WEBVTT 1 00:00:00.840 --> 00:00:03.240 All right, So Rheumatology Part two. We're going to go over a number 2 00:00:03.240 --> 00:00:08.160 of topics polymocytis, dermato myocytis, reactive arthritis, rheumatoid arthritis, chilgrin syndrome, 3 00:00:08.199 --> 00:00:10.320 sclerodermae, and lupus. So a number of stuff. I'll try to 4 00:00:10.400 --> 00:00:13.119 keep it as brief as possible, just the high yield stuff. If you 5 00:00:13.160 --> 00:00:16.519 do want to buy merchandise and support the podcast, I did leave a link 6 00:00:16.800 --> 00:00:20.000 in the show notes to shirt smugs things like that if you want to support 7 00:00:20.000 --> 00:00:22.839 the channel. I really appreciate that, and as always, thank you so 8 00:00:22.920 --> 00:00:25.679 much for the really nice comments and the support. Let's go ahead and get 9 00:00:25.679 --> 00:00:29.399 started. So we'll start with polymiocytis and dermato myocytis. But to group these 10 00:00:29.399 --> 00:00:35.399 together because they're both idiopathic inflammatory myopathies a lot of overlap. Dermato Myocytis is 11 00:00:35.479 --> 00:00:39.479 essentially just polymonocytus with the rash, So don't overcomplicate these because they can easily 12 00:00:39.479 --> 00:00:42.880 become overwhelming if you try to memorize every detail. Let's just know the basics 13 00:00:42.920 --> 00:00:46.679 for the exam and move on. So real quick overview of the patho both 14 00:00:46.719 --> 00:00:52.880 polyendermato myocytis are autoimmune conditions. Polymiocytis involves CD eight T cells that have essentially 15 00:00:52.920 --> 00:00:57.000 gone rogue and are attacking and destroying your muscle fibers. Dermatomocytis, you have 16 00:00:57.079 --> 00:01:00.399 an unknown factor that activates your C three protein. This leads to a cascade 17 00:01:00.399 --> 00:01:06.239 of events which ultimately results in attack and destruction of the muscle capillaries and small 18 00:01:06.359 --> 00:01:10.879 arterials. So both poly and dermato myocytis are much more common in women, 19 00:01:11.159 --> 00:01:14.200 so be looking for a female in the vignette. Pretty much every condition I'm 20 00:01:14.200 --> 00:01:18.319 going to talk about today, as most of your immune conditions are are going 21 00:01:18.359 --> 00:01:19.760 to be more common in female. So you'll notice that's the trend today. 22 00:01:19.799 --> 00:01:25.200 It's we're going over these. Let's talk about clinical manifestations next as well as 23 00:01:25.239 --> 00:01:27.359 the physical exam findings because there's a lot of high yield stuff here. So 24 00:01:27.480 --> 00:01:33.159 first, muscle weakness proximal, progressive, symmetric, So muscle weakness is the 25 00:01:33.280 --> 00:01:38.159 most common clinical manifestation of both poly and dermato myocytis. Classically, it's going 26 00:01:38.200 --> 00:01:42.959 to be symmetric and involve the proximal muscle, so the hips and the shoulders 27 00:01:42.120 --> 00:01:46.120 are going to be really common and It's the reason why in a vignette, 28 00:01:46.159 --> 00:01:49.000 the patient they're normally going to mention is going to have difficulties climbing stairs, 29 00:01:49.000 --> 00:01:52.920 getting up from a chair, raising their arms above their head. You'll commonly 30 00:01:52.920 --> 00:01:57.879 see that in vignettes. Usually the muscle weakness will be progressive with gradual worsening 31 00:01:57.920 --> 00:02:00.840 over a period of weeks to months. Real real quick recap. Remember, 32 00:02:00.920 --> 00:02:05.879 main clinical menifestations for both is going to be muscle weakness. Muscle weakness will 33 00:02:05.920 --> 00:02:08.879 be commonly proximal, so shoulders and hips progressive over weeks to months. And 34 00:02:09.000 --> 00:02:14.319 symmetrics so both sides affected, all right. So for probably midocytis, muscle 35 00:02:14.319 --> 00:02:17.520 weakness is really the only clinical manifestation you need to know. And dermato myocytis, 36 00:02:17.520 --> 00:02:21.159 though, in addition to the muscle weakness, you also got some pretty 37 00:02:21.240 --> 00:02:24.000 high old skin stuff, so let's talk about that next. So there's three 38 00:02:24.080 --> 00:02:29.599 things you have to know for dermato myocytis gotron papules, heliotrope rash, and 39 00:02:29.639 --> 00:02:35.080 photodistributed poiskyloderma. WHF, let's actually talk about what that means. So gotron 40 00:02:35.120 --> 00:02:39.479 papules, they're violaceous papules that are often scally and ulcerated that often occur over 41 00:02:39.520 --> 00:02:44.759 the bony prominences. Most commonly you'll see these over the dorsal aspects of the 42 00:02:44.840 --> 00:02:51.039 metacarpo, philangeal and endophalangeal joints. So basically you got some scaly psoriasis looking 43 00:02:51.120 --> 00:02:53.919 rash that's on the top of the hands, mainly over the knuckles. That's 44 00:02:53.960 --> 00:02:59.199 gotron papules. Next heliotrope rash, so heliotrope rash or heliotrope eruption. This 45 00:02:59.280 --> 00:03:01.639 is just a rap on the periorbital skin. So a rash around the eyes. 46 00:03:01.919 --> 00:03:06.960 It's most common on the upper eyelid. Disremember a rash surrounding the eyes 47 00:03:06.960 --> 00:03:12.560 for heliotrope rash. So gotron papules and heliotrope rash, those are pathenemonic features 48 00:03:12.560 --> 00:03:16.159 of dermato myocytis. Anytime you see that they mentioned something is pathhenemonic for a 49 00:03:16.159 --> 00:03:19.960 certain disease. That should mean to you this is going to be on the 50 00:03:20.000 --> 00:03:23.000 exam. So if you see those two ever mentioned right away, be thinking 51 00:03:23.080 --> 00:03:25.919 dermato myocytis, memorize those. There is one other finding that also seems to 52 00:03:25.960 --> 00:03:30.719 come up on exams a lot. That's photo distributed pokloderma or the shaw sign 53 00:03:30.840 --> 00:03:34.719 or the V sign. It's a complicated name to say, but very simple. 54 00:03:35.000 --> 00:03:38.039 This is a rash or hyperpigmentation that shows up anywhere that's exposed to sunlight. 55 00:03:38.319 --> 00:03:42.159 But classically and the way that it's going to be in the vignette and 56 00:03:42.199 --> 00:03:44.919 the way they should learn it is on the upper back, neck, and 57 00:03:45.039 --> 00:03:47.199 upper chest, which is why they sometimes call it the Shawl sign or the 58 00:03:47.280 --> 00:03:51.639 V sign because that's kind of where those areas are distributed. So again for 59 00:03:51.719 --> 00:03:55.719 dermato myocytis, remember gootron papules rash on the knuckles, heliotrope rash around the 60 00:03:55.759 --> 00:04:00.199 eyes, and photo distributed poiklodrma rash around the upper chest and back. I'll 61 00:04:00.199 --> 00:04:03.520 have a way for you to remember them at the end. Now for diagnosis, 62 00:04:03.560 --> 00:04:08.919 you're going to start with labs. Specifically, first your muscle enzymes. 63 00:04:08.919 --> 00:04:13.400 So there's a bunch of different muscle enzymes you can test for creating kinne s 64 00:04:13.400 --> 00:04:16.360 though is the most sensitive muscle enzyme and it's the one most commonly tested for. 65 00:04:16.519 --> 00:04:20.120 But also be on the lookout for your elevated aldolays. Even you can 66 00:04:20.160 --> 00:04:26.240 even have an elevation of the muscle derived enzymes like LDHAST and ALT. Next, 67 00:04:26.519 --> 00:04:30.439 we have your myocytus specific antibodies, anti Joe one and anti ME two. 68 00:04:30.519 --> 00:04:34.199 There's actually a bunch of myocytis specific antibodies, but these are really the 69 00:04:34.240 --> 00:04:38.680 only two that you'll you'll ever really hear about and the ones you should really 70 00:04:38.680 --> 00:04:43.319 focus on. I have a couple way to remember these antibodies. One way 71 00:04:43.439 --> 00:04:48.920 is to remember instead of dermato myocytis and polymyocytis, instead remember my Jocitus, 72 00:04:49.040 --> 00:04:56.519 like M I JO citus, so polymydositius dermato mydositis and the m I and 73 00:04:56.560 --> 00:05:00.360 the JO and my Joe. Situs helps you remember the anti joe an anti 74 00:05:00.480 --> 00:05:03.560 ME two antibodies. So that's just kind of how I remembered it. So 75 00:05:03.600 --> 00:05:09.720 instead of remembering poly myocytis, remember polymjocytis, am I JO. That can 76 00:05:09.959 --> 00:05:12.720 kind of help you remember those two. And then I'll have another mnemonic at 77 00:05:12.759 --> 00:05:15.319 the end that I'll go over. Anyways, these myocyte is specific antibodies, 78 00:05:15.399 --> 00:05:19.160 they're far from perfect. They're really only positive in around twenty to forty percent 79 00:05:19.160 --> 00:05:21.759 of people, and they also take weeks to come back. They're just one 80 00:05:21.759 --> 00:05:25.920 of those labs that take a long time to get back, but they love 81 00:05:25.959 --> 00:05:29.199 to ask about them on exam, so you have to know them. So 82 00:05:29.319 --> 00:05:32.040 make sure you're thinking of poly or dermato myocytis. If you see these mentioned, 83 00:05:32.240 --> 00:05:36.120 and specifically, if you see anti ME two mentioned, be thinking of 84 00:05:36.199 --> 00:05:42.399 dermato myocytis. As anti ME two antibodies, they're highly specific for dermato myocytis. 85 00:05:42.879 --> 00:05:46.079 And then you have your biopses, your muscle and skin biopsied. You're 86 00:05:46.079 --> 00:05:47.959 not going to do these in every patients, but if you can't if you''re 87 00:05:47.959 --> 00:05:50.120 not going to do it an every patient, but if you can't confirm the 88 00:05:50.160 --> 00:05:55.439 diagnosis from clinical manifestations and lab results, then you canform a biopsied to confirm 89 00:05:55.519 --> 00:05:59.319 the diagnosis and rule out your other differentials. Now there's other diagnostic tests that 90 00:05:59.319 --> 00:06:03.079 I'm not going to go over, electromiography, skeletal muscle imaging for the exam, 91 00:06:03.120 --> 00:06:06.879 there's four things you need to know for diagnosis, creating kinase, anti 92 00:06:08.000 --> 00:06:11.600 JOE and ANTIME two and then your biopsy in that order, and you're done 93 00:06:11.600 --> 00:06:15.319 for diagnosis. Let's talk about treatment next, because really there's only one magic 94 00:06:15.439 --> 00:06:17.160 that you need to know, and that's going to be your glucocorticoids. So 95 00:06:17.240 --> 00:06:20.560 steroids are your first line treatment. It's nice and easy. The steroids are 96 00:06:20.560 --> 00:06:25.240 started at high doses for the first several months, then you slowly taper these 97 00:06:25.240 --> 00:06:29.600 to the lowest effective dose normally for a total of around nine to twelve months. 98 00:06:29.959 --> 00:06:32.720 You have some other agents that I wouldn't memorize, but hydroxyl chloroquin is 99 00:06:32.759 --> 00:06:38.120 great to clear up skin manifestations intermato myocytis, but does nothing for the muscle 100 00:06:38.160 --> 00:06:41.920 disease. And then you have some steroid sparing agents like methotrex site that can 101 00:06:41.959 --> 00:06:45.160 be used as well. For the exam, high dose glucocorticoise is what you 102 00:06:45.199 --> 00:06:48.800 need to know, all right. So between polymyocytis and or mato myocytis or 103 00:06:48.839 --> 00:06:54.439 manto myocytis is the one you'll likely get tested on because it has those pathanemonic 104 00:06:54.439 --> 00:06:59.040 findings we talked about before, as well as the more specific antime two antibodies. 105 00:06:59.360 --> 00:07:01.399 So that's the one you should focus on. And here's how you're going 106 00:07:01.439 --> 00:07:05.480 to remember everything that you need to know for dermato myocytis. So first on 107 00:07:05.519 --> 00:07:08.920 the exam, they're not going to give you a forty nine year old female 108 00:07:08.920 --> 00:07:13.480 that presents at the office today complaining of gotron papules, heuliotrope rash and photodistributed 109 00:07:13.560 --> 00:07:16.120 poiculo derma. So it's not crucial to remember those ridiculous names because in the 110 00:07:16.199 --> 00:07:19.759 vignette they're just going to say she presents with a rash or papules on her 111 00:07:19.800 --> 00:07:25.519 hands around her eyelids. It's really important just to know where they're going to 112 00:07:25.560 --> 00:07:30.399 have these dermatologic findings and then also remember the specific antime two antibodies. So 113 00:07:30.480 --> 00:07:32.839 how do you remember this for the exam? Well, you remember for now 114 00:07:32.879 --> 00:07:38.560 on dermato myocytis in your mind is going to be remembered as permatto myocytius. 115 00:07:38.639 --> 00:07:41.240 All you're gonna do is to replace the D with the pep, and now 116 00:07:41.279 --> 00:07:46.639 you have perm prm permatto myocytis. Why perm Because whenever you think of this 117 00:07:46.759 --> 00:07:48.759 disease, I want you to think of a lady getting a PERM, sitting 118 00:07:48.759 --> 00:07:51.680 in the chair. Her hair is in that little perm helmet thing, and 119 00:07:51.759 --> 00:07:55.920 she's getting the works, she's getting her nails done, her eyebrows wax, 120 00:07:56.160 --> 00:07:59.199 she's got that cape or a little shawl over her shoulders that you normally wear 121 00:07:59.199 --> 00:08:03.040 in the salon or the arbershop and she's just relaxing and having some me time, 122 00:08:03.279 --> 00:08:07.560 me time spelled with an m I. This is very hard without a 123 00:08:07.639 --> 00:08:11.720 visual, so check out the YouTube channel if you need one, But basically 124 00:08:11.759 --> 00:08:15.160 that's the visual you need to create in your head. Lady in a chair 125 00:08:15.199 --> 00:08:18.399 getting her hair permed, getting her nails done, eyebrows wax, with the 126 00:08:18.480 --> 00:08:22.199 cape or shawl that you wear in the salon, having some me time m 127 00:08:22.199 --> 00:08:24.519 I time. Now, how does that help you remember what you need to 128 00:08:24.560 --> 00:08:28.920 know? While she's getting her eyebrows wax, this helps you remember the heliotrope 129 00:08:28.000 --> 00:08:31.720 rash that's common around the eyes, the upper eyele is especially, she's getting 130 00:08:31.759 --> 00:08:35.320 her fingernails done. That helps you remember the gotron papules that are most common 131 00:08:35.360 --> 00:08:39.639 on the top of the fingers. And she's wearing that cape or shawl. 132 00:08:39.960 --> 00:08:43.000 That helps you remember the shawl sign or the photo distributed poikilo derma, which 133 00:08:43.039 --> 00:08:46.159 is most common in the upper back, neck and upper chest. Exactly where 134 00:08:46.159 --> 00:08:50.360 that cape is that you wear, exactly what's distributed anytime you get your hair 135 00:08:50.399 --> 00:08:54.879 done and have that cape on, and then remember she's having some me time, 136 00:08:54.000 --> 00:08:56.960 me time spell with an m I. That helps you remember the anti 137 00:08:58.039 --> 00:09:01.919 me too antibodies, which are highly specific for dermato myocytis. So remember change 138 00:09:01.960 --> 00:09:05.360 the D two A P. You have permatto myocytis. Lady getting a perm 139 00:09:05.440 --> 00:09:09.080 having some me time, her eyebrows waxed, her nails done, and wearing 140 00:09:09.080 --> 00:09:15.919 a cape, and that is dermato myocytis aka permato myocytis. Moving on to 141 00:09:16.200 --> 00:09:18.879 reactive arthritis, This one's fairly simple. There's really just a few things to 142 00:09:18.879 --> 00:09:24.240 know here. So reactive arthritis is just an arthritis that starts after an infection. 143 00:09:24.360 --> 00:09:26.759 That's it. Now, what infection should you be looking for? This 144 00:09:26.840 --> 00:09:31.879 is generally going to be a GI or a GU infection, so gastro intestinal 145 00:09:31.960 --> 00:09:35.399 or genital urinary infection. So if you suspect reactive arthritis, make sure you're 146 00:09:35.399 --> 00:09:37.919 looking for them. To mention in the vignette that the patient recently had a 147 00:09:37.960 --> 00:09:43.399 bout of diarrhea or urethritis. So what specific bugs should we be looking for? 148 00:09:43.120 --> 00:09:46.960 So when we're talking about our GI bugs or our enteric bacteria, there's 149 00:09:46.960 --> 00:09:50.360 a bunch sell Manella, Shagela, campbelow, bactor C diff. Don't get 150 00:09:50.399 --> 00:09:54.399 too hung up on memorizing those. But when it comes to your GU bugs, 151 00:09:54.399 --> 00:09:58.159 your genital pathogens. There's really only one you need to know, and 152 00:09:58.200 --> 00:10:01.759 that's clamidia Clamydia trachomatic. So if you only remember one infective organism that can 153 00:10:01.840 --> 00:10:05.440 lead to a reactive authritis, definitely let it be chlamydia. This is the 154 00:10:05.440 --> 00:10:09.679 one they'll often give you in the vignette, So remember that clinical manifestations. 155 00:10:09.679 --> 00:10:15.000 So generally one to four weeks after infection, your clinical manifestations will start to 156 00:10:15.000 --> 00:10:18.759 pop up. The immune system commonly targets the lining of the joint spaces, 157 00:10:18.759 --> 00:10:22.080 which is why we get our arthritis, but can affect a number of other 158 00:10:22.120 --> 00:10:24.159 areas as well that will go over. So first let's start with the peripheral 159 00:10:24.159 --> 00:10:31.279 authritis. This is commonly going to be asymmetric olego authritis, mainly affecting the 160 00:10:31.399 --> 00:10:37.200 lower extremities, especially the need some other MSK manifestations just to be familiar with. 161 00:10:37.279 --> 00:10:39.840 They may have amphaesitis, which is an inflammation of the insertion site of 162 00:10:39.879 --> 00:10:45.240 tendons. They may have dactylitis, which is an inflammation of the digits sometimes 163 00:10:45.240 --> 00:10:48.519 refer to as sausage digits. This is going to be more common in your 164 00:10:48.519 --> 00:10:52.320 patients with chlamydia infection and that's basically all you need to know if you're MSK 165 00:10:52.440 --> 00:10:54.639 stuff. Mainly focus on the authritis. Next, let's talk about some of 166 00:10:54.639 --> 00:10:58.159 the extra articular signs and symptoms. There's two that you need to be familiar 167 00:10:58.200 --> 00:11:03.679 with. So first is your ocular symptoms, so inflammation of the eye, 168 00:11:03.000 --> 00:11:09.159 whether it's conjunctivitis, anterior uveitis, keratitis, the ie, whatever part is 169 00:11:09.200 --> 00:11:11.759 inflamed. And then we have our gu symptoms, so disuria, pelvic pain, 170 00:11:11.919 --> 00:11:16.919 urethritis, servicitis, prostetitis. So the three things I just went over 171 00:11:18.000 --> 00:11:22.519 form this triad. Triad consists of arthritis, conjunctivitis, and urethritis. This 172 00:11:22.639 --> 00:11:26.159 is actually only going to occur in a small subset of patients, around a 173 00:11:26.240 --> 00:11:31.720 third of all cases. There's definitely other clinical manifestations that can prevent present from 174 00:11:31.720 --> 00:11:35.279 this condition or allusions cardiac manifestations, But for the exam, you damn well 175 00:11:35.320 --> 00:11:39.200 better know that triad because they love to ask about it. So how do 176 00:11:39.200 --> 00:11:41.360 you remember the triad? Well, you can remember this famous demonic It's not 177 00:11:41.399 --> 00:11:45.159 mine, but it's a good one, and it's can't pee, can't see, 178 00:11:45.200 --> 00:11:48.399 can't climb a tree. So they can't pee because of the genital urinary 179 00:11:48.399 --> 00:11:52.200 symptoms, the urethritis, they can't see because of the ocular symptoms, the 180 00:11:52.240 --> 00:11:56.320 conjunctivitis, and they can't climb a tree because of the authritis. So can't 181 00:11:56.320 --> 00:11:58.919 pee, can't see, can't climb a tree, And you remember the clinical 182 00:11:58.919 --> 00:12:03.159 manifestations you need to know for reactive arthritis. Now, clinical diagnosis is going 183 00:12:03.159 --> 00:12:09.440 to be a clinical diagnosis. It's because there's no single definitive diagnostic test. 184 00:12:09.480 --> 00:12:13.679 There's no validated diagnostic criteria. Even if you're getting cultures to check for an 185 00:12:13.720 --> 00:12:16.879 underlying infection, often by the time the authritis presents, the pathogens may no 186 00:12:16.960 --> 00:12:20.559 longer be retrievable. So the idea here this isn't something you need to memorize 187 00:12:20.559 --> 00:12:24.600 for the exam, but you're basically looking for three things. One does this 188 00:12:24.720 --> 00:12:31.879 patient have the characteristic authritis MSK finds we describe before asymmetric involvement a enthesitis dactylitis. 189 00:12:31.960 --> 00:12:35.919 Two do they have evidence of a preceding infection diarrhea urethritis? And then 190 00:12:35.000 --> 00:12:39.440 number three is there a lack of convincing evidence for another more likely diagnosis like 191 00:12:39.480 --> 00:12:45.720 psoriatic authritis, rheumatoid athritis an fyi If they have joint effusion. You do 192 00:12:45.799 --> 00:12:48.799 want to do an author a sentsis to rule out septic authritis, which can 193 00:12:48.799 --> 00:12:52.279 present in a similar way. So again this is a clinical diagnosis. There's 194 00:12:52.320 --> 00:12:56.360 nothing really to know for the exam in real life. Do they have the 195 00:12:56.399 --> 00:12:58.639 typical MSK symptoms, do they have a preceding infection, and have you ruled 196 00:12:58.639 --> 00:13:03.919 out other possible causes And that's it to make the diagnosis with one little exception 197 00:13:03.960 --> 00:13:07.879 that I did want to mention because this may come up. So something known 198 00:13:07.919 --> 00:13:11.960 as HLA B twenty seven. In case you hear about human leukocyte antigen B 199 00:13:13.039 --> 00:13:16.080 twenty seven, know that this is found to be positive and around thirty to 200 00:13:16.120 --> 00:13:20.720 fifty percent of patients with reactive authritis. So it's something that if it's positive, 201 00:13:20.720 --> 00:13:24.559 can help support the diagnosis. But by no means does a negative test 202 00:13:24.679 --> 00:13:28.960 rule out reactive authritis. So just a little fy in case that comes up. 203 00:13:28.360 --> 00:13:31.919 So for treatment, there's not a lot to know here. Basically, 204 00:13:33.519 --> 00:13:37.279 first treat the underlying infection. This is pretty straightforward. If they have chlamydia, 205 00:13:37.360 --> 00:13:41.399 given them doxy etc. For the aarthritis, there is one main treatment 206 00:13:41.399 --> 00:13:43.120 option that's agreed upon first line though that you should know and that's what you 207 00:13:43.159 --> 00:13:46.679 should focus on. And that's end said. So nd sets are first line 208 00:13:46.679 --> 00:13:50.320 treatment for authritis. So unless contraindicated, end sets are going to be what 209 00:13:50.360 --> 00:13:54.440 you're going to use for the authritis. If they are refractory to the end 210 00:13:54.480 --> 00:13:58.399 seids, you can use steroids. It's rare to require disease modifying agents like 211 00:13:58.480 --> 00:14:03.399 methotrek state that we use in other types of authritis like RA because the symptoms 212 00:14:03.399 --> 00:14:07.039 of most of these patients is generally self limited. So basically for the treatment, 213 00:14:07.120 --> 00:14:09.519 focus on your end sets. That's the key takeaway here. So three 214 00:14:09.639 --> 00:14:13.679 main high old things to know for reactive authritis. One look for your preceding 215 00:14:13.679 --> 00:14:18.759 infection GI BUGG or chlamydia. Two remember can't pee, can't see, can't 216 00:14:18.759 --> 00:14:22.120 climb a tree for your clinical manifestations. And three remember treat the underlying infection 217 00:14:22.159 --> 00:14:26.159 if need, be an end sets for your authritis. And that's all you 218 00:14:26.200 --> 00:14:30.240 need to know for reactive authritis. Let's talk about rheumatoid authritis next. So 219 00:14:30.279 --> 00:14:33.200 this is a monster of a topic. There's a decent amount to know here. 220 00:14:33.399 --> 00:14:37.440 Let's just focus on the need to know stuff. So rheumatoid authritis is 221 00:14:37.480 --> 00:14:43.600 a chronic systemic inflammatory autoimmune disease of unknown etiology that can lead to the destruction 222 00:14:43.639 --> 00:14:46.519 and deformity of joints due to erosion of cartilage and bone. Let's do a 223 00:14:46.639 --> 00:14:52.000 thirty second path will breakdown on rheumatoid authortis, so RA, like so many 224 00:14:52.039 --> 00:14:54.840 things in rheumatology, is an autoimmune disease. So we have these T cells 225 00:14:56.480 --> 00:15:00.519 that are recruiting macrophages. They're all just hanging out in the joint space producing 226 00:15:00.519 --> 00:15:03.759 cytokinds unnecessarily. This is leading to a bunch of inflammation. And then this 227 00:15:03.840 --> 00:15:07.000 is the important part. These cytokinds that are being produced in the joint, 228 00:15:07.240 --> 00:15:13.639 they're also causing the synovial cells to proliferate. So your synovial cells form your 229 00:15:13.679 --> 00:15:16.559 synovial membrane. Remember that's your connective tissue that lines the joint space. So 230 00:15:18.200 --> 00:15:24.000 synovial cells are proliferating and this causes this thick inflamed synovial membrane which is called 231 00:15:24.039 --> 00:15:26.960 a panis. And this panis is growing in the joint eventually runs out of 232 00:15:28.000 --> 00:15:31.720 space. The panis gets bigger and bigger, eventually starts breaking down and eroding 233 00:15:31.759 --> 00:15:37.240 the joint space. And this leads to our clinical manifestations. So again cytokines 234 00:15:37.320 --> 00:15:41.320 are causing this panis to grow bigger and bigger, eventually too big and it 235 00:15:41.320 --> 00:15:43.960 just starts breaking stuff inside the joint. That's your thirty second path of explanation. 236 00:15:45.679 --> 00:15:48.360 Next, who are you going to see this in? So that's going 237 00:15:48.399 --> 00:15:52.679 to be females. So incidence of RA is twice as high among females compared 238 00:15:52.759 --> 00:15:56.000 to males, So very likely for this to be a female in the vinet 239 00:15:56.200 --> 00:16:00.600 number of other risk factors, lower socioeconomic status, cigarette smoking, obesity, 240 00:16:00.720 --> 00:16:03.919 just really focus on the female sex. Though. Let's move on to clinical 241 00:16:04.000 --> 00:16:10.159 manifestations because there's a lot of high yield stuff here. So there's a lot 242 00:16:10.200 --> 00:16:12.879 of things to know for the clinical manifestations for r A. But luckily they 243 00:16:12.919 --> 00:16:15.519 all start with an S, or at least I've created away from them to 244 00:16:15.559 --> 00:16:18.240 all start with an S for the sake of memorization. So if you can 245 00:16:18.279 --> 00:16:22.720 remember your seven s is for clinical manifestations for RA, you're good for the 246 00:16:22.720 --> 00:16:25.840 exam, and of course I'll have a way for you to remember them at 247 00:16:25.840 --> 00:16:30.200 the end. So the first S is symmetric, so symmetric joint involvement in 248 00:16:30.240 --> 00:16:33.080 rheumatoid arthritis is a characteristic feature, meaning if the left hand is affected, 249 00:16:33.279 --> 00:16:37.919 the right hand will likely be affected. To this, obviously in real life 250 00:16:37.000 --> 00:16:41.000 isn't one hundred percent. It may be less apparent early on in the disease, 251 00:16:41.360 --> 00:16:45.240 but you need to start doing is separating real life findings from exam questions 252 00:16:45.240 --> 00:16:48.240 because they're two completely different things. You're studying for the exam, and an 253 00:16:48.240 --> 00:16:51.919 exam is going to give you the most common presentation. So for an exam 254 00:16:52.080 --> 00:16:55.960 question that they're going to describe symmetric joint involvement because that's the most common. 255 00:16:56.080 --> 00:16:59.080 So count on that. Next S is going to be swollen. This is 256 00:16:59.080 --> 00:17:03.039 an inflammatory athritis, so it makes sense the effected joints would be swollen, 257 00:17:03.159 --> 00:17:06.880 tender, and warm. Next S is soft, So soft, warm, 258 00:17:06.960 --> 00:17:10.640 boggy joints are typical of RA A. If they mention the joints are hard 259 00:17:10.720 --> 00:17:14.559 and bony, this is not RA You should be thinking of osteoarthritis instead, 260 00:17:14.559 --> 00:17:18.640 So remember soft boggy joints. Next s small joints. So rheumatoto athritis has 261 00:17:18.640 --> 00:17:23.039 a predilection for the small joints hands, feet, wrist, so it's not 262 00:17:23.119 --> 00:17:26.480 impossible, but less likely you'll see this in the shoulders, hips, spinus, 263 00:17:26.480 --> 00:17:30.880 ceterup. So remember your small joints are more common to be affected hands, 264 00:17:30.920 --> 00:17:36.279 feet, wrist. Next S spares the dip, so classically RA spares 265 00:17:36.319 --> 00:17:40.440 the last knuckle, the distal interphalangeal joint, the DP. It's not impossible 266 00:17:40.480 --> 00:17:44.079 for the dip to be effected, but if you see DP involvement, this 267 00:17:44.160 --> 00:17:48.559 is more likely to suggest a diagnosis of osteoarthritis rather than r A. So 268 00:17:48.640 --> 00:17:52.640 remember ra A classically spares the dip. Next s sixty minutes or more sixty 269 00:17:52.640 --> 00:17:56.920 minutes or more so in all types of inflammatory polyarthritis like rheumatoid athritis. You're 270 00:17:56.960 --> 00:18:02.000 looking for morning stiffness for extended periods of time. Depending on the literature, 271 00:18:02.079 --> 00:18:03.440 some are going to say over thirty minutes, others are going to say over 272 00:18:03.480 --> 00:18:08.119 sixty minutes. But the key here is that the morning stiffness RA is sustained, 273 00:18:08.160 --> 00:18:11.720 and that's what's going to help you differentiate from osteoarthritis, where the morning 274 00:18:11.759 --> 00:18:17.400 stiffness and osteoarthritis, if it's present at all, is usually transient or only 275 00:18:17.480 --> 00:18:21.400 last for a few minutes at most. Last s swan neck deformity. So 276 00:18:21.440 --> 00:18:25.680 there's a number of different joint deformities that are can cause botneer, swan neck, 277 00:18:25.759 --> 00:18:29.240 boastring deformity. We don't see them as often as we once did due 278 00:18:29.240 --> 00:18:33.240 to the early innovation of d MARDs, but I do remember Swaneck deformity coming 279 00:18:33.279 --> 00:18:34.799 up on an exam question. So if you see this mention, this is 280 00:18:34.839 --> 00:18:41.440 typically seen in advanced RA and commonly caused from the erosion of the extensor tendon 281 00:18:41.519 --> 00:18:45.960 from chronic sinovitis. It causes the dip joint to flex and the pip joint 282 00:18:45.960 --> 00:18:51.359 to hyper extend, so the middle knuckle is hyper extended, last knuckle is 283 00:18:51.359 --> 00:18:53.279 flexed, and it kind of looks like a swan neck. So that's your 284 00:18:53.319 --> 00:18:57.200 last s, all right. So for clinical manifestations and physical exam findings, 285 00:18:57.240 --> 00:19:02.680 remember your seven s is some metric swollen, soft small joints, spares, 286 00:19:02.720 --> 00:19:06.039 the d I P sixty minutes, and swan neck deformity. If you need 287 00:19:06.119 --> 00:19:08.559 help remembering those seven ses, I got you. Here's the second part of 288 00:19:08.559 --> 00:19:15.279 the mnemonic. So I used to remember rheumatoid authritis like with seven ses at 289 00:19:15.319 --> 00:19:18.680 the end. So rheumatoid authritis, and when you say that out loud, 290 00:19:18.160 --> 00:19:22.680 what do you sound like a snake? So every time you see rheumatoid authritis 291 00:19:22.680 --> 00:19:26.039 on an exam question in school, say it like that in your head, 292 00:19:26.200 --> 00:19:29.200 not out loud, and then picture a snake. But this is not just 293 00:19:29.279 --> 00:19:32.839 any snake. It's a stuffed animal snake, so like a little plush toy 294 00:19:32.960 --> 00:19:37.319 snake. Stuffed animal snake is obviously soft. That helps remember the joints are 295 00:19:37.359 --> 00:19:41.880 soft and boggy. Next, this snake oddly has hands and feet. That's 296 00:19:41.000 --> 00:19:45.839 very weird. Snakes don't normally have that, but this helps remember that the 297 00:19:45.960 --> 00:19:48.559 ri commonly affects the small joints of the hands and feet. Next, the 298 00:19:48.599 --> 00:19:53.480 snake is holding an identical timer in each hand and each time is set at 299 00:19:53.559 --> 00:19:57.599 exactly sixty minutes. So the identical timer in each hand helps you remember the 300 00:19:57.640 --> 00:20:02.799 symmetric findings because timers are identical, and the fact that the timers are set 301 00:20:02.839 --> 00:20:06.359 at sixty minutes helps you remember the morning stiffness for you know, sixty minutes 302 00:20:06.400 --> 00:20:10.799 or more over sixty minutes. Next, his stomach is super swollen, like 303 00:20:10.880 --> 00:20:11.880 most snakes when they eat too much, you know they have that like big 304 00:20:11.880 --> 00:20:15.559 swollen belly and the swollen stomach helps you remember the joints will be swollen in 305 00:20:15.720 --> 00:20:18.759 ra a and what did he eat that made his stomach so swollen? Well, 306 00:20:18.759 --> 00:20:22.759 if we look inside his stomach, we see two things, a swan 307 00:20:22.880 --> 00:20:26.319 and a bowl of asparagus dip. That helps. The swan helps you to 308 00:20:26.359 --> 00:20:30.759 remember the swan neck deformity, and the asparagus dip he ate helps you remember 309 00:20:30.039 --> 00:20:36.279 spares. The DP A spare a guest stip d ip A spare I guess 310 00:20:36.359 --> 00:20:41.400 d ip dip. So that visual combined helps remember the seven ss of hematoid 311 00:20:41.400 --> 00:20:45.079 athritis. So quickly recap you have a soft plush snake helps remember soft joints. 312 00:20:45.200 --> 00:20:48.799 He's got human hands and feet. Affects the small joints of the hand 313 00:20:48.799 --> 00:20:52.359 and feet. He's holding an identical timer in each hand set to sixty minutes 314 00:20:52.599 --> 00:20:56.559 symmetric and morning stiffness over sixty minutes. It's got a swollen stomach, swollen 315 00:20:56.640 --> 00:21:02.240 joints because he ate a swan and asparagus dip swaneckity spares the dip. For 316 00:21:02.319 --> 00:21:04.440 my podcast listeners, definitely check out the YouTube channel. I made an amazingly 317 00:21:04.480 --> 00:21:08.400 horrible snake and Microsoft Paint then helps paint this visual all right, So next 318 00:21:08.480 --> 00:21:12.279 let's talk about diagnosis. There's a number of different tests and labs for diagnosis 319 00:21:12.319 --> 00:21:17.079 of r A, but there's two that you absolutely need to know, and 320 00:21:17.119 --> 00:21:21.599 that's your rheumatoid factor and your anti CCP. So let's start with the rheumatoid 321 00:21:21.599 --> 00:21:25.839 factor. So rheumatoid factor will be positive in seventy to eighty percent of patients 322 00:21:25.839 --> 00:21:27.880 with URA, so it's a very sensitive test. Problem is, it's not 323 00:21:29.039 --> 00:21:33.640 very specific. Even healthy individuals can be positive for rheumatoid factor. Patients with 324 00:21:33.799 --> 00:21:37.119 lupus hepatitis, see a bunch of other inflammatory conditions can all wind up with 325 00:21:37.200 --> 00:21:41.680 a positive rheumatoid factor. So it's a sensitive test. It's just not very 326 00:21:41.680 --> 00:21:45.279 specific. So if we want a specific test, that's when we use our 327 00:21:45.319 --> 00:21:52.200 anti CCP also known as our anti citrullinated peptide antibodies or anti sickl citrulinated peptide. 328 00:21:52.200 --> 00:21:56.319 That's why we just call it our anti CCP. So this test is 329 00:21:56.400 --> 00:22:00.279 very specific rheumatoid author rights, meaning if it's positive, this is very likely 330 00:22:00.559 --> 00:22:03.839 going to be RA and not something else. The specificity is generally over ninety 331 00:22:03.839 --> 00:22:08.160 percent, some literature stating as high as ninety five to ninety eight percent specific 332 00:22:08.279 --> 00:22:12.480 for ra AW. I'm not going to get into the necessary details, but 333 00:22:12.839 --> 00:22:17.960 both of these can actually be negative, the rheumatoid factor and anti CCP. 334 00:22:18.079 --> 00:22:21.559 That can both be negative and you can actually still make the diagnosis of RAA. 335 00:22:21.720 --> 00:22:25.279 It's called zero negative ra Just a little extra knowledge, not really anything 336 00:22:25.279 --> 00:22:29.119 to know for the exam. So in conclusion, know your rheumatoid factor, 337 00:22:29.240 --> 00:22:32.359 know your anti CCP. And a little quick tip. If you get an 338 00:22:32.359 --> 00:22:36.200 exam question and they say which are the following is most specific for the diagnosis 339 00:22:36.839 --> 00:22:40.759 diagnosis of rheumatoid authoritis, and you've narrowed it down to your rheumatoid factor and 340 00:22:40.759 --> 00:22:42.920 your anti CCP, and you can't remember which one it is, remember the 341 00:22:42.920 --> 00:22:48.240 words specific in it has two c's in it, and so does anti CCP. 342 00:22:48.440 --> 00:22:49.880 At least that help me on the exam. So two things again, 343 00:22:49.880 --> 00:22:53.559 and know for diagnosis rheumatoid factor and anti CCP. That's the high yield stuff. 344 00:22:53.559 --> 00:22:57.400 There's obviously other immodalities you can use in diagnosis X ray ultrasound, but 345 00:22:57.519 --> 00:23:00.880 for the exams, they're like going to mention your crologies and that's what you 346 00:23:00.920 --> 00:23:04.119 need to know. Let's talk about treatment next. Now, two things you 347 00:23:04.160 --> 00:23:07.400 need to know for treatment, and that's end sets and DMARDs. So end 348 00:23:07.400 --> 00:23:11.440 sets, plain and simple, they're just for symptom control. If the patient 349 00:23:11.519 --> 00:23:15.799 has contraindications to end sets or inadequate response to end sets, you can give 350 00:23:15.799 --> 00:23:19.319 them corticoids. Like can really anything else. Not much to know here except 351 00:23:19.359 --> 00:23:22.799 for the fact that end sets they're only going to treat the inflammation and pain. 352 00:23:22.880 --> 00:23:26.480 They don't do anything to alter the course of the disease or to prevent 353 00:23:26.480 --> 00:23:30.720 the destruction it can cause. And that's why your next class of meds is 354 00:23:30.759 --> 00:23:34.519 the most important for rheumatoid athritis, and that's your DMARDs, so disease modifying 355 00:23:34.599 --> 00:23:40.680 anti rheumatic drugs. There's a few meds in this class, hydroxy chloroquin soulfasalazine, 356 00:23:40.960 --> 00:23:44.680 but if you're going to memorize one, make sure it's methotrex sate. 357 00:23:45.039 --> 00:23:48.279 Metho trek sate is the most commonly used DMARD because compared to the other meds, 358 00:23:48.279 --> 00:23:52.519 it has the faster on set of action, greater efficacy, better long 359 00:23:52.640 --> 00:23:55.559 term tolerance. So just remember methotrex sate and you should be good for the 360 00:23:55.599 --> 00:23:59.279 exam, let's talk about why it's so important to start patients on this class 361 00:23:59.279 --> 00:24:02.759 of meds in the first place. So patients diagnosed with RA should be started 362 00:24:02.759 --> 00:24:06.440 on these drugs as soon as possible because with R, once the damage is 363 00:24:06.480 --> 00:24:10.079 done, it's irreversible. There's no going back. So starting this class early 364 00:24:10.119 --> 00:24:14.960 on in the diagnosis can reduce or prevent this irreversible joint damage. So remember, 365 00:24:15.200 --> 00:24:18.920 unless there's a contraindication, everybody with RA will get a d MART and 366 00:24:18.960 --> 00:24:22.279 that DMART will likely be methotrex SATEN. So for an exam standpoint for treatment, 367 00:24:22.359 --> 00:24:27.359 remember two things N sets for symptoms and dmarts to prevent disease progression. 368 00:24:27.640 --> 00:24:30.200 In real life, obviously it's a little bit more complicated in that than that. 369 00:24:30.240 --> 00:24:33.480 There's other options, but for the exam, that's what you need to 370 00:24:33.480 --> 00:24:36.400 know, all right. So for rumatoid authoritis, there's a lot of info. 371 00:24:36.720 --> 00:24:38.680 What do you absolutely need to know? Remember your seven S is for 372 00:24:38.680 --> 00:24:44.200 clinical manifestations, symmetric soft, swollen small joints, sixty minutes or more, 373 00:24:44.559 --> 00:24:48.440 spare the dip and swan neck. Remember your rheumatoid factor in your anti CCP 374 00:24:48.480 --> 00:24:52.920 for diagnosis. Anti CCP is the specific one. Remember two seasons, specific 375 00:24:52.920 --> 00:24:57.039 and anti CCP. And the main takeaway with treatment is your dmarts specifically methotrek 376 00:24:57.079 --> 00:25:00.759 sate and that is a rheumatoid authoritis. Movie on to Schogrin syndrome, So 377 00:25:00.759 --> 00:25:04.519 there's just a few things to know here. This is a systemic autoimmune disease 378 00:25:04.599 --> 00:25:10.279 characterized by lymphocydic infiltration of exocrine glands. So there's a problem with the X 379 00:25:10.319 --> 00:25:15.880 screen glands. There's this autoimmune induced inflammation of the exocrine glands, especially the 380 00:25:15.039 --> 00:25:19.799 lachrymal and the salivary glands. This leads to diminished function of these glands with 381 00:25:19.960 --> 00:25:25.559 result in dryness of the eyes and the mouth, among other problems. You 382 00:25:25.599 --> 00:25:29.960 have primary and secondary types of this condition. Primary isn't associated with any other 383 00:25:30.000 --> 00:25:36.000 conditions where secondary is associated with another underlying rheumatic disease like rheumatoidatis, lupus, 384 00:25:36.000 --> 00:25:38.920 etc. Your focus should be on the primary type. So clinical menifestations, 385 00:25:38.920 --> 00:25:42.079 there's three things you need to know when it comes to Schogrin syndrome. That's 386 00:25:42.160 --> 00:25:45.480 dry eyes, dry mouth, parodic glands, swelling. Those are the most 387 00:25:45.480 --> 00:25:48.119 common ones you need to know that will definitely be in the vignette. In 388 00:25:48.119 --> 00:25:52.880 real life, the disease spectrum is much more broad. It's a systemic disorder 389 00:25:52.880 --> 00:25:56.319 and you can have extraglandular organ involvement of the skin joints, heart GI tract. 390 00:25:56.359 --> 00:26:00.240 But for the exam, all you need to rememorize is dry eyes, 391 00:26:00.519 --> 00:26:04.559 which is also known as corrado conjunctivite. A SICA patient may complain of irritation, 392 00:26:04.680 --> 00:26:08.319 greediness, itching, of foreign sensation in the eye, so look out 393 00:26:08.319 --> 00:26:12.559 for that. Next dry mouth, which is also known as zerostomia. By 394 00:26:12.599 --> 00:26:15.359 the way, these two symptoms, dry eye and dry mouth will be the 395 00:26:15.480 --> 00:26:21.319 most common. Dry eyes and dry mouth are president and over eighty patients with 396 00:26:21.359 --> 00:26:26.079 primary schogrin syndrome, and then the last one parodi swelling. So other salivary 397 00:26:26.079 --> 00:26:29.079 glands can be involved, like the submandibular, but the product is going to 398 00:26:29.079 --> 00:26:32.000 be the most common. So again for the exam, if you see dry 399 00:26:32.000 --> 00:26:36.680 eyes, dry mouth, product gland swelling, be thinking schogrin syndrome next diagnosis. 400 00:26:36.680 --> 00:26:40.799 So for diagnosis, there is no one single best test to diagnosis condition. 401 00:26:41.240 --> 00:26:45.920 Make the diagnosis based on the appropriate clinical features combined with laboratory testing, 402 00:26:45.960 --> 00:26:48.559 but no one specific test rules in or out the disease. What that kept 403 00:26:48.559 --> 00:26:52.680 in mind, let's talk about the two diagnostic tests they will likely ask you 404 00:26:52.720 --> 00:26:56.559 about on the exam. First one is your anti ROW and anti law antibodies. 405 00:26:56.599 --> 00:27:00.480 Anti ROW also known as SSA and anti LAW also known as SSB. 406 00:27:02.160 --> 00:27:04.880 This is probably the one you're going to be tested on. Anywhere from sixty 407 00:27:04.880 --> 00:27:08.960 to eighty percent of patients with primary schogrins will test positive for one or both 408 00:27:08.960 --> 00:27:12.599 of these antibodies. The problem is these can also be positive in patients with 409 00:27:12.680 --> 00:27:17.440 lupus, even some healthy individuals without cholgrins. So it's a good test, 410 00:27:17.480 --> 00:27:21.599 but it's obviously not perfect. Go usually order these antibodies in conjunction with your 411 00:27:21.640 --> 00:27:25.640 ANA and your rheumatoid factor. And then the other diagnostic test that you should 412 00:27:25.680 --> 00:27:29.400 know one that's often tested on is the Schermer test. So the Schermer test 413 00:27:29.519 --> 00:27:33.119 is quite literally taking a dry piece of sterile paper and putting it in someone's 414 00:27:33.119 --> 00:27:37.119 eye you suspect to shogrins, and then you measure how what the paper gets 415 00:27:37.119 --> 00:27:38.920 over a period of five minutes. That's really it. It's a simple idea, 416 00:27:38.960 --> 00:27:41.720 but it works well and you should know it. For the exam, 417 00:27:41.759 --> 00:27:45.480 and then just a final note, if there's still any lingering doubt about the 418 00:27:45.519 --> 00:27:49.039 diagnosis after doing your lab's physical exam, etc. You can use a salvary 419 00:27:49.079 --> 00:27:55.240 gland biopsy to establish for the diagnosis. So outside of the clinical manifestations, 420 00:27:55.240 --> 00:27:57.839 really the highest yield thing to know for schogrins are the diagnostic tests that went 421 00:27:57.920 --> 00:28:00.839 over. You need to remember that. The way that I used to remember 422 00:28:00.880 --> 00:28:06.359 anti row and anti law and the Shermer test is by instead of remembering Shogrin 423 00:28:06.480 --> 00:28:11.119 syndrome, I would remember slowgreen syndrome. So instead of shogrin slow green slow 424 00:28:11.160 --> 00:28:17.240 green syndrome, so slow green instead of shogreen, and what slow and green? 425 00:28:17.480 --> 00:28:21.400 A frog? That to remember a slow green frog landed in my cup 426 00:28:21.440 --> 00:28:23.279 of Sherbert. So create that visual in your head. You have a cup 427 00:28:23.319 --> 00:28:27.799 of Sherbert, that little frozen fruit treat and a frog landed right in it. 428 00:28:27.880 --> 00:28:33.279 So shogreen is now slow green and a slow green frog landed in my 429 00:28:33.359 --> 00:28:37.000 cup of Sherbert. So frog. The second two letters are ro o that 430 00:28:37.119 --> 00:28:44.039 helps remember anti row landed. First two letters La helps remember anti law, 431 00:28:44.400 --> 00:28:47.440 and then Sherbert helps you remember the Shechrmer test. So that worked for me. 432 00:28:47.559 --> 00:28:51.799 Just remember slow green instead of shogreen, and remember that slow green frog 433 00:28:51.920 --> 00:28:56.000 landing in your cup of Sherbert. Remember second two letters of frog r O, 434 00:28:56.559 --> 00:29:00.400 first two letters of landing R l A, and then Sherbert helps remember 435 00:29:00.480 --> 00:29:03.559 schermer remember that visual. So for treatment, it's not very much to know 436 00:29:03.640 --> 00:29:07.640 here. If their eyes are dry, give them artificial tears for dry mouth, 437 00:29:07.680 --> 00:29:10.559 make sure they're staying hydrated, stop smoking. They can use salive vary 438 00:29:10.559 --> 00:29:14.839 stimulants like sugar free candies or lozenges. That stuffs usually not test it on. 439 00:29:14.880 --> 00:29:19.039 But what they will test you on is the muscarinic agonis or your cholinergic 440 00:29:19.119 --> 00:29:26.759 specifically pilocarpine and sevemoline. These met stimulate secretion of the extracrine glands, opposite 441 00:29:26.759 --> 00:29:30.880 of your anticholinergics like atropine and atrovent that most of us are more familiar with 442 00:29:30.920 --> 00:29:33.279 that can dry you out. So if they ask you what meant to put 443 00:29:33.279 --> 00:29:37.920 a patient on the shogrins, remember your coolinergics, pilocarpene, sevemoline. All 444 00:29:37.960 --> 00:29:40.880 right, So that is Schhogrin's just a few takeaways here. It's an autoimmune 445 00:29:40.880 --> 00:29:44.000 disorder of the exocrine glands. So remember your dry eyes, dry mouth, 446 00:29:44.039 --> 00:29:48.000 prodig gland enlargement, remember your row in law and your schermer tests for diagnosis 447 00:29:48.240 --> 00:29:52.759 and then treatment. Just remember pilocarpine and sevemoline and you're good to go. 448 00:29:52.240 --> 00:29:57.480 Talk about systemic sclerosis or scleroderma next. So this is a systemic connective tissue 449 00:29:57.480 --> 00:30:03.200 disease or excessive collagen deposition leads to progressive fibrosis of the skin and internal organs. 450 00:30:03.440 --> 00:30:07.240 So the path that is not completely understood, But what we do know 451 00:30:07.799 --> 00:30:11.240 is there is a genetic predisposition combined with some sort of triggering event like a 452 00:30:11.319 --> 00:30:15.680 viral infection, drugs, etc. This is followed by an endothelial injury. 453 00:30:17.000 --> 00:30:22.279 Our T cells come in start overproducing cytokinds, causing excessive inflammation. The cytokinds 454 00:30:22.319 --> 00:30:27.920 activate fibroblasts and the fibroblast this is the important part, start depositing collagen and 455 00:30:29.039 --> 00:30:32.599 because of this, our nice, soft and squishy skin and organs are now 456 00:30:32.640 --> 00:30:37.400 being replaced with this hardened fibrotic tissue. And this fibrosis also causes reduced blood 457 00:30:37.400 --> 00:30:41.599 flow and result in eschemic tissue damage. And this combination is what leads to 458 00:30:41.640 --> 00:30:47.119 our clinical manifestations. So in conclusion, two damn much collagen and fibrosis. 459 00:30:47.480 --> 00:30:49.640 So this is going to be much more likely in a female. In the 460 00:30:49.640 --> 00:30:52.880 female population, so the female to male ratio can be as high as eight 461 00:30:52.920 --> 00:30:56.920 to one, like pretty much everything we're talking about today, so much more 462 00:30:56.920 --> 00:31:02.920 common in women. There's a few different types. Systemic sclerosis is generally classified 463 00:31:02.960 --> 00:31:07.200 based on the extent of skin involvement and organ involvement. There's really only two 464 00:31:07.200 --> 00:31:11.000 main types that you need to know. The first is limited cutaneous systemic sclerosis 465 00:31:11.559 --> 00:31:15.880 aka Crest syndrome. So for limited disease, I just want you to remember 466 00:31:15.880 --> 00:31:21.680 two things. One, it normally spares the trunk that's important, mostly affects 467 00:31:21.720 --> 00:31:23.920 the distal limbs. And then the second part, and probably the most important 468 00:31:23.920 --> 00:31:30.079 thing to know, is this limited form of the disease is associated with something 469 00:31:30.160 --> 00:31:33.319 called Crest syndrome. So what is Crest syndrome? What? Luckily, Crest 470 00:31:33.440 --> 00:31:37.359 actually comes packaged with its own pneumonic that explains exactly what it is. So 471 00:31:37.480 --> 00:31:44.480 CREST actually stands for the C stands for calcinosis. Cutis are stands for raynod 472 00:31:44.519 --> 00:31:49.039 phenomenon. The esophageal dismotility. The S stands for sclerodactylly, and then the 473 00:31:49.119 --> 00:31:53.000 T stands for telangiectagium. So let's talk about what of these actually mean. 474 00:31:53.519 --> 00:31:57.039 So, calcinosis cutis is a calcium deposition in the skin, so you just 475 00:31:57.079 --> 00:32:01.680 get these clumps of calcium to it's all over raynot phenomenon. Is the same 476 00:32:01.680 --> 00:32:05.880 thing that happens when your fingertips get really cold and vaso constrict and they turn 477 00:32:05.960 --> 00:32:07.880 white and blue. This is just an exaggerated form of this, and it 478 00:32:07.920 --> 00:32:13.680 can also happen in response to stress esophageal dismotility. This one's pretty straightforward. 479 00:32:13.680 --> 00:32:17.759 They may have dysphasia, choking gird, etc. Sclerodactyle is this claw like 480 00:32:17.839 --> 00:32:21.599 appearance of the hand due to the tightening of the skin of the fingers, 481 00:32:21.880 --> 00:32:27.559 and Sclerodactyle sounds very much like pterodactyl, which is that big bird dinosaur bird 482 00:32:27.599 --> 00:32:31.880 with the claude feet, So pterodactyl claude feet, sclerodactyle clawde hand, so 483 00:32:31.920 --> 00:32:36.599 that kind of helped me remember it. And then finally tlangiac taja, which 484 00:32:36.680 --> 00:32:38.880 is in so many other conditions so probably not the first time you're hearing this 485 00:32:39.000 --> 00:32:45.200 term. But all this is these dilated superficial vessels that create this weblike appearance 486 00:32:45.200 --> 00:32:47.079 on the skin. So that's your limited form of the disease. Again, 487 00:32:47.200 --> 00:32:52.680 remember two things. One, this mainly occurs in the distal limbs but generally 488 00:32:52.799 --> 00:32:57.200 spares the trunk. And then to remember crest syndrome, calcinosis, raynats, 489 00:32:57.440 --> 00:33:04.559 esophageal sclerodactyly and telangiac taste. Moving on to diffuse cutaneous systemic sclerosis, so 490 00:33:04.640 --> 00:33:07.359 the word diffuse is in the name, so as you'd imagine, this is 491 00:33:07.400 --> 00:33:12.680 more widespread. So the areas we discussed before that limited sclerosis didn't really affect 492 00:33:12.680 --> 00:33:16.160 like the trunk for instance, and proximal limbs, diffuse will affect this area. 493 00:33:16.440 --> 00:33:19.759 This, of course isn't one hundred percent in real life, but for 494 00:33:19.799 --> 00:33:24.240 the exam definitely learn it that way. Biggest takeaway with diffuse cutaneous systemic sclerosis 495 00:33:24.440 --> 00:33:28.599 is that it involves the organs. That's what you need to remember. These 496 00:33:28.599 --> 00:33:31.799 patients are much more likely to have involvement of the internal organs, especially the 497 00:33:31.880 --> 00:33:37.640 heart, lungs, and kidneys, so look for lung fibrosis, scleroderma, 498 00:33:37.720 --> 00:33:42.880 renal crisis. That's the key here. So recap. For diffused disease, 499 00:33:42.920 --> 00:33:47.319 remember trunk involvement and organ involvement heart, lung and kidneys. For limited disease, 500 00:33:47.440 --> 00:33:52.160 remember the distal limbs and spares the trunk, and then of course crest 501 00:33:52.200 --> 00:33:55.680 syndrome one last time, even more condensed diffuse trunk and organs, limited, 502 00:33:55.839 --> 00:34:00.400 distal limbs and crest done. That's it. Don't make it more complicated, 503 00:34:00.400 --> 00:34:02.200 and it has to be. Let's move on to diagnosis. So for diagnosis, 504 00:34:02.400 --> 00:34:06.640 there's really two labs you need to know. The first is your anti 505 00:34:06.759 --> 00:34:10.519 centromere antibody. So the presence of this antibody is most often associated with the 506 00:34:10.679 --> 00:34:15.960 limited subtype. Only about five percent of patients with this antibody will have diffused 507 00:34:15.960 --> 00:34:20.000 disease. So if they give you a positive anti centromere antibody on an exam 508 00:34:20.079 --> 00:34:23.440 question and they ask you which is the most likely diagnosis is one hundred percent 509 00:34:23.519 --> 00:34:28.920 on an exam question, limited disease aka Crest syndrome. And that's why instead 510 00:34:28.920 --> 00:34:31.920 of remembering this antibody is anti centromere, you're going to remember it as anti 511 00:34:31.960 --> 00:34:37.880 crustomere. So no longer anti centromere and is now known as anti crustomere. 512 00:34:37.199 --> 00:34:40.400 Just replace it in your head and you'll get the question right. Next anibody 513 00:34:42.039 --> 00:34:46.719 is your anti topoisomerase, also known as your anti SCL seventy antibody. This 514 00:34:46.760 --> 00:34:52.079 one you need to know is most commonly associated with diffused disease, and it 515 00:34:52.119 --> 00:34:55.599 also carries a higher risk of severe interstitial lung disease if positive. So remember 516 00:34:55.639 --> 00:35:02.960 anti crustomere associated with limited disease, anti topoisomerase SCL seventy anibody associated with diffused 517 00:35:04.000 --> 00:35:07.039 disease. There's a couple other labs you can use, of course, your 518 00:35:07.079 --> 00:35:09.519 ANA, your anti nuclear anibody, which is a very sensitive test and it'll 519 00:35:09.519 --> 00:35:15.079 be positive and approximately ninety five patients, but it's just not specific for anything. 520 00:35:15.559 --> 00:35:19.679 You can be positive in so many other conditions. Your anti RNA polymerase 521 00:35:19.800 --> 00:35:23.320 three anibody. For the exam though, focus on your anti centromere and aka 522 00:35:23.360 --> 00:35:28.360 anti crustomere and your anti topoisomerase. That's what they're going to test you on, 523 00:35:28.800 --> 00:35:30.320 all right. So for treatment, it's unlikely they're gonna ask you about 524 00:35:30.320 --> 00:35:36.000 treatment because treatment is mainly targeted at treating the symptoms or organ that's involved. 525 00:35:36.000 --> 00:35:38.280 So if they If they have GIRD, you're going to give them a PPI. 526 00:35:38.360 --> 00:35:42.719 If they have a NO phenomenon, give them a calcium channel block calcium 527 00:35:42.800 --> 00:35:45.280 channel blocker. Renal problems, give them an ase inhibitor. Patients with severe 528 00:35:45.280 --> 00:35:52.400 inflammatory organ involvement or diffuse skin involvement are usually treated more aggressively with systemic immunal 529 00:35:52.440 --> 00:35:57.280 suppressive therapy like methotrech sate. There's no first line agent and certainly nothing I 530 00:35:57.280 --> 00:36:00.360 feel is necessary to memorize for the exam. They're going to ask you a 531 00:36:00.440 --> 00:36:02.599 question. It will be about the clinical manifestations or the antibodies that went over 532 00:36:02.639 --> 00:36:08.599 in diagnosis, so focus on those, so key takeaway here. First, 533 00:36:08.639 --> 00:36:13.400 this will be a female and divignette. Know your two main types limited, 534 00:36:13.599 --> 00:36:17.960 crest and diffuse. Know that limited involves mainly the distal limbs and your crest 535 00:36:19.000 --> 00:36:22.840 findings, but spares the trunk. No that diffuse involves the trunk and your 536 00:36:22.960 --> 00:36:28.519 organs. No anti centromere aka anti crustomere is associated with limited or crest disease, 537 00:36:29.159 --> 00:36:32.280 and then anti topoisomerase is more likely to be associated with diffused disease. 538 00:36:32.599 --> 00:36:37.599 Treatment again is organ symptoms specific done. Last topic is going to be systemic 539 00:36:37.679 --> 00:36:43.159 lupus erthematosis. Another really big topic, but let's distill it down as best 540 00:36:43.199 --> 00:36:47.119 we can. So this is a multi system immune mediated disorder characterized by antibodies 541 00:36:47.280 --> 00:36:52.960 to nuclear and cytoplasmic antigens. So the specific cause of SL is unknown, 542 00:36:52.000 --> 00:36:55.800 but we do know that many of the clinical minifiztations that we see are caused 543 00:36:55.800 --> 00:37:01.119 by antibody formation and the creation of immune complexes. So these complexes deposit all 544 00:37:01.159 --> 00:37:05.719 over the body, the heart, kidneys, skint, They cause a number 545 00:37:05.760 --> 00:37:08.880 of problems inflammation, tissue damage, cell death. This whole process, by 546 00:37:08.880 --> 00:37:14.760 the way, is a type three hypersensitivity reaction. Surprise, surprise, This 547 00:37:14.840 --> 00:37:17.880 is much more common in women, especially women of childbearing years, so look 548 00:37:17.880 --> 00:37:21.400 for a younger female in the vignette. So a couple of reasons why, 549 00:37:21.440 --> 00:37:22.599 which I'm not going to get into, but it has to do with estrogen. 550 00:37:22.719 --> 00:37:27.079 Also some factors related to the X chromosome which seem to play a role. 551 00:37:28.800 --> 00:37:31.480 This is also going to be more common in African American patients in the 552 00:37:31.559 --> 00:37:37.079 US. African American women are two to four times more likely to have SLEE 553 00:37:37.199 --> 00:37:39.719 than Caucasian women. So the vignette I would be looking for an African American 554 00:37:39.800 --> 00:37:45.880 female. Clinical manifestations literally everything you can think of, hypertension, arthritis, 555 00:37:45.920 --> 00:37:49.480 alopecial and patternopathy, dysphasia, thrombosis, fever, weight loss, fatigue. 556 00:37:49.960 --> 00:37:52.840 Lupus is the great imitator. It affects almost every organ. Therefore, it 557 00:37:52.880 --> 00:37:58.280 can present like so many different diseases. There's a ton of clinical manifestations and 558 00:37:58.320 --> 00:38:00.639 it would be very unwise to memory rise them all. Let's just talk about 559 00:38:00.639 --> 00:38:05.360 the ones they'll likely test you on and the more common one. So first, 560 00:38:05.679 --> 00:38:09.519 you have your constitutional symptoms, fatigue, fever, weight loss. These 561 00:38:09.559 --> 00:38:14.840 are all very non specific, but almost every patient with sl will have these 562 00:38:14.840 --> 00:38:17.960 symptoms at some point during the course of their disease, so they're very common. 563 00:38:17.960 --> 00:38:23.079 They're just not very specific. Next, rash and a melar distribution aka 564 00:38:23.119 --> 00:38:28.599 your butterfly rash, sparing the nasal labial folds, ring the alarms. This 565 00:38:28.679 --> 00:38:31.639 is the thing to know when we're talking about clinical manifizations for lupus. If 566 00:38:31.639 --> 00:38:35.639 you can only pick one thing to know for the exam for clinical manifizations, 567 00:38:35.679 --> 00:38:38.079 this is going to be it. So a melar rash can occur in other 568 00:38:38.119 --> 00:38:43.079 conditions, but if you see it on an exam question, especially if they 569 00:38:43.079 --> 00:38:46.280 see it spares the nasal labial folds. It is lupus, so it's the 570 00:38:46.280 --> 00:38:51.239 most common skin lesion to have in lupus, and it presents as erithema and 571 00:38:51.280 --> 00:38:54.639 a melar distribution over the cheeks and nose, sparing the nasal labial folds. 572 00:38:54.960 --> 00:39:00.280 It normally appears after sun exposure, or almost always appears after sun exposure. 573 00:39:00.400 --> 00:39:04.880 So you have this photosensitive rash that shows up. And remember that word photosensitivity 574 00:39:05.239 --> 00:39:08.719 as it's really important because many of the skin manifestations in lupus appear after sun 575 00:39:08.760 --> 00:39:14.400 exposure. The rash looks like a butterfly over the nose and cheeks, and 576 00:39:14.480 --> 00:39:16.760 it's in a melar distribution. In case you're not familiar with the term, 577 00:39:16.800 --> 00:39:20.760 melard is just another name for the zygomatic bone of the face, which are 578 00:39:20.800 --> 00:39:24.840 your cheekbones. So basically malar distribution just means over the cheekbone area. Remember 579 00:39:24.880 --> 00:39:29.519 this rash spares the nasolabial folds, which are the little creases from the corner 580 00:39:29.519 --> 00:39:31.079 of your nose to the corners of your mouth, and this gives it that 581 00:39:31.159 --> 00:39:36.239 butterfly wing appearance. So remember rash over the cheeks and nose, sparing the 582 00:39:36.320 --> 00:39:39.960 nasolabial folds after sun exposure. Remember that rash, memorize it, know it 583 00:39:40.000 --> 00:39:44.679 well. It will come up next your discoid lesions. While we're on the 584 00:39:44.679 --> 00:39:46.920 topic of skin manifestations, the next one that you should know is your discoid 585 00:39:46.960 --> 00:39:51.840 lesions or discoid rash or discoid lupus, however you see it come up. 586 00:39:52.440 --> 00:39:58.920 These are these round or coin shaped erathematose raised patches that often cause this pretty 587 00:39:59.000 --> 00:40:02.159 dramatic atroph scarring. Just an fyi. If you know who the singer Seal 588 00:40:02.280 --> 00:40:06.159 is, who's really big in the nineties, He's sang that song Kiss from 589 00:40:06.159 --> 00:40:09.239 Arose. The scars in his face are actually from discoid lupus legions. So 590 00:40:09.239 --> 00:40:14.840 maybe I'll help you remember and to associate those two. Authritis. So authritis 591 00:40:14.920 --> 00:40:17.280 and arthrologists are going to be seen and around ninety percent of patients with slee 592 00:40:17.679 --> 00:40:22.000 it's often one of the earliest manifestations and a lot of times it's the reason 593 00:40:22.079 --> 00:40:24.880 why these patients come into the office to be seen to begin with. And 594 00:40:24.920 --> 00:40:29.679 then we have our cardiovascular findings. So pericarditis is going to be the most 595 00:40:29.679 --> 00:40:34.599 common cardio complication of sl so that'd be the one to focus on, but 596 00:40:34.639 --> 00:40:37.840 they can also have issues related to endocarditis, higher risk of myocardial in function, 597 00:40:38.159 --> 00:40:43.199 so just remember the increased risk of cardiovascular problems in these patients. Next, 598 00:40:43.280 --> 00:40:45.960 renal, So around fifty percent of patients will have some sort of renal 599 00:40:45.960 --> 00:40:51.559 manifestation, whether it's the materia, proteinuria, nephrodic syndrome, glomarulan nephritis, 600 00:40:51.840 --> 00:40:57.679 secondary hypertension. Look for problems with the kidneys, and patients with sl thrombot 601 00:40:57.719 --> 00:41:01.679 embolic disease. So throwmbot embolic disease is a potential complication of SLEE, particularly 602 00:41:01.679 --> 00:41:07.559 in the context of antiphospholipid antibodies. The tenure risk of a thrombotic event for 603 00:41:07.599 --> 00:41:10.480 a patient with SLS actually around thirty three percent. And then we have our 604 00:41:10.519 --> 00:41:15.039 hematologic abnormality, so all three blood cell lines can be affected in patients with 605 00:41:15.159 --> 00:41:19.079 sl so your red blood cells, whitelod cells, platelets, so I'll be 606 00:41:19.119 --> 00:41:23.559 looking for anemia, leukopenia, thrombocytopenia. So even though I just went over 607 00:41:23.599 --> 00:41:27.800 a ton of clinical manifestations, there's a bunch that I didn't even touch on, 608 00:41:28.119 --> 00:41:31.320 and I'm not going to neurologic problems like strokes, seizure, GI problems 609 00:41:31.320 --> 00:41:37.400 like aesophagitis, pancreatitis, pulmonary issues like pleuritis, pneumonitis, oral ulcers. 610 00:41:37.719 --> 00:41:39.239 The list just goes on and on. But the ones I went over, 611 00:41:39.360 --> 00:41:43.119 those are the ones that I'd focus on. Those are the ones that often 612 00:41:43.119 --> 00:41:46.280 come up on exam questions. And the way if you want to remember those 613 00:41:46.360 --> 00:41:52.440 is by remembering the mnemonic MD chart. So MD chart an MD chart like 614 00:41:52.599 --> 00:41:59.159 doctor medical doctor chart, like a doctor's chart that stands for The M stands 615 00:41:59.159 --> 00:42:01.559 for me lar rash, the D stands for discoid lesions, and then the 616 00:42:01.679 --> 00:42:06.920 chart part of it. The C stands for both constitutional and cardiovascular manifestations. 617 00:42:07.239 --> 00:42:10.800 The H stands for hematologic abnormalities, the A stands for arthritis, the R 618 00:42:10.880 --> 00:42:15.960 stands for renal, and then thromboembolic disease is what the T stands for. 619 00:42:15.000 --> 00:42:19.400 So just remember, if you want to remember some of the main clinical manifestations, 620 00:42:19.559 --> 00:42:22.480 remember m D chart, and that's how you remember some of the main 621 00:42:22.519 --> 00:42:25.320 ones. So for diagnosis of lupus, like so many things in rheumatology, 622 00:42:25.320 --> 00:42:30.760 it's a complicated one. It's not often based off of any single lab result 623 00:42:30.800 --> 00:42:37.199 or imaging study, but rather recognizing this spectrum of clinical manifestations accompanied by supportive 624 00:42:37.719 --> 00:42:42.880 serologic studies and excluding a number of alternative diagnoses. With that being said, 625 00:42:42.880 --> 00:42:45.679 from an exam standpoint, it's a bit easier as it often is. There's 626 00:42:45.679 --> 00:42:49.360 really just a few labs that you need to know. So first let's start 627 00:42:49.400 --> 00:42:52.400 with your ANA, your anti nuclear antibodies. I've mentioned this and some of 628 00:42:52.400 --> 00:42:55.000 the other ones too, but this is your screening test. This is where 629 00:42:55.039 --> 00:43:00.199 you start your anti nuclear antibodies ANA. Because this test is very sensitive, 630 00:43:00.239 --> 00:43:06.679 it's positive and virtually all patients with sl The problem is it's not very specific. 631 00:43:06.920 --> 00:43:09.239 It's positive in a number of other conditions. The reason you get this 632 00:43:09.320 --> 00:43:14.159 test is because if it's negative, well not to say that it's impossible for 633 00:43:14.199 --> 00:43:17.320 the still to be lupus, but much much less likely. So screen with 634 00:43:17.400 --> 00:43:22.119 your ANA if they have lupus, around ninety five percent chance this is going 635 00:43:22.159 --> 00:43:24.079 to be positive. And then you move on to your more specific labs, 636 00:43:24.119 --> 00:43:28.000 the ones that you really need to know. And first that's going to be 637 00:43:28.119 --> 00:43:32.760 your anti ds DNA or anti double stranded DNA and then your anti Smith antibodies. 638 00:43:32.800 --> 00:43:35.760 Those are the two that you really need to focus on for lupus. 639 00:43:36.039 --> 00:43:40.119 They're both highly specific for lupus. So remember that Smith and double stranded DNA 640 00:43:40.199 --> 00:43:46.159 associate those two very important. So antids DNA anti double stranded DNA anti Smith 641 00:43:46.159 --> 00:43:50.159 antibodies very specific for lupas. You need to know those. And then I 642 00:43:50.199 --> 00:43:52.199 have a mnemonic for those two. This is definitely a weird one, but 643 00:43:52.320 --> 00:43:55.360 sometimes the more weird it is, the easier it's to remember. So the 644 00:43:55.440 --> 00:44:01.239 word lupus sounds like the name Lu, like Lulu and piss like taking a 645 00:44:01.239 --> 00:44:06.079 piss being so I used to remember a guy named Lou taking a piss on 646 00:44:06.159 --> 00:44:09.000 his Smith and Wesson double barrel shotgun. Not sure why he's peeing on a 647 00:44:09.039 --> 00:44:13.679 shotgun. Maybe he's very anti gun. Anyway, Smith and Wesson is the 648 00:44:13.679 --> 00:44:16.679 famous gun manufacturer and a double barre shotgun as well, a double barrel shotgun. 649 00:44:17.039 --> 00:44:21.679 So Smith and Wesson helped me remember the anti Smith antibodies, and double 650 00:44:21.719 --> 00:44:25.159 barrel shotgun helped him me remember the anti double stranded DNA antibodies. So remember 651 00:44:25.199 --> 00:44:28.360 when you see lupus, I want you to think of a guy named lou 652 00:44:28.480 --> 00:44:31.719 taking a piss on a Smith and Wesson double barrel shotgun. Create that visual 653 00:44:31.880 --> 00:44:35.320 in your head and you'll remember the two main labs you need to know for 654 00:44:35.400 --> 00:44:39.199 lupus, anti SMITH and anti double stranded DNA. One other lab I wanted 655 00:44:39.239 --> 00:44:43.280 to really quickly mention that you should be on the lookout for is your anti 656 00:44:43.280 --> 00:44:46.599 fospiphul lipid antibodies. Forty percent of patients with sl will be positive for anti 657 00:44:46.599 --> 00:44:52.639 FOSPIHL lipid antibodies, which is associated with anti fospiphil lipid syndrome, and patients 658 00:44:52.639 --> 00:44:57.719 with the syndrome matter of high risk for venus arterial thrombosis miscarriages. So I 659 00:44:57.760 --> 00:45:00.840 just wanted to mention that in case it does come up treatment. So there 660 00:45:00.920 --> 00:45:02.760 is a lot to know overall for lupus. But the good thing is for 661 00:45:02.840 --> 00:45:07.039 meds, there's really only one medication that you should focus on for the exam, 662 00:45:07.079 --> 00:45:10.039 and that's hydroxy chloroquin. So all patients with lupus need to be on 663 00:45:10.159 --> 00:45:17.920 hydroxychloroquin lupus. Hydroxy chloroquin lupus, hydroxy chloroquin, hydroxychloroquine lupus. Associate the 664 00:45:17.960 --> 00:45:22.440 two in your brain forever. This medication improves constitutional and MSK symptoms, it 665 00:45:22.480 --> 00:45:28.039 improves the mucocutaneous manifestations. It even has a positive impact on patient survival. 666 00:45:28.239 --> 00:45:32.320 So remember this medication for lupus and then also be aware of your steroids and 667 00:45:32.440 --> 00:45:37.519 end said. Some immunosuppressive agents like microphenolate can also be used for treatment and 668 00:45:37.559 --> 00:45:43.559 combination with hydroxychloroquin. Steroids are especially helpful in severe or life threatening cases where 669 00:45:43.599 --> 00:45:46.559 you can use these high dose ivy pulses of methylpredness owe to control the disease 670 00:45:46.760 --> 00:45:52.320 and all tissue injury. But the main takeaway here is just to remember hydroxychloroquin 671 00:45:52.760 --> 00:45:55.000 so lupus triple distilled. This will be a female in the vignette. She 672 00:45:55.039 --> 00:46:00.360 will likely present with the MD chart manifestations we went over screen with your ana. 673 00:46:00.480 --> 00:46:02.079 Then remember Lou took a piss on a Smith and West and double barrerow 674 00:46:02.119 --> 00:46:06.880 shotgun, anti smith and anti double stranded DNA. Those are the specific ones 675 00:46:07.079 --> 00:46:09.840 treatment. Remember hydroxy chloroquin and that is lupus. Let's wrap it up with 676 00:46:09.840 --> 00:46:15.679 five quick questions. Question one, forty two year old female presents the office 677 00:46:15.719 --> 00:46:19.519 complaining of heartburn, small white lumps on her fingers as well as a tight 678 00:46:19.599 --> 00:46:22.480 feeling in her hands that makes it difficult to make a fist on physical exam, 679 00:46:22.519 --> 00:46:27.119 you knowe tolange ectasias on the palms and face. Labs are positive for 680 00:46:27.159 --> 00:46:31.920 both anti nuclear antibodies as well as anti centromere antibodies. What diagnosis should be 681 00:46:32.000 --> 00:46:37.400 suspected in this patient? So that would be limited systemic sclerosis aka CREST. 682 00:46:38.039 --> 00:46:42.519 So this patient has a very classic presentation and presents with a number of the 683 00:46:42.559 --> 00:46:45.960 manifestations of CREST syndrome. So we see the calcinosis cutus, those small white 684 00:46:46.000 --> 00:46:50.800 calcium deposits in her hands. She has heartburn which is from the esophageal this 685 00:46:50.920 --> 00:46:55.840 motility disorder tolange ectasia's as well as the tightening of the skin of the hands. 686 00:46:55.880 --> 00:47:00.360 This is a classic limited systemic sclerosis which we know will generally have a 687 00:47:00.360 --> 00:47:06.320 positive a NA and then most importantly a positive anti centromere and a body test 688 00:47:06.559 --> 00:47:09.880 aka anti Crestomere. Two, a forty seven year old female presents to the 689 00:47:09.880 --> 00:47:15.119 office complaining of dry mouth and dry eyes for several months. She has used 690 00:47:15.119 --> 00:47:19.760 over the counter eye drops with minimal improvement. Physical exam reveals dry mucous membranes 691 00:47:19.800 --> 00:47:22.440 and swollen swollen, parodied glands. You explain to the patient you will be 692 00:47:22.480 --> 00:47:25.960 performing a test to assess for tear production. What is the name of the 693 00:47:27.000 --> 00:47:30.480 test that will be performed? So that is going to be the Schermer test. 694 00:47:30.559 --> 00:47:32.920 So this patient very likely has Schoburn syndrome. Of course we would also 695 00:47:32.960 --> 00:47:37.199 need to perform some labs anti row, anti law NA, but she has 696 00:47:37.199 --> 00:47:39.920 all of the classic clinical manifestation's dry eyes, dry mouth, product land enlargement. 697 00:47:40.000 --> 00:47:44.280 And the test we've performed to assess for tear production is the one called 698 00:47:44.280 --> 00:47:46.800 the Schermer test. Question three. A fifty one year old female presents to 699 00:47:46.840 --> 00:47:52.480 the office today complaining of muscle weakness. She describes difficulty combing her hair rising 700 00:47:52.519 --> 00:47:54.679 from a chair. A physical exam, you know, a rash around the 701 00:47:54.760 --> 00:48:00.800 eyes and the eyelids, violaceous papules of the dural dorsal aspect of both hands, 702 00:48:01.079 --> 00:48:05.400 as well as erathema across the shoulders, upper back, and upper chest. 703 00:48:05.800 --> 00:48:09.079 Labs are drawn which show an elevated creating kinase level as well as a 704 00:48:09.159 --> 00:48:15.159 positive antime two antibodies. What treatment should be initiated in this patient for the 705 00:48:15.199 --> 00:48:19.639 suspected diagnosis. So that is going to be glucocorticoids. So this patient has 706 00:48:19.760 --> 00:48:23.880 dermato monocytis, She has the gotron papule's heliotrope rash, decreased muscle strength, 707 00:48:23.960 --> 00:48:29.679 the Shaw sign, plus elevated CK and antimeto antibodies. That's about as clear 708 00:48:29.679 --> 00:48:32.559 cut as you can get, and then we know for dermato myocytis. Glucocorticoids 709 00:48:32.599 --> 00:48:37.320 are the cornerstone of your initial therapy. This is usually pregnos own added dose 710 00:48:37.400 --> 00:48:40.920 of one miligram per kilogram per day. Question four. A forty one year 711 00:48:40.960 --> 00:48:45.199 old female has symptoms consistent with rheumatoid athritis and labs are drawn to assist in 712 00:48:45.280 --> 00:48:50.719 making the diagnosis. The physician assistant informs the patient that a rheumatoid factor as 713 00:48:50.719 --> 00:48:55.039 well as a very specific antibody for rheumatoid arthritis are both elevated. Which antibody 714 00:48:55.159 --> 00:49:00.039 specific to rheumatoid arthritis is likely elevated in this patient. So that is going 715 00:49:00.079 --> 00:49:07.039 to be your anti citrilinated anti citrilinated peptide antibodies, aka your anti ccpanibodies. 716 00:49:07.280 --> 00:49:12.519 So your anti CCP anibodies are very specific for rhumatoid. Right, it's usually 717 00:49:12.519 --> 00:49:15.960 over ninety percent specific to the disease. So if they ask for the most 718 00:49:15.960 --> 00:49:19.480 specific test for RA, generally this is going to be your anti CCP, 719 00:49:19.719 --> 00:49:22.760 whereas rheumatoid factor is more sensitive but not as specific. And as I mentioned 720 00:49:22.800 --> 00:49:29.599 before, if you can remember which is the specific antibody for RA. Specific 721 00:49:29.760 --> 00:49:32.519 is spelled with two c's, look for the antibody that has two season it 722 00:49:32.719 --> 00:49:37.480 that's your anti CCP. Question five. Last one to twenty six year old 723 00:49:37.519 --> 00:49:39.960 female presents to the office complaining of fatigue, joint pain, and a low 724 00:49:40.000 --> 00:49:44.599 grade fever for the past few weeks. She also reports that she develops a 725 00:49:44.679 --> 00:49:46.920 painful rash after being in the sun just for a short period of time. 726 00:49:47.800 --> 00:49:52.880 Physical exam, you notice a rash that is distributed over the cheeks and nose, 727 00:49:52.400 --> 00:49:58.880 sparing the nasal labial folds, as well as diffuse discoid lesions. What 728 00:49:58.960 --> 00:50:02.639 would be the best initial test to order in this patient? So that is 729 00:50:02.679 --> 00:50:07.559 going to be your anti nuclear antibody, your ANA. So this patient has 730 00:50:07.679 --> 00:50:12.480 systemic lupus or athematosis or obviously this should be at the top of your list 731 00:50:12.679 --> 00:50:15.559 of differentials. Anytime you have a young female of childbearing age, complaining of 732 00:50:15.639 --> 00:50:20.480 joint pain, rash and fever, always be considering lupus. On exam, 733 00:50:20.639 --> 00:50:23.719 she has the classic melar butterfly rash that spares the naso labial folds as well 734 00:50:23.760 --> 00:50:29.000 as the discoid lesions, and she describes a photosensitive rash that burn after a 735 00:50:29.000 --> 00:50:31.760 short period of time in the sun. So the best initial or screening test 736 00:50:31.800 --> 00:50:36.440 in a patient you suspect may have lupus is going to be your ANA anti 737 00:50:36.519 --> 00:50:38.840 nuclear antibody. So it's not a specific test, but very sensitive, and 738 00:50:38.880 --> 00:50:43.480 this is where you'll always start when screening for lupus. Then after obviously you 739 00:50:43.519 --> 00:50:46.559 proceed to your more specific antibodies, your anti double stranded DNA and your anti 740 00:50:46.599 --> 00:50:51.719 Smith antibodies. All right, So that was Rheumatology Part two. I hope 741 00:50:51.760 --> 00:50:53.639 that was helpful. Thank you as always for listening to the podcast, and 742 00:50:53.719 --> 00:50:57.719 good luck on your pants, your pantor your ears, and good luck in 743 00:50:57.760 --> 00:50:58.400 PA school.