Scott Webb (Host): Lupus is a chronic autoimmune disease that generally affects women more than men, and causes inflammation and damage that can affect multiple organs. And today I am speaking with rheumatologist Dr. Mehwish Khan about lupus, systemic lupus, the manifestations and treatment options.

Dr. Kahn, it's nice to have you here today. We're going to talk about lupus and specifically systemic lupus, SLE. So, as we approach this, let's talk about the diagnosis or diagnostic criteria for SLE and lupus.

Mehwish Khan, MD: So, first of all, you know, SLE is a systemic autoimmune inflammatory disorder, which affects women more than others, but of course it can affect anybody. So in terms of diagnostic criteria, we follow the American College of Rheumatology and ULR guidelines. There were three diagnostic criteria that are made so far.

The most recent ones are from 2019. There are very lengthy. So we don't have to go to details, uh, but it's the point system. The most important thing is that you have to have positive ANA to enter into the diagnostic criteria. So you have to have positive ANA of titer of at least one is to 80 or higher.

Then we have clinical domains and we have immunological or lab domains. Among the clinical domains, again, there, there could be many, for example, hematological, that is blood related, constitutional, neuropsychiatric. And I'll just give you examples. Like say in the hematological system, you can have low white count or leukopenia, or you can have low platelets. So thrombocytopenia. Similarly in kidneys, you can have lupus nephritis proven by a kidney biopsy. So everything is given point and you have to choose only one from say for example, it's hematological. You're going to choose only leukopenia, not the thrombocytopenia. Like you don't combine them. So in that way, these clinical criteria, you give the point system and then among the immunological or the lab, you have double stranded DNA antibody, Anti-Smith antibody, which are given maximum points six, six. But we have other antibodies too, like compliments or antiphospholipid antibodies. So together, depending upon the person, when you calculate the score, it has to be 10 or above and then at least one clinical criteria. So it's kind of a little bit complicated, but, just to be like in simple words, have the positive ANA and then if your patients have clinical features, American College of Rheumatology website has all these diagnostic criteria. You, take that criteria, you give the point score, and then you make the diagnosis at the end.

Host: I see. So then let's talk about the major clinical manifestations, you know, before folks are diagnosed or before you apply the diagnostic criteria. How does it manifest?

Mehwish Khan, MD: So it's very heterogeneous disorder. Virtually it can affect any organ in the body from head to toe to be honest. But the three most clinical organs and systems, that could be skin, that could be kidneys, musculoskeletal or joints, and of course nervous system, heart, any organ can be affected.

Again, everybody's different and even the organ manifestations could be different. And the skin photosensitivity is a hallmark of the lupus. People can present in the form of acute rash, which is malar rash, which is very popular. And everybody, you know, most of the people think they have that rash and they come to us worried about that rash, subacute lupus.

And then you have chronic lupus, example being discoid, lupus, and the kidneys, again, in the kidney, most of the times you have to do kidney biopsy depending upon the indication. And an indication could be like if there's rising creatinine or if say, a person has lot of protein, massive proteinuria, and the kidney biopsy is again, divided into multiple stages, stage 1, 2, 3, 4. And that's important because that determines the treatment. In the joints, it can present in the form of inflammatory arthritis or just like rheumatoid arthritis or you know, like inflammation in the joints. It can present in the form of inflammation around heart, which is like, you know, the pericardium, that membrane, that surrounds the heart, pericarditis.

It can present in the form of inflammation around the lungs called pleuritis. It can present in the form of interstitial lung disease or even ofa serious manifestation of alveolar hemorrhage where people bleed in their lungs. So there's a long list and everybody, presents differently.

Host: Right. Yeah. A long list for sure. So let's, as best we can, doctor in podcast form here, let's talk about some of the updated treatment guidelines.

Mehwish Khan, MD: Right. So, just like I said that the clinical manifestations could be different, the treatment guidelines also depends on the organ manifestation, upon the clinical manifestation. But, I would like to say the most important drug for the lupus is hydroxychloroquine or the Plaquenil. All lupus patients, they need to be on hydroxychloroquine unless there's contraindication or they cannot tolerate.

And this medication prevents the complication and it helps probably in the ways we even don't understand, then, you can have different, like say if there is mild rash, you can use topical steroid, but if the organ manifestation is kind of serious or, very impressive, you can use steroids, oral steroids, and again, the steroid dosing can also depend upon the clinical manifestation. For example, if your organ is threatened like kidney nephritis, lupus, nephritis, and you have stage three and four, you will, use very high doses of steroids, maybe pulse dose, which is injectable for three days, followed by high dose with taper. And then if you have just the joints involvement, you can just do low dose of prednisone while you are using other immunosuppressive therapy. Right. So it depends upon the clinical manifestation. But the other medication, that are specific for the lupus could be Benlysta or belimumab, which is available in the form of injections and infusions. And it's very good for the skin.

It's used for lupus nephritis or kidney lupus. It's also used for the joints and you generally it is combined with the steroids. Steroids are, you know, tapered down, stopped, and then you use other medication. While we are talking about lupus nephritis, the next generation, the very, very latest, one of the guidelines suggest you use voclosporin, which is calcium urine inhibitor.

That is excellent for lupus nephritis. We also use other medication like methotrexate, azathioprine depends on the organ manifestation. We have immunosuppressive therapy like mycophenolate, which is also called CellCept which is used for skin, it's used for inflammation in the kidney. We have a very latest 2021 FD approved medication called anifrolumab, or the Saphnelo, which is interferon receptor antibody, and it's very good for the skin.

So just like the organ manifestation clinical expressions, these medications are used, in you know, variable fashion in different people.

Host: Right. Yeah. It sounds like you've got a lot of tools in the toolbox, so to speak. So, doctor, let's finish. Yeah. Let's, let's finish up here today and talk about drug induced lupus. I don't know much about that, but I wanted to ask an expert.

Mehwish Khan, MD: Yeah, drug induced lupus is interesting because again, it's a very long and exhausting list of the drugs that can cause lupus. The interesting, one would be the minocycline, which is commonly used amongst our youngsters. Your. You know, for the acne and later on they can develop lupus like symptoms.

It's an antibiotic. Other medication could be hydrolyzine. I'm just giving you the common medication names, hydrolyzine. It's used for blood pressure, but it can cause lupus. We have procainamide, which is used for arrhythmias and it can cause lupus. Interestingly, we have some medication that we use ourselves like say, for rheumatoid arthritis and psoriatic arthritis, like Humira and Enbrel these are injectable biologics.

They can cause lupus. But the most important thing, would be that, all patients with drug induced lupus, they will have ANA positive. The expression could be constitutional, fever, rash, some joint pain, or sometimes, serious manifestations like inflammation around the heart or pericarditis or pleuritis. But the one thing that we all need to do is stop the drug, stop the stimulus that is causing the lupus, and most of the times that's sufficient. But if it is not, then you can use prednisone or some other tools to treat that.

Host: Sure. Well, I certainly appreciate your time and your expertise today. Great to learn about the updated treatment guidelines. Good stuff today. Thank you so much.

Mehwish Khan, MD: Thank you so much. I appreciate it.

Host: For more information and to get connected with one of our providers, please visit carle.org. And that wraps up this episode of Expert Insights with the Carle Foundation Hospital. I'm Scott Webb. Stay well.