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Interstitial Lung Disease

Apr 15 | 3:30 PM

Interstitial lung disease (ILD) is a term used to describe a collection of about 100 chronic lung diseases that are characterised by inflammation and scarring, making it difficult for the lungs to get enough oxygen. Pulmonary fibrosis is the term for the scarring. The symptoms and progression of various disorders can differ from one person to the next. Inflammation is a common thread that runs across all of the different types of the disease. Bronchiolitis, alveolitis, and vasculitis are all characteristics. Let's learn all about the pathogenesis, clinical presentation, diagnosis, and management from this outstanding Medflix select creator, Dr. Salil Bendre.

[Music] good evening dear doctors on behalf of team netflix i dr fatima welcome you all we are truly grateful for all of your presence here tonight we have the honor of having among us dr salil benray who is a very leading pulmonologist and transplant physician currently currently practicing in mumbai sir is head of department of pulmonary medicine at nanawati hospital today sir will teach us and guide us with his expert perspective on the topic of interstitial lung disease thank you fatima and good evening all the doctors who have joined today for this session on a very very common yet a little complicated disease which is interstitial lung disease as i think all of you doctors students practicing physicians pulmonologists are very much aware of this entity which we call very commonly as interstitial lung disease we'll try to start from the basics of understanding what do we mean by interstitial lung disease subsequently we'll try to you know go through some radiological images now for those who have who are just you know starting to learn about respiratory diseases i need to first tell you that uh you'll hear very often that a person is having a dry cough persistently going on for a few weeks breathlessness which is exertional going on for a few weeks to months and though we know that copd is a very common disease tuberculosis is a very common disease but please keep in mind that even interstitial lung disease is a common disease and more so because now with ct scan interstitial lung diseases are being diagnosed much more easily and in the much more early stage okay so let's let's just go with a a simple x-ray before we start with the anatomy the pathology and physiology i would like all of you to just look at this x-ray and those who have been able to appreciate it's very nice but there is a opacity which is like a whitish areas all throughout both the sides of the lung which we in the terms of a description call it as inhomogeneous opacities or ground glass opacities on an x-ray now maybe some of you may not appreciate the changes on an x-ray right at this moment but such a patient with an x-ray like this and who comes to you saying i'm having cough for last few weeks or months specifically dry cough specifically dry cough so he says that i am getting a cough but hardly any sputum and my problem is that when i walk i feel breathless and that too for a couple of weeks two months or maybe even years you should be suspecting first of all a interstitial lung disease so remember as far as history is concerned chronic history means long-term history usually however there are diseases which can present with the acute history also so not that every patient with ild has to have a chronic history most of them have a chronic history when i say chronic it means more than six months more than nine months so a long term history characteristically dry cough okay characteristically dry cough if a person has got a long term history but he says i am getting a lot of expectoration you will virtually not think of interstitial lung disease so it has to be dry cough which is going on for a long time it has to be breathlessness which is exertional unlikely to be a sudden onset breathlessness so in that scenario what you will think about is is it the disease in the interstation there are many interstitial lung diseases it's virtually like saying a patient is having say a fever and fever has got 1000 causes right from viral fever to malaria to dengue to typhoid to urinary infections everything similarly interstitial lung disease is like a big basket it just means that the interstitium is affected it does not this word ild doesn't mean anything specific to the etiology our treatment is based on the etiology our treatment is based on the cause all right so this diagram actually tells us in a very simple fashion about what do we mean by interstitium now you need to really understand this and the next few slides because your entire basis of understanding the diagnosis treating the disease will be based on this particular slides few next slides in this particular slide so the alveolar has a basement membrane as everyone knows the alveolar has the basement membrane they are like sac and there is a basement membrane which has got two type of cells again try to go back to your physiology there is a type 1 pneumocyte type 2 pneumocyte this is the cell wall of the alveolus outside that is a space which is the interstitium and there is a pulmonary endothelium that is the capillaries which are lining it the endothelium so there is a space between the alveolar epithelium and the endothelin of the pulmonary vessels that space is called as the interstitium so when we say this patient is having ild when we say that x-ray is showing a ground glass you need to remember that where is the disease it is in the space outside the alveoli it is in the interstitial space all right now this is again a diagram to make it a little more clear that if there is a interstitial disease then where exactly it should be now if you can see this diagram you can try to see it a little by magnifying it or holding it and trying to see it in more you know magnified view the space or the lumen is the alveoli okay it's a cross section it's a cut section so it's a lumen is the alveoli now you can also see that there is there are cells in the alveoli there is a type 1 alveolar cell type 2 alveolar cells which are the cells in the lining of the alveoli and then there is the pulmonary capillaries which are around the alveoli which obviously help in gas exchange the pulmonary capillary veins the capillary lumen all these are the vessels which take help in the gas exchange so when i say there is a disease in the interstitium please remember it is outside the basement membrane and between the capillary endothelium so that is the space which are very very microscopic space here we are showing a diagrammatic representation obviously it's going to be in microns that space is going to be really really very thin and that space is called the interstitium that's just the anatomical location now at this point please understand that every part of the body has its own function okay every part of the body then it may be the nasal mucosa may be the bronchi bronchioles all the parts of the body similarly interstitium has a function and when that function is not fulfilled the patient becomes unwell so what is the function of the interstitial the function of the interstitial need to maintain the gas exchange which happens so the basement membrane the gas enters when we pitch when the patient breathes and the gas crosses through the interstitium into the capillaries so that is the function of the interstitial if there is damage in the interstitial the lungs are going to be irritable there is going to be affection of this gas exchange so when we say gas exchange there is a word for it in is that the gases diffuse through the interstitial so in short if there is a problem in the interstitium the defect is in the diffusion so anatomically the disease will be in the interstitium and physiologically the defect will be diffusion so these two things will happen and whenever there is a diffusion defect the outcome is that the gas exchange will not happen and so obviously the oxygen is going to become lesser and lesser in the pulmonary vessels i hope you are getting it i am trying to go a little slow on that because it is not about the etiology it is always about the pathophysiology etiology you are going to anyway investigate and find out but what happens inside is the pathophysiology so just to go back on that interstitium the space the normal physiology diffusion problem in the interstitium now when i say problem it's a very simple and very basic word but we will go to that what exactly i mean by saying a problem then it happens is that the diffusion gets affected and the outcome is that the oxygen becomes less so now where all is the interstitium that also matters because as you graduate to seeing ct scans or reading ct scans you will hear of different terminologies intra-lobular interlobular pulmonary lobules okay these things are all going to come come to you when you talk about ct scans they may not be pertinent so when you actually examine a patient or when you see an x-ray but ct scan is a three-dimensional picture so it helps us to know the exact location of the interstitial lung disease so the lung as we know as we as the alveoli the bronchi branches and branches and branches they finally go into lobules okay and the in between the two lobules are the inter intralobular the interlobular reticular septae or the interstitium and within that also there is small branching which is the intra lobular interstitium so interstitium is distributed throughout it's not only in the lower zones lower lobes only in the upper lobes no it is not so it is distributed throughout now why this is important okay why this slide is important because as you read ct scans you will realize that certain interstitial lung disease affect only the upper part of the lung only the interceptal area certain interstitial lung disease affects only the basal areas of the lung so that's where all these things will matter to you okay now when i talk about ild the question asked is why do you need to really identify what is a different type of ild why it's the treatment we have why can't we just do a x-ray diagnosis and start treatment so the reason for later on knowing the etiology is because every cause of an ild has a different outcome different prognosis and most of them differ in the treatment also so it doesn't have a blanket treatment that okay diagnosed with ild start with this particular medicine diagnosed with iodine start with steroids it is not so the prognosis differs in different ilds the treatment also differs in different ilds so you need to know a little bit of the details of the interstitial lung diseases now you can see this particular change in the interstitium or in the anatomical changes which happen with the process of disease now to understand when i say disease please remember i am talking of inflammation that's just the hallmark of the interstitial disease inflammation okay inflammation means there will be inflammatory material there will be exudates there will be mediators which which collect or which start affecting the space that is the interstitial as you can see in this diagram interstitium is smooth in the normal lung so the oxygen and the carbon dioxide diffusion is very easy and very simple and very straightforward however if the interstitium gets affected or inflamed then naturally the impact is going to be on the oxygen and the carbon dioxide okay so i hope you have been you're understanding exactly what i'm trying to explain to you that interstitium means oxygen diffusion effect and as the process of inflammation continues more and more problem in the diffusion defect now this is again a very simple and very basic diagram which is seen in every book of anatomy of physiology uh guyton ganon whichever you talk about respiratory physiology which tells us about the terminal bronchiole so there is a terminal bronchiole which is depicted here in the green color and accompanying that you can see a artery then there are the alveolar sac and all this unit is kept or is stuck up in a septe which is the interlobular septa okay inter globular septic and we saw in the previous slide that the septe also has interstitium the interlobular interstitium the intralobular interstitium so in between the alveolar sac also there will be interstitium plus they will be stuck up in a particular cascade a particular capsule kind of thing that is called as the interlobular septa okay so these are the different places where the interstitial lung disease gets affected or starts affecting now at this point please remember all of you that interstitial lung disease is not a disease which is only in one lobe it is a bilateral lung disease it's a diffuse pulmonary disease diffuse diffuse means both lungs affected pulmonary obviously lungs are getting affected so it is a diffuse lung disease so if you see a x-ray which shows a localized patch only in the right upper lobe only in the left lower lobe don't think of an interstitial lung disease as the first diagnosis okay don't think of it as the first diagnosis try to think about a localized disease could be an infection whereas if you see bilateral diffuse abnormality opacities think in terms of a interstitial lung disease now this is this slide is just trying to explain what so the one with that picture the diagrammatic picture is just the anatomical representation and exactly what is seen on a ct scan so you see it's a very vague picture on a ct scan but as we sometime come across ct scans in the later few slides you will be able to start interpreting them but try to see how the ct scan looks like you will see lot of lines white white white lines which are actually the septe which we discussed that is the inter lobular septa they are lines lines which are seen and in between them small small boxes triangular rectangular boxes are actually the lobules which have the multiple alveolar alveolar sac so as the interstitium in the septa become inflamed as they become inflamed septa start looking more and more white or they become more and more prominent normally you will not you may not be able to see the septie on a ct scan it will be just like a black area but when you start seeing those lines properly demarcated lines it means that the septae is getting thickened or safety are getting inflamed so that is how the ct scan would look like all right so this is how a cd scan would look like of course this is just the beginning so you may have to interpret it considering what could be the you know the picture on the ct scan now i'm i put this x-ray again so on this x-ray what is seen is if you if you can imagine a normal x-ray then normal x-ray is not as white or as whitish as compared to the x-ray on the screen it is more like a patchy ground glass or face it is groundless this word you remember this word ground glass so one is a glass which is very clean and you can actually see through everything and second is a powdered glass or a ground glass so there is a irregularity or a self design on the glass so that's why it's called as a ground glass appearance so this is about the ild i am showing you a classification of ild which is very very important okay why this is important is because we will be talking about sarcoidosis you will be talking about interstitial lung this is called as hyper sensitivity pneumonitis but all these are now classified you can see one classification is called as idiopathic interstitial pneumonia this classification you google it out and you will get it you don't need really need to you know start writing it down or noting it down you just put up the recent classification of ild and you will get this classification but i need to explain to you each of them so one is what is called as idiopathic as the name suggests the etiology is not clearly known okay it is not clearly known so it is that's why it is called as idiopathic interstitial pneumonia idiopathic interstitial pneumonia the second group is the autoimmune this name suggests that there is a auto there are antibodies formed which are damaging the interstitial so it's a auto immune interstitial lung disease so which one was the first one idiopathic where we did not know the cause which one is the second one the auto immune interstitial lung disease the third one is the hyper sensitivity pneumonitis hyper sensitivity so there is excessive sensitivity and presenting as inflammation so there is a hyper sensitivity pneumonitis that the third type fourth is a completely different entity which has to have which has to be decided on its own merit which is sarcoidosis sarcoidosis and the fifth one is what is classified as other interstitial lung diseases which could be drug induced radiation induced many other chemical induced so there are other ilds so let's go through this one two three four five basic classification one is idiopathic idiopathic interstitial pneumonia iip second is autoimmune third is hypersensitivity pneumonitis it is also commonly termed as hp hypersensitivity pneumonitis fourth is sarcoidosis and fifth is other ilds okay so this is the broad classification we are going to know about each one because each one has got its characteristic presentation each one has its characteristic outcome prognosis there could be some ilds which progress in a matter of two years and the patient dies and there could be some ilds which might go on for 15 years but no progression and there could be some interstitial lung disease where at diagnosis itself the patient is having hypoxia so it can be so variable so please remember that you need to first of all try to diagnose different types of ilds okay now in the iip that is the idiopathic interstitial pneumonia there are many subclassifications okay there are many sub classification the common one you should remember is ipf that's the first box which is in idiopathic pulmonary fibrosis idiopathic pulmonary fibrosis so this is the first box which you have to remember now if this classification is put up in front of you and you ask me which is the commonest type of ild in our city in mumbai then the first one to be ranking in the order of prevalence is hypersensitivity pneumonitis okay hp is a common ild the next in line is idiopathic pulmonary fibrosis then of course is sarcoidosis and other ilds follow so there are many different different different ilds which are present but you have to remember this in some way or the other first is idiopathic interstitial pneumonia out of them the common one is ipf that is idiopathic pulmonary fibrosis there is one more common variety of iip this is called as non-specific interstitial pneumonia nsip nsip maybe you can go through this classification later on but i am going to repeat this again and again because this is something which you really need to you know sort of mug it up or understand in the way you wish to know so simple thing is iip next will be autoimmune hp sarcoidosis and other ilds in iip you have to remember idiopathic pulmonary fibrosis and non-specific interstitial pneumonia nsip okay so i hope we can proceed now further we are going to go into a little details about the molecular change which happens in these ilds because each of these ilds have their characteristics though this diagram appears a little more you know elaborate and too complicated you can just refer to the boxes at the base at the bottom which talk of the pathogenesis different changes of fibrosis which are happening and subsequently there is also the post inflammatory changes which are happening with deposition of different material i hope you can actually see the different points which are mentioned there right from the inflammatory cells coming in the epithelial damage which sets in then there is even the vascular involvement which is there so multiple few multiple changes which happen at that point of time and since subsequently every inflammation has a repair process and that repair process is going to be fibrous tissue now it's not only the fibrous tissue and fibroblasts but also there is lot of extra cellular matrix ecm and this matrix further causes lot of damage and distortion of the interstitial so to make it very simple fibrous tissue once it is deposited it starts pulling the tissues around it towards itself you very well know that a scar or fibrous tissue starts pulling the different different structures towards itself so once we have that happening there is a huge involvement of even the epithelium the macrophage the activation factors platelet activation factors tumor necrosis factor as well as the what we call the vascular endothelin growth factor so multiple factors come into place and then the final outcome is what is happening is this so two things happen when a patient will come to you as doctors and you diagnose this patient and say sir i feel you've got interstitial lung disease or you tell man you are suffering from a interstitial lung disease they may not understand that word interstitial lung disease so you may try to make it simple and say fibrosis my fibrosis okay so the question which the patient is going to ask you why did i develop it i am not a smoker i don't have any bad habits so why am i developing the fibrosis so the answer is in this particular slide so there are factors which predisposed to developing interstitial lung disease one of course is going to be a genetic factor that is called as the genotype we always hear of genotype and phenotype but one is the genotype and the phenotypic is the manifestation so there is a genotype of an individual the constitution of an individual and there is an injury which happens which could be even as simple as a pigeon feather dust exposure pigeon feather dust exposure i'm just giving a very example simple example that a pigeon feather dust exposure is a simple reason why a patient may develop lung fibrosis and not a very uncommon cause as well people who are in spray painting aniline dies people who are in industries so all of these inhalation factors can predispose if the patient is having a genotype which predisposes to that and finally all these things are going to the alveolar epithelial phenotype alterations what happens next is that the epithelial apoptosis now this word apoptosis means there is a cell death means there is a cell suicide the cell kills itself so there is a epithelial apoptosis there are myofibroblast proliferation matrix deposition and angiogenesis okay so multiple changes are happening so it gives rise to diffuse parenchymal lung disease this is another word which is very commonly used these days dpld diffuse parenchymal lung disease dpld so please remember that this will be the line of action which happens when a patient undergoes the changes of fibrosis and so as i was just elaborating multiple changes come into picture now this becomes important because for some ilds you will use steroids again i come back to that now if there is a myofibroblast deposition if there is an extra cellular matrix deposition if there is fibrous tissue deposition the steroids do not help steroids don't have any action they don't help at all steroids help in the early phase before the extracellular matrix get deposited before the myofibroblasts come into picture okay so they come in the latest steroids are useful in the early phase not in the later stage so once the fibroblasts are deposited fibrous tissue is deposited the only thing which may prevent further deposition of this fibrous tissue is anti-fibrotics anti-fibrotics okay so there will be different different mechanisms of each interstitial lung disease so for practical purposes genetic predisposition chronic repeated insult injury that leads to inflammatory cascade mediators come to the place and it leads to fibrous tissue and it causes lot of vasogenesis and changes in the vascular injuries also are added on to that so that's how it will work on and this is again perhaps a repeated new slide but i just wanted to make sure that each of you can understand that there is different different steps which happen right from stage one step two everything is put up in these boxes and these are very diagrammatic and simple uh you know mechanisms which can tell us where exactly are we going to hit the treatment part so if you have this particular cascade and you don't know where the patient is landing up is in the first few weeks of the disease is it in the second few weeks of the disease is in the later part of the disease so once he comes in the later part of the disease which is as you can see in the later boxes for four five six in that time the patient is actually breathless he is perhaps on oxygen he's developed secondary hypertension which is for core pulmonary is there he's got a dilated ventricle and so on and so forth so multiple complications come into picture now at now this is a ct scan which which you will have to see you know you will very commonly see this i am just putting up a picture because when you see radiologically asked linations first thing is you want to see let's see the cd scan now those who have not seen the scans till now this is the simple way a lung looks like right now just remember that the hole or the black black circle in the center is the trachea on the right side the entire black and gray area is the right lung and on the left side is the left lung this is how a normal lung would look like now what will happen and how will an interstitial lung disease look like in the stage of inflammation okay in the stage of inflammation so remember there are two basic stages of ild inflammation fibrosis so if this is a normal ct scan then this is how a inflammatory stage of interstitial lung disease will look like you can make out the difference very easily in this particular ct scan the one in the center is again the trachea that is the bifurcation of the trachea you can see and on the sides you can make out those arrows are pointing towards whitish grayish areas which are called as ground glass opacities so if you see a ct scan like this this would actually mean that it's in the inflammatory phase of interstitial lung disease so just try to compare it with the previous cd scan you can see the lung the lung is very clean there are those small white dots but they are vessels and few septa are seen but if you see this area this can you can see that there are extensive ground glass opacities which are seen in the ct scan now let's go through a couple of specific uh you know interstitial lung diseases you can see this in the specific interstitial lung this is the cascade which is there this is about a sarcoidosis you remember we just talked about sarcoidosis first was the group of iip the next was autoimmune third was hp and fourth was sarcoid now sarcoid is also a common disease and you will very often hear this diagnosis because it mimics tuberculosis very much so what happens in circuit i'm going to just show couple of maybe three to four slides to be more specific about uh you know different types of ilds so one is about the sarcoidosis so there is an antigen there has to be an antigen which happens now unfortunately the there is a lot of controversy or gray area about what exactly is the antigen which causes sarcoid some books talk about even tubercular baxilla itself being a triggers precursor on an antigen enough to cause the sarcoid granuloma so there is sarcoid tuberculosis also in some patients but this antigen it unites or it has an antigen presenting cell and the macrophage comes into picture then subsequently all our immune system comes into picture which is through the t cell mechanism so remember that we have read about b lymphocytes and t lymphocytes but this is where the t cell immunity comes into picture and then subsequently the interferon gammas the tumor necrosis factor and repeated repeated exposure leads to a granuloma formation that is sarcoid so sarcoid is a granulomatous ild there are granulomas formed but the difference in sarcoid is it's a non-necrotizing granuloma tuberculosis is a necrotizing granule now if the in the early phase itself the trigger or the antigen is removed either by treatment or by avoidance then sarcoid actually goes into remission means sarcoid may go away without treatment no treatment nothing required it just vanishes so is sarcoid a curable disease yes if it is diagnosed in the early time of the disease is interstitial lung disease or curable disease yes it is treatable if it's in the inflammatory phase and the response is very good but if you are diagnosing a iod in the fibrous stage where there is collagen there is extra cellular matrix there are myofibroblasts there is vasogenesis then you are not going to be able to actually give him symptomatic relief so you need to remember about these particular diseases then comes the common disease which i have talked about is hypersensitivity pneumonitis maybe as a clinician we may not want to read all these different different details of what happens here what happens there so you may take a picture of this and go through it later but for all the clinic clinicians i will tell hp in about three to four minutes so remember one hp happens in three stages first is what we call that acute hypersensitivity humanities when i say acute you imagine a patient comes to you a young man or a young lady comes to you and says doctor i'm getting dry cough for last couple of weeks okay since last three weeks i am getting dry cough and i feel a lot of chest tightness since last 10 days i have started feeling breathless also so what is the history two to three weeks symptoms dry cough exertional breathlessness you examine the patient and you hear bilateral mark my words bilateral inspiratory so there is a inspiratory foreign sound which is bilateral and inspiratory foreign sounds are called as repetitions so bilateral inspiratory repetitions so this patient is in front of you acute history dry cough exertional breathlessness bilateral repetitions you take an x-ray and x-ray is exactly the way i showed you ground glass pattern you do a ct scan ground glass pattern you need to ask history is there any pigeon exposure do you have any pets at home have you visited any farm or any local area where there is a lot of haystack because any of these can give rise to an antigenic exposure and lead to hypersensitivity pneumonitis so acute exposure right acute hp now you have treated this patient let's take another example patient comes to you says doctor since last three to four months okay mark my words again three to four months i'm getting dry cough my cough is just on worsening it's going on going on i get treated i feel better again it starts and i feel a lot of breathlessness again there are bilateral repetitions ct scan is showing ground glass think in terms of a sub acute hypersensitivity pneumonia so subacute and lastly a patient comes and says doctor i have got this cough for last two to three years i have visited five different doctors but the cough isn't going i don't know what's happening and it's a dry cough i feel the cough should come out but nothing is coming out and i feel so breathless when i walk i start feeling breathless i'm short of breath this could be chronic hp so in simple words hp can present as acute subacute and chronic and how do we confirm hypersensitivity pneumonitis well there are two ways to confirm it one the ct scan picture is almost we can say pathognomonic of hypersensitivity pneumonitis when we do a session on ct scan in ild you'll be able to know how exactly and how typically the hp ct scan picture looks like so it can really look like look like a particular pattern and you know that we are dealing with a patient of hp and of course the conformation will come on a biopsy you need to take a lung biopsy and the histopathology will show that yes as dr shubham is saying mosaic attenuation so for we have not yet discussed ct scan picture so maybe this word mosaic attenuation may not appeal to everyone but when we are discussing ct scans in ild we will be able to appreciate the different patterns of ild now this today's session i thought it should be more related to you know the clinical presentation or what exactly happens when we talk of a patient is having ipf idiopathic pulmonary fibrosis what exactly happens why are we dealing with it what's the biggest problem we are dealing with it so there are multiple changes which happen in a hp patient also and multiple cells get involved neutrophils the macrophages and all these cells cause repeated inflammation repeated inflammation and damage now when i say damage the fibrous tissue or fibrosis will cause distortion and pulling of the tissues around it so that was about sarcoid and hp and coming to another important ild ipf now we'll just take a break for break in the sense wait for 2-3 seconds and try to remember the classification i want each of you to remember the classification which we discussed i'll give you three seconds to mentally remember it before we move on to that remember the different ilds which we thought of so now we are going to talk about ipf idiopathic pulmonary fibrosis this is again a common presentation unfortunately i should say because we virtually can't do much in a patient of idiopathic pulmonary fibrosis and there are multiple factors involved in this again but since this exact cause is not known be classified as ipf so there are particular there are chemicals there are autoimmune mechanisms viruses everyone everything has been supposedly having to have some action and leading to that and it leads to activation of a coagulation cascade even for that matter it causes different uh fibroblasts and problems related to the fibrous tissue deposition macrophage activation multiple things come into picture and then there's an imbalance because of all this there is a pro fibrotic mediator as you can see the different pro fibrotic mediator and there is an anti-fibrotic component also but there is an imbalance so the pro fibrotic obviously surpasses the anti-fibrotic component that's why this this particular change or fibrous tissue gets deposited and finally the endothelium the epithelium the fibroblasts so everything comes into picture and the extra cellular matrix turnover comes in and there is excessive of this matrix deposition and leads to ipf okay so there is a lot of things about the idiopathic pulmonary fibrosis which each of you should know it's not just about a ct scan picture suggest your ipf when you're treating ipf you need to know what you're treating that's so important now this is a ct scan picture of these different changes which happen just to make a life a little simpler when we talk of ct scans maybe in the next session sometime so a normal lung at this point of time patient is getting just exposed to different viruses or chemicals or are different instigating agents and that time the ct scan is going to be normal patient may not have anything on a ct scan the moment the inflammatory cascade comes into picture which could be lymphocytes in sarcoid it could be neutrophils and idiopathic pulmonary fibrosis it could be neutrophils even in hp or granuloma formation in sarcoid the moment those changes come into picture what will be the symptoms patient will start having dry cough patients will have exertional breathlessness you do a ct scan you will see a ground glass pattern you can see this is called as a ground glass pattern so these are the inflammatory changes the vegf the tnf the interleukins the interferon gamma everything is come there the next stage if the instigator agent continues there is a repeated onslaught of the triggers and there is a genetic factor which is predisposing this individual to develop fibrosis or for that matter there is an autoimmune component where antibodies are constantly getting formed and that is leading to repeated accumulation of myofibroblast extracellular matrix the next thing is there is a lot of cyst formation cis means it's all it's called as honeycombing okay cyst formation and those sis formation is what is we typically say on a ct scan is a fibrotic ild it's a fibrotic ild so just to bring everything in line all the inds which we talked about okay all the id's which we talked about at the end of the their tenure i can say or the end of their the way their progression is fibrosis everything ends into fibrosis even sarcoid presents as fibrosis in the later stage hp also presents as fibrosis in the later stage all right so all of them can at some point present to you as a fibrotic ielt it may have started as hp three years ago but patient may come to you come to you and they may actually present with fibrotic ild so there is a big difference in bronchitis and honeycombing of ild please don't confuse or get mixed with those two terminologies honeycomb being on an x-ray on an x-ray is what we saw talk about in bronchitis whereas honeycombing pattern on ct scan is multiple cysts small small cysts which are stacked upon each other i as i said ct scans we will take up in some session later on today's session is just to make a primer of you know what are the different types of ildn somehow there is a myth that interstitial lung disease means it has to be chronic so there are of course acute ilds also so don't be under the impression that they can't be in 10 days or 15 days they can be in weeks and days also so the one which is commoner one is acute hp as we have discussed earlier acute interstitial pneumonia acute interstitial pneumonia we have seen viral infections even covet for that matter it presented as an interstitial pneumonia right and that's why their patients had breathlessness they used to have dry cough and they used to have hypoxia so there are different entities like cryptogenic organizing pneumonia what is cop so try to remember these things because these are going to be more patterns related to the radiology and to the treatment i'll just give a few tips on what is cryptogenic organizing pneumonia cryptogenic again the names suggest everything cryptogenic means we are not clear with what is the cause the genesis is cryptic so we don't know what's the cause organizing pneumonia now pneumonia this word or this term is usually reserved for lung parenchymal disease means alveolar disease but here we are using it in the interstitial lung disease parameter so there is a formation of a pneumonia-like picture in the interstitium as well as the alveolus and it is almost presenting as a pneumonia because the patient also has fever okay so there is presentation of fever bilateral diffuse opacities so this mimics an ammonia that's why cryptogenic organizing pneumonia is usually not the first diagnosis you see a ct scan and you diagnose as cop it doesn't happen many times a cryptogenic organizing pneumonia is confirmed or rather say i would say thought of when the pneumonia doesn't respond to the antibiotics which are supposed to work and since it is a organizing pneumonia and the interstitium is involved the best way to treat it is with steroids so there would be a patient out of hundred one or two patients who would have fever persisting for two weeks or three weeks ct scan is showing patchy areas of consolidation not responding to a good amount of antibiotics fever is continuing breathlessness is progressing and it could be actually a cryptogenic organizing pneumonia so that is where we have to think about cryptogenic organizing pneumonia not a entity which we can just think about at the first first ct scan itself you need to have a very strong clinical uh background to really think about a cryptogenic organizing pneumonia all right so uh can i can i ask you one question can you tell me two examples of idiopathic interstitial pneumonia just two subdivisions or sub classification of iip idiopathic interstitial pneumonia whoever are attentive they can please try to answer that let me see who are attentive for that two examples or two sub classifications of idiopathic interstitial pneumonia very good so i'm getting a few answers i can see there someone has written nsip someone has written nsip ipf so nsip yes so so so good you people are now using the words correctly nsip and ipf and trust me on this if you really really see ct scans and if you start treating respiratory cases at least in a week you will land up seeing a report of cd scan suggest you of ild easily you will see three four ct scans so the report is suggest you of interstitial lung disease so uip pattern nsip pattern so i'm using the word pattern because it's all based on the ct scan picture so this is a good way to start a discussion on interstitial lung disease and of course we have got the sub acute to chronic interstitial lung diseases as well so in that it can be a idiopathic pulmonary fibrosis and another word for autoimmune is connective tissue diseases associated you will hear this word again very commonly ctd ild ctd means connective tissue disease associated ild so ctd ild so they can be sub acute to chronic ipf can be sub acute to chronic sarcoidosis can be sub-acute to chronic so there are multiple entities which can present from three months to six months and diagnosis could be interstitial lung disease clinically what should be the finding on presentation bilateral repetitions bilateral inspiratory repetitions people call it runs and crackles whichever adjectives you can use you want to use you may use it but they are end inspiratory crepitations now just to make a little things uh clear about repetitions by and large the explanation for repetitions is that there are secretions in the alveoli so when air goes in there is bubbling of secretions and that makes a sound which is called as repetitions however in ild we talk that there are no secretions the disease is outside the alveoli that's why we are talking of a dry cough so why should there be repetitions first of all so once the interstitium gets inflamed which is outside the allele it actually compresses the alveoli there is a pressure outside the alveoli which compresses the algae and once the patient starts taking a breath at the end of the inspiration at the end of the inspiration there is so much pressure that it suddenly opens up the alveoli that opening snap or that sudden opening up of the alveoli at the end of the inspiration makes a sound which is called as repetition so it's an inspiratory foreign sound heard at the end of inspiration and that is again very characteristic of interstitial lung disease yes people call it velcro crackles you can actually feel your hair and rub the hair so you hear a particular sound of rubbing so that description has also been given in books but not many books talk about why do we hear repetitions in interstitial lung disease few important points you should remember as doctors as clinical perspective on examination can there be vision present in ild yes the answer is yes in hypersensitivity pneumonitis patient may have wheezing along with repetitions so remember because hypersensitivity is very bronchial okay it is if there is a bronchi and there is a pretty bronchial interstitial around the bronchi also we saw that there is interstitium so what will this interstitium do they will try to compress from outside this interstitium will try to compress from outside so hp is typically very bronchial so it causes narrowing and so when air passes through the narrow airway while exhalation it causes the sound which is the wrong kind can there be patient having pleuritic chest pain in ild in very specific patients again with connective tissue this is ild-like serositis polymyositis there are patients who can present with pleuritic chest pain also so this slide i put up because we need to know that ild can present with different complications like a patient with ild may come with recurrent pneumothorax now how will it cause pneumothorax so there are certain ilds not common i am trying to say they are not common there is a disease called as lymphangio leo myomatosis okay lymphangioleomyomiatosis lam it is in simple words it is called as lamb so there are multiple cysts which rupture and then there is repeated pneumothorax so if you're asked can a patient of ild have wheezing yes it can example is hypersensitivity pneumonia is why in hypersensitivity pneumonia is because there is very bronchial fibrous tissue deposition can there be pleura involved yes there can be pneumothorax can happen so this is also a presentation can an occupational disease lead to lung interstitial lung disease so the answer is yes asbestosis is one of the diseases which can present as ild it can present as a plural effusion as well and asbestos can lead it can be a pre-cancerous entity as well so asbestos exposure chronic asbestos exposure which could be in the rubber linings electricity wire linings on shipping so many of those occupational hazards or repeated exposure can give rise to ild and problems related to the ild again now this is again a very important slide because not every ild has only and only respiratory symptoms it's a systemic disease so patients can have joint spellings arthritis especially those with rheumatoid arthritis so r a i ld rheumatoid arthritis interstitial lung disease so there is a there is a very simple statement which makes which talks about it rheumatoid arthritis can have respiratory symptoms following the symptoms of arthritis or it may also have symptoms of ild prior to the arthritis so if if a patient of rheumatoid arthritis comes to you already on treatment but he or she says that i am getting joint pains i am getting joint pain my treatment for ra is going on for a couple of years but since last few months i have started to get presenting with this dry cough and you do a ct scan and you find that there is a interstitial lung disease pattern then please consider that this could be rheumatoid arthritis related ild and in that situation you may actually have to discuss the case with the rheumatologist who is treating the patient so that you get to a common platform of what exactly to treat this patient with swollen fingers there could be swollen fingers which are there they're not phenomenal scleroderma there are patients with dysphagia okay there could be patient with dysphagia especially in scleroderma the esophageal motility is affected so if you as i was starting in the beginning that i ld is such a big term and just to label a patient that oh you've got ild it's actually just saying that i think there's some disease happening in your lungs it's ja is as much vague as that you need to get to the root of it why this this slide is so important because certain iles and if they are associated with connective tissue diseases the treatment is completely different there could be drugs this is modifying drugs which are used which could be methotrexate okay which could be methotrexate which could be any other drug even hcq is being used in rheumatoid arthritis there is one more drug called as mycophenolate so the treatment will differ once you talk about the different reasons of ind eye involvement even drying up of the eyes is a symptom of a patient having ill repeated uveitis there is redness watering of the eyes patient has uv it is this could be because of sarcoidosis so though i am a pulmonologist and i am talking of interstitial lung disease we cannot overlook this general examination we cannot overlook the other systems i'll give an example sarcoidosis can present with liver sarcoid liver involvement there will be multiple granulomas in the liver and patient has jaundice you do a liver biopsy you will diagnose a sarcoid so sarcoid can present with any particular presentation it could be a skin lesion and the biopsy is showing sarcoid so though we say sarcoidosis is of course ild but don't overlook any other symptom if a patient has it you should please go into the details of it and last few slides before we stop for today because there is too much material i have suddenly bombarded you on with ild is that when we talk of a specific entity like ctdi and the connective tissue disorder there are a few blocks i have written here systemic sclerosis ra sle jogarin syndrome myocytis so these are the common ilds which present with ctd ild the connective tissue disease and there are certain numbers or certain alphabets written because ctd ild has to be diagnosed with lot of markers there are antibodies to specific antigens and then we can diagnose that okay this is sle or this is ra or this is yograin syndrome so that's the different group so this is a for those who are interested in connective tissue diseases and who would like to you know know more of them there are specific markers so as i have written here in the blocks also that for a particular disease like say myocytis there is a jo1 antibody then there is a a test is of course going to be done ccp ccp and ra factor for rheumatoid arthritis there is a anchor test which is done which is of course all these are blood tests which can tell us about vasculitis so this this particular slide actually encompasses or tries to think of patients with connective tissue disorders and you need to remember you you you'll get this somewhere or the other in the google also but you need to remember that this slide is very important and if you have a middle aged lady say a patient comes to you around 55 56 and says i'm getting joint pains i've got skin tightness i feel there's tingling in my lower limbs while i walk i get joint pain sometimes there's watering of the eyes when i try to eat food i feel there's something stuck please get this workup done these are blood tests any of the diagnostic labs do these blood tests and you get the results in about four to five days and if they are positive by any chance then you are struck upon a connective tissue disease which needs to be treated correctly so you need to bring this all together and speak to the rheumatologist because that's how the work will be done faster and faster so i'm not going into this but this again was summarizing and uh this as i said was the ct scan of the ild and if you go into the electron microscopy picture then it's it is exactly like what is seen in this slide there are multiple small small small small pockets and you can see the interstitium would be a very very small area which is which is affected and it damages so again this is uh we'll stop at this because i think maybe in some other session we can take this uh slide and discuss it a little in detail but but for this moment of time these are the pattern sometime later on we can discuss about the different actual ct scan pictures because it needs a complete session on ct scan and x-rays we cannot have everything put together but these are the different patterns you may take a picture of this and keep it maybe in the next discussion we can you know have a better uh details about how to diagnose these different ilds so i leave it today with that for all of you to think about we have discussed the pathogenesis the different mechanisms the classification understood that ild doesn't mean only the lungs systemic involvement is equal you need to pay attention to the other systems and the presentations investigate accordingly history taking is of again a very very significant parameter you need to know the occupation also exposures prior to these symptoms when the disease has been started and of course reflux do not forget gastroesophageal reflux and the medications there are certain medicines which can induce an ild so everything has to be put together so ild patient comes to you spend at least 20-25 minutes just knowing what is the lifestyle of this patient and continuing further so i think that will help you all and i'm sure there are a lot of queries and questions if not then we'll meet again next time so thank you uh fatima again for giving me this opportunity to be with all my friends and colleagues thank you so much sir it was so incredible learning from you once again this has been incredible our audience can join us for part two of the session on 29th april at 9pm dear doctors please put in your queries in the comment section so we have a raise hand request could we take it it's from dr madhiv desai yes yeah good evening sir good evening sir dr bender right now i am in usa and have made it a point to see that i attend your lecture because in 40 years of my practice i have never heard this kind of in-depth discussion so i just wanted to say excellent talk and thank you so much i don't have any questions but in the comments yeah my pleasure thank you so much sir thank you thank you yeah all of us agree with dr maddie if they say this is really incredible so you've meticulously explained every aspect of ild so beautifully yes uh coming to the co we enjoyed seeing you teach with so much dedication agreed agreed thank you sir great session thank you thank you best teacher excellent session thank you that's fine yes uh it seems like a lot of questions on the treatment of ilt so so what we can do is whenever we are meeting next next no half the session will keep on scans and half on the therapy we can try to cover that time sure there are multiple questions on the treatment aspect of it uh regarding the medications um there's also a question lung transplant done for end stage ile so i take it so we will be covering that also in the next session so just to just to make one thing uh one of my old patients who is a ctd ild has just undergone a lung transplant about 48 hours ago in mumbai at global hospital so yes we are doing lung transplants for these patients okay and the prognosis of it uh was the follow-up question though yeah so prognosis i mean if you are talking of lung transplant is that specific for lung transplants yes yes one of our doctors difference between systemic sclerosis lyroderma and ilds are yeah so so again see these particular things about uh i think we will have to have some some point where we can you know talk about ctd when i'll cover this point in the scans that may be a little easy because even there's a radiological typical presentation of systemic sclerosis and sle so we'll cover that keep it in mind okay doctors we will be covering it in the following session uh sir dr mahadev desai asks your experience of code and ild and its outcomes so as uh sir is saying and a lot of questions i could see from a coveted point of view i would say around two to four percent of severe poverty when i say severe covet means those who have been on high flow oxygen those who have got extensive lung involvement almost say uh 60 to 70 percent lung involvement have landed up with post-coverage lung damage post coverage i would not call it fibrosis in fact we have stopped using the word fibrosis here we have called it post covid scarring and they are presented with persistent changes on the scans for even five months to six months but out of those four to five percent who develop these changes 50 of them the changes have reversed and cleared up completely however few of them have persistent to be on nasal oxygen at home even after six months of therapy so in covid yes a small chunk of patient have been on oxygen needing uh you know high flow oxygen even at home but they have been in the severe stage of oxygen and early phase early days of covid where the treatment was not known we never knew how to treat but now fortunately the treatment has been more strategized and we can avoid these complications thank you sarah uh one of our doctors asks um how can one start an ild clinic that's a excellent uh you know initiative in fact we should be starting you can start i mean if you are a pulmonologist then obviously you are going to keep it once a week or once in 15 days if you if you are really keen to start it and you need to educate you need to create awareness amongst your own peers and your family physicians because the patient is going to come to the family physicians first so that's where your awareness should start with and subsequently you should have a spirometry with dlc at your clinic because we are going to monitor these patients by doing a spirometry with dlc so you need to have a dlc or six minute walk test you need to have a good radiologist to discuss these things and naturally you need to have a rheumatologist also who should be a backup with you so iid clinic is superb no doubt you need to plan it maybe start is as i said once in 15 days gradually you know start creating awareness in your locality or amongst your doctors and that's how it should work out okay thank you sir uh we have one more question where uh final confirmatory test for diagnosis of ild so finally yeah so so i cannot say there is one final confirmation for ild because there is a reason for that idiopathic pulmonary fibrosis doesn't need a lung biopsy idiopathic pulmonary fibrosis is confirmed on the ct scan itself you don't need a biopsy however if there is a young patient who is undiagnosed we are not able to know what exactly is the ild young i am saying around 34 year old 37 year old diffuse parent or lung disease you need to do a open lung biopsy or a cryobiopsy so it will vary according to the presentation it will vary according to what you are looking at if there is a patient with bilateral hilar adenopathy bilateral hyaluronidopathy ground glass opacity is in the upper zone and serum ac level angiotensin converting enzyme level is more than 50 it virtually points towards saying this is sarcoidosis you really don't need to do a biopsy so it will vary according to the different ilds a ctd is something against those antibodies which i told you about if there is a conformation with those blood tests that there is either a uh syndrome or an sle and a ct scan pattern suggest your file you don't really need to do a lung biopsy so you will have to take help of the blood test clinical presentation but undiagnosed difficult to diagnose younger individuals please go ahead and ask for a lung biopsy that's the only way to confirm okay so thank you uh so it seems like a lot of questions are in the treatment and investigations aspect which i'm sure we will be covering in the next session yeah yeah dr ganesh says we're looking forward to the second session we have another question ct scan blood markers biopsy do you want to add anything so we will be covering the investigations again in the next session correct yes absolutely we will cover them up see ifd is such a huge huge disease i mean we can't we will try to finish in the next session but let's cover all these things thank you so much we are now looking forward for the part two thank you so much thanks it was a pleasure see you again okay bye good night thank you sir good night

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dr. Salil Bendre

Dr. Salil Bendre

Pulmonologist & Transplant Physician, Nanavati Max Hospital, Global Hospital & Masina Hospital, Mumbai

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dr. Salil Bendre

Dr. Salil Bendre

Pulmonologist & Transplant Physician, Nanavat...

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