Lupus QD Clinics - lessons from the Clinic - Week 1 Save

This is QD clinic. It's lupus QD clinic. This whole month we'll be having QD clinics and cases of interest that pertain to lupus. I'm Jack Cush with RheumNow. Today's case is the curious darkening of skin.

So it's a 30 year old Indian woman who's had lupus for about five years, and her lupus was documented and manifest as malar rash, serositis, polyarthritis, alopecia, CNS lupus, class III and IV, glomerulonephritis, hypocomplementemia, lupus profundus, leukopenia, lymphopenia, positive ANA, double stranded DNA, SM, RNP, and painless oral ulcers, overwhelmingly lupus. She's been managed with chloroquine. The doctor that she was seeing prior to me had treated her with chloroquine and CellCept, and five to ten milligrams of prednisone. And on the day that I see her, this is like second or third visit, I see her. She's doing well, but she has some GI complaints, and she is having some darkening of of the skin on her arms and her legs, somewhat about her toenails periungally.

And she wants to know what she can do about this. So I send her to the dermatologist, knowing what the problem is, and her chloroquine is discontinued because she has chloroquine induced cresiosis. Cresiosis is a skin finding where there's hyperpigmentation, usually in a bluish gray, slate gray, sometimes grayish purple looking skin, depending on whether it's on white skin or darker skin. It, can be seen, on the extremities, especially in sun exposed areas, that's like the key. It can be seen, in the cornea, it can be seen on the eyelids, it can be seen periungally.

And, corsiasis has classically been described in association with gold salt injections for arthritis. It began back in the 40s when gold salts were being used in heavy doses to treat tuberculosis, and you would get this slate blue grey skin changes, mostly on sun exposed areas when it wasn't found in the cornea, in the eye. So, the question is, what can you do about it? Again, it's an uncommon thing. It's much more common with gold than it is with antimalarials.

It's been described more so with chloroquine than hydroxychloroquine, or in this case, Adabrin, because this the dermatologist changed her to Adabrin, also called, what's it, quinacrine, and, and I don't know that I believe that that's going to be making it any better. The problem with this, it's a pigmentary change and it's permanent. It doesn't go away. And that's sort of the sad part. Sun exposed, permanent, the distribution, and there's not much you can do about it other than discontinue the drug and ask the patient to avoid sun exposure on especially the areas where they have this pigmentary change.

This patient, over the years since, has noted a fading, even though she's been on quinacrine, a replacement antimalarial, for years. And her now this therapy in her started back over ten years ago, and so she's not currently on a biologic her therapy is steroids, mycophenolate, and two thousand five hundred milligrams of mycophenolate, and her renal disease has been kept in check. So that's today's case pigmentation due to crissiasis. Tune in for more Lupus QD Clinics.

Hi, welcome to QD Clinic. My name is Eric Dyne, I'm a rheumatologist in Summit, New Jersey, and today I'm going to talk about a case called polar opposite approaches to lupus. This patient was a 37 year old female who was having fatigue and joint pain and was referred to see rheumatology. Her primary care doctor had ordered some tests, found that she had a positive ANA and other subserologies. But when I was looking at her chart a couple of days before the visit, I find, wait a minute, she's in the ICU.

37 years old, no other past medical history, never really seen a doctor before. Apparently, she was found down and unresponsive just a few days before the appointment. She had a cough leading up to this, and when her family arrived, they found her unresponsive on the ground and EMS found that she was in hypoxic respiratory failure. She arrived to the hospital, was diagnosed with multifocal pneumonia, given Solu Medrol in the hospital, recovered very rapidly, discharged on prednisone forty milligrams. So what's going on here?

She isn't on any kind of immunosuppressive medicine. Why is this seemingly previously healthy patient coming in for some abnormal serologies all of a sudden in the ICU? And how do we approach this? So I come in to see her a couple days after she left the hospital. Actually did have quite a bit of serologies that are notable, a positive ANA.

It was direct, the one that was sent prior to me. She had an SSA, SSB, Smith, RNP, double stranded DNA. She had high inflammatory markers. On her exam, she had Liveno reticularis. She had Raynaud's.

She had a pretty extensive erythematous rash on her hands with what looked like cutaneous vasculitis of her fingers, and she had a rash on her trunk. And so certainly a very active lupus picture clinically. What's going on with the lungs? Is it infectious? Certainly the most important.

She was continuing to have dyspnea, continued to have lung findings on her imaging. So we very quickly got her to get a bronchoscopy to rule out any infection. And very importantly, in lupus patients, thinking about diffuse alveolar hemorrhage, Fortunately, for both of them. So this appeared to be acute lupus pneumonitis. It's relatively uncommon.

It's listed between one to twelve percent. It presents often with this rapid onset of fever, cough, dyspnea. In about half of patients, it's their first manifestation of SLE. So she responded very well acutely for prednisone. So what's the approach that you would take with this patient?

Well, the other thing I did not mention initially is that when she comes in to see me, she's also wearing magnets that are stick to her body. So she's got this magnet approach that she feels is the reason why she has responded very quickly. Certainly, is outside of conventional medicine. My approach is you could continue to do these therapies as long as they're not going do anything that interferes, but let's also really focus on goal directed therapy. So we worked on prednisone.

Prednisone's certainly key in the short term for her. We know that eighty percent of organ damage in fifteen years is attributable to prednisone, so very important to get her on other therapy. My plan ideally would be to start both hydroxychloroquine and probably a very early intervention of a biologic type medicine like belimumab. But for her, my plan is to her is to go slow, slowly come down on the prednisone. I think of it like tortoise in the hare, not to rush it and end up back where we are in someone who compliance is a concern, starting with the hydroxychloroquine, trying to get buy in for her and discussing it with her family members who are more inclined for the medical intervention, being honest upfront, telling that it is a lifelong diagnosis, she will need long term treatment, and likely will need multiple, but taking it slowly.

So building rapport, not accomplishing everything all in the first visit, seeing this patient often, and checking in with her often to continue the conversation. So the interesting things about this patient, who I'm still very much getting to know right now, is this acute, pretty dramatic presentation of lupus pneumonitis. And then also not always taking the initial approach that you would want, but taking things time by time, having this relationship building with the patient and getting their buy in to find the treatment that works for both the patient and the physician to control their disease. Tune in to RheumNow for lots more coverage for QD clinic and more for lupus month with the RheumNow faculty.

Welcome to lupus QD clinics. I'm Jack Cush with RheumNow. Today's case, refractory renal disease, what would you do? This is a 25 year old African American female who I've been following with class three and four lupus nephritis, biopsy proven, with, moderate activity and minimal chronicity. The lupus diagnosis is based on clear criteria, and in addition to her nephritis, she's had polyarthritis, lupus skin rashes, I don't remember malar rash, and ANA double stranded DNA, SSA, SSB, positive CCP, leukopenia, very low complements, a positive anti cardiolipin G, negative lupus anticoagulant, but did have a positive beta-two glycoprotein antibody.

And her nephritis and lupus has been treated with CellCept. Did well, but stopped when she got a CMV infection. Then methotrexate couldn't tolerate the GI side effects. She was on hydroxychloroquine at the time, and then went on to, oral, and then IV cyclophosphamide, developed zoster and neutropenia and, didn't seem to respond. Azathioprine was tried, but that caused further, problems with her leukopenia and was inefficacious.

She was given hydroxychloroquine, but told she had eye issues and had to stop it. Oh my goodness. This girl's got, you know, problematic, active, glomerulonephritis. What are we gonna do to manage this? So, her current medicines are she's on lisinopril, she's on prednisone, five milligrams a day.

She was given, two courses of Rituxan, and then when that was no longer being paid for by the insurance, she was switched to belimumab and has since done well on belimumab. But she's only on prednisone and belimumab, and by today's standards, you know, she needs to be on more. You know, the recent ACR treatment guidelines say everybody's on a background hydroxychloroquine, and then you get triple therapy, which starts with steroids in high doses. We've already done that with her, she's now on a stable dose steroids, and then their triple therapy is going to be the steroid plus mycophenolate plus something else, usually belimumab or calcineurin inhibitor. She's just on the prednisone, can't do the mycophenolate, can't do the hydroxychloroquine.

If you look at the menu that is prescribed by the experts that came up with the guidelines, It's basically hydroxychloroquine and mycophenolate. She can't take either of those. I need a replacement for either of those. And she is on Benlysta or belimumab, and that's good. But I think this patient actually needs to go on, an anti proliferative drug, and so your choices there are very few.

Right? She could go on leflunomide, but there's very little data on leflunomide in patients with lupus nephritis. So, I think this patient should be on a calcineurin inhibitor. And yes, a calcineurin inhibitor plus a B cell depleter, I think, would be ideal. It's not that combination isn't recommended, but this patient has exhausted the recommendations of the guidelines.

And it just makes sense, does it not? It's an oral, calcium neuroinhibitor that has its immunosuppressive effects, and then a B cell depleter along with low dose steroids, I think she's going to be fine. Now, could find another antimalarial to give her, but, she was very worried about that and her eye problems. So she had blurry vision. She stopped the hydroxychloroquine.

She still has blurry vision, so anyway, maybe I can talk her into adding another antimalarial in the future. But right now, we're going to add in a calcineurin inhibitor. So what are the choices? Historically, it was cyclosporine tacrolimus, and now, the approval a few years ago of voclosporin. Voclosporin's the hands down better as far a drug as far as safety and tolerability, and even the data.

I mean, there's it's actually got an FDA indication. It's one of very few drugs that are approved for patients, with lupus nephritis. So, we're gonna add that to her current regimen. Why? Because she still has active refractory renal disease.

She has this problematic leukopenia. Oh, by the way, she and her white count's 1.7, she has 700 lymphocytes. She has had a, bone marrow biopsy showing she's making plenty of neutrophils, so she's probably gobbling them up, although she doesn't have splenomegaly by clinical exam. I haven't done imaging for that, but I wouldn't be surprised. I think she has peripheral destruction of her white count of her white blood cells by her lupus.

She has no swollen joints, but four tender joints. Double stranded DNA is still very high, 152, very low c three and c four, still has, low levels of proteinuria. We need to be more aggressive in her. The other thing I want to do about her is actually make sure that she's going to be co managed by a nephrologist. She has some mild renal insufficiency creatinine 1.3.

I worry about patients who have nephritis, and that their CKD and renal disease is being optimally managed, and I'm going to ask the nephrologist to see her again. She's already on lisinopril, but he's probably going to optimize therapy in other ways that I would not have thought of. This is my approach to refractory renal disease. Tune in for more Lupus QD clinics this month.