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Lipid syndrome, dermatomyositis, polymyositis, androgens, just to get a picture of some of the main antibodies that might come up in your KL MMC Qs. So we're first gonna have a little look at systemic sclerosis. So this is an autoimmune condition which affects your connective tissues. It's characterized by increased fibrosis and it often affects the skin and then some of your internal organs, the cause of it is um unknown at present. They think it involves the activations of your fibroblasts which then leads to excessive collagen production which causes your skin manifestations of the condition. And then you also get um angiopathy and blood vessel remodeling. So this can present with your tang aas. There is also an association between uh systemic sclerosis and other msk rheumatological conditions um which are autoimmune. So things such as sle myositis and sros, you can sometimes have a 20% overlap. What you'll find is um once you, once you have one autoimmune disorder, you're at more risk of further autoimmune disorders. So you often find that patients will also have uh other things going on alongside it. So there are two main types of that. You need to be aware of the first one being limited cutaneous systemic sclerosis. So this one involves skin, which is often below the elbows and the knees. So this would be things like your hands and your feet and then your face, you're less likely to have internal organ involvement with lim limited cutaneous a um stomach sclerosis. The symptoms, you can remember it with the pneumonic crest because it used to be referred to as Crest syndrome. They moved away from calling it Crest syndrome. But it's a good pneumonic for remembering some of the symptoms that you might see in patients. So that can be things like calcinosis, which is build up of calcium under your skin, which you can see in that photo there, you can get Raynaud's, which is um also shown in the image under the R. So it's where you get uh discoloration of the fingers due to um poor circulation and blood supply. Often patients will present first with Raynaud's and then go on to develop the other symptoms. You get esophageal disability. So this can present with symptoms of reflux or if patients are having swallowing difficulties with both liquids and solids, you get sclerodactyly, which is the tightening of the skin and you can see it on the hands here. And that is where you get a thickening and that will be from your excess collagen production that we talked about before. And then you get tang, which would be your angio uh the uh blood vessel uh abnormalities that you get with uh systemic sclerosis. Some patients will go on to develop pulmonary arterial hypertension, but it's less common and limited as compared to the other type of systemic sclerosis. But it's just something to be aware of that. It might come up in the, in um in the question. So they might have all those symptoms and have some internal organ involvement. Um It's just less common with limited. The second type is diffuse cutaneous systemic sclerosis. So this is where your skin involvement is in your proximal limbs and your trunk, you have higher rates of internal organ involvement. So if there's internal organ involvement, it's more likely to be diffuse uh symptoms you can get is you can get the tight dry or itching skin. So this can be in your proximal limbs, the closest to you or your trunk. Um You can get Raynaud's as well like you can get in limited patients might present with shortness of breath and this can be as a result of lung fibrosis. You might get palpitations due to cardiac involvement and you can also develop a sclerodermal renal crisis. So we can see this or identify this in patients if they've got a sudden rise in their BP or if they've got baseline hypertension and it's just getting slightly worse. Um That can be some of the first signs that they're having a scleroderma renal crisis. So, what is a scleroderma renal crisis and what causes it. So there's lots of different theories. Um but from what the reading that I did around it, you get endothelial cell activation, which can lead to smooth muscle proliferation in the vessels, which leads to hypertrophy of the vessels. And then the blood is then at a higher um a higher rate and less of it is getting to the kidney which can lead to ischemia and then patients can present with acute an acute kidney injury. So they might have a rising creatinine, rising urea on their blood test and then they get severe hypertension cause the the kidney is trying to compensate for this lack of blood flow by increasing the BP to see if it can um increase blood to the kidney. Um When you're thinking about investigations, you're thinking, oh, I think this might be systemic sclerosis. What sort of investigations might you offer or if it's an ACY, for example. So I always split it into bedside blood and then imaging. So at the bedside, you can do BP. So this can lead you to a diagno diagnosis of a um renal crisis. You can do an ECG to look at um any heart involvement. So you might get some right ventricular hypertrophy, which you can see on an ecg. Um if they've got pulmonary artery hypertension, then urinalysis as well to look for renal involvement. So that can be things such as protein urea or hematuria blood wise, we do an F PC patients might be anemic. And then our using these will help guide ours on their renal function and then we can send off some autoantibodies. So, what autoantibodies are associated with systemic sclerosis? So you can widely do an antinuclear antibody which will be uh positive in about 95% of patients, but it's not specific to systemic sclerosis. So just because they're antinuclear positive, doesn't mean that you, you can say it's a diagnosis of that, you've got more specific antibodies. So these include anti centromere. So these are more likely associated with limited cutaneous systemic sclerosis. And then you've got anti 70 which are more likely positive with your diffuse. So more likely to have internal organ involvement. I remember it as 70 is quite a high number and diffuse is like more, it's more severe. So I remember 70 is with the more severe type which is diffuse. Um and then there was also anti RNA poly three antibodies, which is a new one from when I was revising. So these are more likely to be positive in patients who are associated with a rapid skin disease and renal crisis. So with the more severe forms, but for your exams, I would just know that anti centromere is limited and then anti 70 is for diffuse. Um management wise, there's no cure for systemic sclerosis. Um Treatment is focused on alleviating the symptoms that patients feel and then preventing any further deterioration in internal organ involvement or preventing it altogether. So some things that you can suggest for patients is education around Raynaud's. So avoiding certain triggers. So keeping your hands warm by using gloves or hand warmers and then managing of an episode of Raynauds. So teaching patients to slowly warm up their hands and feet and they can do massages to increase circulation. Physiotherapy and ot have a role, especially if hands and legs are affected. We can give emollients for the skin, the dry skin um that you see and then we can use systemic agents to manage the skin disease and then internal organ involvement. So these would be things like methotrexate cyclophosphamide, my clophen aate and then biologics if those have not been effective, a thing to note with systemic sclerosis is that high dose or long courses of steroids can cause a renal crisis. So we need to use them in caution. So in a question, it is very unlikely that the answer will be steroids as a treatment for systemic sclerosis. Um So here's just a multiple choice question. It'd be really good if you guys could put the answer that you think it is in the chat. So a 57 year old female has noticed that the skin on her hands has become very tight and that her fingers sometimes turn blue. She's also had difficulty swallowing both liquids and solids. What autoantibody is most associated with these symptoms? Do we think it's ABCD or E so it's most likely going to be a Yeah, you're correct. So, a anti, so if you look and break down the question, we can see that they are trying to get that she has sclerodactyly because the skin on her hands has become very tight and her fingers sometimes turn blue. So fingers turning blue makes you think Raynaud's, she's got sclerodactyly, she's having problems swallowing. So she's got esophageal disability. So those would be leading us towards limited cutaneous. So and the antibody associated with that is anticentromere. So, yeah, really good. Moving on. Has anyone got any questions before we move on to about systemic sclerosis that they want to ask in the chat? I take that as a no. So we'll move on. So se systemic lupus arrhyth is um a condition. There's an a another autoimmune condition which is multisystemic multi sys, it affects multiple systems in the body. The cause of it is mostly unknown. It's thought to have both genetic and environmental factors that influence it. It's been found that carriers of the HLA Dr two and Dr three are more susceptible to developing s um also environmental factors can include drugs. So drugs can cause um certain drugs can trigger you to develop se. So these are procainamide