#115 Interstitial lung disease

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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. Hello listeners and welcome back to the Pre Paces podcast and it's the episode that has been requested by so many listeners for a very long time and surely is one of the most common stations to come up in Paces. It's interstitial lung disease. I'm absolutely stoked for this episode of the podcast and I don't want to keep it from you any longer but I absolutely have to give some love back to the people who keep this podcast afloat and say huge thank you to all of the bimia coffee heroes. So the thank yous for this episode go to Kate who spent long runs listening to the podcast and past what are you going to do on your long runs now Kate. Thank you to Elizabeth Chandler who passed first time and donated. Thank you to Jacob Williams. Another of the Williams clan who passed first time well done Jacob. Thank you to you and Davies who left a really lovely message. So thank you you and thank you to James Stracken who passed second time and said everything that came up in his sitting had also been covered in an episode of the podcast. Absolutely remarkable stuff there James. Fantastic work from you all but without further ado let's get into this episode of the Pre Paces Podcast. Welcome to the Pre Paces Podcast. My name is Dr Sam Williams and yes listeners I've caved to the pressure from popular demand for this topic which is almost synonymous with sitting paces. This is surely one of the most common topics which is more than likely going to come up in any given paces center and I'm absolutely thrilled to have another fantastic renowned expert on the topic for this groundbreaking episode in digital lung disease. I'm delighted to welcome consultant respiratory physician with a special interest in digital lung disease at North Bristol NHS Trust Dr. Hosefa Adamarly. Thank you so much for joining me on the podcast today. Hi Sam thank you for having me. You're most welcome. I'm absolutely thrilled to finally get you on to talk about this. We've been plotting this episode for a really long time and I'm delighted to finally get the chance to talk to you about everything ILD and without further ado I'm just really excited so I think let's get into it. So first of all if we start off with the absolute basics from your perspective Hosefa what is in digital lung disease and why is this such an important condition that comes up in paces so often? So in digital lung disease and I think we're going to call it ILD is an overarching term for group conditions that are characterized by information and fibrosis in the pulmonary interstition and that often leads to drug alveolar gas exchange and reduce lung compliance. Now interstition lung disease ILD they're almost about 100 different types but I think it's important to know them because recently over the past 10 years there have been an explosion of new drugs that are coming into the market or already in the market we are making an impact on survival of these patients and we're getting better at managing their care. So in the olden days with one particular type idiopathic pulmonary fibrosis you will give a survival of about two and a half sorry three and a half to five years but now they're living longer and longer so you're bound to see them in your eye patient clinics or as an inpatient and the fun bit about interstitial lung disease is you always have to be a bit of a detective to figure out what has caused those changes in the pulmonary interstition and I hope I can almost impart that excitement and empower your listeners on how to approach these diseases. Yeah absolutely and absolutely you couldn't agree with you more that whilst this is such a common thing that comes up in pace is actually the getting the diagnosis of interstitial lung disease isn't the end of the story and that you need to have an appreciation for the wide variety of causes. So with that in mind I think we're going to start off with that and we're going to go through some of the most common causes of interstitial lung disease and hopefully this will just inform our listeners as later on when we go through their examination in their shoes of how they would approach a patient the types of things they should be looking out for on examination. So I wonder if maybe we can start off by just going through some of the few causes I know you mentioned there's over a hundred but we're going to focus on probably the mainstream ones which might be most likely to come up in paces or at least those you'd be expected to mention in your differential diagnosis in your discussion with the examiner. So why don't we go through some of those some of the most common causes of pulmonary fibrosis or ILD that will come up in paces. Sure. So the way I like to approach and it'll almost lend itself in your history taking is non-causes, causes that we have a cause for and then you have the unknown causes. Now the non-causes are and in your history you'll take an occupational history and it's really important that you ask patients about their occupations and often you will ask them about asbestos exposure because large amounts of asbestos exposure can cause plural plagues but asbestosis. Then we'll have patients who work in call mines. Now in certain areas you will find special in whales and bases where they had lots of call mines. They you can develop call workers and immunocoses and then you've got patients who work with silica or a silica dust and those are the ones that work as stone masons and they develop a condition silicaose. So that was where kind of the occupational exposures. Then I guess the other non-causes you will always explore with patients drug exposures. So the classical one that is always quoted in rheumatological patients in method tracksate and you can develop a method tracksate, pneumonitis and acute cases. The other drugs that are often used and we don't ask or probe about these medications like nitroferantone. Now it's commonly prescribed in elderly patients and those patients who have recurrent urine track infections and they could be on it for six months. So it's really important to take that antibiotic history. People who have arrhythmias may have been on amiodarone so it's important to ask them about that. And then there are other drugs which are rare and what. People who have cancers in the past may have been exposed to chemotherapy to agents like bleomysin. If you're not sure about what drugs can cause eponomi fibrosis, there's a great website called pneumatox.com, www.numatox.com. And if you type in the particular drug on a post-war drug, I mean your consultant will be so impressed because not only will that highlight the drugs that cause that particular type of fibrosis but more importantly you have a list of references that you can impress your consultant with. And then other things about the social history which I often ask about the environment that patients left. These are non-courses again. We have been very much exposed in the media to mold and elderly patients who try to retain heat in the house and want to open their windows. We have a condensation on the walls and that will predispose to more growing on the walls and then inhale the more. I often have a thing about sniffer tests. You can smell a mould of patient's coats and clothes and I have a tendency that when I've helping patients wear the coat, I'll just give it a sniff. You can smell that off them, especially a bit in coats are stored in cupboards. They will have that mould exposure, but more importantly that will almost give you a clue that there's lots of more growing in the house and it'll be important to outrule that. The other thing that patients have are pets. Now I think birds are often associated with a particular type of garring of the lung, which is known as hypersensitive immunitis, pigeon fancius disease is one of the types, but you can have any other birds in the house like parakeets, budgies, I had recently a rock pebler who was the culprit for causing hypersensitive immunitis in patients. Don't forget about stuffed pillows. You can almost sniff away bird feathers in duvets or pillows and having stuffed with bird feathers and we have had cases where that could cause hypersensitive immunitis. Almost anything in particular inhalation can cause hypersensitive immunitis. Recently we have actually described patients who play musical instruments and we published a really good case about hypersensitive immunitis secondary to inhalation through a tenor horn of a particular type of a fungus called plylosinum. So musical instruments they can grow things and that can cause you to inhale the spores and that too can cause hypersensitive immunitis. So these are again normal causes. Now the other bits that you need to know about and it'll blend itself in your history is patients who suffer from autoimmune conditions. Now connected tissue disease, one type, I'll come back to that, but the most common one is rheumatoid arthritis and patients who have rheumatoid arthritis and we're getting better in dealing with joint pains and muscular skeletal problems but these patients are living longer. But what we're noticing now is that many of them develop lung fibrosis and it's important to take a good history from them about things like rheumatoid arthritis, the matter of my site is scleroderm shogun's disease, systemic sclerosis, you need to take a history because they will often have lung fibrosis and it's important that when you're taking a background history check for those and they will be even in your examinations you'll be able to pick up those signs and we'll talk about that later. So those are kind of occupational and environmental exposures we talked about. Drugs we've talked about connected tissue disease, those are all known causes but then when you have known cause and you have an outward everything else it's the condition called idiopathic, known on cause, pulmonary fibrosis and it's almost a default diagnosis but there are characteristics that you can have on history, a radiology and so on but it's really really important that you rule out all the known causes before you start labeling patients as idiopathic pulmonary fibrosis and about 60% of patients will have idiopathic pulmonary fibrosis so they are out there and I'm sure in some of those carousels they'll be a patient with idiopathic pulmonary fibrosis sitting there staring at you but we'll talk about how to examine them and get the diagnosis correctly. Yeah absolutely brilliant. What a great overview of all the different causes of pulmonary fibrosis there's just a couple of additional ones which I'm just going to mention I think that I think these two are really important to mention so the only because there are potential signs that the candidates could pick up and that's radiotherapy fibrosis and then sarcoidosis too. Yeah so I mean sarcoidosis can come up, it can lend itself to scarring of the lung but remember it's grade four sarcoidosis that will have the scarring of the lung and you will hear the crackles but in grade one two in grade one and two you won't hear those signs but they will have other skin manifestations they may have things like everything when they do serum or lupus perneal those signs might lend themselves and then of course those patients who've had particular type of cancer that's required ultimately radiation therapy they can have scarring also often you'll have to have the other clues in the history and to figure that out but keep that in your differentials. Yeah absolutely and so with all of those different diagnoses in mind we are going to move into our examination and hopefully along the way we'll be able to pick out some of the signs which will point your diagnostic weather veins towards the correct diagnosis and hopefully mean you'll get full marks in the station and so quite obviously this is hopefully going to be something we find in our respiratory examination station but obviously this is something that could come up in a clinical consultation as well and so the history taking is obviously going to be important you'll ask about a lot of the things that we've talked about however we are going to focus on it in the context of a respiratory examination station and so that's going to start as every examination does from the end of the bed so from the end of the bed as our candidates start their examination of their patient what are the signs as they step back and just observe the patient what are the first signs that they might see that may suggest their patient has intitial lung disease. So these patients have rest might be sitting quite comfortably but some of the advanced disease patients might have evidence of tickipney they're working a little bit harder they may have lots of weight and there's a highly catabolic diseases the breakdown a lot of protein because they work really hard in the breathing and whenever they mobilize they burn a lot of protein so they look quite thin but some of these diseases remember are treated with steroids so what you may see is side effects of steroids and cushion or features is one of the things that might lend yourself to you when you're examining them so that would be around faces or looking they have may have a fat pad behind their neck and so on. Are they tell tell features is if they are on long-term oxygen and they may have a set of nasal prongs delivering oxygen or if they're on ambulatory oxygen there are be they might have a concentrate to just sitting next to them so those patients that are using oxygen will have that with them so they might give you a bit of a clue that listen they get quite hypoxic with amuletion and they may need oxygen therapy so looking at them from a distance that could be telltale signs it's just when you go closer to them and start looking at the hands then you begin to pick up more signs should we move on to the hands then yeah absolutely go for it so clubbing I always say that whenever you do a hand examination put the hands on a pillow it it makes it easier to pick up the signs and just rest them on a pillow and you'll pick up the signs much easily one of the things that comes to mind is clubbing now remember in 50% of IPF patients they will be clubbing in another 50% it doesn't mean that they don't have clubbing they don't have IPF so it's not a pathonomic sign if you have clubbing there and it's a dispute examination they could be a few differentials coming through but IPF where the pathoclomyphabrosis could be one of them but of course you've got to think laterally too and things about bronchi ectasis and so forth to lung diseases but most likely will be idiopathic connephabrosis the other signs that you can pick up especially with autoimmune diseases or or connective tissue disease you can look for symmetrical arthropathy and swan neck deformity use routine yes sign and so on those lend themselves to rhebutoid arthritis the other features that you can see on the hands is sclerodactyl or digital ulcers that could lend themselves to things like a crest or systemic sclerosis and look carefully at the fingertips the DIPP IPE joins the MCP joins because you might see active sign of iters you may see some skin tightening in the fingers and that itself alens to conditions like systemic sclerosis and so on so it's really really important you examine carefully they one of the other subtle features in connective tissue disease like anti-syntatic syndrome they often have very dry skin especially over your fingertips of your thumb your ring finger any your index finger bilaterally and you will see skin quite dry, fisharing and on the owner side of the fingertips you will have skin thickening. So that in itself could lend to a condition for anti-sensitase. There's also the other signs you can see in the hands in the matter of my side as you can see got transpapules and so on. So it's really really important that you pick up those autoimmune signs that quite subtle but it could give you clues. Moving away and moving on to other things they might be on inhaler therapy so it doesn't mean that you don't you have idiopathic pulmonary fibrosis you don't have asthma. So just chef for a tremor that can be quite easily accentuated by putting a piece of paper and that could be you know they might be on a beta agonist and that could be causing that tremor and sometimes not always in ILE patients but they might have COPD concomit with ILD you can have CO2 tension so always check for astrosis and so you get them to extend the arms, cock up the wrist spread the fingers and they will keep them out like that and they will flap away in front of you and that is CO2 retention. So those are features that you can see on hand examination. I don't think I'm missing anything or of course I forgot they might be features of kisexia and muscle wasting and so on in these patients is if they have advanced disease. So those are I think the hand examination completed. Yeah absolutely and as we discussed at the start you really do have to be a bit of a Sherlock Holmes when you approach these patients for the first time but it doesn't stop there because as well as taking all this on board you've got to look for all of this stuff and you might only have 10 or 15 seconds to consider all these possible signs so parts of the paces learning journey I guess is getting the mental agility to absorb all of those signs in a really short space of time before rapidly moving on to the next section of the examination which I think I'll just sort of rattle through the next bit because I'm really anxious to get to the meat of the sandwich but moving up your examini arms and I think the only thing which I really found would be quite specific to have to look at there would be other signs of systemic sclerosis so skin thickening which is becoming proximal to the wrist which would be more in keeping with a diffuse systemic sclerosis rather than limited so that is important to look for and as we mentioned already looking for signs of cushingoid features or excessive use of steroids and you may notice thin skin or easy bruising in the arms as a sign of that correct I think we'll hear right now and and please look at your biceps and triceps the because these patients are often immobile and they're not been exercising and there's a lot of muscle wasting so you'll have a laxity of the muscle the bulk will reduce and you're from a field that they'll have wings and higher over the tricep area and that's because they burn a lot of protein and they'll burn a lot of calories so just to look out for them and of course don't forget to take the pulse because if they have suit you see or too retention they'll have bounding poles if they have hard issues they may develop atrial fibrillation so or the atrial fibrillation maybe the reason why they might be on cord neuron that could be causing the scarring of the loss yeah absolutely so yeah definitely important things to consider as well and then from the arms you'll move up to the face and the neck and here is where you'll get an opportunity to look more closely at the the face and neck and I think the main things to really look out for here is something we actually didn't mention the hands but we will mention now is is psionosis because if they are you know chronically hypoxic and they are psionosis this is a good opportunity to see that centrally in the lips and tongue and whilst you mentioned at the start about being tachypneic it's also the nature of their breathing and I guess one question would be is that in these patients do often see them person lip breathing in the same way you might see a C.O.P.D patient do that is that sort of a part of being generally sort of tachypneic on a chronic basis so if you have a patient who has got combined polymphibrosis and emphysema you may do that but normally in pyrrhyl D patients they don't tend to per slip I think per slip is to increase the peep they don't need an increased peep here they just need more oxygen so they will be air hungry so just to be mindful of that don't forget also in the head and neck as you said central psionosis and the tongue but don't forget in sarcoid patients you might have lymph adenopathy of the neck so go through your lymph nodes off your head and neck carefully because you often get patients with sarcoidosis which enlarge neck nodes yeah absolutely and of course as you mentioned earlier that lupus perneo as well which is a sort of how would you describe the sort of rash of lupus perneo in these patients with sarcoidosis it's quite a volatious red rash over the nose and the cheeks it's classical when you see it I think you almost blends itself I would recap by looking at some images and recently got caught out with a lupus perneo rash on black skin so it's really really important that you look not only at Caucasian but also look for lupus perneo on black skin because they just look a little bit redder and and it's it's it's almost macular popular in feel but you can get caught out yeah absolutely and I guess one of the other sort of differentials for a sort of vialacious rash commonly around the face but particularly around the eyelids would be a heliotripe rash of de-matamysitis so something something else to consider as well yes so I often you know and and patient a heliotripe rash over the forehead is difficult to recognize unless you lift up their hair and pull it back to look actively for that you've got to go and actively look for these signs but I hope well I mean I'm hoping that they will have given you almost clues at the beginning when they give you the instructions to for examination but I guess if you're vigilant for this you will miss it yeah absolutely but now we are heading to the meat of the sandwich which is the examination of the chest which obviously goes in the same fashion in each examination station of the chest which is you're going to inspect palpate and osculate so I wonder can we just run through that each in turn of the types of signs which we should be inspecting for palpating for and osculating for in in these types of patients and and the types of signs which again might give us an idea of an underlying cause absolutely so the first thing exposure please don't have your patient with a shirt on you know so many times as an examiner for the pieces that people have examined on the top of the shirt that will automatically fail you it's important you get them to take off their shirt or whatever they're wearing and don't please if it's a female patient the expectations are that you need good exposure so do ask the examiner and if they say um uh examine over the bra that's completely acceptable but please get that exposure because with exposure you'll pick up lots of interesting signs now remember some of these patients may have had of that spyopsy so they may be scars and they might be under the xilla on the lateral thorax so you've got to kind of lean over look into the xilla look at the front of the chest look at the back carefully and that will help if they've had that spyopsy and the scars will be easily identifiable some of them might have had new with oruses so look for those scars also they may have radiation therapy tattoos so look for that too while i've got them lean forward i'm looking at the spine itself because they might be chifotic or scoliotic and that could restrict the expansion of the chest and that itself lends to difficulty with breathing especially if you have a lung fibiotic disease and so carefully look i guess the next thing you would do is palpation and the first thing i tend to give all candidates to do is go forward chest expansion now people are a bit obsessed whether they do it from the front of the back i find it much easier to do it from the back and what you do is you place your fingers gently over the chest on each side literally get them to take a deep breath in and breathe out all the way all the way all the way all the way get your fingers kind of gently holding holding to the chest and with your thumbs almost together and then when they've held the breath ask them to take a deep breath and when they do you'll be able to measure this distance between your two thumbs and that gives you a good way of measuring the distance of the expansion it takes a lot of practicing and you know those candidates who've been practicing on the wards they do it so easily and quite slick, you know, in a very slick manner. So I think do get your chest expansion done properly. Then you would examine for tactile fremmeters. And once you've done that with all the areas of the chest, you then move on to percussion. Please kind of practice your percussion. The way I get candidates to practice percussion is to do it over a table. Tap away. Those candidates who have been really tapping on the chest, you know how good they are. And make sure you examine over the six different areas of the back. Don't forget the auxiliary on the side and also the super-clavicular areas. So checking for the percussion note itself. And then finally, over the same different areas, six of the back, two on the sides, over the super-clavicular fossa, oscultaid. And listen for the signs. The classical signs in a patient with interstitial lung disease will be the crackles, the velcro-like crackles. And always get them to cough if the crackles still persist, then you're almost in the territory of interstitial lung disease. If they disappear, then you could consider that infection may be a key here. But also other noises that you can have in patients with an ILD. Remember, you can have squawks. And I wonder, Sam, if you've heard of squawks. I have heard of squawks. Have I heard them myself? I don't think I have convincingly heard them myself, but I have heard of them. Let me give you a rendition of what a squawk sounds like. It goes like this. Are you ready for this, Sam? I'm ready. Herbic captures properly. It goes like. [Laughter] It's a chirping note. And I get so excited when I hear squawks. Because a squawks, for me, means three different things. And when I do that again, just if you haven't called it the first time, I'll do that again. Let me help you. It goes like this. That's fantastic. And that squawk will give you a little mean hypersentist and humanitis. It can mean organising pneumonia. And it can also happen in age influenza. So remember, squawks gives you a different differential. And with a good history of exposure and squawks, it can make a good diagnosis of hypersensitine humanitis. It's just you have to get the CT scan to confirm that. And if you want to talk about Brinkosculina later. But don't forget, these patients can have also other noises. You can have a patient with ILD who is asthmatic. So don't forget about the weasers. So you must be able to differentiate normal breath sounds from crackles to squawks to weasers. They give you different clues. Yeah, absolutely. And I think it's worth just saying as well that there probably is a good deal of overlap with some of these other conditions. But I do feel that in the exam, they might be more likely to give you an isolated condition because I think overlapping something something like COPD and ILD, you'd almost say, OK, will are we just going to say it's correct if they say either or both. I don't know. I think it would be a little bit challenging. I agree. I think I'm forgetting those as four pieces. I'm trying to build people, the listeners to be great at all I could use. I do apologize. So yes, they will. And remember, it won't be a respiratory consultant examining a respiratory station. And it's very difficult for a cardiologist to identify. It's going to having said that I'm not insulting our cardiology colleagues at all. Really great ones who can identify all noises in the chest moving on rapidly to the heart itself. Don't forget, well, you got this opportunity with the Oscultation, go and listen to the heart. It's the loud P2 that you're trying to look for because these patients often invariably develop heart failure. You either hear loud P2 additional heart sounds as three and this is really important. Sam, if you hear that loud P2, I would go back and look at the JVP because if the JVP is elevated and you got a loud P2, then inevitably you are in heart failure zone itself. I mean, once you've listened to the heart rush quickly to the legs and look for peripheral edema can be quite extensive in some of these patients. Look to the knees to see how extensive it is. If the patients lean forward, press gently at the back over the cycle area to look for a cycle of theema also. So I think it's very, very important to examine carefully for heart failure zone. Definitely. And I think we'll just dwell a moment on the nature of the crackles which we would expect candidates to find. And as you said, the typical type of crackle we'd expect is a fine in-spiritually crackle. And I think one of the really important things is and this should be sort of routine in your respiratory examination. If you do hear crackles is asking the patient to take a cough and seeing if those crackles change. And another sort of quite crucial distinction which you'd be expected to make is the difference between a fine crackle and a coarse crackle. Because I think those are the types of distinctions where if you get that wrong that it's leading you down almost a completely different diagnostic avenue. And so I think those two things identifying and distinguishing fine from coarse computations and making sure you're not checking that those computations aren't disappearing or changing with a cough. Absolutely. Because most of the time in our early clinics we see a lot of advanced patients, we'll see really coarse, velcro like crackles. But don't forget you're right. The fine crackles can lend itself to other different causes. And you're absolutely right. The number of times when we examine the good candidates will always get the patients to cough. It's a quick way to win to differentiate patients who have infections because if the cough disappears it's leading you to a different algorithm altogether. Of course, you know, you know, it crackles, you know, quite a few different things that can cause crackles. We've got infections with a said, you've got interstitial lung disease, heart failure and the final thing is bronchiectasis. If the patient has bronchiectasis, there might be quite kind to you and left a sputum pot next to the bedside. So if you have a sputum pot there, do open it and have a look at it. And if you find meek, up your own flame, which is coloured, smells a bit awful. Then you know, you might be dealing with a patient with bronchiectasis. Absolutely. And so moving on from there, that pretty much sums up our examination of the patient. But then after that you'd be expected to present your findings back to the examiner. And so hopefully you're going to be incorporating all of the signs which you've discovered through the course of your fluent and systematic examination. And that should bring you to ideally a specific diagnosis. But I guess the other end of it is that the diagnosis could always be idiopathic or it could be related to autoimmune or connective tissue disease or occupational. And there may be overlap, there may be coincidences, there might be red herrings in all of that. And so I think it probably is unfortunately one of those things which you almost have to learn a list of possible causes to be able to re-aloft confidently to the examiner when you come to present them as a patient with ILD. Yes, you're right. And if it's a short case, you know, you can't converse with the patient. So you are rightly to say that listen, I've identified an interstitial lung disease. I would take a thorough history and try to identify a particular cause because my differentials could include connective tissue disease, although I did not find any features on peripheral sigma to connective tissue disease or a drug history or maybe if they've had it secondary to chemotherapy. And then you would say basically, if there is no cause, then I would call this idiopathic pulmonary fibrosis. So you're covering your tracks. In the short cases, that's very easily done. However, in your station 2 and 5 where you will have an opportunity to take a history and examine the patient, then you will be a mismarple or shallow running around looking for those clues. Absolutely. And so you're going to present your preferred diagnosis and hopefully an underlying cause as well. And hopefully going on from there, you're then going to discuss your investigations for the patient. And so as as I've already said, you're going to describe how you're going to take a full history from the patient, asking about specifically about any underlying causes, including pass medical history, drug history, social history, etc. And then you're going to move on to very simple things. So you may say you want to perform a full systematic examination of the rest of the patient. and routine observations, so things like oxygen saturations at the bedside, taking a formal respiratory rate, and any bedside respiratory function test, which you might be able to do. But if we move on to, if we're talking about sort of full-blown investigations, if you're going to talk about it and get your money's worth from the time you've got, lung function test is going to be something really important to mention, isn't it? That's right. And remember, if it depends on the setting, in a acute setting, you know, patients who are not well in the emergency department, you don't tend to do lung function. You would have ever in that patient setting. Yes, absolutely. If it's in that patient setting, I would get a spherometry with a diffusion capacity, and you're looking for a restrictive pattern. You have both a reduction in your FEV1 and FEC with a normal or super normal ratio, FEV1 FC, FEC ratio. And invariably, what these patients have is that there's impermanent of gas exchange, so the diffusion capacity will be reduced. That is it in a nutshell for lung function. The other things that they like to hear, the examinism, I mean, is making sure that you do a full blood count. Tell them what you're looking for in the full blood count, looking for a source of infection to see if there's elevated white cell count, and looking for a Nemia on the hemoglobin to see if there's another cause for the shortness of breath, or they may have, because of the hypoxia, they may have polycythemia. And then you talk about the urea electrolytes to see if they're dehydrated, look for infected markers such as CRP. The O2 antibodies that you check for is the anti-nuclear antigen, the extra nuclear antigen, the CCP, anti-CCP, and then looking for vasculitis, particularly using an anchor. If you're looking for myocytus, you're looking for things like CK. And then you have extended blocks, blocks such as myocytus and scleroderma blocks, before the rare antibodies that you can look for. Don't forget an ace in context of sarcoidosis, and then if you are suspecting hypersensitum immunitis, go and look for the precipitums, even precipitums, or mold, or farmas long, they like to hear that. So that is a comprehensive blood kind of work up. And finally, the imaging is the most important bit. I think you would be forgiven to say that I wanted higher resolution CT immediately, rather than a chest X-ray. But because we don't make the diagnosis of indistitial lung disease based on a chest X-ray, we like in HRCT. And the patterns you're looking for on an HRCT are the UIP pattern. And particularly in idiopathic polymphabrosis, it's a subplural distribution, basal predominant, and there is evidence of reticulation, an air-wedilitation, and if it's a definite UIP, you will have honeycodal. Remember, this is an entry exam. There's not an exit in that exam. So I wouldn't go and worry about probable UIP or indeterminate UIP. If you know the definite UIP, what you're seeing, the subplural distribution, basal predominant, traction air-wedilitation, reticulation with honeycoming, in the realm of UIP, that gives you almost invariably a diagnosis of idiopathic polymphabrosis in the absence of any other course. And then there are other types of things like NISIP and Plural plaques and things like that. For the purpose of this exam, don't worry about that. And then, if they ask you and probe you, would you like any other tests? Well, in the case of hypersensitive immunitis, I do a bronchoscopy and do a bronchoral avial lavage. And that allows you to look for lymphocytosis, anything above 40 percent lymphocytosis, you're in the realm of HP. And I mean, I don't do biopsies anymore because our radiologists are getting so good at getting the diagnosis based on what we tell them and our multidisciplinary team meetings. I think we're doing less and less of that's biopsy, but we are uncertain. We may put forward a that's biopsy. And we're also using cryobyopsy now to getting samples from patients. And it's important to bring everything together and say that, listen, having taken good history, to take an examination, perform the bloods with the imaging and the pulmonary funkant tests, plus or minus biopsy, I would like to discuss this in a multidisciplinary meeting. And that is the key because all our patients go through an MDT because there you will have clinicians like myself and a radiologist, a thoracic radiologist with a thoracic pathologist and all the ally professionals sitting there, including nurses or pharmacists. And you may also have a rheumatologist there in the Connected Tissue ILD MDT. So to get that consensus diagnosis, because it's based on that consensus diagnosis that we can plan treatments. Yeah, absolutely. And that's going to lead in really nicely into when you're expected or asked about how to manage these patients. And so I guess one of the first things to mention in a condition where the team approach is so important is mentioning that MDT approach, not just specifically the MDT meetings, but obviously the team being led by a respiratory physician with an interest in ILD. You will have support of specialist physiotherapists. You'll have ILD nurse specialists. You will have, I'm trying to think of the guys who, the pharmacists, the pharmacists, well, I'm trying to think of, I guess it would be the specialist nurses who would be involved in sourcing long-term oxygen if that were required and things of that nature. So really important, obviously, to mention the MDT approach in a station like this. But going on from there, I wonder, would you be able to give us sort of go through the general management from your perspective? And that stretches all the way through from conservative and sort of lifestyle measures and then medical therapy and whether or not you would ever subject these patients to any form of surgical treatment. Sure. So you're absolutely right. Long gone are the days where it's clinician led. It's that multidisciplinary team led. Let's not forget the community teams and the hospital teams all working together in keeping this patient as well. So the first thing I would say is if they're smoking, please refer to smoking cessation. It's never too late to give up smoking. If they've got mold in the house or they've got pigeons open the cage, let the pigeons fly away. There is, in the Bristol Zoo, there is Dr. Adam Ali's, there's an Avery probably named after me. For homeless birds, birds of lost causes, but it's really really important that you remove the particular antigen that is causing the damage to the lungs. If it's a particular drug, remove that. That's a quick win. And if it's pillows with stuffed birds, get them synthetic ones. If it's mold in the house, get them to clean the house thoroughly. Mold is such a problem in the United Kingdom that government has involved themselves. In Bristol, we are very lucky to have a centre of sustainable energy. That actually looks, provides leaflets, consultations on the phone and they can do home visits to look at the house and advise how to clear mold. So, age UK is very good in providing dehumidifiers, getting people to come and clean the house thoroughly, putting air breaks and so on and so on. Better ventilation in the house. And then the important thing is to make sure that these patients don't develop infections. Remember, and this is a well known fact, if you have an idiopathic gonorrholyphibrosis patient who develops an infection or an acute exacerbation, there's a 50% mortality so say within a month. So it's really, really important that these patients are vaccinated. So don't forget your flu vaccination, your COVID-19 vaccination, if they're between 75 and 80, the respiratory sensitive virus vaccination, and then the pneumococcal vaccination, that can prevent them picking up infections and having a backup prescription for antibiotics is really, really important. And then one of the things I often get patients to think about is keeping active, often keep condition by sitting around. So referring to them to a Pomemary Abilitation Program, is really, really useful. And that you can all do it in your clinic or recommend the GP to undertake the vaccination and so on. Once you've done that bit, you will hand these patients over from your end. You will have educated them about the antibiotic treatments. Now the antibiotic treatments have been around for almost, since 2013, so almost 12 years. So we've had access to perfinodone and mintininib. You should know about these treatments a little bit and I would recommend that if you You're going to mention one clinical trial for an intensive is the impulses study that was published in New England Journal of Medicine almost 13 years ago. And it showed that an intensive slows the progression of the disease. We'll come back to an intensive in a minute. The other antibiotic, the clinical trial was ESSE and D study. And that also slowed the progression of the fibrosis. And the important bit is that it doesn't reverse fibrosis just slows the rates of decline. So if a patient with that treatment they would lose approximately 250 mils in FEC per year with the antibiotic they reduced half of that. But the clinical trials were never set up to look at survival. But we know the real world data coming through that people are living longer and longer now. And that's really important. But the problem with these drugs they have got side effects. And it's up to you to inform the patient about the side effect. Nintendo, it can cause loss of appetite, loss of weight. But it can cause diarrhea and 60% of patients. Also, I forgot Nintendo is two tablets a day. Profinidone is nine tablets a day, three in the morning, three in the afternoon, three in the evening. So there is a tablet burden. But it too can cause loss of weight and appetite. It can cause reflux and it causes a photosensitivity rash. So you have to be covered from head to toe with a sunscreen, factors 50 in the bottom. For the exam purpose, if you can't remember that. Just if you mention Nintendo and Profinidone, you'll get the briny points. And also remember, those are antifibrotic treatments. But we have treatments for example, for connectative tissue disease, you can use steroids, aquatic steroids and immunosuppressive medications and things like, is a thibin, microphenolate and so on. In hypersensitive and humanitis, you also use that. So that is the pharmacotherapy bit. Don't forget that these patients can be referred to a clinical trial because there's no holy grail at the moment. There's no medication that can reverse the fibrosis. So we're looking for newer, newer treatments. It's importantly, you refer them for clinical trials if they're available. So do mention that. And then there are other bits to managing this patients. It's very important that you look at symptom control and management. So they get very short of breath. So you need to think about, we've talked about ambulatory oxygen therapy, all-on-term oxygen therapy, depending on what they're eligible for. The other things you can think about is morphine and antialytics. Now, morphine is often used as an angi-sib, but one of the side effects is reduces air hunger. So you will often find patients going on morphine and antialytics because when you get very hungry for air, you get very anxious. So introduce something like mortasopene can also help in reducing that level of anxiety. So symptom control management is very important. The other bit about symptom control management, please don't forget to look at the cough. These patients can get very easily tied with a cough. So think about medications like Dextrametorfan, Robotocin cough syrup or Coding Lentus. In some patients, we may even use things like Gabbo Pentin and those refractory coughs that just want to respond, we have used Thalidomide also. So cough is a big bugbear of mine and we really go with guns blazing in trying to reduce that because it gets them really, really tied. Finally, there will be some patients who will be eligible for transplantation. Now age is a bit of a factor. We always consider transplantation for less than 65 and you're looking to see the eligible for transplantation and patients who have progressive disease, there is a rate of decline of FEC greater than 10% or DLCO decline of 15% with progressive symptoms or CT progression. Plus or minus their own oxygen therapy, we often think about transplantation earlier on because it can become very difficult to refer them after the age of 65. And then don't forget advanced care planning. Some of these, not all of these illnesses, particular IPF will progress despite all your measures you've instigated. And it's important that you sit down with the patients, advise them about will and lasting power of attorney and don't forget to work with them and get them to complete the respect form. We in fact in our centre have a dedicated nurse-led respect clinic and they come with the nurse for a lease and are and discuss all about advanced care planning and respect. So yeah, that's in a nutshell I think some. Brilliant, it's a bloody big nutshell but yeah, you fitted it all in. Absolutely brilliant. What a brilliant overview of all aspects of management and we've talked about so much in the ILD space. We've run through all aspects of the causes. We've talked through the examination in detail, we've talked through our presentation, range of differentials and the investigations and management. Absolutely. And if you own ILD patients you should be smiling and if I may recommend some people are very good at examining because with respect to examination you do a day in and day out. Just get a little bit slicker. If you're not feeling about ILD you will have an ILD clinic in your hospital. Why don't you go and sit down with a physician who is happy to have you in their clinic and teach you a bit. It'll build up your confidence and if you have an ILD patient it's a given you'll get it and you'll do very well if you know how to approach it. And hopefully what we've talked about in the past RISM will empower a lot of our listeners. Absolutely, I really, really hope so as well. But I think that's just about all the time we've got. So that only leads me to say an absolutely huge thank you to Dr. Huseyfa Adamarli, consultant respiratory physician with an interest in ILD from North Bristol NHS Trust. It's been an absolute pleasure. Thank you so much for coming on the podcast. Thank you so. And listeners that's just about all the time we've got for this week's show. Please don't forget to like, follow and subscribe to the show wherever you get your podcast. We always love to hear from you. So get in touch on our website prepacespodcast.com or via the email which is [email protected]. And as always if you want to directly support the show you can do that at bimeyocoffee.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 will see you next time on the prepacespodcast. Bye.