Zero to GP - GP Revision Podcast

Polymyalgia Rheumatica - Essential GP Revision

• Thomas Watchman • Season 1 • Episode 11

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0:00 | 21:51
SPEAKER_02

Hi and welcome back to the Zero to GP podcast. This is Tom, and in this episode, we're going to be going through Polymyalgia rheumatica, the key things you need to remember for your AKT exam. As always, this podcast will feature a question answer explanation format. So I'm going to give you a case and ask you questions. I want you to come up with your answer, ideally, say it out loud or even write it down, and then I'm going to go through an explanation. If you want the perfect AKT revision package, I would recommend the AKT revision book, the Zero to GP AKT revision book, which contains keynotes on all the topics you need for your AKT exam, plus the zero2gp.com website where you've got short answer questions, practice multiple choice questions, the notes, and uh digital flashcards. But for now, let's get straight into this episode on Polymalgia rheumatica. So let's introduce our case for today. It's a 67-year-old woman, and she comes in and she says, I've got terrible pain and stiffness, and it affects my shoulders and my hips on both sides. She also has a few other kind of systemic symptoms. So she complains about general fatigue, she's had some weight loss, and she also has ankle edema. So you're talking to this patient, taking a history, with the pain and the stiffness, you're suspecting polymyelgia rheumatica. So the first question in this case is what are the demographic risk factors for polymyelgia rheumatica? Key risk factors for PMR are older age, being female, about 65% of people with PMR are female, but I've seen it in plenty of males, so you can't exclude it on that basis. And it particularly affects people of Northern European ancestry. Next question is when are the symptoms worst?

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When when do patients say their symptoms are at their worst?

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Symptoms tend to be worse with rest and they improve with activity. So people say if I've sat down for a few hours, I'll be really painful and really stiff, and then as I get moving and I move throughout the day, symptoms improve. They're also worse in the morning, and a key feature is that there's stiffness that lasts at least 45 minutes in the morning. So a key question to ask patients is how long do you feel stiff in the morning? How long does it take you to get going and loosen up? And if it's 20 minutes, that's less significant. But if they say an hour or two hours, then you think more along the lines of PMR.

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The next question is, what blood test findings would you see in polymyodromatica?

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The key blood test finding is raised inflammatory markers. So raised CRP and raised ESR or erythrocyte sedimentation rate. But an important feature here is that the inflammatory markers may be normal. So I have seen it in several occasions where someone has really, really typical symptoms of PMR. Their inflammatory markers are normal. And then after kind of discussing with the patient, discussing with rheumatology, looking for other causes, we finally settle on likely PMR, and then they have a really good response to treatment. So the inflammatory markers can be normal, so don't exclude PMR on that basis. Okay, so you've got this patient, you're suspecting PMR, she's got all these symptoms, but you need to do some tests to rule out other conditions that can cause similar symptoms. So my next question for you is what blood test would you do for a potential endocrine cause for her symptoms? The important test here is thyroid function tests, particularly thyroid stimulating hormone. Because if she's hyperthyroid or hypothyroid, this can cause symptoms like fatigue, weight loss, ankyledema, pain, and so on. So ruling out thyroid pathology is important.

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The next question is what bloods would you do for a potential malignancy?

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In a patient with fatigue, bone bony type or joint pain, weight loss and so on, you need to exclude myeloma. And the way to do that is a full blood count, usenese, calcium, and serum protein electrophoresis. These are the blood tests that are listed on the Nice Clinical Knowledge Summaries. If you're really suspecting myeloma, you can also do free light chains, and there's potential to do the urine-bence-jones protein as well. The next question is what blood tests would you do for other inflammatory conditions? So polymyodromatica is an inflammatory condition, but there's other inflammatory rheumatoid conditions.

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What bloods would you do to rule those out?

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The bloods that are commonly listed are rheumatoid factor for rheumatoid arthritis, creatine kinase, which is a marker of muscle inflammation, which could happen in myocytis. So there is myocytis as a condition, and other another condition is polymyocytis, but you can also get myocytis secondary to um statins. So if the patients on statins particularly want to check creatine kinase, and the other ones to consider are anti-nuclear antibodies for systemic lupus erythematosis or SLE, and you could consider anti-CCP antibodies, which is a more specific antibody for rheumatoid arthritis. The next question is what other tests, in addition to the ones we've just talked about, would you do for other differentials? Just to rule these out before you go ahead and kind of go down the path of polyodromatica. So one is liver function tests, which will include alkaline phosphatase, which is a marker, which can be a marker of bone pathology like padd's disease or bony malignancy, for example, urine dipstick test for renal pathology, so proteinuria, um, which could and hematuria, which could happen in kidney problems, for example, vasculitis, and you could consider a chest x-ray. Symptoms of lung cancer can often overlap with um these vague sort of nonspecific symptoms like weight loss, fatigue, aches, and pains. So a chest x-ray is always something to consider. Okay, so you've got the 67-year-old woman, and you're suspecting polymyodromatica. The next thing I want to talk about is what's the essential diagnosis to exclude here in this patient where you're considering PMR? So key thing in any patient uh who has symptoms suggestive of PMR, you want to exclude giant cell arthritis, which is a very much a related condition. So often patients with uh one will have the other.

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So, what are the key symptoms of giant cell arthritis?

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Things that will make you think about giant cell arthritis are an abrupt onset temporal headache. So a headache on one side of the temple across the kind of scalp area. Temporal tenderness, so they may uh you you may have tenderness when you actually palpate their forehead on that side. But in the exam, uh key description is someone's brushing their hair and they've noticed tenderness when brushing their hair. Another symptom is jaw claudication. So as they chew, their jaw starts to ache and get sore, as though they're overworking it. And a final key symptom is visual symptoms. So temporary loss of vision, double vision, these kind of things. The next question is what's the risk if the patient has giant cell arthritis and it's not promptly treated?

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What's the risk? It can cause permanent vision loss.

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So giant cell arthritis is a type of vasculitis, and the inflammation in the blood vessels going into the eye can cause a restriction in blood flow, and this cuts off the blood supply to the retina, so there's ischemic damage to the retina, and the patient loses their vision, and this is permanent. And the final question on giant cell arthritis is what is your management if you're suspecting giant cell arthritis? What are you going to do as a GP in primary care? So the first thing is to assess for visual symptoms. If they have any visual symptoms, they need a same-day ophthalmology referral. They need to go straight to ophthalmology to assess their visual symptoms. The next thing is uh that they need high dose steroids, higher doses than with polymyodromatica. So typically, if a patient doesn't have visual symptoms, it's 40 to 60 milligrams daily of prednisolone, given as soon as possible to start with. They also need, before they have their steroids, bloods for inflammatory markers, but you shouldn't delay the steroids while waiting for the results. But if you can get the blood test done immediately, then start the steroids straight away. That's the ideal scenario. Then they should be referred on the fast-track giant cell arthritis pathway. And this pathway will look different depending on where you are in the country, but this usually goes to uh rheumatology, and they should be seen ideally on the same day, at least within three days of a suspected diagnosis. And to confirm the diagnosis, they'll need a temporal artery ultrasound and potentially a temporal artery biopsy to confirm the diagnosis. Okay, so let's get back to our patient. She's 67 years old, you're suspecting polymelatica. Let's say you've excluded giant cell arthritis, she's got none of the features of giant cell arthritis. The next question is how do you make a working diagnosis of polymodromatica? So to make a working diagnosis, you're suspecting it, but that doesn't really get you to the point of making a diagnosis. What you need to do is give 15 milligrams of prednisolone per day, and you you give the prescription, tell them to take 15 milligrams a day, and follow them up after a week. If they have at least a 70% improvement in their symptoms, you can make a working diagnosis of polymodromatica. So what you'll find is when patients come back, either they'll say, didn't really make much of a difference, uh, maybe like a 20% difference or no difference at all. In that case, it doesn't look like polymyodromatica. You have to think about something else, and maybe refer the patient. If they come back and say, Yeah, I notice a huge improvement, at least 70%, 80%, 90%, then that's very typical for polymodromatica. They have a good response to oral stoids. The other feature that helps you make a working diagnosis is if the inflammatory markers become normal within four weeks. So you can recheck the inflammatory markers. If they come down to normal within four weeks of prednisolone, then that also supports the diagnosis. So the next question is you've made this working diagnosis, they've had a great response to steroids. What do you do next with the steroids?

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What's your next course of action? So they've had a great response to steroids.

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The next step is to start a slow and long reducing regime of the steroids. So you start with 15 milligrams, you carry on with that 15 milligrams until their symptoms are controlled. Usually this takes about three weeks. Once their symptoms are fully controlled, then you drop down to 12.5 milligrams of prednisolone for three weeks, then typically 10 milligrams for 4 to 6 weeks, and then you drop by 1 milligram every 4 to 8 weeks. So they'll do 10 milligrams for 4 to 6 weeks, 9 milligrams for 4 to 8 weeks, 8 milligrams for 4 to 8 weeks, 7 milligrams for 4 to 8 weeks, and so on. You kind of do this depending on how the patient's symptoms are controlled. If their symptoms are really well controlled and they're not getting any kind of return of symptoms, you can go more aggressively. For example, every every four weeks you drop the dose. If their symptoms are creeping back in, you may need to carry on with each dose for a bit longer before you can drop it down. So the next question is this patient's gonna be on steroids probably for one to two years, sometimes longer. What additional steps do you need for patients who are on long-term steroids? So the steroids are gonna cause some side effects and risks and so on.

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What are you gonna do to mitigate those risks?

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So for patients on long-term steroids, I have a helpful mnemonic to remember what to do with them. The mnemonic is don't stop. So don't, it refers to don't stop, don't stop the steroids, because they'll develop dependency within three weeks of taking high-dose steroids. So this means that because you're putting in external or exogenous steroids into the body, the body's own system by which it produces cortisol, the key steroid in the body, that will get downregulated. So basically the body says to itself, I don't need to produce cortisol because I'm getting cortisol from the steroids, or I'm getting glucocorticoid um effects from the steroids that I'm taking. This means the own production of cortisol shuts down, which means if you stop the steroids abruptly, you just stop taking prednisolone, there's a risk of adrenal crisis due to low levels of glucocorticoids and steroids in the body. And this can make the patient very unwell. In adrenal crisis, it can be a life-threatening emergency. So very important not to stop them. S, so that that's don't, and then stop is another part of the mnemonic. So S is for sick-day rules. Now, this means if the patient gets unwell, for example, they develop pneumonia. Normally, in somebody who develops pneumonia, your body will produce about, you know, double the amount of um steroids or cortisol that you have at baseline. So the normal response to illness is to produce more steroids. Remember, the the body's own production of steroids has shut down because they're taking prednisolone. So the steroid dose may need to be doubled during acute illness. So if the patient's really unwell with, say, um pneumonia, they may need to double the dose of the prednisolone for a short period while they recover from that infection. T is for treatment card. So they need to carry a steroid treatment card, which you can often get from pharmacies or you may carry them in your GP practice clinic. And this card basically says you're on steroids, they can give it to, say, paramedics if they become unwell. It's just to alert them that uh that they're on steroids, and these steroids are essential and shouldn't be missed. O is for osteoporosis prevention. So steroids will thin the bones, they'll reduce the bone uh density, and this can lead to fractures. So you want to prevent osteoporosis, and you can do this with calcium and vitamin D and bisphosphonate treatment to protect the bones. And the final bit of the mnemonic is P for proton pump inhibitors. And this is because steroids can be quite irritating to the stomach, reduce the um the kind of protection of the stomach, and lead to things like stomach ulcers and bleeding. So proton pump inhibitors help to counteract that. Okay, the next question is how often are you going to review this patient who's on this long reducing regime of steroids? The nice clinical knowledge summaries say review the patient one week after each dose change, and and then at least every a minimum of at least three monthly routinely throughout the first year. So one week after each dose change plus three monthly. And what you're reviewing is how are the symptoms, how are they getting on with treatment, any signs of giant cell arthritis, any kind of complications or any anything that you're worried about? The final question I have for you is at the three monthly reviews, what blood tests are recommended?

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Or what tests in general are recommended.

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The nice clinical knowledge summaries recommend at the three monthly reviews, a full blood count, inflammatory markers, so ESR or CRP, and the use and ease blood test for the renal profile. The other thing to measure as side effects, so they're the ones really to monitor uh how well PMR is controlled, but then you're also going to monitor for some adverse effects of steroids. And this includes measuring the blood glucose level for diabetes. Steroids can increase the likelihood of diabetes and also the blood pressure because steroids will put up the blood pressure. So I hope that episode on polymaromatica was helpful. Do leave me a comment or send me a message if you have any suggestions for episodes you'd like to see in the future. And I'll see you in the next uh next episode, which will be in about a week's time.