Restrictive Lung Diseases

foreign what's up Ninja nerds in this video today we are going to be talking about restrictive lung diseases lots to talk about so let's go ahead and get right into it so the first thing that we have to talk about like every video is the types of restrictive lung diseases the causes and some of the pathophysiology behind that let's take a quick look at the pathophysiology because thankfully in comparison to obstructive it's not that intense you're like oh thank goodness it's not really that bad so what I want you guys to understand is a patient who has a restrictive lung disease there's some type of exposure to a very various amounts of triggers and that's where restrictive lung disease can be somewhat complicated but when we talk about restrictive lung disease the pathophysiology is straightforward there's some type of Trigger or exposure and we'll talk about all those below here's this trigger that we're going to say right these these blue antigens if you will once the trigger gets into the actual pulmonary system it becomes exposed to alveolar macrophages these alveolar macrophages when exposed to these antigens or triggers they become activated you know generally when they become activated they present a piece of that antigen on their mhc2 complexes and express it to T cells and then activates an entire immune response that's what happens these macrophages start producing lots and lots and lots of cytokines inflammatory cytokines there's so many of them I don't think it's that pertinent to talk about them though but what I want you to know is there's many many different cytokines and what these do is they alert the immune system they activate or stimulate neutrophils to come to the area they activate and stimulate more T cells to come to the area and this propagates a worsening immune response now here's what's interesting whenever there's a lot of this inflammation because you know neutrophils will come to the area and they're going to start releasing proteases and reactive oxygen species you're gonna have T cells coming to the area releasing lots of other types of cytokines and maybe even making some immune complexes but all these things are going to just propagate lung injury Whenever there is injury to the lung tissue repeated injury to the lung tissue what happens is this stimulates these special cells within the lungs you know these these cells called fibroblasts so here we have these like these orange cells here these are called fibroblasts now what happens is from repeated repeated lung injury due to this particular trigger the fibroblasts becomes stimulated by these inflammatory cytokines and they start producing lots and lots and lots of fibrous tissue and what this fibrous tissue does is it starts actually depositing into the actual lung parenchyma and this includes a lot of different parts of the actual lung parenchyma we used to call these interstitial lung disease meaning it only deposited in the interstitial spaces but we found over time that it can actually deposit into the visceral pleura it can deposit around the bronchials it can deposit into the alveoli it can deposit into the interstitial spaces around the pulmonary capillaries so we call these diffuse parenchymal lung diseases now but it's very diffuse fibrosis so you're going to get a ton and ton of fibrosis of the lungs why is that problematic well imagine you have these super super fibrotic lungs lungs that don't really want to be able to have much give if they don't have much give that's a problem because the lungs are supposed to be easy to stretch and be compliant when you take a breath in but if they're very fibrotic the lung compliance drops significantly their ability to stretch is reduced and subsequently if you reduce their ability to stretch you've reduced their ability to be able to inflate and take in air and so there is a significant reduction action in the inflation ability belongs and so we call restrictive lung diseases diseases where they aren't able to take in or inhale as much volume of air in comparison to the normal lungs due to excessive fibrosis now here's the big thing when we talk about restrictive lung disease we can actually put them into two categories pulmonary disorders and we actually like to call these intrinsic pulmonary disorders meaning that there is a disease involving some of the lung parenchyma maybe there is fibrosis around the bronchials maybe there is fibrosis around the alveolus maybe there's fibrosis all in this interstitial space maybe there's fibrosis of the pulmonary capillaries if it involves any of this tissue we call this the lung parenchyma tissue so all of these that we just mentioned this is the lung parenchyma and whenever there is fibrosis of the lung parenchyma we call this an intrinsic or a particularly an intrinsic pulmonary fibrosis and we see this very specifically sometimes again we used to call these interstitial lung disease because we used to think the fibrosis primarily only occurred in the interstitial spaces but we've found now it deposits us all over the place it's diffuse so sometimes instead of calling it interstitial lung disease you may also see it called diffuse parenchymal lung diseases so understand that now when we have these diseases these diffuse parenchymal lung disease or interstitial lung diseases where there's lots of fibrosis to lung parenchyma there are so many different causes behind this and we're going to talk about each one of those below because that's the more in-depth discussion but what I want to take a quick second to talk about is remember the lung tissue is not the only thing that's important for the actual expansion of lungs to being able to inhale properly take a good deep breath that's not the only thing that's involved in a part of our respiratory system being able to breathe you know what else is involved the pleura the pleural cavity if there's lots of fluid in the pleural cavity imagine the lung being able to expand against a pleura that's filled and chalked up with fluid are you going to be able to expand this long against a big fluid cavity now no so pleural effusions can also act as a restrictive lung disease but it's not due to a pulmonary tissue issue it's an extrinsic or extra pulmonary Disorder so pleural effusions big fluid in the actual pleural cavity or a lot of air in the pleural cavity like a pneumothorax that's also going to push on the lung and prevent it from being able to expand what if the plural is not a problem what if it's due to the muscles or the nerves that Supply the muscles you know your intercostal muscles your diaphragm they're supposed to be able to contract and help to be able to bring the diaphragm down lift the ribs up and outward and if that doesn't happen we can't expand the actual lungs and particularly the chest and so inflation or inhalation of the actual lungs is going to be impaired what if there's weakness of the muscles due to the nerves that are supposed to be supplying them being damaged like in Guillain-Barre syndrome or there's inhibition at the actual neuromuscular Junction due to antibodies that are attacking The nicotinic receptors like myasthenia gravis or what if there's actually damage to the muscles themselves such as dermatomyositis or polymyositis that could be another reason so it could be the pleural cavity that's the problem could be the muscles that are involved in breathing this problem or what if there's just not a lot of flexibility within the actual chest wall there's problems with the actual skeletal muscle structure not this I'm sorry the skeletal the bone structure the ligaments the joints that are around that in situations like scoliosis or Ankylosing Spondylitis or what if you have a big chunky fatty layer that's sitting on top of that preventing the actual chest bulb from being able to expand in situations like super obesity these are big things to think about for patients who have restrictive lung disease so now quick understanding again lots of fibrosis of the lung tissue causes decreased lung compliance they don't want to stretch they don't want to inhale and bring air in as well as normal lungs this could be intrinsic disorders meaning it's a part of the lung parenchyma that's actually undergoing fibrosis there's many that we'll talk about down here or it's extrinsic remember pleural cavity disorders neuromuscular disorders are chest wall disorders all right let's talk about the particular causes that we can actually see for intrinsic pulmonary disorders within that restrictive lung category all right so now when we talk about these interstitial lung diseases or diffuse parenchymal lung disease again this is the intrinsic types of restrictive lung diseases due to fibrosis that is occurring of the bronchioles the alveoli the interstitial spaces the pulmonary capillaries there's many different diseases that actually fall within that category that lead to that excessive pulmonary fibrosis now when we think about these one of the categories that I want you to put them in is granular kilometers you know granulomas there's different types there's caseiating which you see in like tuberculosis and there's non-case eating that you can see in situations of like sarcoidosis and hypersensitivity to pneumonitis so I want you to group it into this category one of the intrinsic restrictive disorders that I want you to think about are interstitial lung disease or diffuse frequential lung diseases is granulomatous diseases like sarcoid big one to remember and the second one is hypersensitivity pneumonitis that's the big pathophys now granulomas are basically you have this kind of like clump of tissue here which has macro you have a lot of macrophages then around that you have lymphocytes and then you have these like things called giant cells and then lots of fibrous tissue around that and this kind of tissue when it gets deposited into the actual bronchioles the alveoli the interstitial spaces around the pulmonary capillaries it really kind of causes the success of fibrosis and restricts the lungs from being able to expand now we can see this in sarcoid and hypersensitivity pneumonitis both of these that's the cause non-case eating granuloma was the deposit in the lung tissue big thing that you can think about with epidemiologically between these two disorders whenever they present them in the vignette is that sarcoidosis we primarily see this in very young African-American females 20s to 30s and also they may present this as a clinical vignette queue is exposure in the past too borrelia burgdorfi which is the bacteria responsible for Lyme disease or exposure to mycobacterium tuberculosis which is what's responsible for TB so so if they've had some type of exposure to these pathogens in the young African-American female with granulominus infiltration of the actual pulmonary tissue causing a diffuse sprinkle lung disease think about sarcoid if it's a farmer this is the big one think about Farmer's lungs so sometimes we see this in like patients who are actually exposed to lots of spores from the actinomycetes or from certain types of other situations like exposure to Hay barley some of the organic dust that's in the actual hay The Barley or the proteins that you may have in Pigeon droppings are the feathers from pigeons can actually trigger this granulomatous type of response that you see in patients who have intrinsic lung diseases or these interstitial or diffuse parenthal lung diseases so hypersensitive pneumonitis think about a farmer someone who's actually working with hay barley or is exposed to a lot of pigeon protein like the pigeon droppings that have lots of proteins within them that can be inhaled or the feathers that have lots of these proteins on them and again can trigger a granulominus response the second category that I want you guys to remember for interstitial lung disease are these diffuse parenchymal lung diseases is a category called pneumoconiosis so similar to hypersensitivity there's exposure to an organic dust if you will some type of substance that when it gets into the lungs it acts as that particular trigger activates the immune response causes lots of fibroblasts to cause fibrosis of the lung tissue what is really important that they're going to present in the vignette is the type of environment that this occupational exposure came in or the particular organic dust that they were exposed to and it's really critical to think about these well test your knowledge on these a little bit later but what I want you to remember if they present in the clinical vignette that you have a patient who's developed this interstitial lung disease and they work as a sandblaster they work in mines they work in foundries which are like metal factories you really want to be thinking about something called silicosis if they're talking about a patient who works as a shipbuilder or Works in plumbing because they're like fitting different pipes or they're working in Roofing because they're dealing with insulation this is a high risk category for asbestosis if the patient is working in some type of Aerospace industry right where it's exposed to different nasty types of rocket fuel or things like that or Electronics they're working in maybe computers or Ceramics or tool in die manufacturers you really want to be thinking about a disease called borreliosis okay berylliosis and then last but not least is if there has a patient who's exp who's working in particular coal mines or carbon mines it has to be very very specific coal or carbon mines you want to think about what's called co-workers pneumoconiosis so we call this cwp co-workers pneumoconiosis all right so we got again granulominus diseases sarcoid and hypersensitivity one think about the farmer working with barley hay or pigeon droppings then young African-American female with exposure to TB or limes then you got pneumoconiosis you have silicosis asbestosis briliosis and co-workers pneumoconiosis the next thing that you want to think about is is the patient exposed to particular drugs so if they present in the clinical scenario in a scenario patient has been on Methotrexate for some type of immuno underlying immune disorder or some type of like rheumatological disorder they've been on Methotrexate and they've been on Methotrexate for a while Methotrexate has the ability to trigger this type of fibrosis response as well as maybe they have afib and they're being treated with amiodarone or they're on nitrofurantone and they're taking this maybe particularly because they have very frequent urinary tract infections like cystitis or they're taking bleomycin as a type of chemotherapeutic agent for some particular reason these are one of the most common agents to think about so if they present these drugs in the clinical vignette they could be the reason that the patient has developed a diffuse periodical lung disease lots of fibrosis of their uh diffuse printable tissue so think about these the next category so we got granulomatous we got pneumoconiosis we got drugs Methotrexate amiod or nitrofurantoin and again bleomycin the next category is collagen vascular diseases so these are kind of like immune disorders they're almost like an immune disorder where they actually cause a lot of Auto antibodies to be produced and these Auto antibodies may actually cause destruction of some of the actual lung tissue and surrounding maybe pulmonary capillaries it may cause a little bit of vasculitis of the pulmonary capillaries but they're going to inflame some of that actual parental tissue when you inflame the parenchymal tissue constant repeated lung injury activates fibroblasts that start laying down fibrous tissue what are these diseases that have Auto antibodies that are attacking some of the printable tissue leading to this fibrosis over time well the first ones you want to think about is there antibodies that are attacking this lung tissue in a disease called Scleroderma think about anti SCLC SEL 70 or anti-centromere antibodies these could be the ones that are attacking the actual lung tissue and causing fibrosis if they present in the clinical veneer the patient has exposed some type of very particular antibody such as what's called C anchor antibodies C anchor antibodies cytoplasmic anti-neutrophilic cytoplasmic antibodies that are attacking the actual parenchymal lung tissue particularly the capillaries and causing something called granulomatosis polyhangiitis you know what else they call this Wagner's Wagner's granulomatosis the other one is if they have some type of Auto antibodies that are destroying the actual lung tissue or the vascular tissue again the parenchymal lung tissue and these are specifically called p anchas pericytoplasmic anti-neutrophilic cytoplasmic antibodies destroying the actual parenchymal lung tissue causing fibrosis and lastly what if they have exposure to a very specific type of antibody which are going to be attacking the basement membranes around the actual respiratory members so you know the respiratory membrane is the alveolar capillary you get a thin little basement membrane then after that you have the pulmonary capillaries these antibodies love to attack the actual basement membrane tissue and cause destruction of that inflammation lung injury repeatedly and then fibrosis these antibodies are called anti GBM glomerular basement membrane antibodies and so these are also really important because not only do they tack the particularly the antibodies of the glomerular basement membrane they also attack the basement membrane of the pulmonary system as well the respiratory membrane all right this is the collagen vascular disease so for to reduce diffuse printable lung disease or interstitial lung disease think about granulomatous diseases pneumoconiosis drugs collagen vascular diseases in one last category we don't know why they develop it and this actually happens to be one of the more common ones because sometimes we can't find a particular cause for these patients who have diffused parenchymal lung disease we can't find that trigger that that specific reason and for these we call this idiopathic pulmonary fibrosis we have somewhat of a thought as to why these patients May develop this type of fibrosis and we think it may be due to smoking or old age but it's more of a hypothetical reason we don't have a clear-cut idea so idiopathic pulmonary fibrosis is a diagnosis of exclusion it's the only one of these that causes the intrinsic or diffuse parenchymal lung disease or interstitial lung disease that's damaging the actual alveolar tissue bronchial tissue interstitial lung tissue pulmonary capillar tissue that we don't have a cause for that covers the types the causes the pathophys now we got to do is take these disorders particularly the one that we're going to focus on for the rest of this lecture the intrinsic the diffuse Primal lung disease is the interstitial lung diseases we're not going to talk too much about the extrinsic or the extra pulmonary causes we're going to focus on the intrinsic ones now and talk about their features their complications how do we diagnose it and how do we treat it let's get there all right so when we talk about the features and complications of these diffuse parenchymal lung diseases interstitial lung disease is again all the intrinsic types of restrictive lung diseases we went over the granulomatus we went over the pneumoconiosis we went over the drugs we also went over the collagen vascular and then we had the idiopathic one for the most part there's a general classical features that you will see in the clinical vignette for all of these and what they may present with is in the vignette they'll say that the patient is coming to the clinic or to the hospital and they're presenting with dyspnea shortness of breath when you examine them you'll listen to their lungs and what you'll listen for is something called by basil or crackle so at the basis of their lungs they're going to have this velcroy type of sound and that's due to having to actually open or popping up those bronchioles and alveoli during inspiration because they're kind of collapsed because of all that fibrosis that's around them so classic features they'll come to the clinic with a dry cough more specifically distena on exam by basilar crackles that's important you may may see from chronic hypoxemia no clubbing but it is not pathognemonic or diagnostic of interstitial lung disease remember that dyspnea by basilar crackles by far the biggest types of things to think about for their history and for the physical exam other things to think about is when you take their vitals you throw that O2 sat on them what is it going to look like it depends upon the severity of their interstitial lung disease if you have a massive amount of fibrosis in the interstitial spaces around the alveoli in between the actual alveolus and the actual pulmonary capillaries lots and lots of fibrosis look at the distance now that this poor little oxygen has to be able to move to go from the alveolus into the blood that's a long distance and whenever you increase the distance whenever you increase the thickness of the respiratory membranes all of this is your respiratory membrane whenever you increase the thickness of that respiratory membrane so we're going to increase the thickness of the respiratory membrane it'll decrease the diffusion of the oxygen and because of that not as much oxygen is going to be able to get into the bloodstream so if you don't have a lot of oxygen inside the bloodstream what is this call whenever O2 is low inside of the bloodstream hypoxemia if hypoxemia May trigger a reflexive response your brain is going to say oh their oxygen is low they must not be ventilating well if I have them breathe faster breathe deeper maybe it'll bring in more air more oxygen and push more into the blood but that's not the case because that respiratory membrane is going to still remain to be thick and so it's going to cause this dyspnea and this tachypnea that you'll see in the patient as well as a low O2 sat on their actual pole socks now they may also have somewhat of a difficulty being able to Exhale CO2 because in the same way CO2 has to move across this thick respiratory membrane as well and so there may also be some degree of hypercapnia as well but I think the big thing to think about is hypoxemia which causes tachypnea dyspnea listen at the bases buy basilar crackles these are the classic features now in the same way with obstructive lung disease this alveoli is fibrotic this one's fibrotic right if we had a good alveoli imagine that there's a third one sticking out over here it's normal it's healthy there's not a lot of fibrosis over it we would have good ventilation going to that one and good perfusion going to this one right now there's good ventilation here and there may be good perfusion but the actual exchange process is the problematic issue so what happens in these patients with interstitial lung disease look at this you have decent ventilation okay ventilation I should actually be very careful because again they're not going to be taking in as much air as usual why because of the fibrosis because they have significant fibrosis they can't take in as much volume of air but on top of that not only is that the problem but they got this thick respiratory membrane so the ability to cross the respiratory membrane is the other problem so that's impaired so because of this what the actual pulmonary vessels do is they're very smart they say I don't want to send blood flow to these poorly ventilated alveoli or these alveoli that aren't going to get any good exchange I want to send blood to other alveoli that are getting well ventilated and will have good exchange and so what happens is they undergo pulmonary artery vasoconstriction if the pulmonary arteries vasoconstrict what happens to the pressure in these pulmonary arteries it increases so you increase What's called the pulmonary artery pressure that is going to cause this right heart to go ape crap and start freaking out because it's going to have to get super thick and become hypertrophic to push all that blood that it can into these actual very constricted vessels and this will eventually lead to right heart failure and we say that whenever right heart failure is due to an underlying lung disease like an interstitial lung disease we call that core pulmonal these remember how these patients would present what would happen if your right heart stopped working blood would go up the superior vena cava cause jvd go down the inferior vena cava cause a padomegaly ascites and pedal edema so look for that as well in the clinical vignette we have a basic idea the general features of these interstitial lung disease but one of the key things for your actual exam is they're going to try to trip you up and say okay which one of these do you think it actually is which one of the do you think it's one of the granulomatous ones do you think it's one of the pneumoconiosis do you think it's maybe like one of the collagen vascular diseases and so they'll present very particular types of clinical features that point to one of those and I want to focus on some of those a little bit now and talk about some of the specific features of these intercessional lung diseases all right so we have a patient who comes in on the clinical video they say okay they want to determine if this patient has interstitial lung disease bro they want to find the actual cause so they may present this in the vignette where the patient comes in they say okay I got some dyspnea they have some bibasler crackles they have some hypoxemia they have some tachypnea they don't really have any evidence of core pulmonal yet but you have a young African-American female history of tuberculosis Lyme's disease possibly and they come in and they have these other additional findings in their vignette really think about these first one take a look particularly on their chest x-ray if you get one you may see very big higher lymph nodes so we're called a bilateral lymphadenopathy the other thing is really take a look at their skin that's a big one so look at the cutaneous findings they may have for the cutaneous findings some kind of like violacious rash within the face we call this lupus pernia this is what it looks like on top of that they may also have these like nodules that can appear within the skin especially on the lower extremities like on The Shins and this is called erythema nodosum this is what this looks like on top of that take a look at their eyes so not only cutaneous findings but ocular findings they may have actually what's called eye pain ocular pain and then sometimes some hyperemia of the eyes and this can be is called anterior uveitis this is what this looks like all right so we've had our patient come in we've taken a look at them we found some cutaneous findings we found some ocular findings the other thing to think about is that you have a patient comes in they're like oh man I got some flank pain abdominal pain I'm peeing up blood what's going on with me Doc the reason that that may be is because they may have something called nephrolithiasis they may have some actual renal calculus some some actual stones and why that's a problem is because they have lots of calcium that's building up there and here's the reason why those granulomas that you see in sarcoid those macrovay just pop out lots of what's called one alpha hydroxylase you guys remember one alpha hydroxylase it's one of those enzymes that helps to be able to convert what's called 25 hydroxycolic calcifera which is the inactive form of vitamin D into the active form of vitamin D so now we got our stimulated form of vitamin D because of the presence of this enzyme if this granuloma is pumping this enzyme out what are we going to do we're going to increase the amount of active vitamin D what does vitamin D do guess what acts on the git increases calcium absorption if we increase calcium absorption across the git now we have hypercalcemia calcium can then go and deposit into different parts of the actual ureter and maybe lead to kidney stones therefore nephrolithiasis and they may even have lots of calcium in their urine as well so hypercalciuria so look for hypercalcemia hypercalciuria nephrolithiasis and increased vitamin D all right the next thing that you want to be thinking about is look for any types of neuropathic findings so sometimes these granulomas can deposit along the length of a very particular nerve called cranial nerve seven the facial nerve when it deposits the lung that it actually decreases the function of the facial nerve and if the facial nerve which is supposed to be involved with particularly like facial expressions is actually diminished what do you think is going to happen one side is going to droop and you got this poor little guy here he's got a drooping face and so there's going to be massive unilateral weakness of the face so look for facial nerve palsy as a potential implication of sarcoid the last one is that there could be lots of these granulomatous infiltrations of the actual bundle system what if I bundle this around the AV node or the bundle branches I can then lead to like an AV node block a bundle branch block and what if I deposited this into The myocardium of the heart now I'm going to deposit this kind of like fibrotic tissue into the heart and now the heart doesn't want to expand and get filled properly and this can lead to restrictive cardiomyopathy so look for these particular findings in a sarcoid patient all right the next thing if you think that maybe the patient doesn't have sarcoidosis you think okay what if they have hypersensitivity pneumonitis well I know it's a granulonus disease I know it's due to some particular occupational exposure such as pigeon droppings such as working with hay such as working with Barley if they present that in their history as a farmer and it usually only occurs rapidly whenever they're exposed to that actual occupational exposure and then guess what you remove them from that and all of a sudden really quickly within hours maybe 12 hours they quickly improve that is a very characteristic sign of hypersensitivity pneumonitis so when they're exposed to these particular triggers they have the exacerbations they have these findings you've removed them they have gradual improvement over hours to potentially days hypersensitive pneumonitis next one you think okay what is a pneumoconiosis maybe it's silicosis asbestosis bariliosis or co-workers obviously think about the actual history were they a sandblaster were they working in mines were they working in foundries that would be representative of silicosis so think about that in their history on top of that guess what else silicos is at high risk of tuberculosis you're like what dog crap how you know why silicosis actually causes macrophages you know whenever macrophage they eat a particular particle it's called phagocytosis they form something called a phagosome and that phagosome is supposed to combine with things called lysosomes and then it makes something called a phagolysosome it inhibits that it inhibits this phagolizosome where you're supposed to be able to release these lysosomes to break down this particular bacteria and guess what silica prevents this phagol lysosome from forming and now you can't break down bacteria that are really hearty and guess what this bacteria is it's really hearty tuberculosis and so because of that these patients have a very high risk of tuberculosis so think about that in a silicosis patient on top of that if you happen to get a chest x-ray look for any really heavy calcifications that almost they look like eggs of the lymph nodes particularly around the hilar lymph nodes all right the next one is asbestosis so in asbestosis obviously the patient will come in they'll present oh yeah I'm a ship Builder I work as a plumber I work as a roofer and I deal with insulation or pipe fitting that's obviously within their history but also guess what these patients are super high risk for and that's something that you need to be thinking about they can have cancer that develops other actual pleural tissue so they can develop mesothelioma even more fearful than that they have a very very high risk even higher risk than mesothelioma of what's called bronchogenic carcinoma so a tumor that can actually develop with inside of the Airways and last but not least the patient also can develop lots of fibrotic plaques particularly where at the bases of the lungs so silicosis borreliosis co-workers lung guess what they particularly prefer to attack the upper parts of the lungs they have an upper lobe predilection guess which is the only one that has a lower low predilection the asbestosis okay so we've got sarcoid hypersensitivity and some of the pneumoconiosis let's now move on to the next component which is some of those collagen vascular diseases all right so the next one that I want you guys think about is okay what if the patient has a collagen vascular disease obviously drugs are pretty easy you just look at the patient's history so think about those drugs in their medical history have they taken any kind of amiodarone any nitrofurantoin any Methotrexate any bleomycin that's obviously things to look at in the history but then when you talk about collagen vascular disease there's maybe particular clinical features that point out to one over the other in Scleroderma there's this classic way that we can remember them via what's called The Crest now this isn't all patients it could be like a limited systemic sclerosis but and these patients you can remember Crest calcinosis so hard calcium deposits of the actual skin it actually is really interesting it looks like this the other one that I want you guys remember sometimes they can have what's called rainouts phenomena so they can have kind of like a vasospasm of the vessels near their actual digits it can cause them to present in different colors it actually looks like this the other thing that I want you to remember so we have cr Crest CR esophageal dismotility for the E so they can actually present with maybe some difficulty in being able to swallow so some dysphagia maybe even some chest pain because of a lot of the actual dismotility there and the next one is sclerodactyly so sclerodactyl is actually a very interesting one it's it's where they actually have like they're they're so much fibrosis of their actual their skin around their fingers that actually kind of turns on to like a claw like look and so this is what sclerodactyl actually actually looks like the last one that I want you guys remember that you can actually have like these vessels on The Superficial aspect of the skin they can actually appear dilated and this is called talangectasia and so I want you to take a look at what this looks like all right so we have an idea now of how Scleroderma patients may present a little bit differently in comparison to others the next one is how do we know about these other ones like granulomatosis of polyangiitis also known as Wagner's eosinophilic polyangiitis also known as churg Strauss and then the last one which is called good pasture syndrome which is a really cool one but granulomatosis are wegeners one of the big things is that pretty much all of these granulomatosis eosinophilic and good pastures all present with glomerulonephritis so they may present with some type of hematuria proteinuria in that sense one of the big differences though is that when patients have Wagners or granulomatosis they present with a chronic Rhino sinusitis so look for that as a potential feature sometimes it can even eat away at the tissue at the nose and cause like a saddle nose deformity the other one is for eosinophilic look for them to have a history of asthma or some type of allergic rhinitis or allergic sinusitis the last thing that I want you to think about is for good pasture syndrome they may also have a glomerulonephritis but look in their history to have history potentially of hemoptysis so coughing up of blood that is the big ways that we can differentiate some of these other type of collagen vascular disease and what about idiopathic we don't have a particular reason and they're going to present classically as though the general features which is the Disney the tachypnea the hypoxemia the bibasler crackles maybe potentially some nail clubbing and again later stages of the disease core pulmonal with jvd hepatomagliositis and pedaledema I hope this really gives us a good understanding of how to separate some of these particular etiologies of interstitial lung disease or diffuse printable lung disease because now we got to move on to how do we diagnose these bad boys all right so now we're going to talk about the diagnosis of restrictive lung disease but one of the most important aspects of this is really understanding pulmonary function tests let's watch this clip that we talked about in obstructive lung diseases going over pulmonary function tests and really help you guys to just recall that remember that and go back over it again let's go and take a look at that all right so first thing we talk about pulmonary function tests we're going to do some things called spirometry and what we're going to try to determine is we're going to look at a couple different parameters when we take a patient who has normal lungs and can comparison to obstructed lungs versus restricted lungs we're going to look at three particular things with the pulmonary function test what I want you to understand is when a patient is breathing okay they have normal healthy lungs when they're breathing normally normal quiet breathing taking a breath in taking a breath out that normal quiet breathing is this purple kind of line that's oscillating here that's called your tidal volume now whenever a patient decides to take a deep breath in beyond their normal tidal volume inhalation so that's called their inspiratory Reserve volume so it's the amount of air that they can forcefully Inspire beyond their tidal volume inhalation that's the Irv the next one is the amount of air that you can forcefully exhale beyond your tidal volume exhalation that would be your Erv so I'm a little dumb here but go [Applause] that amount of air that I blew out beyond my title volume exhalation is called my expiratory Reserve volume the amount of air that remains in my lungs at the end of that forced exhalation is called the residual volume that's the amount of air that kind of keeps the alveoli open and prevents them from collapsing that's important concept and what I'm going to do is is we're going to take and look at this graph and compare it what happens to the Irv the TV the Erv and the residual volume and obstructed and restricted lungs but before we do that we have to talk about one more thing there's another terminology which you guys see here called Force vital capacity and total lung capacity what I want you to remember is that Four's vital capacity fvc is the combination of your inspiratory reserve Volume Plus your tidal volume plus your expiratory reserve volume this is called your forced vital capacity your total lung capacity is your Force vital capacity Plus your residual volume so if I go all the way from here down to my residual volume this is called my total long capacity what I want us to Now understand is what happens to all of these volumes in a patient who has obstructed lungs versus restricted lungs let's take a look here so when a patient has an obstructed lung disorder what is the classical feature whether it's asthma whether it's COPD whether it's bronchiectasis there's a problem being able to get air out so because of that air stays trapped in the lungs they hyper inflate their big old chunky lungs because they're big compliant very large lungs what would we expect we would expect that they're going to have really big chunky lungs now here's what I want you to think about if a patient has really really big lungs imagine how hard it would be to take in to imagine my lungs are already filled here right and I'm going to try to take a deep breath in beyond my normal tidal volume how hard is that going to be to take in a large volume of air when my lungs are already hyperinflated imagine the lungs being already filled almost and you got to try to take in a lot of air it's not going to happen so in these patients they're inspired to a reserve volume the amount of air that they can forcefully inhale behind their tidal volume inhalation is very low so that is reduced in patients who have structure lung diseases because you can't take in a large volume of air because they're already super puffy and inflated their tidal volume actually doesn't become affected though thankfully right so that's relatively normal the amount of air that they can breathe in during normal inhalation and exhalation is the same their expiratory Reserve volume the amount of air that they have available that they could forcefully exhale after the title volume exhalation is insane because look how puffy these Dangs lungs are they're huge and so because of that their expiratory Reserve volume is increased and their residual volume the amount of air that they remain have remaining in the lungs after exhalation is also increased they're air trapping so they have a very high residual volume we sometimes use a very special term here which I want to write down here for a second it's called functional residual capacity so the functional residual capacity FRC is elevated in patients who have obstructive lung diseases okay now let's use this concept of what force vital capacity is and what total lung capacity is then right and patients of obstructive lungs their lungs are hyperinflated so the amount of air that they have available to be able to Exhale is large the amount of air that they have sitting in their lungs after they exhale forcefully exhale is large so their functional residual capacity is large the amount of air that they can try to bring in forcefully is very little because their lungs are already hyperinflated think about how much air their lungs can occupy at an entire time so total lung capacity is the Irv plus the TV plus the Erv plus the RV in this situation my RV is massively increased my Erv has increased my title volume stays the same I inspired to reserve volume will reduce a little bit if that's the case this is actually going to cause the lungs to have a very very large total lung capacity the total lung capacity for obstructive lungs is increased significantly okay and that is in part due to these two factors the Erv and the RV significantly increase just to put it in perspective let's give it a double arrow let's give this a double arrow here and let's give this a double arrow here okay and therefore the functional residual capacity is going to increase okay The Four's vital capacity is the Irv plus the TV plus the Erv well the Erv is increased the Irv decreases so the FEC May decrease but it almost may be normal so sometimes in these patients it can vary from where it can actually be relatively normal to mildly decreased it can be normal to mildly decreased usually in the early stages of obstructive lung disease it's relatively normal as they get later and later and later into the disease their fvc starts to decrease so that's what I want you guys to remember the FBC may be relatively normal in the early stages and decrease as we get to the later stages okay that's this component here for obstructive lungs now let's go to the restrictive lungs for the restrictive lungs look at the size oh my gosh big old suckers baby suckers right and restrictive lung diseases we haven't talked about this yet but in their situation they're very very fibrotic they're super super kind of like elastic and they just don't want to be able to expand and they just want to collapse naturally they just don't want to be able to bring air in and they collapse really easily so it's really hard to fill these lungs and they don't have a lot of air in them they're very very tiny so because of that imagine this how hard is it going to be able to bring air into these stiff non-compliant lungs very difficult so being able to forcefully inhale a ton of air do you think I'll be doing a good job at that no so these patients Irv is reduced tidal volume doesn't really change thank goodness that's relatively straightforward the amount of air that they can bring in normally during quiet breathing and out during quiet breathing is the same they're Erv look how little their lungs are they don't have a lot of air that are available for them to Exhale so because of that their Erv is reduced and then the residual volume the amount of air that they'll have remaining in their lungs at the end of a forced exhalation is also really little so there's a residual volume will be reduced and the combination of these two which is called r forced our functional residual capacity will be reduced if we take all of this into consideration and look now at the total on capacity which is all of these things added up there's a lot of down arrows my friends so what do I expect to happen to the total loan capacity if I added all of these up I expect it to be low so I'm going to have a very low total loan capacity Four's vital capacity is the Irv plus the TV plus the Erv two down arrows and one it's kind of normal I expect the actual FEC to be low and so in this situation I want you to remember that the fvc is really this is why I want you to stress this fvc is really low in patients with restrictive lung diseases in obstructive lung disease it can be normal to slightly decreased okay that's one of the big features to understand here now we've had a basic concept of these numbers whenever we're having a patient breathe into what's called a spirometer so basically all of these things we're taking a patient as a normal lungs obstructive lungs restrictive lungs having them breathe into the spirometer apparatus and then give us all of these particular things and help us to be able to kind of determine based upon these volumes where they kind of fall within the obstructive versus restrictive versus normal lungs we can further extrapolate this and really what happens when we do these spirometer devices we actually get these things called flow volume loops so what this is going to show us here is on the x-axis here we're going to have volume on the y-axis we're going to look at the flow of air on the top part it's going to be expiration on the bottom part it's going to be inspiration okay and then here's zero liters 2 liters four liters 6 liters eight liters so when a patient takes a breath in normal lungs they're going to inhale when they inhale they're going to potentially inhale beyond what they already have in their lungs so here this patient's going to start taking in air and it's going to go from two liters during their inspiratory process to about seven liters during their inspiratory process then during expiration they're going to have a normal flow outwards and then again that will start to decrease and take them back to their original volume okay now what this gives us is kind of a couple different things from this point here from the moment of inspiration all the way here expiration all the way here this is going to continue think about this I take a deep breath in Beyond my tidal volume inhalation this would be my Irv and then I take and I breathe out as much air as I forcefully can during exhalation this would be my Erv and then the volumes that are actually just going to be during the normal quiet breathing process my tidal volume so my tidal volume plus my Irv plus my Erv is my Force vital capacity right Irv plus TV plus Erb is force Auto capacity so from this point here to this point here is what's called my Force vital capacity the amount of air that remains in the lungs after I've exhaled all the air that I possibly can is what my residual volume and then if I were to take the residual Volume Plus the force vital capacity what would this be this would be my total lung capacity this entire thing here this is for normal lungs so what I want us to do now is imagine this is what the normal flow volume Loop will look like in normal healthy lungs if I have a patient who has obstructive lungs what do I know what do I know about these particular parameters and a patient knows obstructed lungs well they're hyperinflated if they're hyperinflated they're going to have a high residual volume we know that if they have a high residual volume what's that going to do what we know is an increase in residual Volume Plus an increase in their expiratory Reserve Volume Plus a normal title volume and a mild decrease in their Irv causes their total lung capacity to increase and we said that their Force vital capacity which is their Irv plus their TRV plus their Erv can do what it can be somewhat normal to slightly decreased so if I were to kind of do this it would be here to here okay so somewhere between normal to slightly decrease what would that look like in comparison to the normal graph which is here in Black well now I would expect my Force vital capacity to be what potentially the same but I would expect my residual volume to be increased so what I'm going to do is look where my graph starts here now I'm going to take a deep breath in beyond my normal title volume inhalation and then I'm going to Exhale as much as I possibly can during exhalation beyond the title volume exhalation so this is going to be my entire force vital capacity so from here to here is my fourth vital capacity if I think about that one in comparison to that one it might be normal it's almost slightly decreased in comparison to normal but here's the big thing the amount of air that remains in the lungs after that forced exhalation is called what as a residual volume so my residual volume is what in this in this diagram it's super increased because my residual volume increases it shifts the actual curve of this graph to the left that's an important thing to be able to remember so when you're given a graph on your exam and comparing to say which one of these do you think this lung disease is based upon the flow volume Loop is it obstructive or restrictive if the graph is shifted to the left in comparison to the normal lungs it's an obstructive lung disease and in these situations due to a massive increase in their residual volume and what will happen to their total loan capacity look at this look at this son of a gun it's gargantuous they have a massive total lung capacity that's increased and on top of that you know what else is really cool about these patients look for this as well sometimes and these patients who have obstructive lung disease they have this little like coving that you can see sometimes within their graph and that's because they have a kind of a reduction in their Peak expiratory flow rate and so sometimes you'll see this kind of reduction this little coving in thing and that's an important thing to be able to differentiate between obstructive and restrictive so if you see the curve shifted to the left with a very massive increase in residual volume and an increase in total lung capacity you're looking at obstructive lung disease now compare that to restrictive for restrictive here's again our normal and black and then blue we're going to talk about restrictive in this situation what do we know about their volumes we know the residual volume is decreased we know that all of these are decreased so therefore their total lung capacities decrease and we know their Irv and their Erv is decreased and the tidal volume stays the same but since both of these are decreased we see their Force vital capacity decreases okay look at their curve if I take a deep breath in with this blue line here a deep breath in Beyond normal title volume inhalation and then I breathe out as much as air as I can forcefully Beyond title volume exhalation then from here to here from this point here to this point here is my Force vital capacity so from here to here this is my fourth vital capacity look how small that son of a gun is in comparison to obstructive versus normal that's a tiny Force vital capacity and then look at this look how much like I might even overshot it I might have to kind of go like right here from here to here is their residual volume the amount of air that remains in their lungs after a force exhalation look at that that's their tiny little residual volume and then look if I take the force ride of capacity in the residual volume combine them this is the total lung capacity look how tiny it is so and a patient who has restrictive lung disease if they show you the graph what will you see the curve do you'll see that the restrictive Curve will shift to the right so restrictive the curve shifts to the right obstructive the curve shifts to the left and maybe has a little coving in of the expiratory Curve that's what I want you to remember the last thing that we have to talk about here when you look at these graphs the next thing that we can do is we can take and look at whenever they're forcefully taking in air and pushing out air what we can do is we can look at the very specifically the volume of air that they are exhaling so they take a deep breath in from the moment I begin exhaling until the moment that I stop exhaling I'm going to monitor that volume and that's going to be on the y-axis on the x-axis is going to be the time that it takes for me to start exhaling until I stop exhaling what I want to know is what is the maximum volume of air that I can actually exhale during that entire breathing process the maximum amount of air which is at the peak point of the Y so on the y-axis wherever I hit the top the peak point that is my Force vital capacity that would be this point here okay at one second let's say that somehow I breathe for I don't know five seconds okay so during that five second period that was the maximum volume that I could actually reach that was my Force vital capacity at one second in the inspiration expiration process I want to know how much air I was able to forcefully exhale we call this the forced expiratory volume of one second what you need to be able to take from this my friends is this ratio where we actually say fev one over the fvc this is a particular ratio that we're going to use in this example let's say that the amount of air that I could forcefully exhale in the first second was four liters then that's going to be four liters divided by the maximum amount of air that I could forcefully exhale throughout the entire time period let's say that that kind of curve goes around 5 liters-ish so five liters 4 divided by 5 is going to be about 0.8 multiply that by a hundred and I'm going to get 80 percent that's about what the normal ratio is between fev1 and fvc what I need to be able to determine now is what would that look like in an obstructive and what would that look like in a restrictive lung disease let's take a look in obstructive lung disease what we notice here is in this red curve here is that the fvc can be reduced there could be a reduction in FEC we said that it could be anywhere from normal to decreased okay so we see that that is potentially reduced a little bit so let's actually put a down arrow for the FEC the fev-1 though that's the worst situation here look at their fev-1 in comparison to this one so here's their fev-1 let's just kind of I'm going to make up numbers I'm going to say that in the normal patient it was about four liters and this patient oh my gosh this is terrible actually this might be somewhere around like two so now this patient's fev1 is approximately somewhere around two liters what in the heck happened I dropped there fev one significantly so when patients who have obstructive lung diseases their Force expiratory volume and one second is significantly reduced whereas their fvc can be just normal to almost slightly reduced if that's the case and I take into consideration this ratio what will I get in comparison to 80 if a normal fev1 to FEC ratio was four over five liters which was eighty percent and I went from Two and I would drop my fvc down let's just say to four liters I'm definitely going to be less than 80 if that was the norm if the norm was 80 you can tell me that 2 liters and four liters what would that give me that would give me like 50 percent so my fev one over fvc ratio was definitely less than 80 percent that's what we see in obstructive lung diseases important to remember that very reduced fev1 and mildly reduced or normal fvc all right so now let's talk about restrictive now it's very interesting here right so again we're going to take the normal curve here that's their fev one at one second that's their Force vital capacity right for the normal patient now here's the patient who has a restricted lungs look what happens to their fuv1 it drops but not a crazy amount right in comparison to the patient who's the norm one or in comparison look at this look at the fev one here for the restricted and the obstructed lungs massive difference right so restrictive not a crazy reduction in fev1 we'll just give it like one arrow down okay their fvc though does actually reduce and so in comparison here was about five liters if I'm kind of like this is about four liters because that was their fev one there was about four liters here their FEC is about five liters I'm gonna say it's like somewhere around like three three and a half all right so I'm just kind of extrapolating so their fvc definitely dropped maybe like about 3.5 liters and their fev1 it's just mildly dropping even a little bit below that one so let's just like extrapolate that to about three liters if that's the case their fvc definitely was the one that dropped remember what did I tell you was the big big Factory here for the restrictive lung disease is what happens with their fvc drops pretty significantly so what I'm going to do is I'm going to drop my fvc by two arrows and obstructive I dropped my fev-1 by these multiple arrows so now if I were to just kind of extrapolate here that I had 3 divided by 3.5 that's definitely going to be greater than 80 percent or at least about same close to it so maybe about like 80 86 percent so in that situation here what do I notice about my ratio if I look here and my numerator is really small but my denominator is even really small what happens to the overall number it's going to be large because my denominator is really small and so in this situation theirs will be greater than or equal to 80 percent so in patients who have normal lungs what was theirs about 80 percent and the patient has a restrictive lung disease there's about greater than or equal to 80 percent but for a patient who has obstructive lung diseases theirs is less than 80 percent very important to be able to remember that and the reason why theirs is less than 80 is because they're fev one at one second is significantly reduced and their FBC is only mildly reduced or almost normal and the patient was restrictive lung disease they have a mild decrease in their fev one at one second but their FBC is the one that really drops so their denominator drops even though both of them drop their denominator drops even more which allows for their overall number to still be high at least greater than or equal to 80 percent that's what I need you to remember about normal versus obstructive versus restrictive when it comes to the spirometry to put this in a quick little algorithm let's come down and talk about that all right so when we compare obstructive versus restrictive we're looking at this ratio that we just talked about the fev at one second over the FEC for a patient was obstructive what did we just say their fev1 is very very low we know that so because they have a very low fev1 and a really mildly decreased or almost normal fvc what do we expect the ratio to be less than 80 percent so because of that they'll be less than 80 percent they'll have a decreased ratio and a patient who has restrictive lung disease which is the primary problematic issue here which one is it fev1 or FEC the fvc is the primary one that's actually reduced if the denominator is small and the numerator is only mildly small what's going to happen to the overall number it's going to be large so theirs will be greater than or at least equal to 80 percent that is the big differences so when you're looking at a patient and you're given here's their fev1 here's their FPC determine their ratio if it's less than 80 percent this is likely obstructive and again the primary problem with this is which one of those actual variables the fev of one second versus restrictive their primary problematic issue that causes them to be greater than or equal to 80 is they have a very low fvc all right great now that we've talked about that there's a couple other things that we can do all right so we've gone over pfts now now the next thing we have to understand for restrictive lung diseases is the dlco that diffusion capacity of carbon monoxide it's really important and one of the reason why is it really helps you to differentiate is this an intrinsic or you know an actual pulmonary interstitial lung disease diffuse parinkum and lung disease or is it an extrinsic an extra pulmonary cause meaning is it a pleural effusion pneumothorax neuromuscular disorder like Myasthenia gambarre dermatomyositis polymyositis the scoliosis obesity or Ang spawn if it's one of those it will be able to be able to have somewhat of differentiation between these now one of the ways we can do this is looking at the dlco now with dlco it obviously if you guys remember the formula that we talked about in obstructive lung disease it's directly proportional to the total surface area this is a coefficient and then the pressure gradient and then it's inversely proportional to the thickness of the respiratory membrane what is the classical feature of interstitial lung diseases or diffuse prank of lung diseases they have a increased thickness of their respiratory membrane so what does it do to the dlco it decreases the dlco so for intrinsic disease they have a decreased DL Co but for extrinsic has nothing to do with the respiratory membrane there's no effect there's no change in the surface area there's no change in the pressure getting there's no change in the thickness at all with respect to the extrinsic because it's a problem with the pleural tissue it's a problem with the actual bones or the neuromuscular Junction and that situation there is no effect on dlco so therefore it should be normal so a normal dlco would be characteristic of an extrinsic cause this is very helpful whenever you're trying to be able to differentiate and weed out between what we talked about is intrinsic versus the extrinsic cause all right after we've done a dlco the next thing that I want you guys to understand is we can actually take a look at a lot of images there's a lot of different Imaging that we can actually do to help us to differentiate interstitial lung disease not just being able to diagnose them but also maybe specific interstitial lung diseases have a predilection for particular parts of the lungs remember I told you let's see if you guys remember for the upper lobe it's mainly going to be silicosis berylliosis and co-workers pneumoconiosis for the bases of the lungs where it caused lots of fibrosis it would be where it'd be specifically asbestosis and remember the bilateral mediastinal lymphadenopathy is very I mean bilateral Hyler the phenopathy is very common in sarcoidosis we said again for silicos they have a lot of the eggshell calcifications of their lymph nodes so again some of those classic features are very important and we're talking about these so let's take a look at some images that are very characteristic and important in patients with interstitial lung diseases all right guys let's take a look here at some images we have a patient um or particularly a chest x-ray of patients some type of under underlying restrictive lung disease now what I told you guys in patients who have restrictive lung disease it's a very characteristic type of pattern there may be some mild differentiation that we talked about between upper lobe predominance lower lobe predominance and maybe some things like hyalur adenopathy and eggshell classic calcified lymph nodes all those things that we talked about with specific characteristics but the general classification of patients who have interstitial lung disease they look like this if you look here they have all of these like reticular types of opacity so white opacified types of lines that are moving from the central part out towards the periphery and then sometimes you may even notice like a nodularity to it so we call this reticular not reticulo nodular opacifications and this is one of the very obvious very common findings of patients who have interstitial lung disease sometimes they also give this what's called a ground glass appearance but you have to be careful nowadays because obviously we take ground glass and immediately say covet but again this is a very common and finding that you'll see an interstitial lung disease that reticular nodular opacification or ground glass or pacification on chest x-ray very classic of interstitial lung disease let's take a look at another image all right so here's another really interesting image we have a CT scan so right Hemi diaphragm left hemidiaphragm here's the heart here's the vertebrae now what you see here is we see this thing this is a honeycomb type of sign we see this very commonly in patients who have interstitial lung disease very characteristics of something called what we called about idiopathic pulmonary fibrosis so what you can potentially see as we get towards the periphery of the lungs if you really look is you'll see somewhat of a honeycomb type of appearance here in the periphery in some of these areas you may see this honeycomb type of appearance very common very characteristics of interstitial lung disease but more specifically if you see this on a clinical vignette you see honeycombing of their chest CT think about idiopathic pulmonary fibrosis it's definitely going to be one of those pathonic terms to remember but again honeycombing definitely very common due to a lot of fibrosis thickening of the interstitium and again this is basically going to kind of give that honeycomb type of appearance all right that covers this one one more image all right so the last image here is very interesting so we can see some of the particular findings that we've already discussed so again right Hemi diaphragm left Hemi diaphragm if you look towards the edges you do see again more of that honey honeycombing type of look which is very classic and you know patients who have what's called interstitial lung disease especially idiopathic pulmonary fibrosis but one more finding that you want to be potentially aware of especially in interstitial lung diseases and sometimes do that excessive fibrosis it can create these like potentially like dilate what causes what at least to what's called traction bronchiectasis which can really open up some of these bronchials so here you see some traction bronchiectasis and here you see some traction bronchiectasis this is not the same thing as the bronchiectasis that we saw in obstructive lung disease that's due to a lot of that mucous secretions this is usually seen secondary to interstitial lung diseases so remember that so you'll see the honeycombing type of look and you may see this traction bronchiectasis as well and interstitial lung diseases all right that covers the images let's get back to the Whiteboard and talk more about some Diagnostics so we've taken a look at that the next thing that I want you guys to understand is okay we have a pretty good idea that this patient has restrictive lung disease based upon their pfts we are able to differentiate if it was an extrinsic versus an intrinsic type of restrictive lung disease off the dlco we're able to get a good idea of what maybe in an interstitial lung disease off a chest x-ray or a CT scan and maybe even help us to differentiate which types of pneumoconiosis it could be the next thing is what if I think it's an actual granulominus disease so I look at their chest x-ray their CT and I see a little bilateral hylar lymphadenopathia see some of the classical features how do I determine if it's sarcoid well remember some of those things that we said that can happen because of the granuloma releasing lots of what's called one alpha hydroxylase what did that do remember it increased your one alpha hydroxylase which led to an increase in vitamin D so what I expect an increase in I would expect an increase in the vitamin D levels within the blood the other thing I would see is that basically Whenever there was an increase in the vitamin D that increase the amounts of calcium absorption so calcium levels within the blood would be elevated and on top of that we didn't mention this one but this one's really actually pretty cool granulomas these granulomas can actually release lots of what's called Angiotensin converting enzyme which is a very odd thing but it actually will have very increased or elevated Ace levels so these are things to think about for sarcoidosis check the calcium check the vitamin D and check the ace levels the other thing is that we can go down and do a bronchoscopy and lavage out some of the actual pulmonary fluids and if this is a patient who has sarcoid they're going to have lots of lymphocytes and it's very important to be able to kind of determine the predominance of which type of lymphocyte they have so what we do is we take and we determine how many CD4 lymphocytes they have and how many CDA lymphocytes they have and if the ratio is really particularly an increased CD4 to cd8 ratio whenever we do that bronchial alveolar lavage that's very characteristic of sarcoid the other thing is we can actually biopsy an area that we see so maybe we see this area of fibrosis or granulomatous disease we biopsy that and guess what we'll find non-caseating granulomas what other disease if we biopsied it and a patient who's maybe exposed to hay or barley or pigeon droppings will also have non-caseating granulomas hypersensitivity pneumonitis think about that as well all right beautiful for silicosis if I think a patient has silicosis I look at their chest x-ray or their CT scan I see a lot of eggshell calcifications of their lymph nodes I know their history I also potentially have a very strong suspicion that this is silicosis what could I do I could actually biopsy those lymph nodes and guess what I'll find a lot of silica crystals and we call that birefringement silica crystals that will pick up off that lymph node biopsy lots of silica gets deposited there and creates these immune reactions that leads to the egg cell eggshell calcifications of the lymph nodes so we'll biopsy that the other thing is going to be moving on to the other aspects of the pneumoconiosis so now we're going to talk about asbestosis borreliosis and then we'll talk more about some collagen vascular diseases all right so another thing is what do we think this patient is asbestosis so they have mesothelioma broncogen and carcinoma they've got some plural plaques with the bases of their lungs right we look at their history they have some roof building some Plumbing some you know some Roofing insulation shipbuilding things of that nature we're definitely concerned that they have asbestosis what is another way that we can actually confirm this well we can potentially go ahead and take a sputum sample and from that sputum sample we may be able to find a lot of like these asbestosis asbestos molecules and when we look at them under the microscope this is a key term that you may actually see on the exam is when they look at the sputum sample under the microscope they see these like dumbbell appearing things called ferruginus bodies so faruginus bodies is an important term to be able to kind of correlate with asbestosis is a potential cause of the interstitial lung disease the next thing is berylliosis so how do I know about this one again more upper lobe predilection history physical exam is the big things but for this patient what I could do is I could also do a bronchial alveolar lavage take some of their pulmonary fluid from that pulmonary fluid I'll pull out some lymphocytes I'm going to put that into like a little dish and then what I'm going to do is I'm going to expose that dish of pulmonary fluid and lymphocytes to beryllium if in that situation the lymphocytes start increasing in number and populating and populating populating this is an abnormal finding and consistent with so we call this kind of a beryllium challenge test a prillium lymphocyte proliferation test is really what it is we take bronchial fluid with lymphocytes exposed to beryllium and if that in exposure to beryllium those lymphocytes cars are proliferating like crazy it is a positive brilliant lymphocyte proliferation test it's potentially suggestive of berliosis all right we're almost there now we have to some degree based upon their physical exam findings or their clinical features their history this patients maybe all of them have glomerulonephritis this one has the crest syndrome this one has what asthma allergic rhinitis this one has a particularly a chronic sinusitis this one has hemoptysis in combination with a lot of the other things if this happens what we can do is we can send off a panel of like these antibodies and if I think that it's Scleroderma what would it come back as positive anti scl70 or positive anti centromere antibodies if I sent it off and I was concerned that they have Wagner's what would it come back as a positive see anchor it'd be positive C anchor antibodies if I was concerned that it was some type of church strouse or eosinophilic polynergy to come back as a positive P angle we're just testing you guys knowledge here and then lastly if I thought it was good pasture syndrome it would come back positive as anti GBM antibodies so these are the different things that I can go ahead and test for when I'm potentially concerned that they may have one of these types of collagen vascular diseases also look at their medical List look at the potential drugs that they've had if there's any of those exposures of drugs that would also lead you to particularly which type of etiology was responsible for this interstitial lung disease all right my friends on to the treatment all right so last but not least the treatment of restrictive lung disease be thankful there's not a lot because unfortunately these diseases sometimes they're the damage has already been done it's too late to reverse it or to be honest with you it's really just this top part here treat the underlying cause or just remove the actual trigger so if the patient is exposed to very particular environmental agents like pigeon droppings or hay or barley and that's their trigger get rid of it if it's exposure to an occupational like a type of dust again an organic dust like silica or beryllium or asbestos or if it's a co-worker is kind of like a coal mine or carbon mines get rid of those things avoid those things if it's a drug discontinue it stay away from that drug if it's a collagen vasculinity it's a little bit different you can't unfortunately just remove those things but you can try to treat the underlying disease and again if it's idiopathic we don't really know why so unfortunately there's again nothing that we can do for that but if you can try to treat the underlying disease or remove the particular trigger that would be the best situation oftentimes though it's just supportive care so if the patient already has this interstitial lung disease the damage has already been done we're just trying to be able to help them to have a better quality of life and the way that we can do that is kind of perform pulmonary Rehabilitation get the lungs strong man give them particular ways that we can kind of push their ability to work those lungs and get them a little bit stronger and have a better conditioning the other thing is if the patient has underlying hypoxemia guess what we can do give them oxygen do you guys remember what it was for obstructive let's see if I can test your knowledge but the basic one is if it was at rest exercise anything like that they didn't have an underlying core pulmonal or polycythemia what was the spo2 goal less than 88 percent and then if we checked the pao2 Via ABG it was less than 55 millimeters of mercury but if we had the patient who had corpulmonal or polycythemia what could we do we could check and say oh we'll be a little bit more acceptable at less than 90 spo2 or a po2 of less than 60 millimeters Mercury would be more accepting of so that's an important thing to think about for oxygen supplementation in these patients and last but not least you know what else is really actually kind of beneficial avoiding particular infections these patients lungs are already damaged you can actually cause worsening damage if you have them become exposed to infections and one of the most common infections that you want to be able to prevent these patients from having is the common ones so streptococcus pneumonia so having these patients get their pneumococcal vaccine is going to be very very critical and then on top of that having them get their influenza vaccine because we know that influenza is another type of viral agent that can definitely cause injury to the lungs and especially an elderly increase their exposure or risk of staphylococcus aureus pneumonia so good infection control is also going to be key in these patients the next thing to think about is what if I can't treat the underlying cause I can't remove the exposure or get rid of the trigger I provide supportive care actively but a patient has an inflammatory cause like a collagen vascular disease or they haven't really gotten better with supportive care and removing the underlying trigger such as in a granulomatous disease like hypersensitivity pneumonitis or sarcoidosis in those situations we can consider steroids or immunosuppressants so the only time that we'll really use these is to prevent the further damage of the lungs the further fibrosis we can't repair or remove whatever happened and in those situations you could consider this in sarcoid or hypersensitivity pneumonitis to really shut down that auto remember the again when the immune system's Super Active it's releasing cytokines activating neutrophils activating lymphocytes coming to the area propagating more inflammation activating fibroblasts to cause fibrosis if I suppress the inflammation I can potentially suppress further lung injury and further fibrosis so I could use this in granulomatous diseases or where they're going to continue to have attack by their immune system by situations such as Scleroderma GPA EPA and good pasture syndrome so think about that we can use these drugs there's one other potential thing that we could do in the same way that we could shut down inflammation preventing further lung injury and further fibrosis what if we just shot down the fibroblasts in a disease like idiopathic pulmonary fibrosis we don't know what these patients underlying cause is so we have a patient who's idiopathic pulmonary fibrosis we think it could be due to smoking so obviously telling them to stop smoking would be great giving them supportive care like pulmonary rehab oxygen and if need be infection control they are not going to benefit from a reduction in inflammation because that's not their problem we don't know what their problem is we can consider anti-fibrotics we can shut down the fibroblasts and say hey fibroblasts no more fibrosis so we have to prevent the further progression of the disease the further degree of fibrosis that's all it's going to do preventing the progression we can consider things like profenadone and nintendib which is going to inhibit the fibroblasts from causing further fibrosis unfortunately especially in idiopathic pulmonary fibrosis we've tried everything we treat the underlying disease or try to reverse it we provide supportive care to these patients with pulmonary rehab oxygen and infection control if they do qualify for steroids or immunosuppressants we try that if they're an idiopathic pulmonary fibrosis we can try antifrabiotic agents but oftentimes these patients lungs are so damaged that we have to unfortunately do a lung transplant and that would be the last line of treatment for these patients and probably the best treatment for these patients with restrictive lung diseases ninjas in this video we talked a lot about restrictive lung disease but guess what we're not done we had to do some cases and really put to practice what we learned so let's go and do that now so we're back we're going to start doing some more cases now we're going to talk about restrictive lung diseases all right so first things first we got case study number one we had a 35 year old uh African-American female presented an engineering Clinic with dyspnea and dry cough past medical history is pertinent for Lyme's disease now when we look at the physical exam of this patient very very important we have bibasler crackles which is the classic features General features of interstitial lung diseases nodules particularly on The Shins we also appreciate a violacious kind of facial rash ocular hyperemia and right facial weakness very interesting here so we have a patient who's young middle-aged African-American female what did I tell you was a very important disease that was associated with this is one of the granulomatous diseases it's a sarcoidosis plus it was very common in patients who had an underlying history of tuberculosis exposure or a Lyme's disease exposure she has limes so she's fitting a lot of the epidemiological kind of features of that she has some of the general features like buy basilar crackles she has erythema nodozum so the nodules on the shin she has the lupus pernia which is the violation's facial rash she has ocular hyperemia likely indicative of anterior uvitis and she has right facial weakness which could be ridiculative of the seventh nerve palsy so very very interesting types of features here of sarcoidosis it seems so 92 arrests so not too high not really hypoxic respiratory rates normal heart rate just to actually touch low here so 36 beats per minute that's actually a touch low and then blood pressure is relatively normal so very interesting what could be the cause of this low heart rate it could be an AV block right it could have been from the past medical strip lime she was actually treated for it but it also could be the sarcoidosis depositing within the AV node or bundle system that causes an AV no block so very very interesting right so likely maybe block and that was one of the things where it can cause restrictive cardiomyopathy or it can cause a b no Glock so which one of the restrictive lung diseases do you suspect I am leaning more towards the sarcoidosis in this situation what labs should you check for this patient remember that granuloma makes high levels of Ace so check Ace levels and they're also going to have a lot of that Alpha One hydroxylase enzyme that's going to help to activate vitamin D and then really help to amp up the calcium level so you'll see hypercalcemia high vitamin D levels and increased Ace levels on these patients so how else would you go about diagnosing these diagnosing these patients well first thing do the pfts support more of a restrictive lung disease case so what do we look for we look at the fev1 slightly low but the FEC is significantly low so when we put this into the ratio the bottom number is low increases the overall number so it's going to be greater than or equal to 70 percent uh respiratory I'm sorry residual volume until loan capacity the slungs are really small they don't want to expand they don't want to bring air in and so they're very tiny lungs so decrease to lung capacity and decrease residual volume more supportive of restrictive lung disease check the dlco remember in these patients if it's very thick respiratory membranes there's some type of problem with the respiratory membrane itself due to fibrosis or granulomas or some type of parenchymal lung disease of its nature it's intrinsic that's more suggestive of an interstitial lung disease but if it's normal it's more likely nothing to do with the intrinsic or parenchymal lung disease it's something to do with the chest wall something to do with the muscles it's something to do with the nerves that are supplying the muscles so that would be situations of the pleural disorders like a massive pleural effusion or pneumothorax it'd be situations where there's some type of uh neuromuscular Junction type of problem so Guillain-Barre syndrome myasthenia gravis or like myopathies or some type of like severe weakness of the muscles it would be something that's actually due to like the chest wall deformity so Ankylosing Spondylitis scoliosis or maybe even some type of severe obesity so in this situation it's low more suggestive of an intrinsic type of pericable lung disease and that comes down to granulomas it comes down to pneumoconiosis it comes down to collagen vascular disease it comes down to drugs and then again from there we can also say it could be idiopathic we don't really know the cause so with this patient I think we do and we're suggesting it's a granulomatous cause such as sarcoid one of the things that we would do is get a chest x-ray or CT scan to the chest and it would really give us a good look at that bilateral Hyler lymphadenopathy they would also have that kind of like reticular nodular opacities maybe within the lung tissue itself so you would get this classic type of uh in interstitial lung disease presence where they have these kind of like interstitial reticular nodular types of opacities moving from the central to the periphery this is a very classic interstitial lung disease look if you were to get the patient's um CT scan again you would find more of that interstitial lung disease type of look very nasty kind of ground glass opacities that you would appear okay very very common of interstitial lung disease and again the reticular nodular opacities the other thing is that you could see honeycombings is pretty common in interstitial lung disease but very very suggestive within uh idiopathic pulmonary fibrosis so you can see kind of like this like little honeycomb look that you see here on the edges all right all right so if you did what's called a um a Bronco alveolar lavage and you took a biopsy particularly of areas near the lymph nodes or near the actual printable lung tissue and you checked it out and you're looking at the lymphocytes remember those non-caseine and granulomas are going to pop up so you would pick up non-case seed and granulomas if you did do a biopsy but on top of that if you lavaged out some tissue it'd be rich in lymphocytes and so you have a very high CD4 cd8 ratio for these patients okay and non-case eating granuloma so definitely supportive of sarcoidosis so I think the big thing is how you treat these patients is usually supportive you can kind of you know consider just steroids at some point in time if they need to be on D Mars you can also consider that but I think the other big thing to think about is that there's one particular thing to think about with treating interstitial lung diseases and you know there's a idiopathic pulmonary fibrosis in that situation you don't really know the cause it's suspicious that it could be due to increasing age or could be due to smoking but and these patients you don't really have a cause so what you want to try to do is slap that fibrosis of the lungs and so in these situations you can give them drugs like profession or nintendib and this would be things like idiopathic pulmonary fibrosis all right so what I want to do is instead of doing through cases I want to really help to test your knowledge and see if you guys remember this because this is a key thing that you're going to see on your exam that'll pick about which type of interstitial lung disease it is so let's save some time and help you guys to remember so if I have some exposure and I'm working in an industry that has sand blasting mines or foundries like metal factories which one am I most susceptible to co-workers as best as silicosis berylliosis or hypersensitivity it's likely going to be silicosis okay if I have some type of exposure for ship building Plumbing or Roofing which one would it be asbestosis whoops and then if I have some type of work in Aerospace industry electronic Ceramics tool dies and Manufacturing it would be berylliosis and then the last one if I'm working in Kohler carbon lines it would be co-workers pneumoconiosis and if I'm working as a farmer maybe and I'm exposed to Pigeon droppings um spores from barley or hay what would it be hypersensitivity pneumonitis so remember these and be able to match those up with the patient's history the other thing is important to remember is which lobes are likely involved in these patients who have these pneumoconiosis so if I had the first one which would say like co-workers that always involves the upper lobes and then you have which one asbestosis involves the lower lobes more of those pleural plaques at the bases in mesothelioma and then silicosis and broliosis should involve the upper lobes as well which kind of complications or organ involvement is also really really key here that'll help you to pick out the interstitial lung disease right so we we talked about some of those especially with sarcoidosis but let's go back and see if we can figure out which organs or complications they may present in the clinical vignette that would Point towards which one of the interstitial lung disease causes it would be we already know the exposures for the pneumoconiosis that's key but what else would be important that would help us so first thing with uh we're looking at all of these and testing these out if I have a patient who's very high risk for TB or they have calcified lymph nodes on their biopsy this would be more suggestive of remember that eggshell lymph node calcification silicosis if I have a patient who has a risk of music has mesothelioma bronchodic carcinoma and they have these by uh basilar pleural plaques with ferruginous bodies in their sputum it would be more suggestive of asbestosis if I have a patient who has calcinosis of the skin sclerodactically talangectasia raynance phenomenon it's more suggestive of Scleroderma if I have a patient who has Rhino sinusitis they have glomerulonephritis they have sea ink antibodies this is more suggestive of Wagner's or granulomatosis or polyangiitis if I have a patient who has asthma allergic rhinitis glomerulonephritis and Panka antibodies that's more suggestive of EPA eosinophilic polygangiitis and last but not least if I have a patient with hemoptysis glomerular nephritis and anti-gbm antibodies it's more suggestive of good pasture syndrome so think about those especially if they present that in the clinical vignette all right my friends that covers restrictive lung disease I hope it made sense I hope that you guys enjoyed it as always until next time foreign [Music] thank you

Channel: Ninja Nerd

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Id: v645-pg9DB4

Length: 86min 2sec (5162 seconds)

Published: Tue Apr 25 2023