Treatment of Rare SARDS: Systemic Lupus Erythematous and Idiopathic Inflammatory Myopathies - Episode 2

An Overview of Systemic Lupus Erythematous

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An overview of systemic lupus erythematous including possible causes, prevalence, symptomology, diagnosis, and the goals of therapy.


W. Hayes Wilson, MD: They say that lupus is one of the great masqueraders and since you brought it up, I remember when I was interviewed for a fellowship position, one of the first questions I was asked is what causes rheumatoid arthritis? But I am not going to ask you that question, I am going to ask you what causes lupus?

Kostas N. Botsoglou, MD: An even easier answer, the short answer is we don’t know, but of course it’s multifactorial: genetic components, environmental factors, there are bacteria and viruses in our system. But whatever it is, our immune system is overreacting, and we need to bring it back under control.

W. Hayes Wilson, MD: I had a patient, actually a friend, who contacted me just the other day and said that she is having hormonal changes, and she heard a podcast that talked about how hormones trigger the body to have more autoimmune disease. In fact when you look at the prevalence of systemic lupus erythematosus, it’s much more in women and I guess hormones might be part of it. What do you estimate the prevalence of lupus is?

Kostas N. Botsoglou, MD: I estimate it’s about 700,000 patients in the United States who may be suffering from lupus, but the problem is it’s so heterogeneous, so we’re not capturing all varieties of lupus. Some of our patients who are underserved may have a more aggressive form than some of our patients who just have simple mucocutaneous or skin lesions. I am sure the number is higher than what we’re seeing in our practice, and that can be because of access as well and understanding of the disease and recognizing the disease.

W. Hayes Wilson, MD: I have heard that it’s 1 in 1000 women or 1 in 2000 women, depending on who you talk to. I guess Michelle Petri, [MD, MPH,] has said that a positive ANA [antinuclear antibody test] is found in 10% maybe 15% of women. So when a woman comes in and says, “I had the lupus test and it was positive,” I often caution them and say, “I am sure what you’re talking about is your positive ANA.” It is more common to have positive ANA and not have lupus than to have a positive ANA and have lupus. I like to point that out to them, but what other signs and symptoms do your patients typically present with, the clinical manifestation?

Kostas N. Botsoglou, MD: The clinical manifestations include: photosensitivity, oral ulcers, nasal ulcers, alopecia, hair loss. They might have chest pain with pleurisy or pericardial effusion, muscle aches, inflammatory joint symptoms mimicking rheumatoid arthritis, brain fog, fatigue, the list can go on.

W. Hayes Wilson, MD: Like we started off with, that’s what makes us important because we must take all these pieces of information and put them together, and then we also have other diagnostic work-up that we do, laboratory and such. Do you want to touch on typical laboratory work-up, testing, and imaging that we might do?

Kostas N. Botsoglou, MD: Classically, like you said we start off with that screening test, ANA, which sometimes causes more anxiety for the patient than it’s worth. But we also complete the work-up, and we check in our EMR [electronic medical record], we have an ENA [extractable nuclear antigen] panel that checks double-stranded DNA, complements, Sjögren antibodies, Smith/RNP [ribonucleoprotein]. Some of our patients are able to use the AVISE test, which can help us determine the likelihood of lupus. There are trending inflammatory markers, typically sedimentation rate and CRP [C-reactive protein]. We’re always getting baseline chemistry, liver, and kidney functions on our patients as well as a CBC [complete blood count] to monitor for any cytopenias. Then imaging, we may radiograph some of the affected joints, perhaps their hands or their feet.

W. Hayes Wilson, MD: That speaks to the multisystem nature of it, the hematologic system, the dermatologic system. We get to interact with a lot of other subspecialists, dermatologists, nephrologists, cardiologists, and sometimes imaging I guess might be echocardiograms. They like to do MRIs of the heart at our organization now. We have more than 10 times as many cardiologists as we have rheumatologists; they like to imagine every way they can of the heart. And of course, we have other comorbid conditions, you can have pulmonary involvement as well. When we look at all these organ systems, what are your goals of therapy?

Kostas N. Botsoglou, MD: Our shared goal is to preserve quality of life, minimize disease progression, prevent end-organ damage, and it’s a case-by-case scenario. How it affects their daily life, are they able to perform their daily tasks? The lab work will guide as if there is any other kidney or liver involvement. Are they spilling enough protein and need to perhaps have a biopsy and see a nephrologist? It’s multifactorial, and it can involve multiple specialties as you mentioned.

W. Hayes Wilson, MD: You summed it up very well. We want the whole person to be well. We want them to live the best life that they can. We want them to be as productive as they can be. If they are working we want them to stay at work, and if they are at home taking care of their kids, we want them to be at home taking care of their kids.

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Transcript edited for clarity.