Lupus QD Clinics - lessons from the Clinic - Week 2 Save

This is QD clinic. Hi. I'm Jack Cush, RheumNow. QD clinic this month is devoted to lupus. Our lupus campaign is lupus unlocked, and it's sponsored by Aurinia.

Thanks. Aurinia. Today's case is SLE get ready. A 24 year old presents to me this was a few years ago, and I'm gonna reconstruct the case. But she presented with a diagnosis of lupus.

And at the time, she was just on hydroxychloroquine, azathioprine, and prednisone. And her declaration to me was that she wanted to get pregnant. And in fact, she was about to get married. She worked at the same institution I did. She was a scientist.

And, you know, that was our plan. Her husband wanted to have children, and she knew she had lupus, but she knew there was some concern there, and that's why she was coming to see me. So when I saw her, I documented that she had lupus, and her lupus was clear. She was, she had a lupus skin rash. She had polyarthritis, anemia.

She had sclerodactyly. She had a history of serositis and bruising and purpura and petechiae. Lab wise, she was ANA positive, DNA positive, SM and RNP positive with leukopenia, anemia, hypocomplementemia, and proteinuria in the last year. My words to her were, no. You can't get pregnant, which was quite traumatic for the patient.

So after I backtracked and explained to her, now is not the time to get pregnant, yes, you can get pregnant in the future, but you got to be ready for it. And this was a really hard pill to swallow, especially since it was the first visit. And the only thing she knew was about me was that you need to go see him, and he'll tell you how to better manage your lupus. And it was a slow process. And I told her the first thing we had to do well, first off, you can't get pregnant when you have this much activity, including at the time she had proteinuria.

She had active serologies. She had an anemia of chronic disease at some point, and and I told her we had so many things we had to work through, including a better regimen than what she was on, which is azathioprine prednisone hydroxychloroquine. Kept her on a low dose prednisone and hydroxychloroquine, changed the the azathioprine. Initially, the discussion was methotrexate or mycophenolate. And she initially went on methotrexate, mainly because of really bad polyarthritis.

And she had sclerodactyly, and I and I thought she was an overlap at that point. But the polyarthritis was severe. I think her initial joint exam, I believe she had, you know, like, 12 tender and no. It was it was, yeah, 12 tender and six swollen joints, PIPs, MCPs, elbows with contractures. So she understood, ultimately, and we made these changes first to methotrexate, prednisone, hydroxychloroquine.

Then after six months, we switched her over to mycophenolate for other multisystem disorder management, but her arthritis got worse. And then, ultimately, she went on Humira or adalimumab in addition to hydroxychloroquine prednisone and mycophenolate. And and, again, the patient was told right from the start, you cannot get pregnant on methotrexate. Cannot get pregnant on mycophenolate. Mycophenolate is a one clear cut teratogen in rheumatoid arthritis.

Way, way, way, way, way more than methotrexate or loflinamide. It is the no go teratogen, more so even than cyclophosphamide. So and I impressed that upon her at the first visit and every visit. So the first visit, she had all these changes. The first visit, she went on birth control, and she, you know, she had an implant put in, and that worked well.

And, anyway, it took about four years before we got to the point that she could go off the mycophenolate. She could be on hydroxychloroquine, very low dose prednisone, and adalimumab and conceive. And in fact, she did conceive. And in the years since this happened, she's gone on to have two children. Now she's had very difficult lupus, hard to manage.

But once you get to the point that, you know, the patient is stable, and there is little signs of active disease now she's got, you know, deforming arthritis at this point, and sclerodacty never progressed, but we had to, you know, get her under control. So, again, the big move in her was, treat the polyarthritis first with methotrexate, second with TNF inhibitors, and she conceded on TNF. Because she was on prednisone, she went on calcium and vitamin D, and she got a baseline, DEXA to assess where that was going to go in the future because it looked like she was gonna be on long term low dose prednisone. Early on, the big issue in her was weight loss. She had when I first saw her, she had lost 12 pounds in twelve months, and she had an anemia of 10 over 30, with sort of normal indices on the low end.

The question was, was this GI bleeding? Did she have gastritis? Was this anemia chronic disease? We did a workup with, h pylori and GI, a Coombs test, haptoglobin LDH. She had no hemolysis.

She just had the anemia chronic disease. We'd never proved a GI bleed, but with better disease control, her HNH ultimately came up to, like, around twelve five I'm sorry, eleven five over, like, 35, and that's where she's been for the many years I've been managing her. So, again, the hard thing in this lesson is all about, you have to prepare your patients when they want to get pregnant. And it has to be a slow process, where they have to understand that mom has to be ultimately healthy to make a healthy baby. Tune in for more QD Clinics.

This is QD Clinic, lupus QD Clinic. I'm Jack Cush with RheumNow. Today's case, Rhopus. That's right, r h u p u s. Have you seen these patients?

I have. Here's one such case I saw recently. She's a 40 year old who has been diagnosed with RUPES, meaning she has lupus and seropositive RA, also has a history of fibromyalgia. I think the RUPES was diagnosed, oh, almost fifteen, twelve years ago, something along that line. Why do I say that she has that?

Well, she clearly has a history of lupus manifest as polyarthritis, anemia, leukopenia, lymphopenia, ANA, RNP, photosensitivity, malar rash, alopecia, Raynaud's, dysphagia, dry eyes. She's had two miscarriages, but no tests for antiphospholipid antibody. For RA, she's had symmetric polyarthritis with deformity, morning stiffness, very high CCP greater than two fifty, RF of positive 58, and in the past she's been treated with prednisone, methotrexate, hydroxychloroquine, leflinamide, rituximab, Atabrine, etanercept, and most recently, tofacitinib. So she comes to me at a last visit taking hydroxychloroquine, tofacitinib, trazodone, and Lyrica, and yes she has secondary fibromyalgia. At this point she's done very well.

This regimen she's been on for a while, it's working in her. She has no swollen, no tender joints, but she clearly does have evidence of deformity with swan neck deformities that are static and a prayer sign in the hands with contractors of the PIPs and DIPs. She does have a malar rash today, but no other signs of cutaneous lupus. Her Rupus is stable. Now what's the deal with Rupus?

Can you have both? Well, certainly you can because it's just yet another example of the wide, wacky, screwy world of rheumatology, where it's not a one it's not enough to have one autoimmune disease. You can get two, and we call that overlap syndromes, and I always describe them rather than cutesy names like Rupus. Usually, I say or UCTD, MCTD, I say overlap with an overlap of blank and blank. Myositis and scleroderma, or rheumatoid and inflammatory myositis.

But I'm okay with the term RUPES. I think it still is something that's not yet dead and buried. Some interesting facts on RUPES. How many lupus patients will get lupus? No one knows, but it's certainly no higher than ten percent.

And in some studies it's like three to six percent. Hard to know. How many patients with lupus have positive serologies for rheumatoid? Well, it's up to twenty percent will have a rheumatoid factor, and up to nine percent will have ACPA or one of the other ones like CAR P antibodies or fourteen-three-three ADA. Forty percent of RA patients will be ANA positive.

When these two collide, you know, that's how you get Reese's peanut butter cups. You know that commercial where chocolate and peanut butter collide? Never mind. It's kind of what happens in Rupus. Maybe there's a confusion here when patients have rheumatoid arthritis, they get a TNF inhibitor, they get ANA positivity, which is very common as a result of TNF inhibitors, but uncommon is drug induced lupus from a TNF inhibitor, which is zero point two to zero point four percent of all people on TNF.

But that's a lot of people that will get drug induced lupus. There you stop the TNF inhibitor, it'll go away. Rupus is something that's permanent, and again, exemplified by being double stranded DNA or SM positive along with ACPA or RF positive, the arthritis tends to dominate the picture and they don't have as much organ involvement like heart, lung, kidney. Arthritis they have a lot of. It's inflammatory, it's symmetric, it's polyarticular, it can lead to deformities, erosions, and disability.

But not all of them do, but clearly, you know, the subset of lupus patients that have severe RA like arthritis, you should wonder whether this is a RUPES kind of picture. There is a lack of consensus on what the proper definition is. My definition is, someone who meets criteria for lupus who has asymmetric polyarthritis and seropositivity for, rheumatoid disease. The treatments, are going to be dominated by, as is the case with all overlap disease, by the dominant disorder. And if arthritis is the dominant disorder, for me the treatments are hydroxychloroquine, methotrexate, AT and F inhibitors, but you can use calcineurin inhibitors, and you can use belimumab.

Belimumab seems to work well at arthritis, as does anifrolumab. Works well at arthritis and skin disease. And the take home here is RUPES patients tend not to have as much serious organ involvement. The arthritis can be polyarthritis, it looks like RRA, or can be Jacuzzi like, reducible deformities. And of the biologics that are out there that you could use, I think belimumab, rituximab, I don't like rituximab for lupus, but I do for this subset.

And some people have advocated for abatassa. I find this case interesting. I hope you do too, more lupus QD clinics to follow.

Welcome to QD clinics. I'm Doctor. Megan Close, a professor of medicine and rheumatology at Duke University. I'm going to talk about pregnancy planning with some of our more complicated lupus patients that can be complicated both for medical reasons and because their ability to take medicines really well can be challenging. So this is a young woman I have been taking care of for a while.

She is 29 years old. She had a pregnancy actually and delivered in the summer of twenty twenty two at thirty three weeks with some preeclampsia. That pregnancy had been unplanned and she actually was taking mycophenolate when she conceived. She stopped it really promptly and didn't have problems with the mycophenolate. The pregnancy went along okay until the end when she developed significant proteinuria.

It looked like preeclampsia. It actually persisted for some time after delivery such that we actually thought that she also probably had a component of lupus nephritis because she continued to have proteinuria for multiple months after delivery. So we put her onto azathioprine to control this. She was breastfeeding. It seemed to actually get better pretty fast within a few months.

And she was breastfeeding and she kind of disappeared for a little while and showed back up to the hospital about six months later with a platelet count of six. Whoops. So she'd had ITP before as part of her lupus, and it seemed she had not really been taking any of her lupus meds, and here she was with an exceptionally low platelet count. So the hematologists sort of took charge in treatment and started her on mycophenolate. She came then to see me, and I know her pretty well.

What I learned over pregnancy and before and after is that she is not great about taking medications by mouth. She's also really not great and doesn't like birth control. So I had a pretty frank conversation with her about mycophenolate and the risks of it from a pregnancy standpoint. She told me when I asked her that she wanted to get pregnant again. She had a baby who was about a year, year and a half old, and she wanted to have another one and that she really was not gonna use birth control.

And to be honest, she hadn't really been taking the mycophenolate recently because she doesn't like taking medicines by mouth. So the hematologist and I kind of went back and forth on what to do, and knowing her and knowing patients, when a woman tells me that she wants to get pregnant, I just have come to believe her, that's what she wants. And me telling her not to get pregnant almost never works. Me trying to convince her that mycophenolate really is the best drug for her right now, even though it might cause birth defects and she couldn't stay on it in pregnancy, in my experience does not work. And what we end up with is a woman who either is taking mycophenolate and falls pregnant or a woman who just doesn't take the mycophenolate because she actually wants to get pregnant and she knows that there's a birth defect risk and therefore her lupus just goes untreated.

So what I actually did is I put her on rituximab, and that actually worked great, fortunately, because rituximab often works great for ITP. So since then, we have just continued her on every six month rituximab. Her lupus nephritis has been very well controlled after that azathioprine. Her platelet counts have been stable, and she's now just coming to see me periodically while she's trying to get pregnant. I'll be honest, given her history every time, I'm a little nervous, but I'm doing all the stuff that she will allow me to do, which is have her on a medication that I'm okay with if she were to fall pregnant and a medicine that keeps her platelets well controlled.

Is it maybe the optimal plan? I don't know. She just refuses to take hydroxychloroquine. I would much rather her take that medication, but it's what she will do. And I'm in the case of taking care of people who just will do what they'll do and making the best out of it.

So my sort of learning point on this today, number one, it's important to talk to your patients about whether they want to get pregnant. You just don't know whether or not they want to get pregnant. And if they tell you they do want to get pregnant at some point in the next year or two, believe them. Don't think that you can talk them out of that because you probably can't. The women are making the decisions much more based on their social situation and their family than they are on their lupus.

If they are interested in getting pregnant, just don't put them on a teratogen unless you know they're going to have an IUD or something like that that's exceptionally effective because either she will fall pregnant on it or she just simply won't take it and will continue with active disease. So that's my learning point today. Ask your patients, listen to your patients, believe your patients when they tell you that they wanna get pregnant, and then just work the best lupus meds you can right around them. Thanks so much. I hope you learned something today.

Hi, I'm Jack Cush, and this is QD Clinic, lupus QD Clinic. Today's case is about young patients who have SLE. What made me think of this was this case of a 20 year old African American female who I saw who was diagnosed about seven years ago. At that time, she presented with a lot of systemic features, arthritis, abnormal LFTs, but ultimately was found to have a positive ANA, positive SM, positive RNP, inflammatory polyarthritis, evidence of proteinuria and nephritis. Although she was never biopsied, it was presumed she had nephritis.

She had acute malar rash and other acute erythematous rashes, leukopenia, thrombocytopenia, and Raynaud's. So, over the years, she has, was initially diagnosed at Children's Hospital, and as they get older and bigger, she gets referred to me, the adult rheumatologist. And I've been managing her for a few years, and she's a great patient. Her disease has been complemented intermittently by abnormal LFTs, followed with hepatologists, hypertension, about or two of leukocytoplasmic vasculitis, and nosebleeds. She has been managed over the years with a number of different medicines, hydroxychloroquine, steroids, and then, a multitude of other things.

Her current medicine, when I saw her, she was on mycophenolate, hydroxychloroquine, lisinopril, and not on steroids. But I bring this case up because I think I want to, bring up the challenge of young people. It's a hard diagnosis for them to to, understand and manage and cope with. There's a lot of resentment and animosity and denial. You know, they'll do some research, and sometimes they'll do no research because they don't wanna know anymore because they don't wanna believe that they have it.

This manifests with noncompliance with visits and noncompliance with medicine. Coping, is a really difficult issue, and my issues are how what are the steps you need to take to manage a young person who's starting out with a diagnosis? How do you get them to become a better patient? You know, and basically you have to build a better patient with all your patients, and that's through slow work, concern, and education. So in her, the initial step was steroids, You know?

And steroids went from made her feel good, but you give steroids to a teenager, they're gonna be pretty, pissed off when they come back in two, three months, and they're eight to twelve pounds heavier, and they've got acne, and they notice all kinds of steroid side effects, including that they're not sleeping and maybe that's giving them more pain and ache and fatigue. So, you gotta deal with steroids in them and set expectations and try to you know, I don't put anybody on steroids without, you know, number one, it having an expiration date on when I'm gonna stop it. And two, with that, I first scare everyone with all the side effects of steroids so that they're motivated to get off of them. So, the next step in her was to understand let her understand the importance of co management between me and her, and I need another person. You cannot manage a young person, a teenager or a 20 year old, by themselves, because that's just a bad formula.

You need another very very close person. Usually it's going to be a parent, but it might be their sister. It might be their best friend. It might be their husband or live in boyfriend or girlfriend. But it's got to be someone who you turn to at every visit after you see tell the patient, I'll see you back in three months.

And then you turn to the other person, and you point at them and say, I'll see you back in three months. I need you to be here as well. I need you to be involved in this care. She needs you. I need you.

Let's make it a team. And I think co management by the family is really important. You look for signs that things are not going well. That would include non compliance with visits, non compliance with medicines. You do compliance with medicines by putting them on hydroxychloroquine and then checking hydroxychloroquine levels, you know, aiming for a thousand to fifteen hundred as your your target dose.

People have asked I heard people ask Michelle Petrie recently, do I wanna do trough levels or peak levels? What's the deal? And Michelle says, it doesn't matter. It behaves like an a one c. As long as you don't get the blood drawn, like, twenty minutes after they took their their dose, you're going to be fine with whatever the value comes back as being representative of them taking the drug alone.

The next so you want to find signs, and you can do it through of noncompliance by checking blood levels. You also wanna see what are they doing. Are they going to school? Are they working? Do they have a career?

Is their life stable both at work, avocationally, relationships, family? Who are they living with? What's going on there? When you get the picture that they're floundering, and they have no relationships and they're not coming with someone else, I worry about that. You ask about their habits.

Do they smoke? Do they drink? Are they sexually active? Because they'll tend to tell you the truth on these things. And, are they sexually active?

And again, you have to assume everyone in this age group is sexually active and push hard to get everyone either on an IUD or a progestin implant. That way you don't have to, worry about compliance and worry about the potentially additive harmful effects of an estrogen dominant, oral contraceptive. So, again, you know, showing concern at each visit is going to be really important to get that patient to self reliance. And then if the patient is a pediatric patient, you know, the transition from pediatrics to adult rheumatology is going to be a rough one. It's got to be a very close, if not person to person handoff between the doctor they trust, the pediatrician, pediatric rheumatologist, to the doctor who they're going to be asked to trust, which could be the adult.

Again, I have a greater degree of concern and involvement in young people with lupus. I think you should too. Tune in for more Acuity Clinics.