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Non-malignant haematology and connective tissue disease: Finals Revision

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Summary

This medical lecture explores two medical cases, examining the differential diagnosis and management of lupus and rheumatoid arthritis. Specifically, attendees will learn about which is the most sensitive antibody test for lupus, the clinical criteria for lupus diagnosis and the management of lupus and anti-phospholipid syndrome. Other topics of discussion will include the diagnostics and management of rheumatoid arthritis, the most specific antibody test for it and which medications are commonly used. This thought-provoking and interactive session is a must-attend for medical professionals.

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Description

Sasha Bill (MBBS) provides an overview of the key concepts relating to haematology and connective tissue disease. This lecture is aimed at UK medical students taking their final written examinations.

Learning objectives

Learning Objectives:

  1. Identify the top differential diagnosis of a 35-year-old female with a long-standing history of mouth ulcers, alopecia, and fatigue.

  2. Explain the most sensitive antibody test for screening for systemic lupus erythematosus (SLE).

  3. List the criteria for diagnosing SLE.

  4. Describe the management of anti-phospholipid syndrome.

  5. Recall the most specific antibody for investigating rheumatoid arthritis and name the common drug used to manage it.

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Computer generated transcript

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The following transcript was generated automatically from the content and has not been checked or corrected manually.

Thank you so much for signing on. I'm sure it's the last thing you want to be thinking about right now, thinking about another exam, but you're nearly there. You've got one down, you've got for you to go. So hopefully I'll keep it kind of concise and you'll learn something from this lecture. But um yeah, so I'll make a start. So, so case one, so a 35 year old female presents to your G P with a long standing history of mouth, ulcers, alopecia and fatigue. She has had joint pains for the past few weeks. So, on examination, she's got tender swollen, MCP and P I P joints bilaterally and on a past medical history, it says that she's had a recent admission to hospital with pleurisy. So what is your top differential diagnosis in this case, if you just want to pop the fee in the chat if you want to and hopefully everyone can hear me as well? Yes. So I can see if you're coming in there. So that's it. So, um sle so lupus is the top differential diagnosis. Um So you can kind of see that from obviously kind of rheumatoid is probably the other thing that people were thinking of but and just with the mouth ulcer is alopecia and fatigue. Um And the past Matic history, the recent pleurisy that kind of would point you towards um lupus in this case, more so than rheumatoid arthritis. Um So what is the most sensitive antibody test used for screening sl A again if you just want to pop in the chat when I ask a question, that would be amazing. So, yeah, we've got a bit of a mix of answers. I can see why people are putting down um D S D N A M to see, but the answer is um see um anti nuclear antibodies. So it's a bit of a trick question but kind of when you're asked this question and it's the most sensitive antibody test for sle um and if anti nuclear antibodies there, it's the correct answer. Um So it would be the most sensitive antibody and um kind of anti DST and it is actually an anti nuclear antibody. Um It comes under that bracket, but if you see anti nuclear antibody there, that will be the right answer for sensitive antibody. So um it's present in over 98% of people with Lupus um in terms of anti D S D N A antibody. So it would be kind of the commonest anti nuclear antibody that's, that's seen in Sle. So it's present in about 70% of people. Um and it's the one that's commonly used to monitor the disease um as the levels kind of vary with the disease activity. Um And then in terms of be empty Smith antibody. So they're actually the most specific test um for sle but they're not that sensitive. Um And then Auntie row antibodies, they can be present sometimes as well, but they're more associated with Sugen syndrome. Um So, yes. So if it's most sensitive antibody will be anti nuclear. Um if anti nuclear antibodies, not there, an anti DS DNA is there, then choose that one. And then most specific is anti Smith antibodies. Um And also any questions at any point just pop them in the chat as well. I've got Charlie monitoring the chat. Um and if I see any, I can try and answer as well if I can. Um So yes, Chinese, right. I think that is probably a typical SBA question. Um OK. So just to kind of go over the criteria for diagnosing S L A. So it's the slick criteria. So um it requires four or more of these criteria and it needs to have at least one clinical and one lab criteria or biopsy proven lupus nephritis um with positive ana or anti D N A. So in terms of the clinical criteria, then the kind of acute cutaneous lupus that's referring to that kind of mallard rash, the butterfly rash that you get with it. Um the chronic cutaneous lupus that's kind of the discoid rash that you get sometimes with it and you can get oral or nasal ulcers, the alopecia, um arthritis. So that's kind of synovitis. This, if you've got evidence of sinusitis, I think it's um involving two or more joints um or kind of two more tender joints um or something like that and then serositis. So, um that's kind of when you get complications of it. So like pleurisy or pleural effusions or pericarditis and are kind of some complications of it and then some renal neurological complications and then you can also go and get anemia, low white cells and low platelets as well on your blood counts. And then in terms of the lab criteria, that's the, the test that we were talking about as well. Um Okay. So that's the kind of the butterfly rash that I'm referring to. I'm sure you've all seen it before. Um Just a reminder. So in terms of the management of S L A, so um I feel the one to remember, the one demarte remember is probably hydroxychloroquine. Um it's the one that's typically used for people um to maintain kind of S L A. So there's no kind of cure with sle it's a relapsing, remitting disease. So, the aim is to kind of control the disease. Um and, and that's how you kind of date with hydroxychloroquine. Um And yeah, in terms of fires and things you can add in kind of a low dose of prednisoLONE or if it's more severe, you can add in a high dose of prednisoLONE. Um But as you, I'm sure you've seen with like all these um autoimmune disorders, you can go on to kind of biologics and loads of other drugs as well. So yeah, the want remembers hydroxychloroquine and then flares. I feel like I remember prednisoLONE is commonly quite used. Okay. So back to our patient then. So are 35 year old lady has unfortunately represented to the G P with recurrent miscarriages. Um Can anyone kind of think about a diagnosis for this? Mhm. Yeah. So I can see some coming in. So anti phospholipids syndrome is right. So, um so it's something that can occur secondary to um some of the other some autoimmune disorders and more commonly lupus, it can occur secondary to. So the diagnosis is kind of one clinical and one lab criteria to diagnose it. So clinical, it's characterized by kind of recurrent venous or arterial thrombosis. Um So like peas and DVTs and, and or fetal loss. So if someone is presenting with her current miscarriages and they've got, they've got an autoimmune disorder. Um that's when you kind of start to think about anti phospholipids syndrome. And I think um you actually can you screen for it as well in their person um in women as well, I think sometimes um and then in terms of the laboratory criteria. So it's a positive positive antiphospholipid antibody on to equip on two occasions, 12 weeks apart. So um those antibodies there, the lip is anti coagulant, anti cardio lipin antibodies and things. They're, they're all anti phospholipid antibodies, I think. Um so any of those antibodies and two equations 12 weeks apart. And then in terms of the management of empty phospholipids syndrome, so you can manage it with warfarin to kind of prevent the blood clots or, and kind of in, in the pregnant lady who's got anti phospholipids syndrome. Obviously, you want to avoid the miscarriages and things. So you can, they can be on a low molecular weight heparin um an aspirin with it as well. Okay. So I've just kind of, I mean, rheumatoid arthritis, I feel like it crosses with lupus a little bit. So I've just added a little bit about rheumatoid as well. So, um if you wanna pop your answer into this question into this question, which is the most specific antibody when investigating rheumatoid arthritis. Yeah, you're all completely right. So um it's anti CCP antibody. So, um I think this is quite a common question as well that you'll probably get in your exams. Um And obviously, rheumatoid factor is the, is the one that you would think about otherwise, probably. Um So anti CCP is the specific antibody when you're looking for kind of rheumatoid arthritis and it's the diagnostic antibody um in terms of rheumatoid factor, it actually, it, I mean, I'm sure you're aware, it's, it can be negative and rheumatoid arthritis. Um but when it's positive and when anti CCP and rheumatoid factor, a positive, that kind of indicates some probably a more kind of severe severe disease. Um So, so yeah, so just remember that the empty CCP is the most specific antibody. Um So just a quick summary and rheumatoid arthritis. Um So it's inflammation of the synovial joints mainly affects the kind of small joints of the hand. So the P the mcps and the P I P S, um it's more common in females pres sense kind of in like peaks around the 40 40 year old and then investigations then for it. So you do some bloods, you'd want to do some ultrasound of the joints. So that's quite sensitive for picking up synovitis. Um So when they get a rheumatology clinic, that's what they'll be doing. Um, if they're having a flare and then you'd want to do some joint x rays to kind of rely other differentials and also um just as a baseline, I suppose. Um And then chest X ray, you'd want to do just as a baseline as well. Um I, I think the methotrexate can cause kind of chest problems. So you'd want to do it before starting on any medications, any dmards. And also I think really it didn't TB can be a differential as well. So it can relate that. Um and then monitoring then, so you do the does 28 they do that when they kind of come to Rheumatology Clinic, um where they count the number of kind of swollen and tender joints out of all the 28 joints. Um, and that kind of gives them and I think it's a CRP as well and that gives them a number um to see how it's going. And then diagnosis is that you are a cr criteria, which is another thing that you just kind of have to learn off. Um And then in terms of management, then, so first line you'd start your DeMars. So they normally take about six weeks to actually work. So um they tend to give them kind of empty inflammatories like nsaids or kind of an I am steroid injection, um kind of at the start just to control the disease. Um And, and then the DeMars will start to kick in and the DeMars are more to kind of control the disease. Whereas the empty inflammatories, they're kind of when people are having disease flare ups or at the start just to kind of control it. Um Well, just to kind of settle the flare down, whereas the day dmards kind of help you prevent you going into that flare. So you start off, kind of tend to use methotrexate to start off with. But obviously, there's lots of contraindications and what reasons people can't tolerate it and things so you can start on any of the other dumb Ard's. Um If that doesn't work, use a combination of the dimard and if it's more severe than that, you can obviously move on to your biologics. Um for which there are a lot of, so I've included one example, but I mean, it just depends on the patient, I think for which one you use. Um So that's kind of a summary in rheumatoid arthritis then so we'll move on to kiss to then I hope I'm going at an okay pace, just pop in the chat if you want me to slow down or speed up or whatever. Um Okay. So case too then. So a 59 year old female presents to your GP with new headache, pain on chewing food and painful hips and shoulders. What is the key complication you want to prevent in this diagnosis? Yeah, you're right there. So it's visual loss. Um So the diagnosis here is giant cell arthritis. So um kind of things pointing to that is I suppose your age 59 year old lady, it tends to happen in kind of older age female that happens more commonly and and kind of headache, pain on chewing food, painful hips and shoulders. Very classic symptoms of giant cell arthritis that you can't, you don't want to miss it as a diagnosis because it can cause kind of permanent visual loss. Um And that's the key you need to remember that. I think that's quite common question as well, for SBS um that, that's just why you want to treat it immediately, kind of to prevent that visual loss, especially if they're ever having kind of any um transient loss of vision or whatever. Um it's important to treat it quite early. So in terms of kind of the features of it. So as we said, so, temporal headache, um glass jaw claudications. So pain when they're chewing foods, scalp tenderness. So kind of when, if people are combing their hair or washing the hair, whatever, that's when they'll get pain, um polymyalgias. So that's the pain and kind of the hips and the shoulders. So um in terms of when someone kind of presents just with the kind of pain in their hips and shoulders and it's kind of a 60 year old female and you're thinking polymyalgia rhuematica, um it's important as well to reel out giant seller try to. So you kind of um just just ask about screen for all these symptoms and if they're not present, then it is unlikely. So you don't need to go anything further. But um if they have kind of any of these symptoms and you're suspicious of it, you obviously need to act on that. Um And then obviously, if you get visual symptoms such as blurring or kind of transient loss of vision than um that's even worse and you really would need to start them on treatment immediately. Um on examination sometimes, I mean, you can see tender, decreased pulsation or thicken temporal arteries. Um and investigations raise a esr is kind of the thing that you're looking for in their blood tests. Um when they go to rheumatology, they can ultrasound their temporal arteries and they can have a little look at them to see what they think they might want to do a biopsy if they're unsure. Um and then in terms of management then, so it's a steroid. So they tend to start them on 60 mg orally of steroids um immediately and I think kind of when GPS are suspecting as well, they can start that um kind of in the G P and refer them to rheumatology. Um So they continue that and then you can go on to taper down the dose. Um If you're having kind of any visual loss or translate visual loss, then you can do IV methylprednisolone for that. So, so this is just a short question. Um So spot diagnosis question. So a 12 year old boy goes into the G P with this rash. It developed over a few days past medical history, had recent tonsilitis and he's also got asthma. Does anyone know what it could be? Yeah, it's got hit psoriasis. Um So they like to kind of throw in pictures and things in your SBA exams. So, um psoriasis is one of your core conditions. So I just thought that I feel like psoriasis. I didn't, this is kind of the second most common type of psoriasis. So, um, it's just quite an easy one to remember, I suppose that it's, it kind of presents where, um, it's typically a few weeks after someone has an infection. Typically streptococcal infection, um, cause most common is tonsilitis. Um, and yeah, this kind of rash develops widespread, um, mainly over their trunk, um, and their limbs and, um, yeah. So in terms of kind of diverted the summary, I think I might have. Yeah, so um it often kind of clears in a few months without any treatment or anything. Um but unfortunately, about 25% of people then develop into chronic plaque psoriasis after it. Um So, so yeah, so I think that's just want is to remember as well. So moving on to case four then, so uh 62 year old female patient undergoes investigation for osteoporosis. Her T score is minus 2.3. What can be deduced about her diagnosis from the score if anyone can pop it in the chat, if what they think. Yeah, that's right, everyone. So it is see. Um So it's basically the T score is compared to healthy year old, healthy 30 year old female. Um and a score of minus 2.3 indicates osteopenia. So osteopenia um is between minus one to minus 2.5 and osteo process is below minus 2.5. Um The T score is kind of when you compare it to a healthy, 30 year old female. Um whereas the Z score is when you compare it to someone of kind of their same sex, same age. Um So that's just to remember as well. So in terms of osteo process, so it's a reduction in uh I mean, it's a reduction in bone density. Um So it means that the more potent effect fractures, risk factors to remember just in case is that they'll present to in the SBA so older age, female richest activity, they've got a low B M I um rheumatoid arthritis, alcohol and smoking, long term cortico steroids. Um So that can be even like a few months and then just some other medications can cause it as well. And then in terms of investigations that you want to do, so you do routine bloods, obviously, if they're presenting with a fracture, you'd want to do an X ray. Um You can do a Dexa scan. So just X rays of all the bones and stuff and that can calculate their bone mineral density score, which is reported as A T score and said score. Um and then you can work at a Frax score as well, which is kind of their risk of fragility fracture within the next 10 years. Um So, so another question then, so um you've been asked to start a patient on Alendronic acid, 10 mg once daily to treat osteoporosis, which of these is not a known side effect of this drug. Oh, oops, just keeps getting you the answer. But you've already told me anyway. So, yes, it's the, um, so venous thromboembolism. So it doesn't, it doesn't tend to cause BTS, but more pointing towards it can cause a lot of side effects. Um, and you have to counsel, patient's a lot on this medication. So, and you've got to warn them of these sort of side effects. So, atypical femoral fractures, osteonecrosis of the jaw and a soft jail ulcer or a common side effects. Um And, and then in terms of kind of the safety advice, um it's important to remember when you're kind of counseling them. So it should always be taken on an empty stomach, sitting upright at least 30 minutes before breakfast with plenty of water. Um So that's kind of to prevent the reflux and this off the jail erosions and ulcers. Um Patient should be told to kind of report any thigh, hip or groin pain and that's looking at the atypical fractures and to maintain good oral hygiene have regular dental checkups because it can cause osteonecrosis of the jaw and report any ear pain discharge from the air air infection because it can cause is it like osteonecrosis of the air canal? Um And then obviously you stop taking if any kind of difficulty swallowing and worsening heartburn or any pain on swallowing as well because that could point towards kind of an ulcer or something. Um And in terms of the treatment of um with bisphosphonate. So you can consider what's called a treatment holiday. Um If the, if they've kind of recheck the bone mineral density and it's improved or they have not had any kind of fragility fractures after 3 to 5 years. And so they take this kind of holiday and then they can reassess the situation after that to see if they need to go on them again. Um So that's just quite a common something to always remember about bisphosphonates all the safety advice. Um Okay, that was quite quick. Actually, I thought that I thought I was that was going to take a bit longer of that. So, um but I'll go through my non malignant hematology cases. Um If anyone has any questions or anything, just pop them in the chat at this point as well. Um So first of all, so kiss one then. So a 64 year old gentleman presents or resents too ambulatory care with a three day history of shortness of breath and chest pain. So his past medical history then, so he's got type two diabetes diet controlled and then in terms of his observations. So his SATS are okay. Um 94% on room air respiratory rate is 19 which is okay heart rates 111. So it's, he's a bit tachycardic and BP is fine 112 over 63 then temperature is 38.1 So it's a little bit high. So differential diagnosis. Does anyone have any? Okay. Yes P is, is the correct answer to be fair. Um Yeah. So wait, did I even? Okay? Well, I'll just go through this. The P is the right answer, but yes. So it describes becoming progressively more short of breath over the last few days and he had this sharp pain on his right side of his chest, worse on inspiration. Also reports a small amount of blood in his state and one coughing um on further questioning, reports pain and swelling in his right leg over the last three weeks. So, so it's very classical p um but yeah, all those other ones, lower spirits, a tract infection. I see s um they're all differentials as well. Um So in terms of kind of the, the kind of blood clot screening questions um for my exams, I don't know why this is the only acronym that I could come up with. I'm sure someone's got a much better one but um spam and then two H is into. Yes, was the thing I used to remember. Um So just make sure you ask all of these with in terms of Maslow's. And um there also just quite useful to remember like on an SBA if someone's presenting with some of these things, I've got a history of these things. It's just important to remember the um that the risk factors. So surgery, pregnancy, um age older age malignancy. Um if they're on the kind of combined oral contraceptive or HRT hospitalization, so we're just mobility um fracture. So if they're in a cast, they're not going to be moving around much. And then a fight, obviously for over four hours is a higher risk as well. So obviously, when this man is presenting to hospital, so you calculate a while score can appreciate, probably can't read, read, that's quite small. Um But yeah, you calculate a well score. So um I think the one on the right is for pain and the one on the left is for DVT. So if you've kind of calculate the well score, which is all kind of the risk factors and things and DVT is likely. So then you kind of move on to the imaging. So if it's, if it's a DVT, you're wanting to do an ultrasound Doppler of the leg and if it's A P and the P is likely over four points, then you want to do imaging. So CT P A um and if it's unlikely in both cases, then you do a D dimer and a negative D dimer in that case will tend to reel out um the blood clot then, so um this is a classic medical skill thing. I feel like that you learn with E C G. So um yeah, S one Q three T three um is the kind of is something that points towards P but it's actually quite rare. So the most common thing is sinus tachycardia. Um So high heart rate um in terms of kind of the S one Q 3 to 3 thing, I don't know whether you can see my mice, but it's like an S wave in lead one Q wave in laid three and T wave inversion inlayed three as well. Um So that's what you're looking for, but you're really looking just for a sinus tachycardia. It doesn't really, it just helps the kind of diagnosis, but it doesn't, it definitely doesn't rely or anything if it's not there. Um Okay. So then I'm moving on to case to them. So an eight year old girl is brought to the G P with a strange rush on the backs of her legs. So her mother tells you when she woke up this morning, she noticed purple spots on the backs of her legs. She's got no other pain or other signs of bleeding, no headaches or next stiffness past medical history, asthma, she had a cold two weeks ago. Observations are normal and she's a fibril. So differential diagnosis pop them in the chat. Okay. Um Also I just kind of forgot to say about the management of P and DVT. So, um the reason I didn't include a slide is because it's quite different just depending on what you're on the kind of the trust that you're working in. So, um commonly just a low molecular weight have been such as Louise um, treatment dose tins apparent in Northumbria. But I know that I think the RBI and things might use Dove works. So it just depends where you are really. But you'll want to anti quick them, them obviously and in some cases of a pa, if it's kind of a big massive pee like a saddle p or if they're hemodynamically unstable, you can, um, use ultra players as well. So like thrombolysis, if it's a big P um and then obviously kind of follow them up in a V T clinic um as an outpatient as well. So in terms of this case, then, so yeah, so manager this CHSP um it just p is there any others that people are thinking of? It's mhm So on examination. Um I mean, those are obviously all um these are obviously all differentials in this case as well. But then yeah, I was looking for I T P as well and so on examination, she's got small petechia on the backs of her legs, um non tender, non blanching. So they don't feed away with the glass test, know prepare a or breezing, visible. Um And then the kind of current eggs and bread zinc's is or negative as well, no photophobia. So those are the meningitis tests. Um And then in terms of your full blood count, then hemoglobin's normal white cell count's normal platelets are 50. So they're low. Um and blood film is normal. So, yeah, we're thinking of I T P. So, um, in this case, but H S P as well, I feel like it is. Um, it's, yeah, that would definitely point towards HSP the initial history I give and you always want to rule out meningitis as well. Um, so that's what we've, we've kind of done there and then in terms of immune thrombocytopenia as well. So just a quick summary on this one. So um it's basically an immune destruction of platelets. It's most common in Children. Um in Children, it tends to kind of go away on its own after a few months or after six months or so. Um adults can get it as well and it's more commonly tends to be a bit of a chronic thing with adults. Um And it's just an abrupt onset of this kind of petesch alert, prepare it rash and, and it often precedes viral, respiratory, viral respiratory infection. And so, yes, the defining criteria then. So it's a diagnosis of exclusion. So you want to rely all those other more important diagnoses. Um So when isolated thrombocytopenia, um so platelets below 100 there's no clear other cause. Um So in terms of the management of I T P then, so it's a watch and wait approach. So most of the cases resolve within six months, as I said, um just with nothing done and in terms of treatment, you want to consider treatment if they're bleeding, whether it's minor, whether it's major bleeding. Um, and it's also rarely indicated of platelets are below 50 and they're not bleeding. So you'd want to treat, I mean, the cortico steroids, um, and IV immuno globulins can be used as well. And then obviously if it's more severe and you can move on to kind of immunosuppressive agents such as is a thigh, a prime, um, kind of a platelet transfusion. They're quite ineffective. So you wouldn't use that even though that's kind of the first thing I would think of. Um So yeah, I actually think that's everything. So keeping it very short and sweet today, but I'm sure it's probably needed after your mom Osler's. Um, and I know I didn't touch on like a lot of your core conditions. I don't want to bombard you too much information, but um, we can send out the slides and things if you complete complete the feedback forms. Um And as a reminder just on tomorrow, yeah, tomorrow night, um the SBA quiz is happening. So I know it's the night before your SBA but if you, if you want to come along, um come along and it will just be some questions. Um And yeah, we'll try to get the slides out as soon as we can and yeah, thank you so much for coming and I wish you all the best. Um, don't envy at all. You'll be done soon just briefly before you. Uh Thank you very much, uh, for doing that session. And as you said, yeah, please tune in tomorrow. Uh, if, if you're free, we're gonna sort of will be about 25 questions all based around what we sort of got for our first exam in the fourth year. Um, to tune in, it will be high yield. It will be very, very quick, 45 minutes kind of try to keep it about two minutes a question and all the relevant information about the answers will be on the slides. Um So that will be for all of you guys to take away from it. And yeah, if you don't turn up tomorrow, good luck on Friday. Uh Sasha says, I'm sure it'll do really, really well. You have you got any questions about anything, just leave it in the chart otherwise we'll see you tomorrow night. Thanks. Thanks everybody.