Chapters Transcript Video Is it Rheum? A Clinical Approach to Identifying Rheumatologic Diseasease-to-identifying-rheumatologic-disease.mp4 Good morning, everybody. Thanks for joining us today. Um, I'm Sarah Blacher. I'm one of the pediatric chief residents, and I am very excited to introduce our speaker today, um, for, um, a lovely grand round at the start of the holiday season. Um, so with us today, we have Doctor Nicole Ling, um, who we're thrilled to have joined us. Doctor Ling is um one of our amazing rheumatologists here on both sides of the bay at UCSF. Um, she earned her medical degree from the University of California Davis and completed her residency at Children's Hospital Oakland, followed by a fellowship in rheumatology at UCSF, and she's now, um, one of our wonderful associate clinical professors in rheumatology. Um, she specializes in caring for Children and adolescents who have diseases such as juvenile idiopathic arthritis, lupus vasculitis, um, juvenile dermatomy dermatomyositis and scleroderma. And in addition to patient care, she works with um the Benny Children's Hospital's QI executive Committee and is developing QI metrics for outpatient care and pediatric subspecialties at UCSF. She's Also a co-chair of the Medication Safety Committee. Um, today she's here to talk with us about, um, a clinical approach to identifying rheumatologic disease, um, which I think everybody, um, if I'm speaking for all of us, um, based on personal experience, could always use a refresher and so I'm very excited to hear, um, from Doctor Ling, and, um, please take, um, take it away. All right. Thanks, Sarah. Good morning, everyone. Uh, thanks for inviting me to come speak to you this morning. I was asked, uh, see if I can advance my slides here. I was asked to give a general talk on rheumatology. So today we'll be talking about a clinical approach to identifying rheumatologic disease, um, I have no disclosures. And really, we're gonna be focusing on the thing that is most common in pediatric rheumatology clinic, which is figuring out, um, if, if arthritis is present. So objectives for today are to be able to differentiate clinical characteristics favoring arthritis versus arthralgia, um, to develop a basic approach to evaluating patients with possible joint swelling, and then along the way, recognize some mimics of rheumatologic conditions. So we're gonna whip through a couple of cases this morning. Let's go ahead and get started. Um, this is the first case. This is a patient that comes into your clinic. She's 9. She has a several-year history of joint pain all over for the general pediatricians in the audience. I know. This is, um, something that does come into your clinic often. Uh, the pain is worse in her knees, and she's often injured after physical activity. So mom reports the pain is worse with basketball, lacrosse, and track. And the pain improves with rest. Mom notes that she seems generally just more flexible than others, but she hasn't dislocated any joints. When you uh do a physical exam, there's no abnormal scars, she doesn't have chest pain, and there's no exercise intolerance. So, this first snippet, or clinical vignette is to really talk about terminology, and we really need to be precise in our thinking and in our terminology when we're talking about joints. And that's really differentiating arthralgia, which is joint pain, which is an unpleasant sensation that's associated with actual or perceived tissue damage. Versus inflammation in the joints or arthritis, where you can have swelling or limited range of motion accompanied by sometimes warmth or pain or tenderness. You can see that of the cardinal features of inflammation, I've excluded erythema or rhubor. That's because in our inflammatory conditions in rheumatology, typically, we don't see erythema or redness of the joints. It makes me think about other conditions which we'll get into a little bit later, like septic joint or acute rheumatic fever. So This Venn diagram is not exactly perfect, but I hope you'll appreciate that there are, there is overlap between these two terms, but certainly, you can have arthralgia without inflammation and you can have arthritis where pain is not really a large component of the presenting symptoms. OK. So even in our first case, when we're taking a history, what are the things that make us think uh pain is more mechanical in nature or related to overuse? That's pain that's worse with activity and tends to improve with rest. These patients lack morning stiffness, and there really should be no swelling um for mechanical joint pain. On the flip side for inflammatory joint pain, there's pain and stiffness that tends to improve with activity and worsens with sedentary periods or rest. Um, you can have stiffness in the morning. And inflammatory joint symptoms are typically associated with swelling and sometimes warmth. I'm gonna show you this, uh, retrospective chart review that was done quite a while ago now, but they looked at patients that presented to pediatric rheumatology clinic, and they looked at the chief complaint or reason for visit that kids came in and sought to figure out of those patients, how many of them were diagnosed with inflammatory arthritis. So what they found in this study is that 16% of patients who were ultimately diagnosed with inflammatory arthritis had pain as part of their chief complaint, but rather it was gait disturbance and joint swelling as the reason for visit that was more highly associated with ultimately being diagnosed with inflammatory arthritis. So isolated pain without any other signs or symptoms is rarely the chief complaint of kids diagnosed with inflammatory arthritis. As a side note, uh, they looked at labs as well, and kids who had a positive ANA or an RF ultimately, uh, that was not associated with being diagnosed with inflammatory arthritis. OK, back to our case. Here's your physical exam. Vital signs are normal, body habitus is normal, uh, HENT, cardiovascular and skin exam, normal. And then You do a very detailed and nice musculoskeletal exam, and you know that there's no swelling, there's no limited range of motion or growth disturbance in the joints. But you do a detailed joint exam and and notice that there's generalized hypermobility. So, the patient is able to oppose both of her thumbs to her forearm, she's able to hyperextend her elbows. She's able to flex her fifth digit beyond or 290 degrees, but she's unable to put her palms flat on the ground with forward flexion and unable to hyperextend her knees. OK. So, let's talk a little bit about joint hypermobility. That's the, when your joint is able to move actively or passively beyond normal limits along physiologic axis axis, and up to 33% of the pediatric population is hypermobile, this decreases with time. Joint hypermobility can be associated with arthralgia, uh, which is why we're talking about it this morning. And when we're thinking about joint hypermobility, we should be considering primary or genetic disorders of connective tissues such as hypermobility spectrum disorders or the hypermobility subtype of Ehler stlos. OK, so how do you assess for hypermobility? You can do that uh a few ways actually. You could take a goniometer and do some measurements and, and, uh, compare those to age-appropriate controls. You can um use a five-part questionnaire, or more commonly in rheumatology clinic, we're doing Byton scoring. So let's go over that. I talked about it a little bit in the vignette. Uh, here are the features of the Byton scoring. So, the 1st 3, A, B, C, and D are 2 points each because you have two sides, and then E is 1 point. Uh, but we talked about all the criteria that go into Byton scoring. If you're able to rest your arm and put your pinky up past 90 degrees, uh, if you're able to fully oppose your thumb to your forearm, you get a point for that. If you're able to hyperextend either your elbow or your knee, you get an additional point. And then our patient couldn't do this, but if you could uh forward flex and put your palms flat on the ground, you get a point. Uh, you all are probably very aware of this, and you see this in your clinics. But let's talk a little bit about treating hypermobility arthralgia. You can use supportive footwear. Sometimes taping or bracing troublesome joints. Although I think, uh, referrals to physical therapy to strengthen muscles around the joints to reduce their laxity is key. Uh, weight loss can reduce some of the arthralgias from hypermobility. And you can use PRN NSAIDs or Tylenol. I think if there's widespread pain, CBT or biofeedback or referral to one of our amazing pain programs, um, would be of consideration. And then if you're worried about any of the genetic conditions of connective tissue disease, then a referral to genetics. Um, we do sometimes get patients, uh, sent to our clinic without joint pain, that is just for genetic conditions, uh, Of connective tissue and those really should go to genetics. I will say that um there is no genetic testing for the hypermobile subtype of Ehlers-Danlos. So um really the, the hallmark of treatment is supportive care. OK. I have spent the 1st 10 minutes talking about hypermobility and arthralgia, and I think it's important because the differential for arthralgia without arthritis is quite different from the differential, um, when you have a kid in your clinic that has arthritis or inflammation in their joints. Um, this is a pretty good list. It's not all-inclusive, but there are disorders of hypermobility and hypermobility, overuse syndromes, osteochondrosis, developmental disorders, uh, really a whole host of things when kids have joint pain, but they don't have inflammation. This will be in the slide deck for your reference. OK. Our true quest today is to figure out if there is arthritis or not. That's the main quest for rotators in our clinic, um, and that we're doing in rheumatology all the time with new referrals. OK. So we're gonna go through uh a high-level overview, some physical exam findings, and talk a little bit about imaging. But, um, first, let's go over the physical exam. It could be its own talk, so, uh, we're not gonna get too in detail about all the joints, um, but give you a basic approach. So for the joint exam, you really need to look. And what are you looking for? You're looking for anything that looks more swollen, anything that looks slightly asymmetric and has grown differently, contractures of the joints or, or changes in muscle bulk. Really, when you're looking, asymmetry is your friend cause things can be quite subtle, and I'll show you some pictures coming up. When you palpate, what you're feeling for is that effusion. And when I, uh, teach the residents who come and rotate in our clinic, what you're feeling for is a water balloon feeling. So, um, it really does feel like that. It's different than soft tissue which can feel kind of more foamy, uh, and just a little more dense. So, a lot of practice palpating. Uh, we do a lot of range of motion in clinic going for the extremes. We did a lot of range of motion over telehealth during the pandemic. We'll talk about some of those maneuvers in the upcoming slides. And then tenderness. So, you really need to palpate forcefully enough that you think it might hurt and you look for nonverbal cues of discomfort in your patient. OK, let's go through some examples. It's, you know, this format isn't ideal for teaching a joint exam, just looking at pictures and, and looking at slides. Um, but let's try our best anyways. This is a patient of mine who showed up in clinic. You might recognize the chairs, um, who you can see just by looking, if you, if, uh, if you had them take off their socks and their pants, um, You can see the swelling draped around the lateral malleolus of this ankle, and even the, the, see if I can pull up my cursor. Even this ankle back here posteriorly, you can see the swelling. If you moved this foot, it would be tender, um, and certainly, she would have decreased range of motion. OK, let's keep going. Here's another picture, uh, a classic picture that we use in rheumatology clinic. So in this picture, you can see that her right knee is quite swollen. When you're looking at it, if you look at the medial gutter here, you can see uh a loss of landmarks. And then also for this little girl, you can see that she's kind of leaning over to her left. That's because she has some leg length discrepancy with knee arthritis, the affected limb will get longer. And you might also appreciate that this knee is a little more bent and she has a joint contracture. If you had her walk-in clinic, you would note that she's probably limping, not just from the leg length discrepancy, but because of the swelling in her knee. OK. Here's another picture. Uh, this is where again, landmarks are really helpful and asymmetry is helpful as we talked about. You can see that there's uh a loss of the medial gutter of this left knee here. And you can also see that there's supra patellar swelling, uh, which does communicate with the, with the joint up here. And I hope you could appreciate also that this calf is perhaps a bit smaller and this quad is a bit smaller um from decreased use. All right. We talked about range of motion. Here are more pictures. This is the, I should probably try to get some videos in here of joints moving, but, uh, this will suffice for now. Uh, this is a patient, the, on the left are patients who have wrist arthritis. We're not only palpating the wrist, but we're also asking them To do several motions. I'll give you a reference for that in a couple of slides. Uh, but they really are having trouble opposing the, the dorsum of both of their hands together, and that's because there's swelling in the wrist and it's quite painful. So in clinic for wrists, I will often hyperflex their wrist and sometimes they'll withdraw if it's painful. And then this picture on the right is a picture of DIP arthritis. It's pretty actually uncommon to be an isolated finding, um, but you can see how if it wasn't all that symptomatic, you might miss it for a little bit, um, if you weren't really looking. OK. We talked about growth disturbance with the knee. You can also get growth disturbance with the jaw, and certainly, you can have TMJ inflammatory arthritis. When that happens, you get something called micronathia. So in this top picture here, you can see that this, there's um Uh, as a microathia, and then in this bottom picture, you can see this is, again, where asymmetry is your friend. There's left TMJ involvement. You can see that this mandible is shorter, and when he opens his mouth, his jaw cans to the left. You may feel things like popping and clicking, those are non-specific, cause you can certainly have non-inflammatory TMJ dysfunction. But all of these, uh, the growth disturbance, particularly, especially when it's as asymmetric. Um, can help you figure out what's going on. You'll also note that he can't really open his mouth all the way, so decreased oral aperture is one of the findings that we see associated with TMJ arthritis. OK, I told you physical exam could be. It's own talk, um, and probably not in the scope of today, but there is a validated tool for general pediatricians and others to use to screen for arthritis in clinic. I know it's a busy slide, but it's just meant to be for your reference here and give you a general flavor of some of the movements that we ask children to do. We actually leverage the peals and some of these maneuvers during the pandemic, and as we continue to do telehealth, if we struggle to get patients back into the clinic. Um, because it gives us at least a visual idea of, uh, their range of motion. We certainly can't palpate through the screen. OK. If you're still um not sure about the joint exam in clinic, I think it can be helpful to look for extra-articular manifestations of JIA. We're gonna go from head to toe. So, the first thing we're looking at and paying very close attention to in clinic are the eyes. This is an example of something called seniya. Uh, that is, that you'll notice that this pupil is irregular, and that's because there are adhesions, um, that are holding points of the pupil fixed while other points of the pupil dilate. And you can also see hypopion, which is actually layering of inflammatory cells at the bottom. This is actually, uh, a pretty severe case, you may see, I've seen lots of Seneca, I have not seen very much hypopion. Um, I bring this up first, not only because we're going head to toe, because it is often tested on on pediatric boards, and it's really important for kids with inflammatory arthritis, but you can have inflammation in your eyes associated with inflammatory arthritis, uh, specifically JIA. This is typically anterior and it's non-granulomatous. It can be asymptomatic and insidious in onset. And can really cause permanent vision loss. So it's something that we take very seriously. Everyone in the crowd has probably seen us order an ANA for kids with arthritis. And that's not really to diagnose if they have arthritis or not, but rather to assess their risk for Uveitis. So patients who have a positive ANA and are similar to that picture I showed you of the girl with the swollen knee, young, um, and ANA positive. Those are the kids that are at highest risk for Uveitis. OK. So how do we actually diagnose it? We send it to the, send them to the optometrist or the ophthalmologist where they do a slit lamp exam. And when they do their slit lamp exam, this is what they see. You can actually see the inflammatory cells floating in the anterior chamber in this picture. I've rotated through um our UVI clinic. It is very difficult to see in real life, and they are very talented for being able to actually count or quantify the number of cells floating in the anterior chamber. OK, just a reminder of the anatomy of the eye. The uvea is the vascular middle layer of the eye. It's highlighted in pink around the eye here, and it's comprised of three things, the iris, the ciliary body, and posteriorly, the the choroid. And you can see when there's anterior uveitis, um, How if there's inflammation of the iris, it can then scar down onto the lens and cause that fixed point that you would see on physical exam. OK, I'm gonna show you one more scary eye picture. I don't think anyone would miss this, but this is called band keratopathy. It's where you have uh calcium deposition in Bowman's membrane after long-standing and chronic inflammation in the eye. And certainly, you would urgently refer anyone you saw with an eye that looked like this to optometry or ophthalmology. All right. The pearl here, and I just did an outpatient consult yesterday. I'm on service right now with an outpatient pediatrician. Any child that you're worried about having juvenile idiopathic arthritis should be screened for uveitis by an ophthalmologist. If you don't have an ophthalmologist, uh, local to you and you have an optometrist who's comfortable seeing children, I think that's fine as well. Um, but the PCP that called me yesterday, she put a referral in for us because she was worried about swelling, and I asked that she also put A referral into their local ophthalmologist. OK, so we just started with the eyes in terms of extra-articular manifestations. There are two subtypes of JIA that have rash, we'll go into the subtypes a little bit later. The first is, uh, the rash of systemic onset JIA. On the left here, you'll see a maculopapular rash that's usually evanescent in nature, meaning it comes with a high fever and disappears when the kid is afebrile. And SJA is also associated with the Kebner phenomenon, which is a, an exaggerated um erythematous response to trauma. So you can see that this person on the left here was scratching and you can see the linear excoriation resulting in the erythema. OK, that's the first flavor of rash that's associated with inflammatory arthritis. The second is psoriasis. This is a uh very clear cut picture of psoriasis, which is erythematous plaques with a silver scale. There also is a predilection for behind the ears and around the gluteal crease. Um, so this is something we're always looking for in rheumatology clinic in patients who have joint swelling as well as more subtle findings, like nail pitting, which is associated not only with psoriasis, but, um, you can see it in isolation and also associated with other things like alopecia areata. But really looking at the nails is important. I like to move the nails under the light to see um the nail pits a little bit easier. OK, the last extra-articular manifestation is rheumatoid nodules. This is something that's pretty uncommon in the pediatric population, so I won't spend too much time on it, but these are, uh, soft, mobile, soft tissue nodules that tend to have a predilection for extensor surfaces. This is a picture of a PIP and a nodule here, and an elbow, and a nodule here. They're non-painful um and not very common. All right. We've whipped through physical exam pretty quickly. Um, sometimes we need imaging to help us figure out if there's swelling or not. And actually, the PCP that I talked to yesterday, I asked, um, that she send the patient for an ultrasound so we could capture their arthritis if they were having it. OK. More and more we're using ultrasound and pediatric rheumatology. It's low cost, it's easily accessible, even in remote areas of the state. There's no sedation required, and rheumatologists are getting trained in it to guide injections as well. Here are some examples of what you would see if you looked at the ultrasound. Um, on the right here, you can see that there's synovial thickening, that's this thick white band. And then there's this dark hypoechoic fluid here that represents the the effusion. And if you threw Doppler on there, you could see that there's increased vascularity, two different pictures, but illustrating some of the findings that you would see if you ordered an ultrasound. This is good for large joints. If you're not sure if there's swelling or not, or if the swelling comes and goes, trying to capture it with an ultrasound is great because um, That can be a little easier to get in terms of scheduling than an MRI. OK. MRI uh has increased sensitivity for detecting inflammatory arthritis. Usually, we're ordering this with and without contrast. Although more and more for large joints, we are ordering it without contrast and certainly of the pelvis, we're now able to see um enough on T2 and other and other sequences to not uh need contrast. And this picture illustrates what you'll see. Again, the same thing. You have this hypertrophic inflamed synovium that happens with inflammatory arthritis. You have this dark effusion you see here. And then because you can see more of the contour of the bones, we're able to see erosions, um, with more sensitivity than X-rays and also tenosynovitis and bone marrow edema. OK. Lastly, um, radiographs, these are not always sensitive for effusion. So I've seen kids with huge large knees with, uh, reportedly normal knee X-rays, and that's because it's difficult to look for a fusion. I think there are some areas, such as the elbow, where there's fat pad displacement, where you may have some increased sensitivity for detecting. A fusion by X-ray, but really, we're getting X-rays in our clinic to screen for other ideologies or to assess for damage. So, um, Here in this picture, here, this is a child who has juvenile arthritis. And I, I know it's probably not projecting perfectly, but I hope you can appreciate around the MCPs and the PIPs that it's a little bit darker. So that's peri-articular osteopenia. Um, if you were really good at outlining the contours of the bones, you could probably see that there are erosions here and there's joint space narrowing and crowding of the carpal bones. And then even more rarely, um, especially these days with the medicines that we have, you can see joint subluxation or misalignment and ankylosis. OK. So is, is there arthritis? Um, I hope I've convinced you in the 1st 25 minutes of this talk that that's a clinical diagnosis made by physical exam findings and sometimes imaging. There's really no lab test that indicates the presence or absence of arthritis. Um, and let's jump to the next case. All right, so this is um something probably very common for you all. A 3 year old presents to urgent care. For fever for the last couple days. She had a cough that began 5 days prior. And overall, she seems to be improving, so she gets discharged from urgent care and supportive care is uh recommended. 2.5 weeks later, she wakes up with a warm and swollen right knee. OK, so we're gonna, we're gonna pause here and start to think about what should we be thinking about as a patient approaches us with a swollen joint. So with this history that we have so far, when I see a referral like this, I start thinking or if a, if a rotator or resident is seeing this patient, I start thinking this as they're presenting. Did the patient have pharyngitis? Um, there is post-strep reactive arthritis. There's acute rheumatic fever. And so I wonder, with this initial illness, was there any sore throat? Um, Did the patient take any new medications? So, when they went to urgent care, were they actually diagnosed with an acute otitis media and took amoxicillin, and 2.5 weeks later, there's a warm swollen knee. Now, serum sickness-like reaction, um, is a type 3 hypersensitivity reaction, and the, the clinical triad is fever, urticarial rash typically, and, um, arthralgia or arthritis. So these are, are things that are still going through our mind. Was there any trauma that's looking for ligamentous or cartilaginous injury? She actually fell at school 3 days prior to presenting with the warm knee, but she fell from standing. So not much of an impressive history in terms of trauma, but if there was large swelling, you might think, and not a lot of trauma, you might think about things like hemophilia in a different patient. Uh, was there any tick exposure? So Lyme arthritis is a, a late finding and typically can, can migrate. It's a late finding of Lyme. So things that we're always thinking about in clinic. This patient, because of the acute onset of the warm right swollen knee was actually sent to the emergency room. Uh, and here are the vital signs. You can see that the patient is afebrile and the vital signs look pretty good. And this is the exam of uh NO chief resident that rotated with us in rheumatology. So for the emergency room, this is a very detailed musculoskeletal exam and kudos. The patient appears well. Um, there's moderate swelling over the right knee. She can bend her knee up to 90 degrees. There's mild warmth to palpation, and, but no tenderness and no redness. Because you're in the emergency room, you can get some more workups. So you get a basic CBC and you see that the patient is inflamed, uh, a little bit anemic, uh, with elevated inflammatory markers. And you get an X-ray of the right knee and the pelvis just to make sure there's no hip pathology. And you see that there's a large joint effusion of the right knee, but the hips otherwise look normal and you can't see anything else. Here's another point that we'll pause in the history and think about how this. has gone for other patients and the subtle clinical things that make us think about other things. So, this patient overall appears well, and there's not a whole lot of tenderness. If there was a large joint involved and something called pseudoparalysis, or there was pain out of proportion to exam, that would change my thinking of the patient. Um, I would think about things like septic arthritis or malignancy even. If the patient was febrile, you might even think more about septic arthritis. And then some of the more subtle cases that we've seen are relative cytopeniaas in patients, meaning they have high inflammatory markers and maybe they're, um, white blood cells, red blood cells, platelets, they don't meet criteria for being low, but they're lower than you would expect, um, as acute phase reactants with elevated inflammatory markers. And some of those patients have been Uh, we've seen patients present with arthritis, both with malignancy and um septic. OK, again, I, she's in the emergency room, so you have the luxury of calling ortho, orthos consulted, and I think, you know, in a well-appearing patient, Uh, that's afebrile. I think it's a shared decision making for whether or not you would tap the joint, but they went ahead and did that for this patient, and they had, they pulled out 2 ccs of cloudy but not purulent bloody fluid. There are the cell counts for this uh synovial fluid, and we'll fast forward 5 days, and the blood and synovial cultures didn't grow anything. Because she was well appearing, um, no antibiotics were started, there was no surgical intervention, but she was admitted just for some observation and ID was consulted. They elicited a history that she had moved from New York 4 months prior, but she had no tick exposure or rash. They recommended some infectious testing, uh, both in the serum and also in the synovial fluid. They also did a Kella PCR which was negative. So, due to persistent swelling, she was started on naproxen BID referrals were made to our clinic and ophthalmology. And I had the luxury of seeing her 5 to 6 weeks after the onset of swelling was first noted. Now, sometimes, uh, patients come in and maybe it's been 2 weeks, but you can look back at pictures and see that, in fact, things have been swollen uh for longer than parents may have noted. OK. So this patient comes to rheumatology clinic and she has no GI symptoms. She's following her growth curve. She's afebrile still, and her right knee is still swollen and in mild flexion contracture, which is a clue to the chronicity of this patient. But there's no leg length discrepancy and um she also has some moderate swelling of another spot, a left index PIP. You're diligent, you do your eye exams. She doesn't have any photophobia. You don't see any sneaky eye of her pupil, um, and there's no rash or nail pitting. Actually, this patient came um to see many others who did labs before me. So she was actually noted to have a positive ANA sent to ophthalmology, who she saw before she saw me, um, and was diagnosed with left-sided uveitis. So, even in this situation, we've kind of talked about presenting symptoms and what permutations clinically you might be thinking about in your brain. So let's continue to do that. Um, If she had GI symptoms, or I reviewed her growth chart, and she was crossing percentiles for weight, we might think about other things like IBD or bachettes. Um, if she had gone to the ophthalmologist and been diagnosed with pan uveitis or had oral or nasal ulceration, we might think about really rare things, especially if you're in rheumatology clinic like sarcoidosis. And then, this is just the tip of the iceberg about, of things that we see in rheumatology clinic, but if she had other things on physical exam, again, nasal oral ulceration, any rashes or nail fold, uh, capillary abnormalities, I'll show you a picture in a second. Um, other things, or if the patient was older, we might think about lupus, dermatomyositis, or vasculitis. OK. Let's, uh, let's pause here and these extra symptoms that this patient doesn't have. Let's just look at some pictures, um, I think they're. Interesting. OK. Here. So, um, here's a picture of oral ulceration. This is a patient of ours, uh, mine who has lupus. Um, and it typically has a predilection for the hard palate. Uh, again, this is, uh, cutaneous lupus. This is rash, Gotrin's papules. It's very subtle, um, of a patient with more pigmented skin. Uh, but you can see these flat top slightly erythematous macules over the MCPs and PIPs, that's typical for dermatomyositis. And then if you come rotate, or for the residents who come rotate with us in rheumatology clinic, we're doing nail fold capillaroscopy. So this is an example of normal nail fold capillaries and in conditions like lupus or dermatomyositis, or other inflammatory vasculinities, you may see um more hemorrhagic. dilated, uh, tortuous capillaries with dropout. OK. So, as we've gone through that second case, we've kind of talked through the differential for kids who have arthritis. It's different than the, than the differential for arthralgia itself. This is not all-inclusive, but it's, um, again, pretty good. Infectious ideologies, we're always thinking about, again, febrile patients, if there's pain out of proportion. to findings or sometimes it can be a little less obvious. Uh, post-infectious is a huge category, and remember the Coker criteria for distinguishing transient synovitis versus um septic arthritis in a hip in a child. And every once a year or so, we have patients present to our clinic with arthritis who get diagnosed with malignancy. And then a whole host, and then hopefully that last picture demonstrated there's a whole host of other autoimmune and auto-inflammatory things that can present with arthritis. All right. This patient was ultimately diagnosed with JIA and how did we get to that assessment? Uh, well, the patient was less than 16 and her arthritis was present for more than 6 weeks. And JIA is a diagnosis of exclusion. So anytime we talk about JIA we're always talking about the other things that can cause arthritis in kids, because we really need to be at least ruling those out in our minds, if not with labs. OK. JIA is a general term for a heterogeneous group of disorders that are characterized by inflammatory arthritis. There are 7 different subtypes based on clinical characteristics and labs, and each of these subtypes have different epidemiologic associations and different prognosis and approaches to management. It's um, Uh, probably not in the scope for anyone besides us in rheumatology to know all the nitty gritty details of the subtypes, but I will go through them on the next slide. Um, some of the subtypes do have adult onset analogs, uh, versus a subset of them or really have a phenotype that's clinically unique to pediatrics. OK, let's whip through these subtypes, um, just so you get an idea and a flavor of them. The 1st 3 subtypes are characterized by the number of joints involved in the 1st 6 months of disease. So, the first subtype is oligoarticular JIA. Think of the girl in the dress with the swollen knee. That's where you have 4 or less joints involved in the 1st 6 months. The youngest patients with oligoarticular JIA with the positive ANA, those are the ones that are at highest risk for uveitis. And then the next two subtypes are related to polyarticular arthritis, meaning 5 or more joints. I remember that because 5 is related to the hand, uh, and polyarticular arthritis has a predilection for the small joints. Um, And so, 5 or more is polyarticular. The first subset is if you're RF negative. The second subset is if you're RF positive. This is the adult analog of adult RA and has a more um aggressive course and can be more damaging down the line. We talked a little bit about this before, there is psoriatic arthritis that is a subtype. It's associated with both nail pitting and dactylitis in addition to arthritis and psoriasis. The 4th flavor is andesitis-related arthritis. This is the uh pediatric analog of adult spondyloarthropathy. So this is where the typical profile for this patient is a teenage boy with low back pain and may have sacro ileitis, uh, or reactive arthritis. It could be seen on a spectrum. And then, uh, start for these patients, the systemic onset patients with juvenile arthritis are really different than the 1st 4 that I mentioned. These patients are sick, they have persistent fever, that evanescent rash we talked about. Um, and really could be its own talk to talk about this subtype of JIA. In fact, Doctor Soulsby gave an excellent grand rounds on systemic JIA. So I would refer you to his talk for more details about The complications like MAS or ILD and the adult analog for this is stills. OK, lastly is undifferentiated just to to round out the different subtypes. OK. Here's a treatment algorithm for arthritis. We're, it's actually a really exciting time in rheumatology because we have a whole lot of treatments in our armamentarium for inflammatory arthritis. As you're waiting for patients to come and see us in clinic, I think it's reasonable to start NSAIDs, and you'll note that We will consider uh a myriad of DMARDs, both conventional, like methotrexate, biologic DMAs. Um, Doctor April Zat was talking to me before this talk about how she was excited about the alphabet soup of rheumatology. I think this is what she meant. There are, um, a lot of biologic therapies against different targets like TNF, IL1, IL 6, CD20, and CTLA. Um, and then a new class of medications, targeted synthetic DMAs, which includes the Jack inhibitors. We've probably all seen commercials for these on TV. So we have a lot of medicines to treat inflammatory arthritis in kids and probably less and less we're adding systemic steroids at the onset or, or, um, doing inarticular joint injections with steroid because our systemic medicines are, are so good. For the general pediatrician and others, um, let's just talk about naproxen, uh, which has many different names. The dose is 10 per kilo, uh, per dose given BID. We like naproxen in rheumatology because of the half-life is longer than Motrin. Um, it can cause a rare photodermatitis called pseudoporphyria. Your skin gets fragile and vesiculates, resulting in some shallow scarring, and it looks like little cuts. Uh, for patients who are started on naproxen, who are fair-skinned, uh, they're the most at risk for developing this photodermatitis, and you can prevent it with sunscreen. Here's a picture. Uh, again, this rash is scarring, so if patients develop this reaction to naproxen, we stop their, uh, we stop their therapy. All right. What happened to our patient? She was started on weekly methotrexate. With the addition of adalimumab or Humira shortly after. Um, her left-sided uveitis never came back. It was controlled with systemic therapy for her arthritis. And, uh, since then, she's been in and out of remission, on and off medications for the last 7 years. She's now on methotrexate and uh Enbrel, and she's playing competitive soccer. For HIPAA reasons, I won't tell you who she is, but she's in this picture here. Um, I volunteer at arthritis camp every year in Livermore, um, although our site might be changing. And if you came to visit us in arthritis camp, you would know that though these kids have rheumatic conditions, they run around, um, on and off medicine, and you wouldn't even know, uh, because most of them are doing quite well. OK. I know there's someone in the audience probably thinking, wait, we didn't really talk about labs. This is uh probably one of the most common questions. I get as a curbside, hey, I'm thinking about this patient, what lab should I send? Um, so let's go through it before we finish here. I think at minimum, you could get a basic set of labs, a CBC with diff, an ESR and a CRP and a CMP, um, and that's to think about just the profile of the patient and to think about other things like malignancy or, or infectious arthritis. If there's persistent arthritis present and you're still waiting for us for some reason, I think our availability is pretty good. Um, you could send another set of labs, the ANF, ANA, RF, CCP and HLAB 27. These are more used to characterize the subtype of arthritis rather than diagnose anything unless you're thinking about other rheumatologic things like lupus or mixed connective tissue disease. Um, and while you're at it, if they have persistent arthritis and you're getting blood work, please get a quantifiron also because some of our systemic therapies, we need a negative TB test before we can start them. In the starred patient that you're worried about with persistent fever and rashes and may present very similarly to Kawasaki disease, for example, you could send uh this subset of inflammatory labs looking for hyperfarinemia, um, and other findings that we see with MAS which is the complication of systemic JIA. In clinic, if you're worried about lupus or other classic autoimmune connective tissue disease, I would start with a urine test. We get a UA with micro and uh urine protein and creatinine ratio, as well as an ANA just to screen and make sure you're not missing renal disease, which is a big morbidity for um lupus. And then if you wanted to get the extra credit and wanted to send serologies um for a patient, you could send the rest of the serologies that we send when we're working patients up for lupus. We have a couple of panels here in Oakland. Um, I think there's a bioplex that is an ANA that will cascade to some of these serologies. If you wanted the classic answer, an ANA by IFA is the gold standard. Um, so sometimes you'll see us rechecking that if there's a positive ANA by bioplex. And then a whole host of other things. So, what, uh, depending on how the patient presents, we may send testing for postinfectious things like Lyme or strep. We may screen for IBD, etc. etc. Call us if you have any questions. OK. Here are the, the take home points for today's talk. Arthritis is a clinical diagnosis made by physical exam and sometimes with imaging. I think more and more over time we're, I've, I've been recommending ultrasound if you're unsure or unable to capture um arthritis that may be waxing and waning, which is not the usual presentation of inflammatory arthritis. I think I've hammered home this point that labs don't tell you if the patient has arthritis or not, but it helps you think about other ideologies and causes for the inflammation. And hopefully, I've illustrated, not all arthritis is rheumatologic in nature. We're happy to help you wade through the differential, that's why we're here. Um, and children that you're worried about having JIA should be screened for uveitis by an ophthalmologist. I know wait times can be quite long, so, um, putting that referral in as you're putting the referral in for us is helpful. All right. I think I am. Done and ready for questions. I have references here, but I'll leave it on the question slide. Thank you so much for that talk. Um, I have one to sort of just start us out on, and you sort of, uh, mentioned this earlier when talking about um how so many kids who have JIA and other arthritis involved, rheumatologic conditions, managed to be incredibly active. Um, I just was wondering if you could talk to us a little bit about how you've seen outcomes and severity of illness change in the last, you know, several years with, you know, increased availability of DMARDs and biologic drugs and sort of where you see, um, Where you see additional opportunities for improving quality of life and outcomes for these kiddos in the next um few years to come. Um, great question, Sarah. So, pro, um, The changes in prognosis for these kids in terms of their joints from having long-standing inflammation predates some of even, uh, my fellowship. So when our division chief, uh, was in fellowship and early practice, I think there weren't as many options on the table. And if you went to arthritis camp at that time, I don't think you would see quite as many children running around so robustly and not being able to tell. Um, You know, things like physical therapy, I think we're using less and less because the kids, uh, are able to do everything they want to do on medication if we're treating them correctly because we have so many different agents to help us. So I think the, the prognosis and outcome has really skyrocketed over the past several decades. Um, we are at a point now where we really want to know with precision how our patients are doing. So we're doing things in our clinic like trying to measure disease activity precisely by the joint and being able to do some population management and looking at their outcomes in terms of activity scores. But it's always hard to do long-term outcome, uh, studies, but I think that the trajectory for our patients is um very optimistic. I'm very happy to be practicing medicine in this particular time period. Yes, it is alphabet soup and a lot of medicines to, but um. But it's really cool for our patients. Definitely. And would you mind real quick advancing to the next slide that has the QR code on it as well while we keep it going with the questions. Thank you. um. We've got a couple other questions coming in. Um, any thoughts on psoriasis being connected to UPF consumption and anything that you would recommend that patients avoid eating? Oh. Uh, dietary. diet and its effect on autoimmune disease is of such interest to all of us and especially our patients. I think, um, I'm not as familiar with UPF so I'm gonna table that, but I can get back to you if you tell me who you are and I can look through some of the literature. But generally, um, We recommend a healthy diet. I think our uh dietician recommends a Mediterranean diet. But it's hard for these kids. They're getting labs, they're getting injections, and so any changes that families are interested in making, we just try to emphasize that the whole family does it. Doing randomized control trials with diet are quite challenging because of the variation of diet between, um, different folks. So, uh, it's always an area of interest. I think in other areas like celiac, for example, it's a little more clear cut about how diet might be able to affect symptoms. Certainly, thank you. Um, we also have a question about, um, you know, I, about if you're waiting for a specialist appointment and it's a couple of weeks away, um, based on the time of year or just uh a moment of higher demand, um, a question about how we can be reassuring the parents in the meantime and um about what sorts of things would, um, warrant a call to request earlier appointments. Great question. Um, we are always here, and I think I mentioned in the talk, our availability is pretty good. So, if you, if you are worried at all as a clinician, trust that judgment. Give us a call. I think things like inability to walk is a red flag, fevers with symptoms, with joint symptoms, also a red flag things to look out for. Um, If it's a little more chronic in nature, and maybe there's lots of pain, but not to see, not much to see on exam, um, NSAIDs can be helpful. But again, just give us a call and we can help you triage how urgent it might be because certainly, patients can be really anxious to see us who have amplified pain, for example, and um we don't have immunosuppression to offer those patients that would alleviate their symptoms. Thank you. It sounds like when in doubt ask. I love that. Yeah, there, um, there's just so many nuances to the cases that were available. We're here all the time, especially in the era of Volt. Wonderful. Um, we also have a question, um, that's a little bit, um, more on the distinguishing infectious from rheumatologic ideologies of arthritis. Um, there's, you know, um, a question about Lyme's possibility, um, uh, of being involved in cases, you know, having a difficult time distinguishing between Lyme and, um, and rheumatologic conditions given overlap of fatigue and, and joint involvement. Um, the question is how often do you presumptively treat for Lyme disease, given that testing is relatively Not the most reliable, um, just based on a rash or arthritis and um a patient having exposure to an endemic area. I mean, I, I think there are various schools of thought about the testing of Lyme. In rheumatology, we rely heavily on the EIA and Western blot um to determine if we should be treating for Uh, empirically for Lyme as a cause of their arthritis. We also, I saw Ann Petrou in the list of participants. We also phone a friend and ask our infectious disease colleagues to weigh in if there's some ambiguity about any infectious trigger for the symptoms. I think though it's important, um, there are lots of independent labs out there. Uh, especially related to Lyme and other things. And so, uh, we stick with the ones that are, uh, certified, Clio certified. Wonderful, thank you. And maybe time for just one more question, which is, you touched on this a little bit earlier, but what sorts of things should make us concerned that a patient might have MAS or might be kind of on the sicker spectrum of a new presentation of um a rheumatologic disorder? Yeah, great question. Uh, it's really a different flavor than everything I just presented to you all, where you have some time to think about things and, uh, it doesn't seem so urgent. I think, trust your clinical judgment, but the patients that come in with maybe systemic JIA, you're also probably thinking about other ideologies of persistent fever like Kawasaki disease, for example. Um, so, It's your same criteria for wanting to admit a patient with FUO to the hospital, um, where we meet our patients and diagnose them with systemic onset GIA is almost exclusively in the hospital and we're thinking about other things like Primary HLH or secondary HLH because there's that overlap. Um, so, I don't know if that answers your question, but the patient will look sick and have persistent fever. And usually, it's the arthritis that we discover as rheumatologists later, um, because there are so many constitutional symptoms. Certainly, thank you, that does answer the question. And we've got one more sneaking in under the radar. Um, this is a provider who's realizing that we occasionally get teens who have TMJ issues, um, and, um, they're kind of wondering how often do we think that this might be, you know, a presentation of rheumatologic disorder rather than just a dental issue. Oh gosh, this is such a good question because we struggle with this in rheumatology. Also, because there are mechanical TMJ issues that are very common in patients, and teasing them out from inflammatory arthritis is very challenging because even the symptoms and exam findings that I mentioned during the talk are not pathonemonic for inflammatory TMJ involvement. And so, You know, if they're, if it's isolated. You could get more imaging. We do do MR of the face. And when there are mechanical issues or subluxation of the TMJ you can see things like a joint effusion. You can see things like synovial thickening. And, but you, what you really shouldn't see are erosions. And so, it can be helpful to rule in inflammatory arthritis if you see erosions, but you're kinda still stuck figuring it out. If there is a joint effusion or if there's synovitis because it could be mechanical in nature. Other things like, you know, the growth of the mandible, if it's asymmetric, um, Then I would be worried about inflammatory arthritis and I might get that MRI. So really, have the patient look at you. I put my hands like this to look for symmetry, um, just so I can get a feel and a visual about the growth disturbance. Great question. Thank you so much for answering all of our questions and for that wonderful talk. Um, and thank you everybody for coming. Make sure to scan the QR code, um, to get CME credit or you can click the link that's in the chat. Um, and I hope everybody has a wonderful holiday season. Thanks again, Doctor Lang for everything this morning. Yep. Happy holidays, everyone. Created by
