Lupus QD Clinics - lessons from the Clinic - Week 3 Save

Welcome to Lupus Cutie Clinic. I'm Jack Cush with RheumNow. Today's case is early lupus. A 29 year old Latin female comes to me after she went to the emergency room two months ago. She presented at that time with two months of pain all over with arthralgias in her arms and her legs.

In the ER, she was found to have some swollen joints, pancytopenia, was given I'm Solu Medrol and Toradol for pain, and sent home on ibuprofen. In the eight weeks prior to seeing me, she tried the ibuprofen, but couldn't take it because of nausea and vomiting. Again, two months ago she presented with morning stiffness, she had rain outs, she had two babies in the past, but no history of miscarriage. Her review assistance was positive for back pain, fatigue, myalgia, weakness, hair fall, and occasional rash on her arms and legs. She had dusky fingers, she had a number of tender joints, more than a dozen, but they found only a few that were swollen.

And labs done at that time showed an ANA positive at one to twelve eighty in a speckled pattern. ESR was 35, white count was 9.7, HNH was tenthirty two. Platelet count's normal, 194,000, but lymphocytes were low at six thirty one. So she had lymphopenia and a positive ANA. Her metabolic profile was normal, creatinine was eight, no LFTs.

When I saw her, her pain was eight out of 10. She had fatigue, she had myalgias, she was noticing hair on the pillow when she would wake up in the morning. She said she was having fevers up to 101 and occasional night sweats. She had biphasic Raynaud's and five pounds of weight loss. She had a clear cut malar rash with a sparing of the nasolabial fold, and she had swollen fingers with PIPs that were swollen.

TJC was 12, SJC was eight, MCPs, PIPs were all involved. Labs showed an ANA and a strongly positive double stranded DNA at sixty one ninety, six thousand one hundred and ninety. Now she's leukopenic with a white count of three and a lymphocyte count of 700. Platelets are down to 114,000. She has two plus proteinuria on dipstick and hematuria.

Her C3 and C4 are about half their normal value. So this is a gal who has really relatively early disease, but she is active in skin and joint, and her kidneys, and her serologies, and the question is how should she be managed? Again, I think at this point, now this would happen a few years ago when I saw her, I didn't do a kidney biopsy. The lesson from this month's campaign on Lupus Unlocked is strongly in favor of getting a renal biopsy to know exactly what you're dealing to better guide your therapy, in line with current thinking, in line with the recently released ACR guidelines on lupus nephritis. I did give her high dose steroids, sixty milligrams a day, and got her down to, five milligrams a day after about four months, slower than I would I wanted to.

She went on hydroxychloroquine. The early argument when I saw her was and this was around the time that belumumab, I think, was first coming out, was mycophenolate versus cyclophosphamide. She went on mycophenolate and, and did very, very well, but she had to go on birth control because we worry about mycophenolate. And now, if she's having problems going forward, you know, we could consider, based on biopsy, based on picture, some of the newer drugs, including anifrolumab and belimumab, and also voclosporin, and not have to think about cyclophosphamide in her. But I want to talk about early, and early is a problem in lupus.

There are significant delays in the diagnosis. You know, in some series, it's up to fifty months, you know, five years from time of symptoms to the time they get diagnosis. I would say, and others who are expert in the field would say that the referral and early diagnosis of patients is a gigantic, still unmet need, because early intervention and early diagnosis is going to establish the future trajectory of their disease. And if it's going to take five years or more, or two years or more, the patient's going to incur damage and be in trouble. There are a number of reports out there that show that when patients are diagnosed earlier, they have lower flare rates, less disease activity over time, less damage, better quality of life measures, less health care utilization, and lower overall cost of care.

It's also been shown that in things that you worry about, including cardiac complications, and whatnot. The risk factors for all of that is present in the first few years. And again, the earlier they're diagnosed, the earlier you're thinking about these things, and intervening as early as possible. So luckily, this gal, has done well and hasn't had problems with joint damage over time. Her proteinuria did go away with aggressive management.

Whether or not she needs a future biopsy, I think I would now consider it in the case of a new change in her renal status or in her overall activity of her lupus. But again, this QD clinic is really about, we need to push for earlier diagnosis. And since that means that your referral base needs to refer to you more aggressively, I think you should be marketing yourself as, with a letter to all primary care doctors, all OBs. I am expert in lupus. Send me your early lupus patients, patients who have a positive ANA.

I would be happy to evaluate them. This is how you get early lupus patients, and this is how you make a difference in their outcomes. Tune in for more Acuity Clinics.

Hi. I'm Megan Close, and welcome to Acuity Clinic. Today, I'm gonna talk about medication adherence in patients with lupus and my current strategy for some of my most challenging patients. So the patient we're gonna talk about is a 46 year old woman. She has had lupus for about five years.

She has had really significant lupus nephritis. It was class four or five on biopsy, and we already gave her a round of urolupus. And we have been prescribing her hydroxychloroquine, maximum dose mycophenolate, and monthly IV belimumab. She's been on that regimen for a couple of years now, and she keeps coming into clinic with about the same amount of proteinuria every visit, usually about three grams of proteinuria. She often has some cells in her urine sort of suggesting that she still has active lupus nephritis.

Sometimes she's coming in with pretty significant arthritis as well, occasional rashes. So it's like our medicines, we're giving her the best drugs we've got, and she's just not really getting better. And one of the things we do in our lupus clinic pretty often is check drug levels. And we do it for a couple of reasons. We do actually modify drug dosing to some extent based on the levels.

But to be honest, the main reason we do it is to really check for medication adherence. This patient has been swearing to us up and down that she keeps taking her medications, yet we can tell in our epic medical record that she actually is not picking up her medications, and her drug levels are just pretty much always undetectable. I have, like, five or six levels from her over the last year and a half of both hydroxychloroquine and mycophenolate always undetectable. So what do we do? Right?

It's a huge challenge. So, we do know she gets seblimab. So what I did is, I actually added obenetuzumab. You know, the new data that's come out on it for lupus nephritis suggests that it's quite helpful. It was tested in patients who are also taking mycophenolate and hydroxychloroquine.

And it showed really pretty nice efficacy and improved outcomes over the placebo group. So we're adding obinutuzumab, actually, on top of the hydroxychloroquine and the mycophenolate and the belimumab, just keeping all of them, because I don't think she's actually taking half of those medications. So it feels like a lot of immunosuppression. When I write it in the note, it's like a lot of immunosuppression. But actually, what's physically in the patient's body is actually not that much medicine.

So I'm really leaning into medications like obedutuzumab, rituximab. You know, rituximab does not have FDA approval for lupus because the clinical trials showed that it did not work. And I think often it's not great, but it's probably better than no drug. And I think that that's what's happening to a lot of our patients. So I just now accept that many of our patients are not taking their medications on a regular basis.

I used to blame them for it. I used to feel like they weren't doing their part. They weren't showing up. But I've really come to realize that the medication regimens that we've put together, particularly for lupus nephritis, are pretty much impossible for a young woman to do. And she has to do them for, like, years, ten years, twenty years.

Right? You know, the the new guidelines say we're supposed to be doing triple therapy, but it's really five therapies because they're supposed to be on hydroxychloroquine, an ACE inhibitor, you know, mycophenolate, and, you know, etcetera. Right? So it's a huge number of medications. And I just think it's impossible for them to do, actually.

If you compare it to HIV twenty, thirty years ago with HIV, when the new medications came out, they were exceptionally challenging to do. And people would die just because they could not do the medication regimens. That's what's happening in the lupus world is our regimens, I think, are just too complicated for people to be able to do, particularly when they make you feel bad, and when lupus nephritis, in particular, does not make you feel bad. So, I think that the drugs we have are too hard to take, and what we need are drugs that are easier to take, taken less often, fewer side effects. And I think that's gonna be the game changer in our field.

So what I did with this patient was added obenetuzumab. I kept her on the belumumab. I don't know. You tell me if that was the right thing to do. But I would love to hear what people have to say about how you monitor and how you tackle the medication adherence issue.

Thanks.

Hello, everyone. This is Bella Mehta from New York reporting for RheumNow. And I wanted to talk about managing lupus pregnancy and navigating this almost minefield. It's a tough one whenever you have a patient like this, so I thought it would be nice to talk about it. So I'm going to talk about a case.

This is a 28 year old woman who comes to me for the first time in order to discuss pregnancies. So this is a known patient with lupus lupus nephritis manifesting a few years ago, at least six years of disease, has had a rough course, ups and downs, has had a kidney biopsy which shows class three lupus nephritis, was treated with a whole bunch of things at that point steroids, immunosuppressants. And finally, right now, she came to me in anticipation of pregnancy planning on three drugs hydroxychloroquine, MMF, and prednisone. So MMF or CellCept is not something that you want patients to be on when they are pregnant. So at the first visit, when we talked and sort of discussed, she's been in remission.

The kidney numbers, the protein in the urine have all been low to none. So first thing to learn about in lupus pregnancies is that is this the right time? And the best time to plan a pregnancy is when you're in remission. So seems like a good case for that. And then we discussed about medications.

So some of the medications that we typically use in lupus or lupus nephritis cannot be continued through pregnancy. One of it that the patient had was CellCept or MMF. So we switched the MMF to azathioprine in anticipation of pregnancy. Now, a few months later, the patient does get pregnant, comes back. At that point, we do testing, specifically SSA, SSB, as well as EPS testing.

And again, so these are specific labs that you want to know either preconception or early in pregnancy because of different risks or different things that you got to monitor during pregnancy. So both SSA and SSB increase the risk of neonatal lupus or congenital heart block in babies. So, in this case, actually, we knew that the patient has SSA and SSB positive. So we knew that this is a patient which will require much more monitoring. And we ordered antiphospholipid panels for these patients, which do for this patient, which did turn out to be negative.

Of note, this patient did not have a history of a lot of miscarriages or early pregnancy losses, which, again, put APS at a higher risk. And these these are the patients that you need to monitor, maybe even start aspirin if they've had multiple early pregnancy losses. But for this patient, APS was negative, did not have early pregnancy losses. So now since the patient was pregnant, she was on hydroxychloroquine and azathioprine. Prednisone was tapered almost off.

It was like one or two milligram. At that point, I don't think it was doing much. She thought it would probably help with her joint pain, but eventually, we tapered it off through her first trimester itself. Things that we monitored throughout her pregnancy were the usual CBC, CMP, which includes creatinine, liver function tests. Disease activity monitoring throughout pregnancy is very important, especially double stranded DNA and complements.

And again, complements and pregnancy are a little tricky, but it's good to have a baseline before patients get pregnant, and then you can monitor it through. Since this patient had RO and LAB positive, both, you know, fetal echocardiograms are recommended usually around sixteen weeks to twenty six weeks to screen for congenital heart blocks. And, of course, these are high risk pregnancies, especially patients who've known lupus nephritis in the past and, you know, all of these antibodies. The patient did not have, other medical problems, but a lot of these lupus patients who are pregnant can have comorbidities. So that's also something to watch for.

And for managing these patients, a multidisciplinary team is very, very important. Ultrasounds for monitoring fetal growth every four weeks starting the second trimester is also something that is recommended. And most OBs who you'd work with first managing such cases would do that. And again, hydroxychloroquine was, of course, continued. Prednisone was eventually stopped.

Azathioprine was continued. And one of the things sort of late at around twenty eight weeks, she started having some symptoms, you know, one or two incidences of increased blood pressure. So that's when we were starting to worry about preeclampsia or eclampsia. And, you know, she did well. But around thirty five weeks or so, her blood pressure really started to go up.

And at that time, again, it was managed with cardiology, with us. But eventually the patient delivered at full term and we had to do a C section. But most patients, when we monitor these patients closely, do fine. I just wanted to bring up that when she had these little bumps in blood pressure, we were worried, is this a lupus flare or eclampsia or eclampsia? And there are key differences, and it's very, very important to be able to differentiate eclampsia versus lupus nephritis, especially in pregnancy, like late pregnancies.

Early pregnancy, usually, you know that it's lupus nephritis mostly. But late pregnancies, the picture can be confusing. So some things to note. Hypertension is much more common in eclampsia or preeclampsia than lupus nephritis, which sort of gradually goes up. Or it may not be present in some cases.

And again, preeclampsia and eclampsia usually occur after twenty weeks. And, again, both preeclampsia, eclampsia and lupus nephritis will have protein, and that's when things can get confusing. But in eclampsia, preeclampsia, the protein can be severe. And, you know, it may not have a trend like in lupus nephritis where you see gradually the protein levels going up. It will be like suddenly the patient's protein levels in the urine have started increasing.

One thing that if you can take away, how do you differentiate eclampsia and lupus nephritis is active sediment. So in nephritis, you'll see active sediment as in casts, RBCs, hematuria, cellular casts, you usually don't see in eclampsia or pre eclampsia. And, again, all the usual lupus serological factors, low complements, anti dsDNA, which is high. All of those things is what you see. And, you know, usually if you have a lupus flare, everything flares up like joints, all of that.

So there's some hints. But, you know, sometimes in late pregnancies, patients have joint pain and aches, and it's sometimes difficult to differentiate. So, you know, I know it's not done all the time by nephrologists inpatient. But you if you're suspecting lupus nephritis versus preeclampsia, you could request active urine you know, sort of a spinning of the urine, see the sediment, see look for casts, And that would be would be a key differentiator. So so I think all in all, lupus nephritis is a tough is a tough thing to navigate, especially during pregnancy, you know, because you can't give all the drugs that are out there.

You know, obviously, cyclophosphamide is tough. Giving high dose prednisone might be difficult sometimes given the blood pressure can be high. So overall, I think sort of three things that I would want you to remember for these. Counseling patients to try and plan pregnancies, which would be ideal. Once pregnant, make sure that you do appropriate testing and monitoring, Rho, la, and APS testing.

And if you have any of these positive, you know, do echoes or fetal monitoring appropriately. And lastly, how to differentiate preeclampsia and eclampsia with active lupus nephritis. And most of it, sort of the money lies in the urinary sediment as well as obviously the usual markers of lupus, which is complements, double stranded DNA. With that, I think I wanted to emphasize that in this complex scenario, it's great to have a multidisciplinary team monitoring and managing these patients with rheumatologists, nephrologists, OBs, and depending on what the patient's manifestations are. I hope this case helps you manage these patients better.

And for more of these, please follow me on Twitter at Bella underscore Mether, and follow for more content on RheumNow. Thank you.

Welcome to SLE QD Clinics. I'm doctor Sheila Reyes from The Philippines. And today's case is entitled All's Well, Stip Ends Well. Our patient is a 26 year old female who initially consulted with me via telemedicine. This was during the height of the COVID pandemic lockdowns.

She presented with a six month history of maculopapular rashes on the face and was treated initially as allergy without improvement. And three months prior to consult, she started to develop progressive periorbital and bipedal edema, massive hair fall, and joint pains. As I was already considering SLE, I immediately advised admission. On workup, she had anemia, thrombocytopenia, heavy proteinuria, hematuria, and casts. Baseline Baseline renal function was normal.

Her ANA, anti DSDNA, and anti SSA were very high, and c three was very low. Methylprednisolone pulse therapy for three days was given then shifted to prednisone. Hydroxychloroquine, calcium, and vitamin D supplementations were also started, and she was eventually discharged stable with plans for a renal biopsy as outpatient. However, a day after discharge, she developed severe headache and generalized tonic clonic seizures and had to be readmitted. She also had hypertensive episodes.

The highest SPP was around one ninety millimeters per curie. And except for the alopecia, rashes, and bipedal edema, her physical exam, including her neurologic PE, were unremarkable. Cranial MRI revealed diffuse patchy hyperintensities in the frontoparietal and basal ganglia regions consistent with cerebral vasculitis, but there was also a finding on both occipital lobes that were consistent with posterior reversible encephalopathic syndrome or PREST. In addition, she developed acute kidney injury with worsening proteinuria and decreasing urine output. Now if you were the physician handling this case, what would you do?

Pharmacologic management of lupus should be directed towards the type and severity of organ involvement, taking into consideration patient characteristics, comorbidities, and preferences. Apart from nephritis, our patient also presented with new onset neurologic manifestations. Now are these attributed to lupus or not? And if they are, distinguishing between an inflammatory or thrombotic cause is crucial because we all know management is different. Now another interesting point to take note of is the presence of PRES on the patient's MRI.

The question now is, is this associated with the patient's SLE? So what is PRES? PRES or posterior reversible encephalopathic syndrome is an acute reversible neurological syndrome that is clinically characterized by seizures, headache, visual disturbances, and hypertension with neuroimaging findings of reversible parietal occipital edema, which is also consistent with the MRI findings of the patient. And reports say in the literature that up to forty five percent of patients with PRES have an underlying autoimmune disorder, of which SLE, you guessed it, is the most commonly reported. Now treatment of PRES is directed to addressing the predisposing factor.

So for our patient, what management strategy did we adopt? Well, considering the patient's limited financial resources at this point, we had to prioritize the tests. Referral to neurology and nephrology was also done. And due to the severe organ threatening manifestations that are present in this patient despite the high dose glucocorticoids, nephritis and CNS lupus at that with this patient would press as a potential manifestation of the patient's CNS lupus on top of, CNS vasculitis, IV cyclophosphamide was given, and prednisone was shifted to dexamethasone. After a very stormy hospital stay, BP control, and temporary hemodialysis, the patient was discharged, improving.

She was able to complete her monthly cyclophosphamide infusions with the NIH protocol and is currently doing well with MMF, hydroxychloroquine, and very low dose prednisone. There has been no recurrence of CNS symptoms, and kidney function is back to normal. All's well. It ends well. So what are my takeaways from this case?

Number one, early diagnosis and prompt treatment of SLE is crucial, and multidisciplinary management and approach to and approaching it from a multidisciplinary, perspective is always a good choice. Number two, PRES can be a manifestation of CNS lupus, and high SLE disease activity and renal involvement can serve as a trigger, and maybe that's what caused PRES in this patient's case, and attributing neurologic symptoms to SLE is challenging. That's why it requires a combination of clinical and laboratory data. And thirdly, the presence of life threatening organ involvement should be considered when choosing treatment. At the end of the day, we owe it to our patients to provide them the most effective and safe medical care.

Follow me on x at RheumOrampa, and tune in to RheumNow for more updates on rheumatology. Thank you.