Cram The Pance - S1E47 MSK Rheumatology Part 2 of 2

Episode Date: August 29, 2022

MSK Rheumatology part 2 of 2 review for your Pance, Panre, and Eor's. Merchandise Link: https://cram-the-pance.creator-spring.com/►Paypal Donation Link: https://bit.ly/3dxmTql (Thank you!)Included in this review: Polymyositis & Dermatomyositis, Reactive Arthritis, Rheumatoid Arthritis, Sjogren Syndrome, Systemic Sclerosis (Scleroderma), Systemic Lupus Erythematosus.Become a supporter of this podcast: https://www.spreaker.com/podcast/cram-the-pance--5520744/support.

All right, so rheumatology part two. We're going to go over a number of topics, polymyocitis, dermatomyocitis, reactive arthritis, rheumatoid arthritis, shoulderin syndrome, scleroderma, and lupus. So a number of stuff, I'll try to keep it as brief as possible, just the high-yield stuff. If you do want to buy merchandise and support the podcast, I did leave a link in the show notes, just shirts, smugs, things like that. If you want to support the channel, I really appreciate that. And as always, thank you so much for the really nice comments and the support.

Let's go ahead and get started. So we'll start with polymyocitis and dermatomyocitis. I'm going to group these together because they're both idiot. Phyopathic inflammatory myopathies, a lot of overlap. Dermatomyocitis is essentially just polymyocitis with the rash. So don't overcomplicate these because they can easily become overwhelming if you try to memorize every detail. Let's just know the basics for the exam and move on. So real quick overview of the patho, both poly and dermatomyocitis are autoimmune conditions.

Polymyocitis involves CD8 T cells that have essentially gone rogue and are attacking and destroying your muscle fibers. Dermato myocitis, you have an unknown factor that activates your C3 protein. This leads to a cascade of events, which ultimately results in attack and destruction of the muscle capillaries and small arterials. So both poly and dermatomyocitis are much more common in women. So we'll be looking for a female in the vignette. Pretty much every condition I'm going to talk about today, as most of your immune conditions are going to be more common in female. So you'll notice that's the trend today. We're going over these.

Let's talk about clinical manifestations next as well as the physical exam findings because there's a lot of high-yield stuff here. So first, muscle weakness, proximal progressive symmetric. So muscle weakness is the most common clinical manifestation of both poly and dermatomyocitis. Classically, it's going to be symmetric and involve the proximal muscle. So the hips and the shoulders are going to be really common. And it's the reason why in a vignette, the patient they're normally going to mention is going to have difficulties climbing stairs, getting up from a chair, raising their arms above their head. You'll commonly see that in vignettes.

Usually the muscle weakness will be progressive with gradual worsening, over a period of weeks to months. Real quick recap, remember, main clinical manifestations for both is going to be muscle weakness. Muscle weakness will be commonly proximal, so shoulders and hips, progressive over weeks to months and symmetric. So both sides affected. All right. So for polymyocitis, muscle weakness is really the only clinical manifestation you need to know. In dermatomyocitis, though, in addition to the muscle weakness, you also got some pretty high-ield skin stuff. So let's talk about that next. So there's three things you have to know for dermatomyocitis. Gautron Papuels, Heliotrope rash, and photo distributed poikiloderma, WTF.

Let's actually talk about what that means. So Götron Papuels, they're volacious papules that are often scaly and ulcerated that often occur over the bony prominences. Most commonly, you'll see these over the dorsal aspects of the metacarpal phalangeal and interphalangeal joints. So basically you got some scaly scalycysts psoriasis looking rash that's on the top of the hands, mainly over the knuckles. That's Gotron Papules.

Next, Heliotroper rash. So heliotrope rash or heliotrope eruption. This is just a rash on the periorbital skin. So a rash around the eyes. It's most common on the upper eyelid. Just remember a rash surrounding the eyes for heliotrope rash. So Gotron Papuels and Heliotrope rash, those are pathonomonic features of dermatomyocitis. Anytime you see that they mention something is pathonomonic for a certain disease, that should mean to you this is going to be on the exam.

So if you see those two ever mention right away be thinking dermatomyocitis, memorize those. there is one other finding that also seems to come up on exams a lot that's photo distributed poikiloderma or the shaw sign or the v sign it's a complicated name to say but very simple this is a rash or hyperpigmentation that shows up anywhere that's exposed to sunlight but classically and the way that it's going to be in the vignette and the way that you should learn it is on the upper back neck and upper chest which is why they sometimes call it the shawl sign or the v sign because that's kind of where those areas are distributed so again for dermatomyocitis remember Gotron Papules, rash on the knuckles, heliotrope rash, rash around the eyes,

and photodistributed poikiloderma rash around the upper chest and back. I'll have a way for you to remember them at the end. Now for diagnosis, you're going to start with labs, specifically first your muscle enzyme. So there's a bunch of different muscle enzymes you can test for. Creatine kinase, though, is the most sensitive muscle enzyme, and it's the one most commonly tested for, but also be on the lookout for your elevated alde lace. You can even have an elevation of the muscle-derived enzymes like L-D-H-A-S-T and ALT. Next, we have your myocitis-specific antibodies, anti-Joe 1 and anti-Me2. There's actually a bunch of myocitis-specific antibodies, but these are really the only two

that you'll ever really hear about and the ones you should really focus on. I have a couple way to remember these antibodies. One way is to remember instead of dermatomyocytis and polymyocitis, instead remember my-jocytis, like MI-J-O-Sitis. So polymygocytis, dermatomygocytis, and the MI and the J-O and my-Jocytis helps you remember the anti-Joe and anti-Meet-2 antibodies. So that's just kind of how I remembered it. So instead of remembering polymyocytis, remember polymyocytis, MI-J-O, that can kind of help you remember those two.

And then I'll have another mnemonic at the end that I'll go over. Anyways, these myocytis-specific antibodies, they're far from perfect. They're really only positive in around 20 to 40% of people. And they also take weeks to come back. They're just one of those labs that take a long time to get back. But they love to ask about them on exam, so you have to know them. So make sure you're thinking of poly or dermatomyocitis if you see these mentioned. And specifically, if you see anti-meatomyocitis, be thinking of dermatomyocitis as anti-meetumibositis.

They're highly specific for dermatomyocytis. And then you have your biopsies, your muscle and skin biopsy. You're not going to do these in every patient, but if you can't, you're not going to do it in every patient, But if you can't confirm the diagnosis from clinical manifestations and lab results, then you can perform a biopsy to confirm the diagnosis and rule out your other differentials. Now, there's other diagnostic tests that I'm not going to go over, electromyalography, skeletal muscle imaging. For the exam, there's four things you need to know for diagnosis, creatine kinase, anti-Joe and anti-meet two, and then your biopsy in that order, and you're done for diagnosis. Let's talk about treatment next, because really there's only one magic that you need to know, and that's going to be your glucocorticoids. So steroids are your first line treatment.

It's nice and easy. The steroids are started at high doses for the first several months. Then you slowly taper these to the lowest effective dose, normally for a total of around 9 to 12 months. You have some other agents that I wouldn't memorize, but hydroxychloroquine is great to clear up skin manifestations, endermatolyositis, but does nothing for the muscle disease. Then you have some steroid sparing agents like methotrexate that can be used as well. For the exam, high dose glucose corticoids is what you need to know. All right, so between polymyocitis and dermatomyocitis, dermatomyocitis is the one you'll likely get tested on because it has those pathenomonic findings we talked about before, as well as the more specific anti-meat2 antibodies. So that's the one you should focus on, and here's how you're going to remember everything that you need to know for dermatomyocytis.

So first, on the exam, they're not going to give you a 49-year-old female that presents to the office today complaining of Gotron Papuels, heliotroporash, and photodistributed poikiloderma. So it's not crucial to remember those ridiculous names because in the vignette, they're just going to say she presents with a rash or papules on her hands, around her eyelids. It's really important just to know where they're going to have these dermatologic findings and then also remember the specific antimete-2 antibodies. So how do you remember this for the exam? Well, you remember for now on dermatomyocitis in your mind is going to be remembered as permatomyocitis. All you're going to do is replace the D with the P, and now you have perm, P-E-R-M, P-E-R-M, P-A-M-A-Mocytis. Why, perm?

Because whenever you think of this disease, I want you to think of a lady getting a perm, sitting in the chair, her hair is in that little perm helmet thing, and she's getting the works. She's getting her nails done, her eyebrows wax. She's got that cape or little shawl over her shoulders that you normally wear in the salon or the barbershop. And she's just relaxing and having some me time. Me-time spelled with an MI. This is very hard to, without a while. a visual, so check out the YouTube channel if you need one. But basically, that's the visual you need

to create in your head. Lady in a chair, getting her hair permed, getting her nails done, eyebrows waxed with the cape or shawl that you wear in the salon, having some me time, MI time. Now, how does that help you remember what you need to know? While she's getting her eyebrows wax, this helps you remember the heliotrope rash that's common around the eyes, the upper eyelids especially. She's getting her fingernails done. That helps you remember the Gotron Papules that are most common on the top of the fingers. And she's wearing that cape or shawl that helps remember the shawl sign or the photo distributed poikiloderma, which is most common in the upper back, neck and upper chest, exactly where that cape is that you wear, exactly where it's

distributed anytime you get your hair done and have that cape on. And then remember, she's having some me time, me time spelled with an MI that helps you remember the anti-meet two antibodies, which are highly specific for dermatomyocitis. So remember, change the D2 a P, you have permatomyocitis. lady getting a perm, having some me time, her eyebrows waxed, her nails done, and wearing a cape, and that is dermatomyocitis, aka permatomyocitis. Moving on to reactive arthritis. This one's fairly simple. There's really just a few things to know here.

So reactive arthritis is just an arthritis that starts after an infection. That's it. Now, what infection should you be looking for? This is generally going to be a GI or a GU infection, so gastrointestinal or genital urinary infection. So if you suspect reactive arthritis, make sure you're looking for them to mention in the vignette that the patient recently had a bout of diarrhea or your arthritis. So what specific bugs should we be looking for? So when we're talking about our GI bugs or our enteric bacteria, there's a bunch, salmonella,

shegella, campelobacter, C. diff. Don't get too hung up on memorizing those. But when it comes to your GU bugs, your genital pathogens, there's really only one you need to know. And that's chlamydia, chlamydia trachomatous. So if you only remember one infective organism that can lead to a reaffiruses, that can lead to reactive arthritis, definitely let it be chlamydia. This is the one that will often give you in the vignette. So remember that. Clinical manifestations. So generally, one to four weeks after infection, your clinical manifestations will start to pop up. The immune system commonly targets the lining

of the joint spaces, which is why we get our arthritis, but it can affect a number of other areas as well that will go over. So first, let's start with the peripheral arthritis. This is commonly going to be asymmetric oligo arthritis, mainly affecting the lower extremities, especially the knee. Some other MSK manifestations just to be familiar with, they may have anthicitis, which is an inflammation of the insertion site of tendons. They may have dactylitis, which is an inflammation of the digits, sometimes referred to as sausage digits. This is going to be more common in your patients with chlamydia infection. And that's basically all you need to know for your MSK stuff. Mainly focus on the arthritis. Next, let's talk about some of the extra

articular signs and symptoms. There's two that you need to be familiar with. So first is your ocular symptoms. So inflammation of the eye, whether it's conjunctiveitis, anterior uveitis, keratitis, the eye, whatever part is inflamed. And then we have our GU symptoms. So dysuria, pelvic pain, urethritis, cervacitis, prostateitis. So the three things I just went over form this triad. Triad consists of arthritis, conjunctivitis, and urethritis. This is actually only going to occur in a small subset of patients around a third of all cases. There's definitely other clinical manifestations that can present from this condition, oral lesions, cardiac manifestations. But for the exam, you damn well better know that triad because they love to ask about it. So how do you remember the triad?

Well, you can remember this famous demonic. It's not mine, but it's a good one. And it's can't pee, can't see, can't climb a tree. So they can't pee because of the genital urinary symptoms, the urethritis. They can't see because of the ocular symptoms, the conjunctivitis. And they can't climb a tree because of the arthritis. So can't pee, can't see, can't climb a tree. And you remember the clinical manifestations you need to know for reactive arthritis. Now, clinical diagnosis is going to be a clinical diagnosis. It's because there's no single definitive diagnostic test. There's no validated diagnostic criteria. Even if you're getting cultures to check for an underlying infection, often by the time the arthritis presents, the pathogens may no longer be retrievable. So the idea here,

this isn't something you need to memorize for the exam, but you're basically looking for three things. One, does this patient have the characteristic arthritis, MSK findings we described before? asymmetric involvement, enthesitis, dactylitis. Two, do they have evidence of a preceding infection, diarrhea, urethritis? And then number three, is there a lack of convincing evidence for another more likely diagnosis like psoriotic arthritis, rheumatoid arthritis? And FYI, if they have joint effusion, you do want to do an author or centesis to rule out septic arthritis, which can present in a similar way. So again, this is a clinical diagnosis. There's nothing really to know for the exam. In real life, do they have the typical MSK symptoms? Did they have a preceding

infection and have you ruled out other possible causes, and that's it to make the diagnosis, with one little exception that I did want to mention, because this may come up. So something known as HLAB-27, in case you hear about human leukocyte antigen B-27, know that this is found to be positive in around 30 to 50 percent of patients with reactive arthritis. So it's something that if it's positive can help support the diagnosis, but by no means does a negative test rule out reactive arthritis. So just a little FYI in case that comes up. So for treatment, there's not a lot to know here. Basically, first treat the underlying infection. This is pretty straightforward if they have chlamydia, give them doxy, et cetera. For the arthritis, there is one main treatment option that's

agreed upon first line, though, that you should know, and that's what you should focus on. And that's NSEDs. So NSEDs are first line treatment for arthritis. So unless contraindicated, insides are going to be what you're going to use for the arthritis. If they are refractory to the NSAD, you can use steroids. It's rare to require disease-modifying agents like methotrexate that we use in other types of arthritis like RA because the symptoms of most of these patients is generally self-limited. So basically for the treatment, focus on your insets. That's the key takeaway here. So three main high-yield things to know for reactive arthritis. One, look for your preceding infection, GI bug, or chlamydia.

Two, remember, can't pee, can't see, can't climb a tree for your clinical manifestations. And three, remember, treat the underlying infection, if need be, and inseds for your arthritis. and that's all you need to know for reactive arthritis. Let's talk about rheumatoid arthritis next. So this is a monster of a topic. There's a decent amount to know here. Just focus on the need to know stuff. So rheumatoid arthritis is a chronic systemic, inflammatory autoimmune disease of unknown etiology

that can lead to the destruction and deformity of joints due to erosion of cartilage and bone. Let's do a 30-second patho breakdown in rheumatoid arthritis. So RA, like so many things in rheumatology, is an autoimmune disease. So we have these T cells that are recruiting macrophages. They're all just hanging out in the joint space, producing cytokines unnecessarily. This is leading to a bunch of inflammation, and then this is the important part. These cytokines that are being produced in the joint, they're also causing the synovial cells to proliferate. So your synovial cells form your synovial membrane.

Remember, that's your connective tissue that lines the joint space. So synovial cells are proliferating, and this causes this thick, inflamed synovial membrane, which is called a panis. And this panis is growing in the joint. Eventually runs out of space. The panis gets bigger and bigger. Eventually starts breaking down and eroding the joint space. And this leads to our clinical manifestations.

So again, cytokines are causing this panis to grow bigger and bigger, eventually too big and it just starts breaking stuff inside the joint. That's your 32nd patho explanation. Next, who are you going to see this in? So that's going to be female. So incidence of RA is twice as high among females. females compared to males. So very likely for this to be a female in the vignette. Number of other risk factors, lower socioeconomic status, cigarette smoking obesity, just really focus on the female

sex, though. Let's move out to clinical manifestations because there's a lot of high-yield stuff here. So there's a lot of things to know for the clinical manifestations for RA, but luckily they all start with an S, or at least I've created away from them to all start with an S for the sake of memorization. So if you can remember your seven S's for clinical manifestations for RA, you're good for the exam. And of course, I'll have a way for you to remember them at the end. So the first S is symmetric. So symmetric joint involvement in rheumatoid arthritis is a characteristic feature, meaning if the left hand is affected, the right hand will likely be affected too. This is obviously in real life isn't 100%. It may be less apparent early on in the disease.

But what you need to start doing is separating real life findings from exam questions because they're two completely different things. You're studying for the exam and an exam is going to give you the most common presentation. So for an exam question, they're going to describe symmetric joint involvement because that's the most common. So count on that. Next S is going to be swollen. This is an inflammatory arthritis. So it makes sense the affected joints would be swollen, tender, and warm. Next S is soft. So soft, warm, boggy joints are typical of R.A. If they mention the joints are hard and bony, this is not RA. You should be thinking of osteoarthritis instead. So remember, soft boggy joints. Next S, small joints. So rheumatoid arthritis has a predilic.

for the small joints, hands, feet, wrist. So it's not impossible, but less likely you'll see this in the shoulders, hips, spine, etc. So remember, your small joints are more common to be affected, hands, feet, wrist. Next, S, spares the DIP. So classically, RA spares the last knuckle, the distal interphalangeal joint, the DIP. It's not impossible for the DIP to be affected, but if you see DIP involvement, this is more likely to suggest a diagnosis of osteoarthritis rather than R.A. So remember, R.A. Classically, bears the DIP. Next S, 60 minutes or more. So in all types of inflammatory polyarthritis, like rheumatoid arthritis, you're looking for morning stiffness for extended periods of time.

Depending on the literature, some are going to say over 30 minutes, others are going to say over 60 minutes. But the key here is that the morning stiffness in RA is sustained, and that's what's going to help you differentiate from osteoarthritis, where the morning stiffness and osteoarthritis, if it's present at all, is usually transient or only lasts for a few minutes at most. Last S, swan neck deformity. So there's a number of different joint deformities that RA can cause. Buteneer, swan neck, bowstring deformity. We don't see them as often as we once did due to the early innervation of demards.

But I do remember swan neck deformity coming up on an exam question. So if you see this mentioned, this is typically seen in advanced RA and commonly caused from the erosion of the extensor tendon from chronic synovitis. It causes the DIP joint to flex and the PIP joint to hyperextend. So the middle knuckle is hyper extended. Last knuckle is flexed. And it kind of looks like a swan neck. So that's your last S. All right. So for clinical manifestations and physical exam findings, remember your seven S's, symmetric, swollen, soft, small joints, spares the DIP, 60 minutes, and swan neck deformity. If you need help remembering those seven S's, I got you. Here's the second part of the mnemonic. So I used to remember rheumatoid arthritis, like with seven S's at the end. So rheumatoid arthritis. And when you say that out loud, what do you sound like? A snake. So every time you see rheumatoid arthritis on an exam question in school, say it like that in your head, not out loud. And then picture a snake. But this is not just any snake. It's a stuffed animal snake. So like a little plush toy snake. Stuffed animal snake is obviously soft. That helps remember the joints are soft and boggy. Next, this snake oddly has hands and feet. That's very weird. Snakes don't normally have that. But this helps remember that the RA commonly the small joints of the hands and feet. Next, the snake is holding an identical timer in each hand, and each time is set at exactly 60 minutes. So the identical timer in each hand helps you remember the symmetric findings, because the timers are identical. And the fact that the timers are set at 60

minutes helps you remember the morning stiffness for, you know, 60 minutes or more over 60 minutes. Next, his stomach is super swollen, like most snakes when they eat too much, you know, they have that like big swollen belly. And the swollen stomach helps you remember the joints will be swollen in And what did he eat that made his stomach so swollen? Well, if we look inside his stomach, we see two things. A swan and a bowl of asparagus dip. That helps, uh, the swan helps you to remember the swan neck deformity. And the asparagus dip he ate helps you remember spares the DIP, a spare agis dip.

DIP, a spare, I guess, DIP dip. So that visual combined helps remember the seven S's of rheumatoid arthritis. So quick recap, you have a soft plush snake helps remember soft joints. He's got human hands and feet, affects the small joints of the hand and feet. He's holding an identical timer in each hand set to 60 minutes, symmetric and morning stiffness over 60 minutes. It's got a swollen stomach, swollen joints because he ate a swan and asparagus dip. Swan neck deformity spares the DIP.

For my podcast listeners, definitely check out the YouTube channel. I made an amazingly horrible snake in Microsoft Paint that helps paint this visual. All right. So next let's talk about diagnosis. There's a number of different tests in labs for diagnosis of R.A. but there's two that you absolutely need to know, and that's your rheumatoid factor and your anti-CCP. So let's start with the rheumatoid factor. So rheumatoid factor will be positive in 70 to 80 percent of patients with RA.

So it's a very sensitive test. Problem is it's not very specific. Even healthy individuals can be positive for rheumatoid factor. Patients with lupus, hepatitis C, a bunch of other inflammatory conditions can all wind up with a positive rheumatoid factor. So it's a sensitive test. It's just not very specific. So if we want a specific test, that's when we use our anti-CCP, also known as our antithrullinated peptide antibodies or anticyclic citrullinated peptide. That's why we just call it our anti-CCP.

So this test is very specific of rheumatoid arthritis, meaning if it's positive, this is very likely going to be RA and not something else. The specificity is generally over 90%. Some literature are stating as high as 95 to 98% specific for R.A. Now I'm not going to get into the necessary details, but both of these can actually be negative, the rheumatoid factor and anti-CCP. They can both be negative. And you can actually still make the diagnosis of RA. It's called zero-negative RA.

Just a little extra knowledge, not really anything to know for the exam. So in conclusion, know your rheumatoid factor, know your anti-CC. And a little quick tip, if you get an exam question and they say, which of the following is most specific for the diagnosis of rheumatoid arthritis and you've narrowed it down to your rheumatoid. rheumatoid factor and your anti-CCP, and you can't remember which one it is. Remember the word specific in it, has two Cs in it, and so does anti-CCP. At least that helped me on the exam. So two things again, a note for diagnosis, rheumatoid factor and anti-CCP. That's the high-yield stuff. There's obviously other modalities you can use in diagnosis, x-ray ultrasound, but for the exam, so they're likely

going to mention your serologies and that's what you need to know. Let's talk about treatment next. Now two things you need to know for treatment, that's enseds and demards. So n-s plain and simple, They're just for symptom control. If the patient has contraindications to nseds or inadequate response to nsids, you can give them gluca corticoids like in really anything else. Not much to know here except for the fact that inseds, they're only going to treat the inflammation and pain. They don't do anything to alter the course of the disease or to prevent the destruction it can cause.

And that's why your next class of meds is the most important for rheumatoid arthritis. And that's your demarred. So disease modifying antiromatic drugs. There's a few meds in this class, hydroxychloroquium. peroquin, sulfosalazine, but if you're going to memorize one, make sure it's methotrexate. Methotrexate is the most commonly used demarred because compared to the other meds, it has a faster onset of action, greater efficacy, better long-term tolerance. So just remember methotrexate and you should be good for the exam.

Let's talk about why it's so important to start patients on this class of meds in the first place. So patients diagnosed with RA should be started on these drugs as soon as possible because with RA, once the damage is done, it's irreversible. There's no going back. So starting this class early on in the diagnosis can reduce or prevent this irreversible joint damage. So remember, unless there's a contraindication, everybody with RA will get a DMARD and that DMARD will likely be methotrexate. So for an exam standpoint, for treatment, remember two things, inseds for symptoms and DMARs to prevent disease progression.

In real life, obviously, it's a little bit more complicated than that. There's other options. But for the exam, that's what you need to know. All right. So for rheumatoid arthritis, there's a lot of info. What do you absolutely need to know? Remember your 7 S is for clinical manifestations, symmetric, soft, swollen, small joints, 60 minutes or more.

Spare the DIP and swan neck. Remember your rheumatoid factor in your anti-CCP for diagnosis. Anti-CCP is the specific one. Remember two seasons specific and anti-CCP. And the main takeaway with treatment is your DMARTS, specifically methotrexate, and that is rheumatoid arthritis. Moving on to Shogran syndrome. So there's just a few things to know here.

This is a systemic autoimmune disease characterized, by lymphocytic infiltration of exocrine glands. So there's a problem with the exocrine glands. There's this autoimmune induced inflammation of the exocrine glands, especially the lacrimal and the salivary glands. This leads to diminished function of these glands with result in dryness of the eyes and the mouth, among other problems. You have primary and secondary types of this condition.

Primary isn't associated with any other conditions where secondary is associated with another underlying rheumatic disease, like rheumatoid arthritis, lupus. etc. Your focus should be on the primary type. So clinical manifestations, there's three things you need to know when it comes to Shogran syndrome. That's dry eyes, dry mouth, parodic glands swelling. Those are the most common ones you need to know that will definitely be in the vignette. In real life, the disease spectrum is much more broad. It's a systemic disorder and you can have extra glandular organ involvement of the skin, joints, heart, GI tract, but for the exam, all you need to memorize is dry eyes, which is also known as Corrado Conjunctivitis Sica. A patient may complain of irritation,

grittiness, itching, a foreign sensation in the eye. So look out for that. Next, dry mouth, which is also known as zero stomia. By the way, these two symptoms, dry eye and dry mouth, will be the most common. Dry eyes and dry mouth are present in over 85% of patients with primary chogren syndrome. And then the last one, parotid swelling. So other salivary glands can be involved like the submandibular, but the parotid is going to be the most common. So again, for the exam, if you see dry eyes, dry mouth, parodic gland swelling, be thinking chogren syndrome. Next, diagnosis. So for diagnosis, there is no one single best test to diagnosis condition. Make the diagnosis based on the appropriate clinical features combined with laboratory testing, but no one specific test rules in or out the disease. With that kept in mind, let's talk about the two diagnostic tests they will likely ask you about on the exam. First one is your anti-Roe and anti-law antibodies, anti-Row, also known as SSB. This is probably the one you're going to be tested on. Anywhere from 60 to 80% of patients with primary chogrin's will test positive for one or both of these antibodies.

The problem is these can also be positive in patients with lupus, even some healthy individuals without chagrin's. So it's a good test, but it's obviously not perfect. You'll usually order these antibodies in conjunction with your A&A and your rheumatoid factor. And then the other diagnostic test that you should know one that's often tested on is the Schumer test. So the shirmer test is quite literally taking a dry piece of sterile paper and putting it in someone's eye you suspect to chogrin's. you measure how wet the paper gets over a period of five minutes. That's really it. It's a simple idea, but it works well, and you should know it for the exam. And then just the final note, if there's still any lingering doubt about the diagnosis after doing your labs, physical exam,

etc., you can use a salivary gland biopsy to establish the diagnosis. So outside of the clinical manifestations, really the highest you'll thing to know for chogrens are the diagnostic tests that went over. You need to remember them. The way that I used to remember anti-Row and anti-law and the Shermer test is by instead of remembering Shogran syndrome, I would remember Slow-Green syndrome. So instead of Shogran, slow-green, slow-green syndrome. So slow-green instead of show-green. And what slow and green, a frog, to remember a slow-green frog, landed in my cup of Sherbert.

So create that visual in your head. You have a cup of sherbert, that little frozen fruit treat, and a frog landed right in it. So show-green is now slow-green. and a slow green frog landed in my cup of Sherbert. So Frog, the second two letters are RO. That helps you remember anti-R-R-O. Landed, first two letters, L.A. helps you remember anti-law.

And then Sherbert helps you remember the Shermer test. So that worked for me. Just remember Slow Green instead of show-green. And remember that slow-green frog landing in your cup of Sherbert. Remember second two letters of Frog, R-R-O. First two letters of landing are LA. And then Sherbert helps you remember Shermer. Remember that visual.

So for treatment, it's not very much to know here. If their eyes are dry, give them artificial tears. For dry mouth, make sure they're staying hydrated, stop smoking. They can use salivary stimulants like sugar-free candies or lozenges. That stuff's usually not tested on, but what they will test you on is the muscarinic agonus or your cholinergic, specifically pylacarpine and syvemylene. These meds stimulate secretion of the exocrine glands opposite of your antichs like atropine and atrovent that most of us are more familiar with that can dry you out.

So if they ask you what meant to put a patient on the chogrin's, remember your colonergics, pylcarpine, syvemoline. All right. So that is chogrens. Just a few takeaways here. It's an autoimmune disorder of the exocrine glands. So remember your dry eyes, dry mouth, parotic gland enlargement. Remember your row and law and your shirmer test for diagnosis.

And then treatment, just remember, pyloprine and syvimoline, and you're good to go. Let's talk about systemic sclerosis or scleroderma next. So this is a systemic connective tissue disease where excessive collagen deposition leads to progressive fibrosis of the skin and internal organs. So the patho is not completely understood, but what we do know is there is a genetic predisposition combined with some sort of triggering event, like a viral infection, drugs, etc. This is followed by an endothelial injury. Our T cells come in, start overproducing cytokines, causing excessive inflammation. The cytokines activate fibroblasts, and the fibroblast, this is the important part, start depositing collagen. And because of this, our nice, soft and squishy

skin and organs are now being replaced with this hardened fibotic tissue. And this fibrosis also causes reduced blood flow and result in ischemic tissue damage. And this combination is what leads to our clinical manifestations. So in conclusion, too damn much collagen and fibrosis. So this is going to be much more likely in the female population. So the female to male ratio can be as high as 8 to 1, like pretty much everything we're talking about today. So much more common in women.

There's a few different types. systemic sclerosis is generally classified based on the extent of skin involvement and organ involvement. There's really only two main types that you need to know. The first is limited cutaneous systemic sclerosis, aka Crest syndrome. So for limited disease, I just want you to remember two things. One, it normally spares the trunk, that's important, mostly affects the distal limbs. And then the second part, and probably the most important thing to know, is this limited form of the disease is associated with something called Crest syndrome. So what is Crest syndrome? Well, luckily, Crest actually comes packaged with its own mnemonic. That explains exactly what it is.

So Crest actually stands for. The C stands for Calcinosis Qus. R stands for Reynodysenotinon. The R stands for Acetyl. The S stands for sclerodactyl. And then the T stands for telangiotasia. So let's talk about what of these actually mean. So Calcinosis Qudus is a calcium deposition in the skin, so you just get these clumps of calcium deposits all over. Raynod phenomenon is the same thing that happens when your fingertips get really cold and vaso-constrict and they turn white and blue. This is just an exaggerated form of this and it can also happen in response to stress. Asophageal dysmotility, this one's pretty straightforward.

They may have dysphasia, choking, gurd, etc. Sclerodactyl is this claw-like appearance of the hand due to the tightening of the skin of the fingers. And sclerodactyl sounds very much like teradactyl, which is that big bird dinosaur, bird with the clawed feet. So teradactyl clawed feet, sclerodactylate, clawed hand. So that kind of helped me to remember it. And then finally, telangiocetia, which is in so many other conditions. So probably not the first time you're hearing this term. But all this is, is these dilated superficial vessels that create this web-like appearance on the skin. So that's your limited form of the disease.

Again, remember two things. One, this mainly occurs in the distal limbs, but generally spares the trunk. And then two, remember, Crest Syndrome, Calcinosis, Raynoughts, Sophogil, Scleradactyl, and Talangiocetacea. Moving on to diffuse cutaneous systemic sclerosis. So the word diffuse is in the name. So as you'd imagine, this is more widespread. So the areas we discussed before that limited sclerosis didn't really affect, like the trunk, for instance, in proximal limbs, diffuse will affect this area. This, of course, isn't 100% in real life, but for the exam, definitely learn it that way. Biggest takeaway with diffuse cutaneous systemic sclerosis is that it involves the organs.

That's what you need to remember. These patients are much more likely to have involvement of the internal organs, especially the heart, lungs, and kidneys. So look for lung fibrosis, scleroderma, renal crisis. That's the key here. So recap for diffuse disease, remember trunk involvement and organ involvement. Heart, lung, and kidneys. For limited disease, remember the distal limbs and spares the trunk.

And then, of course, Crest syndrome. One last time, even more condensed. Diffuse, trunk and organs, limited, distal limbs, and crest. Done. That's it. Don't make it more complicated than it has to be. Let's move on to diagnosis. So for diagnosis, there's really two labs you need to know.

The first is your antiscentromere antibody. So the presence of this antibody is most often associated with the limited subtype. Only about 5% of patients with this antibody will have diffuse disease. So if they give you a positive anticentromere antibody on an exam question, and they ask you which is the most likely diagnosis is 100% on an exam question, limited disease, aka Crest syndrome. And that's why instead of remembering this antibody is anticentromere, you're going to remember it as antichristomere.

So no longer anticentromere, it is now known as antichristomere. Just replace it in your head and you'll get the question right. Next antibody is your anti-topo isomerase, also known as your anti-SCO-70 antibody. This one you need to know is most commonly associated with. diffuse disease and it also carries a higher risk of severe interstitial lung disease if positive. So remember, antichristomere associated with limited disease, anti-topoe isomerase, SCL70 antibody associated with diffuse disease. There's a couple other labs you can use.

Of course, your ANA, your anti-nuclear antibody, which is a very sensitive test and it'll be positive in approximately 95% of patients, but it's just not specific for really anything. It can be positive in so many other conditions. your anti-RNA polymerase 3 antibody. For the exam, though, focus on your anti-centromere, and a.karykate, and your antiproisomarase. That's what they're going to test you on. All right, so for treatment, it's unlikely they're going to ask you about treatment

because treatment is mainly targeted at treating the symptoms or organ that's involved. So if they have GERD, you're going to give them a PPI, if they have Raynod phenomenon, give them a calcium channel blocker, renal problems, give them an ACE inhibitor. Patients with severe inflammatory organ involved, or diffuse skin involvement are usually treated more aggressively with systemic immunosuppressive therapy like methotrexate. There's no first line agent and certainly nothing I feel is necessary to memorize for the exam.

They're going to ask you a question. It will be about the clinical manifestations or the antibodies I went over in diagnosis. So focus on those. So key takeaway here. First, this will be a female in the vignette. Know your two main types, limited, crest, and diffuse. know that limited involves mainly the distal limbs and your crest findings but spares the trunk. Know that diffuse involves the trunk and your organs.

No antacentromere, aka antichristomere, is associated with limited or crest disease. And then antitopal isomarase is more likely to be associated with diffuse disease. Treatment, again, is organ symptoms specific. Done. Last topic is going to be systemic lupus erymatosis. Another really big topic, but let's distill it down as best we can. So this is a multi-system immune-mediated disorder characterized by antibodies to nuclear and cytoplasmic antigens. So the specific cause of SLE is unknown, but we do know that many of the clinical manifestations that we see are caused by antibody formation and the creation of immune complexes.

So these complexes deposit all over the body, the heart, kidneys, skin, etc. They cause a number of problems, inflammation, tissue damage, cell death. This whole process, by the way, is a type 3 hypersensitivity reaction. Surprise, surprise. This is much more common in women, especially women of childbearing years. So look for a younger female in the vignette. So a couple of reasons why, which I'm not going to get into, but it has to do with estrogen, also some factors related to the X chromosome, which seem to play a role.

This is also going to be more common in African American patients. In the U.S., African American women are two to four times more likely to have SLE than Caucasian women. So the vignette, I would be looking for an African American female. clinical manifestations literally everything you can think of hypertension arthritis alopecia lymphatineopathy dysphasia thrombosis fever weight loss fatigue lupus is the great imitator it affects almost every organ therefore it can present like so many different diseases there's a ton of clinical manifestations and it would be very unwise to memorize them all let's just talk about the ones that'll likely test you on and the more common one so first you have your constitutional symptoms fatigue fever weight

loss. These are all very non-specific, but almost every patient with SLE will have these symptoms at some point during the course of their disease. So they're very common. They're just not very specific. Next, rash in a malar distribution, aka your butterfly rash, sparing the nasal labial folds, ring the alarms. This is the thing to know when we're talking about clinical manifestations for lupus. If you can only pick one thing to know for the exam for clinical manifestations, this is going to be it. So a malar rash can occur in other conditions, but if you see it on an exam question, especially if they say it spares the nasal labial folds, it is lupus. So it's the most common skin lesion I have in lupus, and it presents as erythema in a malar distribution over the cheeks and nose sparing the nasal labial folds. It normally appears after sun exposure or almost always appears after sun exposure.

So you have this photosensitive rash that shows up. And remember that word photosensitivity, as it's really important because, because many of the skin manifestations in lupus appear after sun exposure. The rash looks like a butterfly over the nose and cheeks, and it's in a malar distribution. In case you're not familiar with the term, maylar is just another name for the zygomatic bone of the face, which are your cheekbones.

So basically, malar distribution just means over the cheekbone area. Remember, this rash spares the nasal labial folds, which are the little creases from the corner of your nose to the corners of your mouth, and this gives it that butterfly wing appearance. So remember, rash over the cheeks and nose sparing the nasal labial folds, Fults after sun exposure, remember that rash, memorize it, know it well, it will come up. Next, your discoid lesions. While we're on the topic of skin manifestations, the next one that you should know is your

discoid lesions or discoid rash or discoid lupus, however you see it come up. These are these round or coin-shaped erythematous raised patches that often cause this pretty dramatic atrophic scarring. Just an FYI, if you know who the singer Seal is, who's really big in the 90s, he sang that song, Kiss for Marose. The scars in his face are actually from disquoid lupus lesions. So maybe they'll help you remember to associate those two. Othritis, so arthritis and arthrogyas are going to be seen in around 90% of patients with SLE.

It's often one of the earliest manifestations. And a lot of times it's the reason why these patients come at the office to be seen to begin with. And then we have our cardiovascular findings. So paracarditis is going to be the most common cardiac complication of SLE. So that'd be the one to focus on. But they can also have issues related to endocarditis. high risk of myocardial infarction. So just remember the increased risk of cardiovascular problems in these patients. Next, renal. So around 50% of patients will have some sort of renal manifestation,

whether it's hematuria, proneria, nephrodic syndrome, glomerul and nephritis. Secondary hypertension, look for problems with the kidneys in patients with SLE. Thrombolic disease. So thromboembolic disease is a potential complication of SLE, particularly in the context of antifospholipid antibodies. The 10-year risk of a thrombotic event for a patient with SLE is actually around 33%. And then we have our hematologic abnormality. So all three blood cell lines can be affected in patients with SLE. So your red blood cells, white blood cells, platelets. So I'll be looking for anemia, leukopenia, thrombocytopenia.

So even though I just went over a ton of clinical manifestations, there's a bunch that I didn't even touch on. And I'm not going to. Neurologic problems like stroke, seizure, GI problems like esophagitis, pancreatitis, pulmonary issues like pleuritis pneumitis oral ulcers the list just goes on and on but the ones i went over those are the ones that i'd focus on those are the ones that often come up on exam questions and the way if you want to remember those is by remembering the mnemonic md chart so md chart um and md chart like doctor medical doctor chart like a doctor's chart um that stands for the m stands for may lar rash the d stands for discoid lesions and then the chart part of it the ccise

stands for both constitutional and cardiovascular manifestations. The H stands for hematologic abnormalities. The A stands for arthritis. The R stands for renal. And then thromboemabolic disease is what the T stands for. So just remember, if you want to remember some of the main clinical manifestations, remember MD chart. And that's how you remember some of the main ones. So for diagnosis of lupus, like so many things in rheumatology, it's a complicated one. It's not often based off of any single lab result or imaging study, but rather recognizing the spectrum. of clinical manifestations accompanied by supportive serologic studies and excluding a number of alternative diagnoses. With that being said from an exam standpoint, it's a bit easier, as it often is. There's really just a few labs that you need to know. So first let's start

with your ANA, your anti-nuclear antibodies. I've mentioned this in some of the other ones too, but this is your screening test. This is where you start your anti-nuclear antibodies, ANA, because this test is very sensitive. It's positive in virtually all patients with S.S. The problem is it's not very specific. It's positive in a number of other conditions. The reason you get this test is because if it's negative, well, not to say that it's impossible for the still to be lupus, but much, much less likely. So screen with your ANA. If they have lupus, around 95% chance, this is going to be positive. And then you move on to your more specific labs, the ones that you really need to know. And first, that's going to be your anti-DS DNA or anti-double-stranded DNA, and then your anti-Smith antibody. Those are the two that you really need to focus on for lupus. They're both highly specific for lupus. So remember that. Smith and double-stranded DNA associate those two very important.

So anti-D-S-D-Na, anti-D-Nand-D-A, anti-Smith antibodies. Very specific for lupus. You need to know those. And then I have a mnemonic for those two. This is definitely a weird one, but sometimes the more weird it is, the easier it's to remember. So the word lupus sounds like the name Lou, like L-O-U, and piss, like taking a piss, peeing. So I used to remember a guy named Lou taking a piss on his Smith and Wesson double barrel shotgun. Not sure why he's peeing on a shotgun.

Maybe he's very anti-gun. Anyway, Smith and Weston is the famous gun manufacturer and a double-barrow shotgun is, well, a double-barrow shotgun. So Smith and Weston helped me remember the anti-Smith antibodies. And double-barrel shotgun helped me remember the anti-double-stranded DNA antibodies. So remember when you see lupus, I want you to think of a guy named Lou taking a piss on a Smith-and-Wessel double-barrel shotgun, create that visual. in your head and you'll remember the two main labs you need to know for lupus, anti-Smith and anti-double-stranded DNA. One other lab I wanted to really quickly mention that you should be on the lookout for

is your antifospholipid antibodies. 40% of patients with SLE will be positive for antifosophyllipid antibodies, which is associated with antifosolipid syndrome, and patients with the syndrome at a high risk for venous or arteriophthalmosis miscarriages. So I just wanted to mention that in case it does come up. Treatment. So there is a lot to know overall for lupus. but the good thing is for meds, there's really only one medication that you should focus on for the exam,

and that's hydroxychloroquine. So all patients with lupus need to be on hydroxychloroquine. Lupus, hydroxychloroquine, lupus, hydropsicloroquine, lupus. Associate the two in your brain forever. This medication improves constitutional and MSK symptoms. It improves the mucocutaneous manifestations. It even has a positive impact on patient survival. So remember this medication for lupus, and then also be aware of your steroids and ensets, some immunosuppressive agents like mycophenylate can also be used for treatment in combination with hydroxychloroquine. Steroids are especially helpful in severe or life-threatening cases where you can use these high-dose iv pulses of methyl prednisone to control the disease and halt tissue injury. But the main takeaway here is just to remember hydroxychloroquine.

So lupus triple distilled, this will be a female in the vignette. She will likely present with the MD chart manifestations we went over. Screen with your ANA.N. Then remember, Lou took a piss on a Smith and West Endo Bowerowbrose shotgun, anti-Smith and anti-Double-Stranded DNA. Those are the specific ones. Treatment, remember hydroxychloroquine, and that is lupus. Let's wrap it up with five quick questions. Question one, a 42-year-old female presents to the office complaining of heartburn, small white lumps on her fingers, as well as a tight feeling in her hands that makes it difficult

to make a fist. On physical exam, you note telangiactas on the palms and face. Labs are positive for both anti-nuclear antibodies, as well as antacetromere antibodies. What diagnosis should be suspected in this patient? So that would be limited systemic sclerosis, aka Crest. So this patient has a very classic presentation and presents with a number of the manifestations of Crest syndrome. So we see the calcinosis cutus, those small white calcium deposits in her hands.

She has heartburn, which is from the esophageal dysmultility disorder, telangiocetias, as well as the tightening of the skin of the hands. This is a classic limited systemic sclerosis, which we know will generally have a positive ANA and then most importantly, a positive antacentromere antibody test, aka antichristomere. Two, a 47-year-old female presents to the office complaining of dry mouth and dry eyes for several months. She has used over-the-counter eye drops with minimal improvement. Physical exam reveals dry mucus membranes and swollen parotid glands. You explained to the patient you'll be performing a test to assess for tier production. What is the name of the test that will be performed?

So that is going to be the Shermer test. So this patient very lucky. likely has Shogran syndrome. Of course, we would also need to perform some labs, anti-row, anti-law, A&A, but she has all of the classic clinical manifestations, dry eyes, dry mouth, parodic gland enlargement, and the test we performed to assess for tear production is the one called the Schumer test. Question three, a 51-year-old female presents to the office today complaining of muscle weakness. She describes difficulty combing her hair, rising from a chair. On physical exam, you know a rash around the eyes and the eyelids, violaceous papules of the dorsal aspect of both hands, as well as erythema across the shoulders

upper back and upper chest. Labs are drawn, which show an elevated creatine kinase level, as well as a positive anti-me2 antibodies. What treatment should be initiated in this patient for the suspected diagnosis? So that is going to be glucocorticoids. So this patient has dermatomyocytitis. She has the Gautron Papules, heliotrope rash, decreased muscle strength, the shawl sign, plus elevated CK and anti-meatoo antibodies. That's about as clear cut as you can get. And then we know for dermatomyocytos are the cornerstone of your initial therapy. This is usually prednisone at a dose of 1 milligram per kilogram per day. Question four, a 41-year-old female has symptoms consistent with rheumatoid arthritis and labs are drawn to assist in making the diagnosis. The physician-assistant

informs the patient that a rheumatoid factor, as well as a very specific antibody for rheumatoid arthritis are both elevated, which antibody specific to rheumatoid arthritis is likely elevated in this patient. So that is going to be your antichralunated peptide antibodies, aka your anti-CCP antibodies. So your anti-CC antibodies are very specific for rheumatoid arthritis, usually over 90% specific to the disease. So if they ask for the most specific test for RA, generally this is going to be your anti-CC, whereas rheumatoid factor is more sensitive, but not as specific. And as I mentioned before, if you can remember which is the specific antibody for RA. Specific is spelled with two Cs.

Look for the antibody that has two Cs in it. That's your anti-CCP. Question five, last one to 26-year-old female presents to the office complaining of fatigue, joint pain, and a low-grade fever for the past few weeks. She also reports that she develops a painful rash after being in the sun just for a short period of time. On physical exam, you notice a rash that is distributed over the cheeks and no sparing the nasal labial folds, as well as diffuse disquois lesions.

What would be the best initial test to order in this patient? So that is going to be your anti-nuclear antibody, your ANA. So this patient has systemic lupus erymatosis, or obviously this should be at the top of your list of differentials. Anytime you have a young female of childbearing age, complaining of joint pain, rash, and fever, always be considering lupus. On exam, she has the classic Malar butterfly rash. that spares the nasolabial folds, as well as the disquoid lesions.

And she describes a photosensitive rash that burn after a short period of time in the sun. So the best initial or screening test in a patient you suspect may have lupus is going to be your ANA, anti-nuclear antibodies. So it's not a specific test, but very sensitive. And this is where you'll always start when screening for lupus. Then after, obviously, you proceed to your more specific antibodies, your anti-double-stranded DNA, and your anti-Smith antibodies. All right.

So that was Rheumatology Part 2. I hope that was helpful. Thank you, as always, for listening to the podcast, and good luck on your pants, your pan or your EORs, and good luck in PA school.