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All you need to do to sign up is go to pacesahead.com and I advertise for these two sponsors because I genuinely believe that combined they essentially guarantee you'll get that all important parts in Paces. So use the discount code Pre Paces 15 at quesimed.com and sign up today. Welcome listeners to this episode of the Pre Paces podcast where I interviewed Respiratory Registrar Dr. Giles Dixon on some of the more uncommon causes of breathlessness which may rear their ugly head in your Paces clinical consultation station. Giles was simply brilliant and it really comes across how much he cares about this topic. So I really hope you enjoy it this episode but as ever we have to pay homage to the absolute legends to keep the podcast going over on the bimia coffee page. Henry and Claire, according to the message that they left, Claire nailed it and Henry scraped through a pass as a pass Henry. Thank you to Michael who passed and to Luke who donated. Thank you to Anu Ripp and Mukherjee who passed their exams. Thank you to Yoga and Jack who donated in advance of their exam. Thank you to Nino Chandra Mohan who passed first time. Thank you to Neil who passed first time after listening to all 136 episodes and left a really lovely message. So thank you Neil. But without further ado let's get into this week's episode. [Music] Welcome to the Pre Paces Podcast with me, Dr Sam Williams. And this week we're covering a topic within the realm of our clinical consultation station and that is breathlessness but not just any breathlessness because let's face it, Paces is unlikely to throw you your bog standard infective exacerbation of COPD or decompensated heart failure. I mean they could and it might be one of the easiest Paces stations you might encounter but it seems unlikely. So I thought it might be helpful to produce an episode looking at funky causes of exertional breathlessness, the causes which you might not immediately consider but which might become a lot clearer when you take a full and systemic history asking slightly more in-depth questions which unearthed crucial features to reveal a given diagnosis. And I'm delighted to be welcomed to this episode of the podcast by Dr Giles Dixon, respiratory registrar in the 17th January. Giles welcome to the podcast. Hi, nice to be here. And Giles this is potentially something really easy for the exammers to bring up in Paces, not least because the examiners might have patients with some of the conditions that we're going to discuss who can provide their original story but alternatively they might also just provide an actor or a surrogate who can give a really detailed history without necessarily having physical signs. So either way I think this is absolutely crucial for our listeners to have a handle on. The value here is the bulk of what we're going to be talking about is some of the respiratory disease. Yeah so I totally agree there and I think the only thing I would add to that is that you know, exesional breathlessness is a very common presentation. Maybe regardless of what medical specialty you end up in the future and knowing how to take that in-depth history of breathlessness is going to be kind of really key for everyone's curious going forward. Yeah, couldn't agree with you more and listeners this is going to help you regardless of your Paces but this is going to help you in your day-to-day clinical practice too. So without further ado let's get into funky shortness of breath. So Giles just to start off I wonder if you can tell us about your approach to taking a history of breathlessness coming from a respiratory specialist and again this may not necessarily be something that even exists but I wonder if you can tell us about your approach to taking the history of someone with exesional breathlessness. Are there any particular approaches or particular ways of phrasing the common questions which you find provide the most value for the patient or enable them to give you the best history possible or is it much the same as we've learned in medical school and beyond of just talking about exercise tolerance or distances walked or time walks before they become breathless or I wonder if you could just give us an insight into your approach to taking a history from these types of patients. Yeah of course so you know being a respiratory registrar I'm obviously obsessed with breathlessness and having just done a PhD in Institutional Lung Disease that's what the majority of my patients present to clinic with it's its exertional breathlessness. I think I probably get quite geeky from the breathlessness point of you and we can all remember back to first days of medical school where you know you hear chest pain and you immediately go into your Socrates history but I think you probably have to be as in depth when you hear breathlessness coming from a patient so that should really stimulate a whole load of questions that can then come from that breathlessness and you really need to pin down with the breathlessness a number of certain features so key things when is it happening so is it something that the patient wakes in the morning with is it something they're having having at night is it something that they're having on exertion if it's happening on exertion you know what are we talking about with exertion so you know in respiratory clinic we might see marathon runners that can run 26 miles and at the end of that they're feeling breathlessness and say you know I can only run 24 instead of 26 miles now or is it someone who can literally get up from a chair and do a couple of yards before they're breathless and then the other key part of that I think from there once you've figured out when it's happening is what's the trajectory of that breathlessness is it something that has happened immediately and has happened very quickly and has come on very severely or something that built up slowly over time for that patient when we then start to think about different kind of causes one of the big ones for me is is that breathlessness episodic and the way I ask about that with my patients is if I got you up and you know sent you for a walk for 50 yards would you always be breathless at about 30 yards is it every time you get to that 30 yards that you get breathless or might you have a day where you can go you know a couple of miles and it or you know a bad day where where you can only go kind of 50 yards and if you're getting into that kind of persistent breathlessness is very typical and reproducible then I'm thinking about things like interstitial lung disease if you get a variable breathlessness there then I'm thinking about something more like airways disease and asthma in that in that kind of patient so when is the patient getting breathless what do they have to do to kind of trigger that breathlessness and then is it episodic and then I think maybe after that I'm into my kind of more refining questions of thinking about particular diagnoses and be able to rule them and rule them in and out at that point I think I probably have to say I do the same medical school history for all of my new patients in clinic and you you you tag on additional questions I think as you work your way through your respiratory training and in you kind of tailor those questions for what the patient has said initially about that breathlessness but I think I would really kind of reiterate that point of once that patient says breathlessness you need to delve into that symptom before you jump into any other associated symptoms. Yeah absolutely absolutely agree and I love your sort of openness with the questions as much as you need to get an idea from the patients about what the breathlessness means to them in terms of their existing functional capacity you need to figure out the nature of it as you've described. One of the things that I thought was which I thought was very interesting in my research and preparation for this episode was thinking about associated features so as well as what you've described as trying to characterize the nature of the breathlessness and try and quantify it as best you can trying to identify any associated features is going to be really important and I guess the thing for our listeners to bear in mind is that if we talk
about a slightly less conventional cause of breathlessness. It's probably going to be in the absence of typical risk factors such as smoking because otherwise, you know, the doors pretty much open for you to say, "Oh, well, you know, this could be undiagnosed COPD." And I don't think the examiner's really wants a lot of candidates coming along and the patient's story giving a history of smoking only for the only for the patient's candidates to say, "Well, they're a smoker." So it's undiagnosed COPD until proven otherwise. You know, I think they're going to want to test you a bit more in terms of your the rigor of your medical knowledge. So I don't know how you would, I don't know how you think about that of, in my gut, it feels as though it would be unlikely that they would be a smoking or at least there would be an absence of easy things to attribute their breathlessness to such as smoking. Yeah, I think so. This probably also the more you spend time talking to people with breathlessness. If you're thinking about COPDs, these patients do have relatively specific symptoms with COPD. So they get that reproducible breathlessness typically. They get that cough with the sputum. They describe exacerbations during the winter period, you know, the bronchitis and the emphyseumemphysema causing that breathlessness, the bronchitis causing that episodic cough. And I think if you went down the lines and you heard the patient was a smoker, but they said, "You know, I hardly ever cough." And I've never had a chest infection before. And actually the breathlessness is more episodic or order us some other more specific features in there that I'm sure will kind of get to. I think it would be difficult to just label things as COPD and I think coming up with a differential diagnosis is all important. As we know from the patients who walk through the door, you know, a good 20-30% of our patients will have smoked at some point in their lifetime. I think not all patients who have had significant smoking history have COPD. That's just me jumping on that hobby horse from a respiratory perspective and finding lots of patients with insitial lung disease throughout my career who have maybe had this label in primary care of COPD. But no, I probably do agree from a patient's perspective that they're trying not to throw you off down a route that would be unfair to throw you down. So I think while taking obviously that smoking history is key, I think there are a number of different elements to your history that might actually point you in a different direction. Yeah, absolutely. And so if we dive into the first sort of differential diagnosis which I wanted to discuss today, when we're talking about taking the extended history from the patient and as we've discussed, you know, you're going to ask the main questions to the patient, the nature of their breathlessness, the episodic nature, when is it worse than it and when you're talking about exertion, that's really what we're talking about. It's breathlessness on exertion. One of those features of your extended history is going to be drugs. One of the differential diagnosis I wanted to talk about was drug-induced lung disease and having just done your PhD in interstitial lung disease jars, you seem like the right sort of chap to talk to us about drug-induced interstitial lung disease. So I wonder if you can explain to the list is what are the most common culprits that might cause drug-induced interstitial lung disease? And what should the candidate be asking their patients to try and tease out this sort of detail? Yeah, so there's a few key ones, but actually it's probably important to say there is a big long list out there. So from the patient's perspective and the history perspective, I probably have two or three big drugs that I ask all my patients about. But if I've got a patient who has an interstitial lung disease and actually there's a suspicion for other reasons this could be drug-related, I will go into every drug that they've ever had in the past and there's a database as you can look up about whether they're related to pulmonary fibrosis or interstitial lung disease. The kind of big ones that I would remember for taking a history in in paces and the most common one is probably nitrofuran tone. So these are our patients typically older patients, maybe with a predominance towards female rather than the male who have had recurrent urin-outract infections in the past and have had nitrofuran tone either recurrent causes for infections or as of kind of a prophylactic antibiotic. And we do see, albeit rarely with those patients, but we do see significant drug-induced lung disease. So I think if I was taking my history and they called out that this patient had been on nitrofuran tone, I'd be then trying to do my timeline for my detectiveness. So, okay, what year did you start your nitrofuran tone when do you think your symptoms started? And do those two things line up there? Beyond the nitrofuran tone, you're then starting to get a little bit rarer. One of the drugs that you guys in cardiology like to use amiodarone is it's the typical one that we learn at a medical school. And so, you know, if does that a patient have any reason particularly not going back into their past, maybe where amiodarone was more commonly used, do they have history of a rhythmia in the past where they might have been prescribed amiodarone? And then the other one that everyone remembers, although we try to dumb it down a little bit more in the pulmonary fibrosis community is metatrexate. Methatrexate can cause an acute humanitis, but actually it relatively rarely causes a fibiotic lung disease. So, do you worry about metatrexate as reduced significantly in more recent years? And actually, part of that has come from a lot of our patients have in intitial lung disease, have rheumatoid arthritis, and metatrexate is a great drug to treat rheumatoid arthritis. And we try not to put off our rheumatology colleagues from using that drug if it is possible. But you may have a patient who has been on metatrexate years and years ago and are not associating what they've got now with that exposure in the past. So, I'll be asking them about connective tissue diseases and I'd be also asking the about treatments for those connective tissue diseases in the past. One thing I did mention before was whether increasingly immunotherapy is used for a range of different hematological and oncology malignancies and they can cause an acute humanitis. So, potentially, if you've got someone presenting more acutely with breathlessness, then that's something to ask about how they had an immunotherapy recently. And then what's tied to that in a way, we always heard about in the textbooks about this chemotherapy drug called Blaemysin. And Blaemysin is a medication that was used for hematological malignancies amongst others in the past. And that is typical for cause and pulmonary fibrosis. And there may be many patients out there who maybe have that in the past who have potentially quite stable, fibiotic lung disease nowadays who could be a good candidate to come and sit and talk to some some some some paces candidates. So, I think they're the big ones that I would not be missing at the nitrofuran turn, amiodarone, metatrexate with the caveats that I've mentioned. And then finally, adding in immunotherapy. But that's a bit more acute probably. Yeah, absolutely great overview of those drugs, giles. And if I can just chuck my topics in from an amiodarone perspective is that amiodarone has a remarkably long half-life up to sort of six weeks to three months worth. So, I guess the one thing with this type of presentation would be trying to formalize the timeline of when they've taken the drug. But as you've detailed, it might be weeks to months even after cessation of the drug. And so something like that would be perfectly reasonable to see three or six months down the line even from stopping amiodarone. Just important to consider that for our listeners. But maybe away from drug-induced intitational lung disease, there was another group of conditions I wanted to discuss giles. And those were the occupational specific intitational lung diseases or the industrial lung diseases. So, we're talking about conditions such as aspostosis, pneumoconiosis or cilicosis. And again, this is going to incorporate our listeners taking a much more extended history. And probably something they may not consider asking about in many other types of station. But in this station, when you're considering a diagnosis like this, taking a comprehensive occupational history is actually critical, isn't it? Yeah, it's absolutely vital. And this is where you're really moving into my area kind of interest here. And getting up on my hobby horse about taking these occupational histories. Because far more people have been exposed to these kind of occupational exposures in the past than we actually think about. And you know, places of short stations, you're not going to be expected to take a history that's going to stand up and court when someone's arguing whether someone's going to ask for a stosis or not. But I think just asking the patient what their current occupation is probably not enough. I think if you've got a patient who has got a deserional breathlessness, you need to be asking about current occupations and then previous occupations as well. So, occupations have come and gone over the years. There's been a big change in what people are working in the UK. And also there's been changes to exposures that you can be exposed to at work. So if we think about something like us best over.
is typically you know asbestos you need to have fairly significant exposure to asbestos and more recently although it is possible due to asbestos being banned more recently it's quite difficult to get that exposure in the near history so you're of commonly having to delve back in these 78 year old patients to what did you do when you left school so and that's my when it might come out that you know I went and did an apprenticeship I went to go and join the Royal Navy of which I was in for five years working in submarines where we were lagging pipes or working around where people were lagging pipes etc. So I think I would be encouraging your listeners to think what do you currently do now but what have you done in the past and then being aware of the kind of key occupations if we're thinking about kind of asbestos exposure key occupations that I'm thinking about are kind of plumbers engineers in times gone by people might or not have heard of lagging of pipes but they used to mix up this kind of asbestos sludge is probably the best way that I can think of it and wrap it around pipes to insulate pipes and mixing up with their hands and manually putting around around pipes and then removing it at the end of that. So anyone who's going to be exposed to those kind of things but even if we're talking about we know that there's significant amount of asbestos in buildings so anyone who does any kind of building work demolition work could be exposed and that may be even extends into people like teachers who work in public buildings doctors who work in public buildings sort of full of asbestos and there is potential exposure exposure there. Definitely not anymore. Once we move into kind of thinking more about things like silicosis and this is kind of really hot off the press now but kind of silicosis one of the big exposures is people working with artificial stone and artificial stone work tops. So most of us have probably got some lovely worktop in their kitchen that's been made out of artificial stone but actually people who are cutting or working with that are a really high risk of silicosis and what obviously goes with that is kind of stone masonry and the construction work there as well and then also you're thinking any kind of anything where it's supposed to silica dust so are they working in mines? Have they been inquiring the past? Have they been sandblasting old buildings? Those kind of things which have exposed them over time to occupational exposures that could have led to an ILD. Yeah, great overview again, Jarls. I guess the main takeaway for listeners if the suspicion is of an industrial under these is that without asking these questions and without going back or being maybe specific about your line of questioning for example your patient may say I'm a handyman or something like that and say well you know what type of handyman you know what actually do you do and rather than just going over that and even though it's a stressful situation you may not think to delve a bit deeper into what they actually mean it's really important to pin down those details because it might be crucial to nailing the diagnosis and whilst you know you might think you're on the right lines by saying oh well you know this could be idiopathic pulmonary fibrosis actually by nailing these elements of the history you're making the specific occupational industrial under these diagnosis which is going to get you the maximum marks. Yeah exactly and you know you get to be that detective that we also will find up from day one medical school of you know not just picking the top diagnosis and going for that but really asking the patients those pertinent questions to try and find you know that exposure that will then point you towards that diagnosis and yeah I would say to everyone that you've got that top line of profession and then you really need to know what they're doing in that profession and what they could have been exposed to. So they're moving on to a sort of similar range of conditions and this is where I have to plead utter ignorance because it's been a long time since I've even looked at anything to do with this condition and truthfully did I understand it when I first revised it for paces maybe I think it's the solid answer to that but hypersensitivity and humanitosis is something which I'm pretty sure I must have discussed on this podcast before but guys I wonder if you can give us some ideas around how you how we can differentiate the industrial lung diseases we've discussed so far from the hypersensitivity and humanitosis is it to do with just the environmental exposure and is that different between the two ranges of conditions and I guess a more broader question is how much overlap is there between these conditions or is it sacrilege to mention them in the same sentence? No so you know symptoms wise and even you know diagnostics wise you're going to do a lot of tests on these patients and they're going to tell you a lot of the same things they're going to have a exertional breathlessness that's going to be there but when I come to think about the differences between the two and obviously they can potentially be an overlap your occupation may expose you to something that leads to hypersensitivity and humanitosis but I think the easiest way to divide the two groups is thinking about organic exposures and inorganic exposures so typically enough in a hypersensitivity and humanitosis you are exposed to something organic that has led to this, led to this immunological reaction within the lungs which has led to a typically we think of an inflammatory process followed by a more chronic and fibrootic process and there's a number of different environmental exposures that typically unless you ask the patient that exact exposure you are not going to find it. The one we all remember from medical school is pigeon fanciers lung and pigeon breeding, pigeon racing is far more common than you might expect it is. It is vital to be asking about birds. I think what goes along with asking about birds is asking about pets so are people keeping cockatiels and keeping budgies and parrots in that house and I am still surprised by asking that question and finding out that they have had an African parrot in their front bedroom for the last 25 years which I think is not an uncommon thing for people to do. So the things I have spoken about in terms of exposures so far so it is pigeons and then birds in the house. My next typical question beyond that is exposure to molds. So I am asking people about in their house have they got damp and mold anywhere and then particularly thinking about aspergillous exposure there and that can be important obviously for you know asthma as well but you know have they got black mold growing in the bathroom behind their bed etc and key question about molds there. Then I keep it kind of open beyond that and I will probably say you know have you got any hobbies what you do in your spare time what you enjoy doing in your free time and that might be when it comes out that you know they love doing gardening and they are out turning their compost heap every other day and the exposure themselves or big fungal exposure at that point. The other one and this is where there is an overlap between occupational and hypersensitivity in terms of you can fit into both would be someone like a farmer and farmer's lung where you are exposed to various different funguses and bacteria through your work typically it is through working with silage for the farmers and that can lead to inhalation of the particular bacteria or fungus that has led to this reaction within the lungs and cause the fibresis there. The other ones they might want to throw in something a bit more weird and wonderful so there is a condition called hot tub lung there is probably more and more people owning a hot tub now but actually hot tub lung has led its way into my common history that I take particularly for patients turning up in interstitial and disease clinic and then if you want to get really weird and wonderful there is a case looked after by one of my mentors in Bristol Dr Adam Marley who looked after a case that was written up of a man who gained hypersensitivity new anitis from breathing in a bacteria in his brass instrument probably mentioned for well he mentioned the story on this very podcast Giles. There you go well he has mentioned to me many a time and I think I have actually met the patient himself so yeah don't forget the weird and wonderful stuff but the typical things that I would be wanting to ask in my general history there would be do you keep birds or pets what do you do for work which you will have already covered and then molds and damps around the house. Yeah absolutely fantastic and for any listeners that want to go back and listen to Dr Hosepa Adam Marley's episode that is episode number 115 on interstitial lung disease where you can hear about about his patient who contracted interstitial lung disease from is a trumpets was it? It was a trumpet or a saxophone but it was an interesting one. It was a brass instrument of some sort yeah really fascinating stuff. One last thing Giles with that which I really think is crucial is that away from this range of presentations you know you probably wouldn't ask a patient about their hobbies in many other types of presentation and so again just reemphasize and tell you what it is.
to the listeners. This type of presentation does change the way that you take your history and what features you're going to ask about. So hobbies, absolutely, in a patient presenting with exertional breathlessness, even if you just sign post to the examiners that you know that there are hobbies which are associated with interstitial long disease, fibrosis or hypersensitivity, pneumonitis, it's demonstrating to the examiner your breadth of knowledge of this range of conditions. But moving on to the next point, Charles, and we don't need to spend maybe too much time on this. I'm sure it will be something familiar to the listeners. But again, keeping on the occupational theme and something which could well be a differential diagnosis in this station is occupational asthma. So I wonder, can you just outline sort of the typical pattern in this condition, the typical pattern of the history? And I know you mentioned breathlessness, but I guess weas as well would be a predominant feature here. Yeah, of course. So I'm probably typically thinking about three things with your airways disease patients. And when we say airways disease, we're obviously narrowing things down to aspirin, COPD, but in this case asthma. And I'm thinking about weas, I'm thinking about cough and I'm thinking about breathlessness. So does the patient have those three things or any combination of those three things? And then thinking about occupational asthma, you've got a patient who's got an exposure to something at work, which is leading to an acute bronco constriction, which is leading them to their symptoms. And they're typically not going to be exposed to this outside of work. So your key question there, and probably the only question that you really need to worry about is does it get better away from work? Then you went on holiday last year for two weeks to Spain. Did you have your symptoms over the Christmas holiday period where you weren't there for two weeks? Did you have your symptoms? And if the answer to that is no, or it got all better, or I have a weekend away from work and it gets a whole lot better, I'd be then delving into what you do for work. What are you going to expose to at work? Thinking about dusty exposures, isocyanite exposures, or paint sprayers, etc. But that's your key question that I would be adding in there, and I don't think I would be going, I wouldn't be asking a load of screening questions for for occupational asthma unless they've got that history of things getting better away from work. Yeah, absolutely. But again, this is something which it might be really important that you ask specifically about. And it might come out as part of your history of percentage complaints. But again, the nature and the periodic or episodic nature of the condition is obviously going to be really, really important. So, Charles, the next condition on our list, for me, was a little bit questionable, but I am going to include it and it's sarcoidosis before I hit the record button. We were talking about breathlessness plus. And so we've talked about some conditions where we've talked about breathlessness plus exposure, whether that's occupational or environmental. But with sarcoidosis, it's seen to come out as breathlessness plus a range of possibly or seemingly unrelated systemic clues or associated conditions with sarcoidosis. So, I don't know, do you have much experience of managing sarcoidosis? And if you do, what are the typical kind of associated features which might point a listener or a patient's candidate towards sarcoidosis rather than anything else? Yes, I think sarcoidosis is a tricky one. As we probably know, sarcoidosis can cause most symptoms and affect most systems within the body, which leads to it being really difficult. There are some key symptoms that can point you heavy and heavily in the direction of sarcoidosis from your history. And often these are other features from your patients. So you might know your patient's ethnicity, for example, we know that in Africa, being patients that sarcoidosis is more prevalent. So if you have a patient from that ethnicity, you might think about asking more questions into sarcoid. The other thing to say is, you know, I've probably looked after a lot of patients with sarcoid and even in the chronic follow-up of these patients, we are still every time we're seeing them, asking them questions through covering the whole body to see whether that sarcoid is now affecting elsewhere. So it is quite common for us to inquire about things like the skin in our patients, asking if they've got any new rashes, have they had any changes to their eyesight, have they got itchy eyes, have they got painful eyes, and then thinking about things, symptoms of cardiac sarcoids. So, you know, have they got exertional breathlessness and they've had a few funny episodes of palpitations maybe there, or have they got more constitutional symptoms that you can get with sarcoids, so have they having night sweats or they have fever, are they having some weight loss to go along with this as well. Kind of typical rash that we think about is, you know, erythemia, no dosim, so have they got a rash that's affecting the front of their shins, or have they had a rash that has come and then gone in their history. So I think you have to really quite broad if you're thinking along lines of sarcoidosis and actually probably quite commonly, we identify sarcoidosis in investigations and it's an incidental finding and that's probably been the majority of presentations that I've seen, but these patients do a 10 primary care and have to spend a number of times going back and from before a diagnosis kind of reveals itself in it with sarcoidosis, so I think it's important having it there in your back of your mind. Yeah, definitely, and I think sarcoid would be an interesting diagnosis to mention, but I think it probably would have to be pretty much a textbook case for it to be fair game for the listeners. I don't think they can expect to throw in a rare diagnosis with an atypical presentation into paces. I completely agree. I don't think you're going to get a cardiac sarcoid case with an arrhythmia and a collapse. I think the beauty of sarcoid is that it can cause anything and everything and that probably means that you know you can't create a case that is specific enough that is fair to examine a poem in paces. So yeah, I'd be unless it's your very classical patient who has maybe presented with constitutional symptoms with a rash in the front of their legs and some breathlessness and you're expected to think about a differential diagnosis of potentially sarcoidosis, maybe something like tuberculosis, then I think it is unlikely to come up. So moving on to my next thought, and we're going over to a slightly different range of conditions here. We're going into the world of pulmonary hypertension and we're going to cover two separate entities here, one of which is going to be primary pulmonary hypertension, which in my work in cardiology, I've come across on a couple of occasions. Charles, you said you've worked in another hospital in our area, which is bath, which happens to be one of the regional pulmonary hypertension centres. So I wonder if you can detail for us what type of sort of typical case you think might be fair for the examiners to present to our candidates when trying their best to paint a picture of primary pulmonary hypertension? Yeah, so I'm going to be a bit geeky here. I wouldn't be an interstitial lung disease physician without wanting to be pedantic about things, but primary pulmonary hypertension is kind of a nomenclature of the past. So they're pulmonary hypertension physicians like changing the names of things as much as we do in ILD and actually the term that's previously known as primary pulmonary hypertension is now thought of as pulmonary arterial hypertension. And then they've gone one step further and split all the causes of pulmonary hypertension to five different brackets and pulmonary arterial hypertension typically sits in that first bracket. And pulmonary arterial hypertension, if we're thinking specifically in a pace is kind of context, I think it would be difficult to give someone an idiopathic pulmonary arterial hypertension to be able to detect, because even respiratory physicians are seeing patients and outpatients clinics, investigating these patients in depth and missing that diagnosis of pulmonary arterial idiopathic pulmonary arterial hypertension. But I think the big one not to miss there and consideration for pulmonary arterial hypertension is is scleroderma. So does the patient have a history of systemic sclerosis and are they breathless? If they have those two together, it is either going to be an interstitial lung disease combined with the scleroderma or a pulmonary arterial hypertension combined with the scleroderma. And I think in the past you've had John Pauling who's an international expert. Another one of my mentors from Bristol talked to you about scleroderma. So I wouldn't dare touch on that subject any further. Sensible, sensible giles. And one thing I will say is that the one occasion where I did come across this condition in my own practice was someone who had been referred to us for the question was about right heart failure. But it was in a patient who was in I think either in her mid to late 30s, possibly early 40s who had presented with sort of
a gradual history of worsening peripheral edema. So the picture was of right-sided heart failure and with, you know, probably hypertension, but it was just profound edema and someone had requested an echo not unreasonably. And that had shown that her right heart was completely blown, dilated with torrential trichospid regurgitation. And it was far out of keeping with her left-sided heart function, which was only affected by the right-sided pressure overload as a result of her underlying condition. And she went on to be referred to either tertiary or quarter-nery centre. But the suspicion then was that she had an undiagnosed autoimmune condition. So again, that might be something which is detailed, but again, we're approaching the limits of paces when they may not be able to bring you a patient with profound peripheral edema who's well enough to have their history taken by a range of budding medical reges. Yeah, exactly. I think if there's a set of examiners who are feeling particularly mean, there are maybe a couple of causes of pulmonary arterial hypertension that could be thought about, that could be picked out, teased out as a history. So there is a familial cause of pulmonary arterial hypertension. So I don't think we've touched on family history yet, but actually, family history is vital from the pulmonary hypertension point of view, but also from the interstitial lung disease point of view. So I'm making going back in that patient's family history and finding out whether anyone else has been affected by breathless thus at a young age is going to be key as well. The second one is there are some drug causes of pulmonary arterial hypertension. The typical one from way back in the past was medications that were used to suppress appetite. I need to double check that that is suppress rather than enhance appetite, but I do think it is suppressing appetite. They were common causes of pulmonary arterial hypertension in the past. If we're thinking about pulmonary hypertension more generally and this is slightly moving on to what I think you wanted to talk about next, there is, and then this is now jumping into group four of pulmonary hypertension and that's a condition called C-TEPH. So chronic thromboembolic pulmonary hypertension. And this is patients who have had chronic clot within the lungs that has formed typically a web-like appearance within the lungs and it kind of lines the pulmonary arteries when the lungs and effects the pulmonary circulation. The key elements to pull out that from a history would be finding out that patients' VTE history in the past. This might be a patient who presents in their 60s who had a DVT unprovoked in their 40s or even provoked following a, they broke their leg on a skiing accident and their 40s or something and I had a PEE and a DVT kind of a number of years ago. Sometimes you'll get patients who have had a number of DVTs in the past and I think if they were going to do something like C-TEPH in paces then it would probably make it pretty obvious and say you know this is a patient who's had one, two, three, pulmonary emboline in the past, have been on some blood thinners or maybe even have stopped the blood thinners erroneously at some point in the past and now they're presenting with breathlessness. So I think I would be adding to my list of direct questions for have you had this in the past, I would be adding have you had a DVT, have you had a PEE in the past and that might lead you in a direction of this chronic thrombo and mollic pulmonary hypertension which is a relatively rare condition but is probably under appreciated and under investigated for. I think the other thing to think about from the C-TEPH point of view is you might have a patient who has had a PEE in the relatively recent past, so say someone who's had a PEE six months ago or something and now they're presenting back to clinic but actually they've got persistent breathlessness after that PEE and that persistent breathlessness at that point of you know three, six months after the initial PEE then triggers us in in respiratory medicine and in follow thrombosis follow up clinics to think what are their investigations to these patients need and typically they'd undergo an echocardiogram and something and a repeat C-TPA to look for evidence of this chronic thrombo and mollic disease in those patients. Yeah, yeah absolutely fascinating stuff and chronic thrombo and mollic probably hypertension is something which I have essentially no exposure to but I am fascinated with the condition because it's these types of patients that I mean I don't know how it works in in real life clinical practice trials but I just would find managing these types of patients so challenging but I am aware that at some very large centers they do you know invasive procedures to take out some of these chronic embolite. Yeah so everyone listen to this podcast needs to immediately google pulmonary end-artorectomy and have a look at the clots that are a cardiothoracic surgeons can pull out of the pulmonary arteries and there's a number of publications with some very pretty looking clots but you know jokes aside the key to that is that these patients can be very symptomatic they can be young and they are can get to the point of oxygen dependency and if this is thought about and diagnosed and they're sent for an operation that operation can be curative so although it is rare it can be potentially curative and I think packed with other the only hospital over in Cambridge in the in the UK that do does this surgery but it can be curative and completely life changing my patients. Wow clearly something really important for our listeners to know about the end of the podcast. I hope that when you're faced with a patient whose main symptom is breathlessness. I hope we've given you a handful of questions that can help you move away from maybe the more common diagnoses and start uncovering these more unusual causes but I ought to mention that we don't want to get stuck in the medical school zebra world when not everything has to be a rare and uncommon condition but all we're saying is it may be more likely in cases but we hope we've been able to help you with some of these diagnoses in this episode. That only leads me to say a huge thank you to Dr Giles Dixon, the spiritual registrar in the 17th century. Giles it's been absolutely fantastic thanks so much for joining me. It's been a pleasure. Thank you very much. But listeners that is just about all the time we've got for this week's show. Please don't forget to like, follow, subscribe to the show or leave a five-star review wherever you get your podcasts. We always love to hear from you so as always give us a shout on our website prepacespodcast.com or the email which is
[email protected] and if you want to go above and beyond and support the show it's buymeocoffee.com/prepacespodcast but for now we're just about out of time. Thank you so much for listening. I've been Dr Sam Williams and we'll see you next time on the prepacespodcast.
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