Pediatric rheumatologist Nayimisha Balmuri discusses what primary care practitioners should look for when diagnosing and treating juvenile idiopathic arthritis.
Hello everyone. My name is Dr Hamish. About mary. I'm an assistant professor of pediatrics and the johns Hopkins School of Medicine and pediatric rheumatologist. Today I'll be talking with you all about juvenile idiopathic arthritis. And this is the specific for primary care practitioners. I have no relevant financial disclosures the objectives of this talk or to help you understand the presentation. Clinical manifestations work up of J. I. A. To identify patients who may have reactive arthritis and for you to feel more comfortable with working up lines arthritis. And there will be um two questions at the end to sort of assess reaching these objectives. And I've always um I tend to like case based um um structures. So the first case is your first patient of the week because come in for a sick visit and two year old female with this colony. So mom noticed that the left knee swelling has happened for about a week. Not sure how long it's been stolen before that though. Um There hasn't been any fevers, maybe a limp. Um Our child doesn't seem too bothered at all. And there's been no recent illnesses. Um No medical history up to date vaccines. Um They've been getting some Tylenol for this. There's no allergies that has type two diabetes. Um No auto immunity within the family and a little girl lives at home with her parents and older sister who is well a cat physical exam. I mean you can look at that picture already, you can see that the knee is quite swollen. Um And so here we have a vital sign vital signs that are normal and growing well. It's always important to look at the growth chart because certain kinds of diseases that present with um arthritis or concern for arthritis can be inflammatory bowel diseases, cancers, these kinds of things. And so it's important to look at growth always. And our child is a federal, her general pediatric exam is negative and she's quite happy and chatty and then you can see that swollen knee. We haven't got ahead and got labs which all looked normal. We found that she was positive and A are negative and CCP negative. So remember in A and A is not diagnostic of literally any disease but we'll talk about why and a and A is important in this context of J. I. Which differential is fast reactive arthritis, septic arthritis always right malignancy, trauma lyme disease J. A. Like we all go typists and definitely one joint that's involved. So what the heck is juvenile idiopathic arthritis? So this is actually one of the most common chronic diseases that affect Children in youth is actually one of the most leading causes for short and long term acquired disability in Children. And so the criteria for J is that the age of odds that has to be less than 16 years of age Arthritis has to be present or arthritis is either swelling or fusion in um joined in addition to um limitation of range of motion tenderness or pain um and increased heat to touch. And so this occurs in one of our joints. The duration diseases six weeks or longer. The reason why it's six weeks is because sometimes you can have kind of post viral reactive sina Vitis that can happen that generally would resolve itself within six weeks. Um And then the onset or type of J. Is defined with the disease in the first six months. And so polly articular is when there is greater than or equal to five joints. I'll go is for less joints, systemic onset is when you have that fever and that's an auto inflammatory. Autumn laboratory disease we have quotidian fever and of course the exclusion of other forms of arthritis. So associated loop is I. V. D. Cancer associated infection. All these kinds of things. There are seven different kinds of J. I. A. We'll talk through all of them so Allah go articular once again that's the four or fewer joints typically impacts younger. Um Kids females more than males, these kids generally are not systemically ill. They're typically in a positive and we care about that because along with being an oligarch articular J. A patient and a and a on top of that increases your risk of developing asymptomatic UV itis or iritis where you have a risk of blindness and other things. So this needs to be treated ASAP and needs to be screened. And the effect of joint typically is in the lower extremity it never ever ever never ever affects the hip or shoulder. And so when you have a patient coming in with warm to touch in the hip, that's nuance that you need to think about subjects arthritis. It's never all ago. Um Kids generally very very happy and well appearing. They can usually bear with weight but typically they have a limb generally in the morning time because it affects kids that are a little bit younger parents might say they were doing well with potty training, potty training and then regressed and sort of wetting the bed and these kinds of things because of that stiffness and difficulty bending the knee or sort of getting up from bed and going to the bathroom. There's a variable course persistent means they continue to have four or less joints. And sometimes they can have extended meaning that when their disease duration is longer. But then also more joints get involved and ends up looking more like a poly articular patient psoriatic arthritis um has sort of two peaks younger kids and sort of teenagers once again um female predominance and it typically presents with colitis which we have a picture of that in the cordillera worry of sausage like swelling two digits. And this is sort of like the soft tissue. Um and not actual arthritis and then you can have mono arthritis usually um um angles with small joints are more frequent psoriasis occurs in 40 to 60% of patients. Sometimes kids can present with psoriasis the rash sometimes it develops it later but it's important to kind of figure out um if there's a family history. So in addition to arthritis on exam you need you need either psoriasis or two of the following nail pitting psoriasis in a first degree relative. Or dr lettuce polly articular J. A. Um is when there's five or more joints. And this is most similar to adult rheumatoid arthritis. There are two different forms of poly particular J. A. There's our f negative rheumatoid factor negative and rheumatoid factor positive. We also get CCPS on these patients too. Which is another auto antibody associated with poor prognosis but are of negative um Have a sort of buy basic um kind of predominance meaning younger kids. And then once you get teenagers tend to be a little more asymmetric tend to have less weight loss. Maybe a little great fevers are a positive tend to be sort of that Tween age 9 to 11. It's very similar to adult onset ira. And then we have upper and lower large and small joints and it's generally symmetric things we worry about is always spine neck and TMJ stick out of fatigue and weight loss. You can get some cutaneous battles with that and the status related arthritis. Um Or er a is kind of similar to what you guys think of. Probably with angles and spondylitis such as not a pediatric disease though but so similar E. R. A. So this is um when the sacred iliac joint is impacted the spine and thesis. And that's the um where attendance and ligaments uh kind of um are attached to the bone. Um These guys tend to be associated with H. L. A. B. 27. Um The onset is insidious and you can have intermittent sort of pain and stiffness, gender lines in the lower parts extremities and um peel pain generally. And so generally the sort of history that I get is a kid who's pretty active and there's a lot of sports who's been having lots of stiffness that is over several years and sort of said oh it's probably because of injuries and everything else and my achilles here because I'm a runner. But generally you know that's sort of the history that I look out for. Um systemic symptoms are minimal back symptoms are absent at onset but then can be evident. Um So the things we want to look for are decreased board flexion. Um Since donald j it's typically it's an auto inflammatory disorder. So it's a little bit different then um Then then um kind of typical J. A. But it's under that umbrella. And so this is where you very typically need to have fever. That's quotidian meeting at spikes at night or twice a day. Very high fevers that kind of go away on their own. Um have evanescent rash that occurs with those fevers as general salmon colored or red. You can have incredible pariahs that happens with that. Um Some kids will have passed on ugly of course arthritis but the zero status. But you know what you do have to have for diagnosis is so quotidian fever and rash. The arthritis, you know tends to happen a little bit later. You can develop macrophage activation syndrome, which is deadly and essentially D. I. C. With fevers. And so this is an emergency and something to kind of keep in mind similar to adult onset still's disease but a little bit kind of more specific. So Jay, you know, the frequency is not known incidents is up nine for 100,000. Hard to know the ideology. You know, there are some auto antibodies and there's some research that shows that auto immunity tends to run in families and kids who have J. A but hard to know. Um the thought processes that both in the in the depth of immune systems play a role in this. So a lot of times people refer patients to me for pain. Pain is one thing but kids with arthritis tend to complain a lot about um stiffness. So stiffness is far more specific and sensitive for arthritis than pain is. And so um you know kids will generally say that um that they have pain with active or stiffness with active or passive motion. Um uh a little bit different versus pain patients who also have our thrall aja kind of always describe their pain is extremely severe um and generally improves with sitting still versus um you know j tends to worsen with sitting still in terms of stiffness. Um So joint stiffness occurs after um when you first wake up or after pulling in activity. And it's a nice question to ask is long car ride or flight or first thing in the morning. Um And so you need to have pain swelling, key loss of function erythema and these are kinds of things that the joints will show um joints loser range of motion, especially an extension. Um And and so it's important to sort of think about that. Um For when hip joints are involved there's a loss of internal rotation reflection the patterns of jr very characteristics that sort of what we talked about before. So when we think about symmetrical involvement of large and small joints, it's apology a um one joint or a couple of joints in the lower extremities, that's all ago, dr lie tous psoriatic arthritis. Um And thinking about the lower extremities with emphasis. Itis right, that's er a so it's a very kind of pattern involvement that's important to kind of get when you get a history and then also for the exam there's lots of complications from j we talked a little bit before about how this is the most common cause of acquired um short and long term disability. And so this comes from just pain, inflammation, you can develop localized growth disturbances um leg length discrepancies contractors, you be honest is something that we really need to keep an eye out on an all kids with J. A. Especially those with periodic and oligarch articular especially remember A. N. A. Um generally even this is asymptomatic unless you have um uh interior UV itis and that's what you see with emphasizes related arthritis. That's sort of like the big red eye. That's very painful um UV itis because it's asymptomatic. Unfortunately if you don't go to screening you can develop chronic disease and not even know until you apply this. Um so screening is key and the DNA helps to sort of determine how often these kids need to be screened. So here there's something kind of for you to screenshot or sort of look through about how often these kids need to be screened. And of course from a rheumatology perspective, when they come to see me, this is something that I keep in mind. But as their primary care physicians and sort of other people on and providers on their teams, it's important to kind of make sure that the kids are doing what they're supposed to be because the consequences are so legit and real right, so the type of J a if they're in a positive or negative and the younger they are, the the more likely they are to develop UV itis. And so these are all things to kind of keep in mind and their earlier on in their disease being to be screened more often and so there is no way to confirm J. A. This is a clinical diagnosis right? And so there is no lab test that confirms it. Um you have to rule out infection or other causes of arthritis and sort of get a good history. Um european east are sometimes are elevated sometimes orange. You know it depends like we talked about before auto antibodies you should are not um diagnostic as we talked about before. It's something that I will get to help especially with an A. And A. To help decide about UV. I do screening does not diagnose aroma to logical disease. And um you know sometimes you do imaging of ultrasounds and MRI's to sort of figure out an X rays to figure out if there's chronic disease um erosions that sort of thing. And so there are our first steps. So if you're thinking about does my patient have J. A. And doing the kind of the initial infectious work up and everything else. Um After you refer them to me you can discuss with them using Motrin and that sort of thing. P. C. O. T. Is really important. And then you know when they see us you know we we if needed we start um rheumatoid demarches or disease modifying anti rheumatic drugs. Um And oftentimes you know labs need to be measured every three months. And so when they see you guys more often sometimes they see us. It's important to make sure that their blood work is is on target and that it's happening to make sure that we're not having real issues, lft elevations um inside of that sort of thing, these kids are at increased risk of infection, right. And so it's really important that they come to see you guys first if infectious um symptoms start to be kind of um swab for flu. Covid strep throughout all those kinds of things. Case # two. So we have 13 year old males coming in with a right knee that's swollen for two weeks. And so what do you think? You know things that you need to know is basically the history of when this all started. What's going on. This child had the most dual several weeks ago and now is having some of the fevers and um had some fevers with that. But they're currently federal. So this is reactive arthritis. So preceding viral illness, especially G. I. Illness followed by several weeks later joined set hurt. And so arthritis occurs immediately or after an extra articular infection. So um it's occurs more more often in adults and Children. So adolescents or people that we want to adolescent age group 3 to 1 male predominance also associate with maybe 27 they tend to have more severe disease and could turn into an arthritis patients and so you need to know about G. I. And G. You infection. So if the kids coming to you with this kind of um um symptom Atala ji besides doing good screening it's important to get um chlamydia gonorrhea that sort of testing. In addition acute HIV can also present with um arthur raja. So um so systemic symptoms that come with reactive arthritis or our throttle weight loss fever, malaise uh took a base metric and large joints and lower extremities. You can see that sort of true. I add right of arthritis, arthritis, nuclear opponent, conductive itis right um from writers try it. Um That's what this is. Um So the questions have already varies. Um So and you know so that's important to kind of talk to families about and can be triggered by a bunch of different pathogens that we talked about before. So you can you know get some different blood work and S. T. A. Screening. That could be helpful treatment is supportive. So n sets cold treatment and avoiding overuse and obviously if any of those geo cultures come back positive treating them and their partners and sort of talking about sex safe sex practices. Um Sometimes if there continues to be reactive arthritis and it takes a while and it's pretty debilitating. Sometimes we can inject story right into that joint that's bothering them. Um If um the reactive arthritis doesn't resolve in about six months then it might be chronic reactive arthritis or presentation of J. Ia. And so then we tend to um you know work that up. So that's sort of how you would treat reactive arthritis in your guesses which they don't need to see room rheumatology. It's just important for you to kind of keep in mind and duration and how severe their disease symptoms are. Lyme disease. So it's really complex disease as you guys know and very regularly cutaneous articular neurological manifestations. Um Some of these components can even happen in isolation and like a timely fashion or altogether uh females and males are affected equally. Um Often times it's painless by after nymphs. Um And so it tends to occur obviously in the northeast mid atlantic and north central us. So how do we diagnose this? It's a two tier testing approach. Right so um uh we get the enzyme immuno assay that's then the I. A. That's followed reflectively by the I. G. M. And I. G. Western blood testing. And so to be positive you need to have I. G. M. Two of the three bands at least and I. G. Five of the 10 bands um generally takes 2 to 3 weeks after a tick bite for antibodies to B. I. G. To become positive and negative. I. G. An untreated patient rules out lime um a positive I. G. M. Alone after four weeks after a tick also is likely to be a false positive and it's not a line. Um I G. G. For limes can persist for many years even after it's been sent to be treated. So it's important to kind of keep that in mind. And positive antibodies are not protective against subsequent episodes of Lyme disease. So there's ever any questions you can always talk to your local infectious disease doctor as well. And so how do we assess patients who have suspected Lyme arthritis? So people who are living in or visiting an endemic area, the presence of documented arthritis. There's no other causes of arthritis. We talked about the enzyme, the E. A. And western blot that should be positive. Um You start therapy which is antibiotics um and then you kind of see how things go. Um Those were kind of the the important thing is to kind of keep in mind um for lives arthritis and so for kids that are younger than eight blocks would be it for 28 days. For kids that are older taxi would be the choice for 28 days. Sometimes you can have post infectious sina bidis in which case that's like the acute infections gum but the kids still have some pain. So sometimes those kids come to see me and will inject a little bit of steroid and and fix them right up so those are other things that rheumatology can help with. Um if there's ever any questions feel free to reach out to me. My email is listed for patient appointments that is the call center to call. Um that number and then option one and then option three. Um And then the Hopkins access line as well for urgent questions. Should there be someone on call and then some questions to kind of see where we are with our learning. Um So you're evaluating a 10 year old girl for joint pains for the last three months. On exams she has symmetric joint symmetric swelling, pain limitation with range of motion of P. I. P. S in our hands risks uh discomfort with her TMJ movement which of the following is most likely so symmetric multiple joints. Three months 10 year old. This is a poly articular J. A patient five year old comes into your office with a few weeks of blimp and pain in her left knee on alexander left knee is mormon swollen although she is a fireball in her office. Her parents say she had a fever all day yesterday you suspect oligarchical day but now you're worried about infection. What test gives you a definitive answer? Um And that would be a synovial fluid analysis. Right? Because if you're worried about an infection you gotta tap that joint one can have an elevated white blood cell count for lots of different reasons. And R. F. Is not diagnostic because you could be positive R. F. And not have any arthritis. And a and a is not diagnostic of any disease ever. Um And can be elevated for lots of different reasons. So here are my references. Thank you so much.
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