with chest pressure, with exercise. Sometimes it feels like indigestion for some people. Some people don't have pain in their chest. It's actually referred to their arm and it's, you know, regular heart issues.

And definitely that should be looked into if there's any doubt, even if one is younger. So you're 40 years old and you have lupus, maybe push people toward doing a stress test or some way to check to make sure there's no blockage of the arteries. People say, oh, you're too young. Again, having lupus, you're at higher risk for regular, as you want to call it regular heart disease.

Host: Mm-hmm.

Marcia Johnson, MD, PhD: Things like pericarditis. You would have chest pain, it'd be somewhat different quality, but it's, it's hard to tell. So you would probably, if it's severe enough, get evaluated. And again, characteristically the pain that you get with pericarditis actually improves if you lean forward. People who are having regular like blockage of their arteries, it wouldn't improve with that at all.

So that's one way to maybe tell the two apart.

Host: For sure. Yeah. And I know one of the hallmarks, doctor of lupus is inflammation. Maybe you can help us sort of, we were talking earlier about connecting dots, like how does that, how does the inflammation with lupus contribute to the development of heart disease?

Marcia Johnson, MD, PhD: Well, inflammation of any sort, whether it is lupus or due to active rheumatoid arthritis or other diseases, does increase the risk of heart disease. One theory is that might be related to damage to the blood vessels due the inflammation. So obviously the controlling the underlying cause can help reduce that risk.

Host: Yeah, definitely. And you were talking earlier about patient history and how you diagnose lupus. So I wanted to go through that for folks. How is it typically diagnosed? What kinds of tests or screenings are recommended?

Marcia Johnson, MD, PhD: Often I have patients sent to me because they have a positive ANA or anti-nuclear antibody, and some rheumatologists believe you have to have that blood test be positive to be diagnosed with lupus. However, there are many people who have a positive blood test, a positive ANA, who do not have lupus.

So it's usually a combination of symptoms that one develops also and and more specific blood tests. So most people with lupus are sun sensitive. They go out in the sun. And they may break out in a rash. The classic is what's called a butterfly rash, which looks like a butterfly over the bridge of the nose, but doesn't go down lower in the face.

So it's not, if it's like below the chin area, that's not as classic for it. And it's not just getting a rash when you go out in the sun, often they will have a low grade fever and feel awful. So that is not a rare symptom of lupus at all. You could have mouth and nose sores with lupus, not that rare. You could have a small joint arthritis, it could be other joints too, but often is where you're stiff and sore and swollen in the morning, you may be stiff for an hour or two, which is not typical at all for anything. And then you improve later in the day with activity, which is what an inflammatory arthritis, is how it behaves.

Lupus again, you can have pericarditis with it. Fortunately, relatively rare. You can have, pleurisy not related to respiratory infection, but from lupus. That's not that uncommon. If you have kidney involvement, actually by many criteria, lupus kidney involvement is all it takes to be diagnosed with lupus.

But nonetheless, kidney involvement definitely you see with it. And the easy way to check for that, at least initially screen, is to just check the urine, which is an easy enough test. You can have neurologic involvement with it, but that's usually later on. And fatigue. I mean, almost everyone with lupus is really tired, more so than would be normal for their age.

 They want to just rest, but they don't feel that much better if they rest. But, and if they have an inflammatory arthritis with lupus by resting, they get stiffer again when they try to get up and do something. So there are multiple factors that are involved. Blood workwise, I mean the ANA is classic for lupus, but again, not sufficient to diagnose it.

They're more specific blood tests like anti double stranded DNA binding, which is more specific for lupus, something called the Smith antigen. More specific, there are proteins called compliments, that can be lower with lupus. So it's usually a combination of different factors to make the diagnosis.

And there are some people one sees where they have some things but not quite enough, but you monitor, and if they're close enough, you may even try some treatment too.

Host: Okay. Yeah. I wanted to get a sense from you as a rheumatologist, you know, how do you help in the coordination of care, working with cardiologists and perhaps other members of the multidisciplinary team for lupus patients.

Marcia Johnson, MD, PhD: Well with cardiologists, if people have had lupus with cardiac involvement is a matter of, you know, they may order tests to be done, to check to see what's going on, to check for coronary artery disease. Echocardiograms sometimes if we suspect there could be some pericarditis, I might order it, but then the cardiologist would probably take over treatment of that, obviously. When lupus affects the skin, sometimes dermatologists you work with, and there are some people who have lupus only affecting their skin. They don't have internal involvement, and there are different types of skin manifestations. It can be tough to sort out. Sometimes skin biopsies are done to look for things.

Other specialists if lupus is affecting the kidneys, obviously, kidney specialists, nephrologists take over there oftentimes. And, we coordinate care basically, you know, they kind of take care of the kidney end of things.

So, there are multiple specialists one might work with.

Host: Right. Yeah, definitely a team effort, patient specific, all that at Franciscan Health. What advice would you give to someone who's newly diagnosed with lupus?

Marcia Johnson, MD, PhD: Number one, to coordinate their care with a rheumatologist. To try to avoid sun exposure because that can be a trigger for it. And I tell people, Hey, by avoiding sun exposure someday when you're older, you look younger than your friends. You got that going for you too. But, you know, sometimes the sun can trigger things.

Not everybody, but most. To obviously reduce their risk factors for heart disease. You know, if they're a smoker, stop smoking. Exercise is good. Watching diet. Some people feel an anti-inflammatory diet helps, other people don't, and reducing stress as much as one can, getting adequate rest. So just a whole, you know, kind of range of things one can do.

Host: Yeah. Let's finish up here today, and I know we're going to talk again soon. We're going to talk about spondylitis. It's just a kind of a fun word to say. I like saying spondylitis, but we'll do that next time. For today, any new promising treatments or research developments in the area of lupus?

Marcia Johnson, MD, PhD: For many years there was only one FDA approved drug for lupus, hydroxychloroquine, or Plaquenil, which is over 60 years old now. Back in about 2011, BENLYSTA, belimumab was approved, which is IV or injectable, which is not actually going to help lupus affecting the kidneys, which is not a super strong immunosuppressive.

That has helped some people. It's helped reduce fatigue and joint pain and other symptoms. And then more recently, about three years ago, SAPHNELO, which is also called anifrolumab, they have horrible names for these. Any rate was also approved, which is only IV right now. But that also has helped a lot of lupus patients.

So I've had lupus patients where on either of those two medications they've gone from, they were taking Plaquenil usually, but adding those on, they've gone from not being able to do much, being really tired, really limited to leading a much more normal life. I mean, some people, it's really helped their energy level, which is what people most want to improve oftentimes, help their joint pain.

It's been very, very useful for some people. In development, they do have other drugs, which I don't know how they'll pan out. Lupus does have a track record of drugs getting to like the final phase of testing, then they flunk. For example, there's a drug called RINVOQ The generic name is upadacitinib. It's a horrible generic name, but at any rate, great drug for some people with rheumatoid arthritis and spondylitis, but also being tested for lupus now. Don't know how that will work out.

And there are several other drugs in development too. And then the most aggressive treatment is they're using CAR T-cell therapy in people with severe lupus. They've only done small trials so far, but they found these were people with very bad disease. And so far, from what I've read, there was a study in Germany, not a huge number.

There are trials ongoing in this country right now. It's totally put it in remission afterwards. People have been able to get off all their medications and do very, very well. And so far it seems durable. So that looks, although that's a fairly aggressive therapy. But that looks, very interesting and potentially quite promising.

So I mean, we'll see in the future.

Host: Right. Yeah. You and I were speaking before we get started today about how quickly things change in medicine and science and research, and it's, it's very exciting and, great for providers and patients and families and everybody involved. Like I said, we'll talk again next time about spondylitis. Until then, thanks so much.

Marcia Johnson, MD, PhD: Oh, you're welcome. Thank you.

Host: And for more information, visit franciscanhealth.org and search rheumatology.

Host: And if you found this podcast helpful, please share it on your social channels, and be sure to check out the full podcast library for additional topics of interest. This is the Franciscan Health Doc Pod. I'm Scott Webb. Stay well, and we'll talk again next time.