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Whether you’re currently living with lupus or caring for someone who is, this disease can sometimes feel unpredictable. Rheumatologist Emily Littlejohn, DO, explains lupus triggers and how to manage flare-ups. She also chats about common treatments, the average timeline for finding a diagnosis and ways to find support.

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Managing Your Lupus with Dr Emily Littlejohn

Podcast Transcript

Cassandra Holloway:

Hi, thanks for joining us for this episode of the Health Essentials Podcast. My name is Cassandra Holloway and I'll be your host. Today we're talking about living with lupus. We're here with rheumatologists, Dr. Emily Littlejohn. Thanks for being here today, Dr. Littlejohn, and welcome to the podcast.

Dr. Emily Littlejohn:

Thank you so much, Cassandra.

Cassandra Holloway:

Today in this episode, we'll be talking about what it's like to live with lupus. We'll do a deep dive into everything from how it's typically diagnosed, all the way to how it can affect your body and life as you know it. Lupus tends to be one of those diseases that many people have heard of, but if you're like most people, you probably don't know anything too specifically about it. So we will be talking to Dr. Littlejohn today about living with lupus and everything that entails.

              So with that being said to start off our discussion, Dr. Littlejohn, I want to first ask you if you'll tell us a little bit about your practice at Cleveland Clinic and the types of patients you typically see.

Dr. Emily Littlejohn:

Sure. I see all types of patients with rheumatologic disorders at the Cleveland Clinic main campus. And in particular, I see a lot of lupus patients. We have one day that's devoted just to lupus and we call it the lupus clinic. And typically I see patients alongside other specialists on that day, so nephrologists or dermatologists. I also run the lupus biobank, which is a bio-repository of lupus patient's blood and urine. And the idea is we see patients, we get a lot of input about their history and their medications and their family, and then we're able to biobank or freeze their blood and urine to then do further testing on in the future.

Cassandra Holloway:

I want to start off by asking in its simplest terms, what is lupus? How do you define this disease?

Dr. Emily Littlejohn:

I get this question a lot. In its simplest term, lupus is the body attacking itself. So the body stops recognizing itself as self. It starts attacking itself in different organs, in different areas, which then causes inflammation at those sites and the inflammation causes the pain or the lupus manifestations in the body.

Cassandra Holloway:

And what are some of those manifestations? What are some of the top symptoms that you often see with lupus? I'm sure they range on a big spectrum, but what do you typically see?

Dr. Emily Littlejohn:

Everyone's Lupus is different. We have this idea of a lupus fingerprint, so everyone has their own lupus fingerprint. But lupus can really span any involvement of the body, so from head to toe, it can cause hair loss, patchy hair loss, what we call it alopecia. Can cause lupus on the face in terms of rashes or sun sensitivity. It can cause ulcers in the nose or in the mouth. Certainly joint pain, patients come in and they have pain in the hands or the feet, the wrists, usually along with swelling and some redness. And then of course, internal organ involvement, so patients can have lupus that affects the heart or the lungs. And one of the most devastating manifestations is called lupus nephritis, when lupus affects the kidneys.

              So really any organ can be involved. I would say the most common, I see a lot of joint pain. I see a lot of hair loss, weight loss, fatigue certainly can go along with lupus, although it's very uncommon for people to have lupus with just fatigue as their main manifestation.

Cassandra Holloway:

I feel like you often hear people talking about these lupus flare ups and you mentioned inflammation. Can you talk a little bit about what it means when people are saying they have a flare up right now because of this disease?

Dr. Emily Littlejohn:

Sure. Just like I mentioned about the lupus fingerprint, so if a patient has lupus in the joints and they're very sun sensitive, they might go out into the sun or they might get sick with a virus or some other illness and they have a flare. Flares can be caused by many things, missing medications, like I said the sun, or just being sick in general from a virus or another infection, but their typical manifestations will then come out, so they'll have joint pain, they'll have rashes on their face. They'll often be very fatigued. They can have changes in their urine. Usually patients with lupus will know when they're flaring. Every patient kind of has an idea of what their lupus is like and they can tell when it's active.

Cassandra Holloway:

Are there certain triggers? Obviously, you said going out in the sun. If you have lupus, should you be aware of what triggers your flare ups to typically happen?

Dr. Emily Littlejohn:

Absolutely, absolutely. Yeah. Usually when patients are diagnosed, that's one of the first things we ask them. What are the main symptoms that you've had in the past that keep recurring or are worsening. And those typically are what are triggered when patients have a flare. We also can see in the blood, and sometimes in the urine, markers for inflammation or lupus activity, so it kind of confirms to us that, yep, this is definitely a lupus flare and these are symptoms from active lupus.

Cassandra Holloway:

And you mentioned the rash that typically flares up. How common is that, or again, does it depend on the lupus fingerprint? You often hear about that butterfly shaped rash on the nose or cheeks. Talk to me a little bit about the skin aspect of lupus.

Dr. Emily Littlejohn:

This is a great question. It's very important because some people can have just sun sensitivity. We go out in the sun and we get a sunburn. We get a little bit of redness on our face or on our chest, wherever the sun was exposed. Sometimes it leaves a red mark and then it go away within an hour or a few hours. Typically, patients with a true malar rash, which is the rash that we call a lupus rash, usually it takes hours to days to really come on. So patients will go away on vacation for the weekend and on Monday they come back and they've noticed they have some new lesions on their face or their chest and they kind of get worse before they get better. So they're palpable typically, they're very dark. They're not transient. They don't come and go quickly. They stay for a while.

Cassandra Holloway:

Are there different types of lupus?

Dr. Emily Littlejohn:

Yeah. There's about four different types. The first one is systemic lupus, which is lupus that's all over the body. We see it in the blood. And that can be, like I mentioned in the joints, on the skin, in the heart, in the lungs, in the hair, in the kidney. That generally is called systemic lupus.

              Some people have isolated lupus on the skin and that's called cutaneous lupus. Patients can have Lucas from drug effects, so certain medications can cause lupus. It really just sort of drives lupus to come to fruition. And the last one is called neonatal lupus. And that's when lupus really passes from the mother through the placenta and affects the fetus. Patients with lupus when they have babies, we're usually very careful about surveillance and monitoring them while they're pregnant to avoid this type of thing. But babies can be born with lupus.

Cassandra Holloway:

Did you say systemic lupus is the most common form of lupus just in general?

Dr. Emily Littlejohn:

Mm-hmm (affirmative). Mostly because there's just so many things that fall under systemic lupus, so lupus nephritis, patients can have lupus pneumonitis or those types of things.

Cassandra Holloway:

I feel like you often hear about this notorious lupus brain fog. What is that and what causes that?

Dr. Emily Littlejohn:

That's a great question. And that's a question that I cannot answer in its entirety. There's a lot to be understood about that process. We think that what really is going on is there is a lot of inflammation at the blood, brain border. So the area that protects your brain, there can be some lupus activity there and some inflammation, and that local inflammation can cause brain fog, sort of like how patients get migraines or they are forgetful. We think there might be an inflammatory reason for patients who have the brain fog. Sometimes when we treat the lupus it gets better and sometimes it doesn't. There's just a lot to really kind of figure out in terms of what's going on with that.

Cassandra Holloway:

With all of these big ranges of where your symptoms fall, what about the mental health side of lupus? I imagine having all of these symptoms can be quite emotionally exhausting, especially if something triggers it or you can't control the flare ups. Talk to me about the mental health side of things.

Dr. Emily Littlejohn:

Absolutely. Lupus in general, I think, is a multidisciplinary disease. So we have a lot of specialists who need to be involved. Here at the clinic when we see patients, we oftentimes will refer them to a cardiologist, a physical therapist, a nephrologist. And one of those specialists who's involved in the care is oftentimes a psychiatrist or a psychologist, because as you mentioned, it does take a huge mental toll. It's a chronic disease. Typically, it's a lifelong disease and there's a lot of anxiety and depression that can go along with it.

Cassandra Holloway:

You mentioned it was a lifelong disease. Can it be cured or does treatment basically focus on quality of life, it won't ever go away?

Dr. Emily Littlejohn:

I would like to describe it in terms of remission. Patients can come in and they can have rip-roaring active lupus. We can give them our strongest medications, which are often chemotherapies, and we can put it into remission and it can be very quiet for a very long time. Now, some patients need to be on lifelong therapy and some patients are able to come off their medication very far down the line. But generally speaking, we don't like to use the word cure. I think because it's a genetic component to this disease, along with some other things, it's usually lifelong.

Cassandra Holloway:

I know you mentioned that systemic lupus is the most common form of lupus, but generally speaking, how common is lupus? Who does it affect and how often?

Dr. Emily Littlejohn:

The most commonly affected people are women of childbearing age, so ages 15 to 44. Like I said, women more than men, in about a nine to one fashion, women are more effected. And it typically affects women of certain ethnic groups, minority, racial and ethnic groups, African-Americans, Hispanics, Asian-Pacific Islanders, more than other groups. Generally, we think there's probably between 200,000 and 300,000 patients right now living in the United States with lupus. It's a tough number to get because a lot of times lupus is either not diagnosed or it takes a while to be diagnosed. But typically, women, childbearing age, usually African-American, Hispanic, Asian-Pacific Islander. Men are also affected, I would just say much less so.

Cassandra Holloway:

That's really interesting. I didn't know it was primarily women, but like you said, obviously, men can be affected as well.

Dr. Emily Littlejohn:

Yeah. And when men are affected, they could be much more severely affected. So for example, in patients with lupus nephritis, which is lupus in the kidney, when men have lupus nephritis, very often, they have a very severe presentation. It happens very quickly and they're very sick.

Cassandra Holloway:

I'm curious, is there a genetic factor in lupus? Is it ever hereditary?

Dr. Emily Littlejohn:

Mm-hmm (affirmative). When patient's ask me why, why they have lupus, I tell them what we know to date, which is that we think it's born of a two hit theory. So there is probably some genetic underlying issue, whether it be in the complement pathway or these other pathways of inflammation markers, but there's really a second hit. So there's something that's really unmasking and letting this to fluoresce and whether that's an exposure to an estrogen or a hormone, whether that's an exposure to a virus such as the mono virus, we have some evidence of that, or whether that's exposure to UVA or UVB light, we're not totally sure. But it's probably both of those things in conjunction together.

              It's not typically seen in first degree relatives. Typically, a person with lupus doesn't have sisters and a mother also with lupus. It does happen, but it's more commonly sort of clustered in families. So I will have a patient who has lupus and maybe her distant cousin also has lupus or maybe her mother had rheumatoid arthritis. We have a lot of these diseases that are sort of cousins and they live on a spectrum of each other, so patients often will have family members with one of those other types of diseases.

Cassandra Holloway:

That's really interesting that you can often find a similar situation going on somewhere in the family line at that point.

Dr. Emily Littlejohn:

Yep. There's a lot of clustering, I would say. That's kind of what we call that. Yep.

Cassandra Holloway:

Can you have lupus and have no idea? Are there always symptoms or can it go undiagnosed for your whole life or for several years?

Dr. Emily Littlejohn:

That's a great question. For the most part I tell patients lupus doesn't hide. If you truly have very severe active lupus, we're going to see it somewhere, whether that be in the physical exam, whether that be in the blood or other imaging. You can see this internally on echocardiograms of the heart or different images of the organs.

              There are some cases, though, where patients come in and they have this very abnormal blood work and they feel great. And those patients I usually don't let leave my sight for very long. I usually will see them back every six to nine months, because my concern is that they're accruing inflammation slowly over time and they're going to become a lupus patient or a patient with an autoimmune disease. It can be silent for a while, but if you truly have strong evidence in the blood of a lupus or lupus like syndrome, I usually like to check up with them at least once a year.

Cassandra Holloway:

So I know you said you like to think about lupus and the cure for lupus kind of being in this remission state. What is the prognosis for lupus in the long-term effect? What are those most severe outcomes if you do have lupus?

Dr. Emily Littlejohn:

What we worry the most about is end organ damage. So when patients are diagnosed with lupus, we want to figure out what their fingerprint is and those who have severe end organ damage are the ones that we worry about the most. For example, if someone comes in and they come in with issues in their kidneys or their urine is very frothy and we do a kidney biopsy and they have very active lupus nephritis, those patients we worry about the most. Because that means that their lupus came on quickly, it's very active and who knows how long it's been active. So the prognosis for a patient who is very ill at the beginning and is perpetually ill, or it takes a long time to get into remission, the prognosis is likely worse. And that typically happens in young women who, like I said, come in with very severe disease.

              We also see patients who are a little bit older. Women maybe over 50, who have lupus. Typically, they don't have severe organ involvement, but those patients tend to do a little bit better. They might have a little bit of arthritis or some skin manifestations of lupus, but they don't have end organ disease. And they tend to do very well.

Cassandra Holloway:

So, with this lupus fingerprint that we keep mentioning and you said managing lupus is this multidisciplinary team approach with your team of doctors and clinical professionals, how long does it take to diagnose lupus? I imagine having all of these symptoms and in between different flare ups and triggers, it might be a bit difficult to find a true source to why you are having these symptoms and why you're feeling this way and what's ultimately causing these issues. Talk to me a little bit about diagnosing a lupus disease.

Dr. Emily Littlejohn:

The big thing with lupus is there are a lot of mimickers. The mimickers can be other autoimmune diseases. The mimickers could be an infection. They could be a malignancy, a blood cancer or a blood disorder. So we have to be very careful when we diagnose lupus, because we have to look at the blood. We have to look at what's going on with the bone marrow. And oftentimes we have to go look at other organs, so the urine or advanced imaging and making sure we're not missing something else.

              So that's sort of the first big issue in that sometimes patients have one or two symptoms that sound lupus like, but we don't see it in the blood. Or the opposite happens, like I mentioned before, we see something in the blood, but they don't have any symptoms. So sometimes we'll wait until we have more symptoms or more evidence that there's true lupus activity. And the reason why we wait sometimes, even if we suspect there's lupus is because our medications aren't without side effects. Our medications are typically very immunosuppressive. So I don't want to start a patient on a chemotherapy or a drug that's going to put them at risk for an infection, unless I'm really sure that that's what's going on. Especially during COVID we want to be very conservative with putting people at risk for an infection.

Cassandra Holloway:

You mentioned the blood test, that's a big one for obviously diagnosing lupus. What other tests or exams are typically performed when you're going through this kind of diagnosis?

Dr. Emily Littlejohn:

Typically, at a first visit, when I suspect lupus, we do get a panel of blood work. Patients will probably be familiar with the ANA test and that's by no means a specific test for lupus, but it is very important that patients have a positive ANA. We also look at their urine because the urine can tell us what's happening in the kidney. And like I mentioned before, that's the one organ that we don't want to miss, if there's involvement of the kidney. Oftentimes I'll also get x-rays of the hands or the feet or any of the joints that we think could potentially be involved with the lupus. And then if patients have issues with their heart or their lungs, we'll certainly get a CT scan of the lungs and an echocardiogram of the heart or at the very least an EKG, so looking at the way that the heart is beating,

Cassandra Holloway:

Is there an average amount of time that it typically takes to find a lupus diagnosis in a patient?

Dr. Emily Littlejohn:

I'm not sure I can say there's a typical amount of time. I do know that patients will come to me with lupus and they'll tell me that back when they were in high school or back when they were a teenager, they had these symptoms. And it wasn't until 10 or 20 years later that they finally are diagnosed. And whether that be because they didn't know to go see a doctor about it, they didn't make their way to rheumatology or no one told them that that could potentially be a lupus diagnosis, I'm not sure, but there often is a very, a long lag time to diagnosis.

Cassandra Holloway:

Do you have any advice for listeners who might be feeling frustrated by a lack of a diagnosis in general, whether it's lupus or not, ultimately?

Dr. Emily Littlejohn:

I think the first thing is, there's no shame in getting a second opinion. We see a lot of second and third opinions here at the clinic. Blood work typically is the same at different institutions, but in some cases, blood work will be run differently. The different assays can be either machine run or human run, so that can be important.

              And I think just getting a second set of eyes on what's going on, because maybe there is something that's more serious going on, like I said, like a malignancy and they need to see the hematologist, maybe they need a bone marrow biopsy. So I think if there's ever an idea of we're missing something or there's some frustration, to always get a second opinion. And also be your own advocate. Be strong about getting a second opinion. Ask questions. Go back to your primary care doctor. Seek out other specialists, because it is a very frustrating disease and it's very elusive, so we have to have a keen eye for it.

Cassandra Holloway:

If you have been diagnosed with lupus, what sort of treatment is available? Walk me through what you first start thinking of in terms of a treatment plan for someone with lupus.

Dr. Emily Littlejohn:

When someone comes in with lupus, we assess which organs are involved. That's the first thing, if it's joint pain versus if it's issues on the skin or issues with hair loss. And like I said, I'll usually engage other physicians, so if it's someone coming in with very severe skin lupus, I'll certainly see them and treat them, but I'll send them to the dermatologist also to help with treatment.

              The first line for temporizing measures for acute flares, we use steroids. And any lupus patient who has taken steroids before, they know it works very well, but there's a lot of side effects with steroids. Steroids are very dangerous long-term. Literally, they can affect almost every part of the body, every organ of the body in a very negative way. That's usually the first line just to get things under control.

              The next step is something called Plaquenil or hydroxychloroquine, which is a medication that I call lupus life insurance. Patients will take it and they'll be on it for life. They're really investing in their future self. It's really one of the only drugs that we have that prolong survival in lupus and can prevent organ involvement. So every lupus patient, unless they can't tolerate the drug, is on Plaquenil.

              After that, we have our more immunosuppressive medications and there's a whole slew of them. We have a few that are just for lupus, like Benlysta, which is a great medication, but there's a lot of other drugs that we've borrowed from the oncology world. Things like mycophenolate or methotrexate, and these are all just drugs that they modify the immune system and they can be taken orally. Sometimes they're given as an injection. And then we have our biggest guns, which are usually infusions, so things like cyclophosphamide or cytoxan or rituximab, which are our big hitters and that requires a patient to come into our infusion center and sit for four to six hours and they get an infusion and those drugs require a lot of monitoring for blood work and for infections and those types of things.

              So there's different tiers of how we treat patients. And that's just a pharmacological aspect of it. We always talk to patients about lifestyle and eating heart healthy diets and making sure they're exercising and if they need to see physical therapy to make sure they're strengthening their ligaments and their muscles around their joints. So it's kind of a multi-pronged attack here. And also some patients find that if they eat a certain diet, they feel great. So patients who avoid heavy sugar diets or gluten or other inflammatory foods, they tend to feel better. We really just recommend a heart healthy diet because patients with lupus are known to have advanced coronary artery disease or atherosclerosis, so we ask them to be very conscious of that. But, like I mentioned, a lot of times the cardiologists will help us talk to them about that, or we can refer them to dieticians.

Cassandra Holloway:

How important is that doctor, patient relationship? It sounds a lot like you're counseling these patients and kind of working through a treatment plan and they obviously have to be having an open conversation with you about what they're experiencing in terms of symptoms. It just sounds like there's a very special bond between a doctor and a patient to try to solve this problem together.

Dr. Emily Littlejohn:

I feel a strong connection to my lupus patients and a lot of that is because I feel sort of like their primary care doctor, even though I'm really not. But because we're taking care of so many different parts of their disease, from really every organ to even just their health maintenance. We talk about vaccinations. We talk about their pap smears. We talk about things that they just need to have every single year. But when they come in and we examine them, we do a full body exam, so you're very familiar with your patients. It's a very intimate relationship. And I think that's important because you don't want to miss anything, just because lupus can pop up in different things over time in different organs and kind of morph and you want to make sure that you have an idea of what's really happening with each patient.

Cassandra Holloway:

Are there any other alternative treatments that you often will recommend to patients? I know you said physical therapy. I'm curious, is acupuncture or massage ever something that you recommend?

Dr. Emily Littlejohn:

If they feel better with acupuncture or massage, I say, that's great. Your mental health is important. If it makes you feel good, you're going to be well. In terms of what the evidence has told us, we know that patients who keep moving do better. So as long as they're active, whether that means pool therapy or walking or any kind of other hobby that they have, even meditating, just staying active mentally and physically, we know patients do better over time.

Cassandra Holloway:

My last question today for you is just about generally coping with lupus. If you have lupus or you were recently diagnosed, you're likely experiencing a lot of emotions from fear to frustration. How important is coping and support when dealing with a lupus diagnosis, either right in the very beginning or as a lifelong kind of disease that they're going to have to live with? What parting advice do you have for our listeners?

Dr. Emily Littlejohn:

This is important. Like I mentioned before, being your own advocate is everything. Bringing people to your appointments who can also advocate for you. There's a lot of really wonderful support groups out there. The Lupus Foundation of America is one, the LFA, which has a lot of different chapters locally and on the bigger levels. The Arthritis Foundation is also very important. I think they offer a lot of very good steadfast information. The biggest problem I have from patients come in is that they've Googled things. And Google can be very scary and there's a lot of misinformation out there. So looking for groups such as these foundations, the LFA, the Arthritis Foundation, that have really strong factual information that can educate a patient, because education and knowledge is really power with this disease. I think kind of taking things into your own hands, but also knowing the appropriate and the best resources is what's really important.

Cassandra Holloway:

That's wonderful advice to end on. Dr. Littlejohn, thank you so much for speaking with us today and sharing your insight.

Dr. Emily Littlejohn:

You're welcome. Thank you for having me.

Cassandra Holloway:

To learn more about managing lupus, visit clevelandclinic.org/lupus.

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