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This is QT clinic. Hi. I'm doctor Jack Cush, executive editor with RheumNow. QT clinic is brought to you by RheumNow live twenty twenty five, February eighth and ninth in Dallas, Texas. You know, my voice is strange today.

I have a little scratchy thing going on. I think I'm talking too much. We got a great session at RheumNow live on Saturday the eighth on JAK inhibitors. Let me just read this to you. I mean, John Giles talking about what we learned from oral surveillance three years later.

Maddie Feldman's talking about when JAK inhibitors go generic. TYK2 inhibitors from Joe Marola, a RheumDerm person at UT Southwestern. And then Christina Charles Schulman is going to talk about JAK inhibitors and dermatomyositis. That's an exciting session. Today's case is a patient who I scratch my head about frequently.

She's 30. She has the diagnosis of ANA negative lupus. Yeah. I know. It's in my chart.

And Sjogren's syndrome and fibromyalgia. And when I see her, she's pregnant, and she's in for follow-up and having some complaints. ANA negative lupus, you say. Yeah, the diagnosis was made prior to seeing me when she presented with arthralgias, arthritis, serositis, an ANA that was weekly positive once, and then repeatedly negative two or three times. But her other serologies showed a positive SM, RNP, SCL70, double stranded DNA, SSB, lymphopenia.

She had nasal ulcers, photosensitivity, Raynaud's, and Sika symptoms. When I've been seeing her, she's repeatedly ANA, SSA, rheumatoid factor, APL antibodies, lupus anticoagulant, RPR negative. Double stranded DNA was also negative since I've seen her. So what's going on? Well, right now, she's doing fair.

She has some joint pain. Her pain is an eight out of 10. Her sleep is only fair. She complains of stiffness, has sixty minutes of morning's, morning stiffness. She also has some fatigue, dry eyes, dry mouth, recent URI.

When I examine her, she has no rash. She has no oral ulcers, her chest and lungs are clear, she has atender joints, no swollen joints, a C. D. I. Score of around 20.

When you look at her labs, she has no cytopenias, platelet count, white count, H and H are all normal, and her CRP is normal. And she's eight months what did I say? Five months pregnant, and having pains. So I'm gonna focus on her sleep, do what I can, have her take analgesic medicines, which she's not doing right now. She's on plaquenil and folic acid, takes Neurontin at gabapentin at bedtime, and she doesn't take any pain medicine.

So I can understand why she wouldn't want to with a pregnancy and all, but she can take Tylenol. And at this point, since she's only five months or twenty weeks or so, she can take nonsteroidals, but she's probably not gonna wanna do that. So here are the considerations here. One, ANA negative lupus. I teach it doesn't exist.

It's a figment of investigations prior to 1982 when the cell substrates for ANAs would allow for, a negative ANA because you were missing Rho or La SSA, SSB positivity. The newer tests should not that should not happen. And, yes, it does occur. We've seen people at the university who would be ANA negative but still be positive for SM, RNP, and double stranded DNA. Are they worse or the same or better?

In general, if you're ANA negative and you got lupus, you're gonna not gonna have a very consequential lupus course. And if you do, you're incredibly, incredibly rare. If they're ANA negative repeatedly, you should be looking to other diagnoses. So what's the deal with this patient? This patient has multiple autoantibodies.

And in those people, I want to, for one, look at whether they have liver disease, hepatitis C, hepatitis B amongst them. Whether they have other autoimmune disorders, especially Sjogren's syndrome, but you have to consider myositis or scleroderma, or an antiphospholipid syndrome. And again, this patient does have Sjogren's, and probably does account for a lot of the autoantibody positivity. But the other thing is, are you treating lupus, or are you treating fibromyalgia? We just recently reported, a recent report, that showed that, what was it, fifteen percent or so?

Certainly less than twenty percent of lupus patients will also have coexistent fibromyalgia. And that needs to be managed, because you're treating lupus, when you should be treating fibromyalgia, guess what? They'll not do very well. If you're not treating fibromyalgia, their lupus treatment's not gonna do very well either. Anyway, these are cases that we do see.

Hopefully, we'll see you at RheumNow Live. Maybe the best part is gonna be the reception and gong show karaoke on Saturday night. That's right. I'm gonna sing. Others are gonna sing.

We're gonna score. There's gonna be prizes. It's gonna be fun because education and meetings like this should be fun. We'll see you in Dallas on February 2025. Welcome to QT clinic.

Hi. I'm Jack Cush with RheumNow. QT clinic is brought to you by RheumNow Live twenty twenty five. We'll see you in Dallas February eighth and ninth, an exciting meeting for sure. Today's case is called autoinflammatory or not.

This is a 27 year old gentleman I've been following for a few years. He was given a diagnosis, by me of autoinflammatory syndrome. He presented with urticaria and daily fevers for a few months. When he was hospitalized and worked up, he really was quite sick. He had generalized lymphadenopathy, two to three fold elevations initially of his LFTs, 40 pounds of weight loss, anemia, hypoalbuminemia, a high ferritin of 14,000, an aldolase of 18, normal CPK, CRP was a 113 per, milligrams per liter.

He had signs of heart failure, pericarditis. His ANA was positive, SCL 70 positive. His TSH was elevated at nine. And again, because of the daily fevers, the urticarial rashes, the incredibly high acute phase reactants, including ferritin, serositis, arthralgia's, he was called Still's disease and referred to me. In my evaluation of him, he does not meet my criteria for Still's disease, which is a major minor criteria, and he has nine points out of 10.

Look it up if you want to know. So I was unwilling to call it Still's disease, and left open the opportunity for him to be called an autoinflammatory syndrome. So why would I make the distinction? Well, I want to get locked into a diagnosis, and then locked into therapies and thinking. And I think that that can be a bit of a problem for both patients and those who manage those patients.

Secondly, he has some atypical features, right? He has some autoantibodies going on. He has urticaria. Now, urticaria and pruritus is seen in up to thirty percent of patients with Still's disease, and it's actually a major feature of a lot of the other autoinflammatory syndromes, some of which are monogenic and inherited and seen in kids, some of which are acquired and seen in adults. So what autoinflammatory periodic fever syndromes are associated with urticaria.

Number one comes to mind is Mucklewell syndrome, usually seen in kids, but can be seen in adults, including adults with an onset in their teens or in their twenties. The cryopyrin associated periodic syndromes, or CAPS, that's, you know, FCAS, no mid synca, and Muckle Wells is part of that same triad. Still's disease, both in kids and adults, has urticaria often as a prominent feature. And Schnitzler syndrome, another adult, acquired, periodic fever syndrome associated with usually a monoclonal gammopathy. So this gentleman had a normal SPEP.

He did not have a childhood onset. He had an adult onset around age 24, and he's now 27. So I have been treating him with steroids and anakinra. Anakinra worked well initially, hundred milligrams once a day given at night. I give it at night because fever occurs at night in most of these patients, and the half life of anakinra is only six hours.

But in recent times, a 100 did not control him. And while he did increase to bid, or two hundred at night, he could not afford it. He's not doesn't have the insurance. He's running out of anakinra, and he has activity. He said that when he went back from BID to once a day anakinra, once a night anakinra, his fever started to recur at a 103 degrees every night.

He was having some more aches and pains and back pain. He did not have side effects with the anakinra. That would include, injection site reactions, or painful reactions, or low white counts, or infections. So the question is, how are we going to manage him? His most recent lab tests are not good.

His aldolase is 12, still elevated. His CK is 72, normal. While his sed rate and CRP are normal, his LFTs are elevated. His AST and ALT are 60 to 70. His ferritin is 2,500, and his H and H is normal, 14 over 47, and his white count is 4.1.

Really confusing. Now, I say he's active and you would say he's active because of the ferritin. I say he's active because of the aldolase. In my hands, in the hands of others that I that have managed these syndromes and patients at Aldolase is the single best biomarker for patients with Still's disease and the IL-one responsive auto inflammatory syndromes. And that's showing up here, his aldolase is still 12.

When he was diagnosed, it was 18, when he was at his worst. And again, he does have, a normal SPEP. So the question is how are going to manage this gentleman? I will still call him autoinflammatory syndrome, and the treatments that would work in him are going to be steroids, we'd like to avoid that. Methotrexate in combination with other DMARDs, not always very effective.

I think that the drugs that we should use would be IL-one inhibitors, or the IL-six inhibitors, or JAK inhibitors. Now, now he's in a situation where he doesn't have insurance and his income is low. You can apply to the manufacturer to get compassionate use drug for either not so much for anakinra, that's kind of hard. But for maybe other IL-one inhibitors, and that include canakinumab, which is a long acting drug, go for either, sorrelimab, the IL-six inhibitor, or, the use of tocilizumab, which is FDA approved for use in systemic JIA. And we now don't distinguish systemic JIA in adults and kids or Still's.

It's Still's disease. It's the same in adults and kids. If it's approved in one, it's approved in the other. So you can get it approved that way. So that's what we did.

I told him temporarily to go up on his methotrexate, try to continue to take his anakinra. I started him on methotrexate as a steroid sparing move. Not sure if that's gonna work very much. And in the meantime, we're gonna apply for, compassionate use canakinamab. These can be difficult cases.

By the way, we'll see you at RheumNow Live, where we've got a great session on RA, where we're going to talk about AI in RA, artificial intelligence in RA, managing older patients in RA, and we got Bryant England talking about multimorbidity management in RA. Our RA speakers include Una Macriese, Jeff Curtis, and Bryant England with a great panel discussion afterwards. We'll see you at RheumNow Live. This is QT clinic. I am Jack Cush with RheumNow.

QT clinic is brought to you by RheumNow live. Today's case is a patient with a problematic past history. Oh my goodness. So a 62 year old white female is referred to you by the cardiologist because she's got, you know, a long history of rheumatic things. She was diagnosed with ankylosing spondylitis, and she has occasional pains in addition to her hypertension and cardiac concerns, which are not all that major.

She's 62, and twenty years ago, she was diagnosed by a local rheumatologist as having ankylosing spondylitis because she had chronic low back pain, and was recently diagnosed with uveitis that required steroid treatment. The rheumatologist managed her low back pain by doing x- Oh goodness, x rays, and by doing, lab tests. I don't have the lab tests. I assume a b 27 maybe. Who knows?

So and she's carried this around, but he's mainly managed her sorry. I'm having some technical difficulties here. Mainly managed her with nonsteroidals over this many years. And the question is, when she comes to see you, she comes to see you because her ankylosing spondylitis and she wants some kind of management, what are you gonna do about it? Well, she has no back pain today.

Her morning stiffness is five to ten minutes. When she does have it, it's kind of general, hands and feet. And on exam, she has what looks to be a pretty mobile cervical and lumbar spine. I don't have any X rays. I don't have any labs.

But she's worried about her ankylosing spondylitis because she has multiple medical problems. And the question is, what are you going to do about this? I'm going to manage what she has. She has some stiffness. She has some poor sleep.

Her exam shows no tender joints, no swollen joints. She has evidence of Heberden's nodes and says that those sometimes bother her. So I think that this happens all the time to us, does it not? Someone comes in with a past diagnosis that handcuffs you, and you're gonna have to deal with it in the context of their current complaints. Luckily, this patient doesn't have current complaints.

And what I do is I like to talk about you back then, and what your diagnosis was with your old rheumatologist. And, you know, I don't have great documentation. I certainly respect what you say. But we're going to move forward with you right now. And the new you is not the old you, right?

So the old you is not your past diagnosis. I'm sorry. The new you is not your past diagnosis. The new new the new you is not your family history. The new you is not what your best friend tells you you should be worried about.

We're gonna deal with what you have today, and that's the case you make. So in this case, she's got some stiffness some stiffness, some Heberden's nodes. We suggest that she takes PRN analgesics with either Tylenol or over the counter Advil, And we then we we give her guidance on how to better manage her sleep, because if she sleeps bad, she's gonna feel bad. She sleeps great, she's gonna feel great. So her problem is not what she had twenty years ago.

Her problem is what she has now, and we wanna maximize her condition now and look forward to how she's going to be in the years to come with preventative measures and good advice. Keep following the patient. Keep encouraging the patient. Keep reassuring the patient that her past diagnosis is no longer in play. This happens all the time with people previously diagnosed with spondylitis, previously diagnosed with fibromyalgia, previously diagnosed with lupus, previously diagnosed with seronegative RA.

But again, you have to deal with what they have today. That's my advice on managing this kind of case. I wanna recommend that you attend RheumNow live. Our second day, which is Sunday, February 9, begins with a lecture by Desiree Vanderheid on the management of spondyloarthritis. Oh my goodness, really.

In that same session, we have Catherine Bakewell talking about imaging in spondyloarthritis, and well known dermatologist Jennifer Cather talking about hidradenitis suppurativa and its management. And then we're going to have a great panel discussion. Be there online or in person in Dallas. We'll see you February eighth and ninth, RheumNow Live.