Cram The Pance - S1E33 Restrictive Pulmonary Diseases

Episode Date: August 8, 2021

Restrictive Pulmonary Diseases review for your Pance, Panre, and Eor's. ►Paypal Donation Link: https://bit.ly/3dxmTql  (Thank you!)--- Support this podcast: https://anchor.fm/scott--shapiro/supportBecome a supporter of this podcast: https://www.spreaker.com/podcast/cram-the-pance--5520744/support.

All right, so we're going to do a quick review of restrictive pulmonary diseases. As always, I'm going to say it. I know it's repetitive, but thank you so much for the really nice comments you guys are leaving. It really does make my day, and I appreciate it so much. If you haven't checked out the YouTube channel yet, please do it scram the pants on YouTube and it has some great visuals to go along with these presentations. So let's get started on restrictive pulmonary diseases. I'm going to say this outright.

Probably not the highest yield of all the topics you can study in poem, but, you know, there's a few things that you may get quite. questions on. So we'll break it down, of course, of the most important things that you need to know. And just some of the tricks to ways, tricks to remember these. I'm going to focus more on sarcoidosis and idiopathic pulmonary fibrosis. Those are the two more important. And then we'll go over towards the end, the pneumoconiosis. So let's start with sarcoidosis. This is an idiopathic multi-system inflammatory disease. And it manifests as what's called non-caseyating granulomas. It's most commonly found in the lungs and the intrathorac lymph nodes.

notes. Now, as far as the path, it's all about the T cells. So the T cells play a central role. You have this exaggerated T cell response. It leads to this excessive cellular immune reaction. And because of this, you have inflammation and granuloma formation. So T cells get out of control. You have this immune system that's gone into overdrive. Inflammation, granulomas form throughout the body leading to the clinical manifestations. We'll see. Now, risk factors, there's two things that you need to know because this is what's going to be on your vignette. This is who they're going to give you on the vignette. So risk factors. African American three to four times more common and then females there's a slight female

predominance so in the vignette they're going to give you an African American female that's the question you'll get on sarcoidosis almost a hundred percent so you look for that in the in the vignette to kind of differentiate from other conditions which we'll go over later where their demographics are going to be a little bit different so sarcoidosis remember african-american female is likely who you'll see in the vignette and in real life too now clinical manifestations bear in mind this is a systemic disease it can affect anywhere in the body. So there's a lot of possible clinical manifestations we could go on and on forever. But let's focus on the common ones and the unique ones because that's the ones that the boards are

going to test you on. Now first before we talk about what you'll see in clinical manifestations, be aware many patients, about half the patient population is going to be asymptomatic. The disease is only going to be detected on an incidental chest x-ray prior to the development of symptoms. That's about half the time this is just going to be an incidental finding on chest x-ray. Now, if they do have symptoms, half of them are going to be pulmonary complaints. The main things you're looking for is a nonproductive cough, dyspnea on exertion, chest pain. All that's very nonspecific. But those are the most common complaints you'll see in sarcoidosis.

Now, these are the unique ones that you should look out for in the vignette. So the first thing, looking into your dermatologic finding, so erythema and odosum. It's those painful nodules that are most common on the interior surface of the lower extremities. It's a non-specific finding because it can be found in strep infection. It can be caused for medications, ulcerative colitis. So non-specific, but the key to erythema and odosum when we're talking about sarcoidosis is a combination of erythema nodosum, polyarthriagis and fever, and bilateral lymphadenopathy. If you see that, that's known as Lofgren syndrome, and it is very specific to sarcoidosis.

So specific that you don't even need a biopsy to diagnose. So again, Lofgren syndrome is a combination of erythrin syndrome. Phthalmidosum, polyarthralgias, migratory polyarthriagiaz, combined with fever, and then bilateral hylar lymphadenopathy, which you'll see on chest x-ray. Lofgren syndrome, remember that if you see that right away, you should be thinking sarcoidosis. Another specific finding in derm is something known as lupus pernio. So it's the most specific physical exam finding in sarcoidosis by itself, and it's pathognomic for sarcoidosis.

It's these indurated plaques. They kind of have this violacious discoloration. It's mainly found on the face. So the nose, cheeks, lips, sometimes the ears too. And it's more common in the African-American patients with sarcoidosis. So lupus pernial, very specific as well. And then on your x-ray, so lymphadenopathy, you may see bilateral hylar lymphadenopathy. So it's this bilateral enlargement of the lymph nodes of the pulmonary hila.

It's a classic finding in sarcoidosis that you'll see on chest x-ray. And then finally, not as important, but just remember that sarcoidosis is one of your causes of restrictive cardiomyopathy. So it's one of the infiltrated diseases that can cause. restrictive cardiomyopathy. It's because of the granuloma deposition in the myocardium. So remember that as well. Now diagnosis, there's no specific or definitive, I'm sorry, there's no definitive diagnostic test for sarcoidosis. You're looking for a combination of a few things. You're looking to make sure they have the clinic, the classical clinical manifestations. You're looking on x-ray to make sure they have those classic findings.

You're excluding other diseases like tuberculosis, which can present in a similar way. And then on biopsy, you're looking for a detection of a number. non-caceating granuloma. So that's generally what you're looking for to make the diagnosis. Don't memorize that. It's not important for the exam, but I just want you to be aware of how the diagnosis is made when you're actually treating patients. All right. So let's actually go down the different tools we use to diagnose. First, chest x-ray, bilateral high-lar lymphadenopathy. Half of affected patients with sarcoidosis are going to have bilateral hylar lymphadenopathy. So that's one of your first expressions of sarcoidosis. It's the big fluffy stuff. I'd like to have a visual here,

but I don't, but it's the big fluffy stuff at the center of the chest x-ray. It's an enlargement of those lymph nodes on pulmonary hyla. So on x-ray, you look for this big fluffy stuff right in the center of the chest x-ray. So very classic finding in sarcoidosis. Now, labs, you have to know this one. Increased ACE level. So your serum angiotensin converting enzyme, the ACE level is going to be elevated in about 75% of sarcoidosis patients that are untreated. So what you should know is that you're definitely going to see this on a vignette.

They're definitely going to mention increased ACE levels, but know that it's not really a great diagnostic test. There's a lot of false negatives. But again, it's going to be on your vignette. Just know that in real life it's not the best test, but you will see this in a lot of your patients. Now, pulmonary function testing, remember, these are all restrictive diseases. So you're going to see a restrictive pattern. So your PFTs, your pulmonary function test will reveal a restrictive pattern. So your FV1 FEC ratio is either going to be normal or increased.

So remember patients with restrictions. restrictive disease, there's no trouble getting air out, it comes right out. It's just having trouble getting air in. So you always think about just putting a big strap or rubber band over your chest. So you breathe in and then it's hard to breathe in because you have all that resistance from that rubber band, which is really the scar tissue and the fibotic changes that these patients have with restrictive diseases. So hard to breathe in. But then remember that big rubber band, as soon as you breathe out, it's pushing it right out. So it comes right out easily. And that's why they have this restrictive pattern. So normal or increased FV1,

FVC ratio you'll see on these patients. The way that I used to remember that because sometimes I forget what is obstructive, what is restrictive, how does it change the ratio? So the R and restrictive diseases stands for raise the ratio or remain the same. So remember restrictive FV1 FEC ratio. Restrictive is either going to R raise the ratio or R remain the same. And then obstructive, I always remembered oppress the ratio. It keeps it down because obstructive diseases keep it down. So O, oppress the ratio, R, raise, or remain the same.

So you remember that for your FV1 FEC ratio. Now, biopsy, this is big. So you can biopsy anywhere there's an accessible lesion. You don't have to go all the way into their lungs, into the intrathoracic lymph nodes. You can get it anywhere. If there's a cutaneous lesion, if there's a palpable lymph node. And what you're looking for on biopsy, the characteristic feature of sarcoidosis is a non-catiating granuloma. That's what you're looking for in biopsy.

It can be biopsied anywhere that there is an accessible lesion. Treatment is very easy. Treatment is just steroids. So oral corticosteroids, first line, that's all you need to know for treatments. Don't waste any time with anything else. Not every patient's going to need treatment because a good number of patients with sarcoidosis will have spontaneous remission or they may have a benign clinical course not requiring treatment. But if they ask you for treatment 100% steroids.

All right. So there's a few things you need to know with sarcoidosis. Let's give you a way to remember that. So remember, we said those ACE levels were super high. So ACE levels super high. ACE levels L super S high H. So that stands for A, African American. Remember, three to four times more common.

The C stands for cough, dry cough. Pulmonary findings are the most common. The E stands for erythema nodosum. The L stands for lupus pernial. Remember your most specific finding. S stands for steroids, first line treatment for patients that need it. And then the H in high stands for Hylar, lymph, adepathy bilateral. So remember, ace level super high, African American, cough, erythema, nodosum,

Lupus pernial, steroids, and hylar lymphadenopathy. Okay, so that's sarcoidosis. Let's move on to idiopathic pulmonary fibrosis. So this is a rare form of fibrodic disease with no known etiology. That's what idiopathic obviously stands for. You're going to have progressive scarring of the lungs over the period of several years. This isn't going to be something that's going to come on quickly. It's an insidious onset. And while there's no specific cause known, there are a couple of important risk factors, which you'll see are quite different than sarcoidosis. So an idiopathic pulmonary fibrosis risk factors, cigarette smoking. Majority of patients with pulmonary fibrosis are going to have a history of cigarette smoking.

Males, so it's actually more common in males than females. This is the opposite of sarcoidosis, which will help in your vignettes, which we remember was more common in females. And then finally, you're going to see this in older patients. Generally over 60. It's most commonly seen in the sixth and seventh decades of life. It's rare to see idiopathic pulmonary fibrosis in patients less than 50. Okay, now the clinical manifestations on pulmonary fibrosis,

I'm just going to start calling it that rather than idiopathic pulmonary fibrosis every single time. There's really nothing specific, which means anytime there's nothing very specific, you're probably not going to get an exam question. But the clinical manifestations are going to be dyspnea. It's going to be generally on exertion, nonproductive cough. Again, remember insidious onset, so generally over the span of a few months, maybe a year. And then physical exam. Baisal or crackles, so they may be absent early on in the disease, but that is something you will likely hear in these patients.

Remember, bi baseler just means at the basis of both lungs. So bi-bazilar, base of the lungs. So crackles at the base of both lungs. And then clubbing, because remember, clubbing of the fingers, anytime you have a chronic hypoxemic state, clubbing of the fingers is something that is definitely a possibility. So all of those really nothing specific to idiopathic pulmonary fibrosis. And then diagnosis, a few things. So remember pulmonary function test. Of course, this is a restrictive pattern.

So normal or increased FV1 FEC ratio, restrictive R remains the same or raises the ratio. Chest X-ray, not very specific. Again, you're going to have these increased reticular markings, which is great. But you can see that in any interstitial lung disease and in heart failure, so not specific. Chest CT is where you're going to have a little bit more specificity as far as diagnosing. And really all patients with pulmonary fibrosis or suspected pulmonary fibrosis need a CT, much more sensitive and specific than a chest x-ray. And it's really essential in a diagnostic workup. So there's three findings you're going to see on CT that are characteristic of pulmonary fibrosis.

The first one is something known as honeycombing. So honeycombing is this peripheral basilar predominant opacities to put that in a way that we can kind of understand it. It's really just these cysts that are spread throughout the lung form. field. They're all different sizes, generally pretty small, about three to 10 millimeters, and they're clustered together. So you have these little cists that look like holes on CT. And when you have all of these cysts that are clustered together, they just look like a honeycomb. If you look at a honeycomb, it's just these little holes all next to each other. So that's what honeycombing is on a CT. And you can see this in idiopathic pulmonary fibrosis. Something else you may see on CT is something known as

traction bronchiectocystis, bronchialectocyst just due to the damage of the bronchules. You have these distorted abnormally dilated bronchial. And then finally, something else known as bilateral ground glass opacities. So when you look at the CT or even a chest x-ray, you have this hazy increased lung opacity, just due to all the scarring and fibrosis that are overlaying the lungs. It can be seen in other interstitial lung diseases, sarcoidosis, etc. But it's generally more prominent in pulmonary fibrosis. So again, ground glass opacity is just this haziness you'll see over the lung fields because of all the fibotic changes and scarring over the lung. lungs. Now biopsy, what you're going to see is alternating areas of normal lung, fibrosis,

and honeycombing. So you have, you look at the biopsy and then you see these fibotic changes with varying levels of severity, so different ages, varying ages, next to normal lung, next to honeycombing. So that's the hallmark finding. You have alternating areas of normal lung, fibotic changes and honeycombing. That's biopsy. And then treatment. So there's no cure for idiopathic pulmonary fibrosis outside of a lung transplant. And most of the measure you're going to take are going to be supportive. So some of your supportive measures, smoking cessation is an obvious one. And then supplemental oxygen. All patients with pulmonary fibrosis are eventually going to require oxygen. Initially, it's just going to be with exertion, but eventually they're going to progress to a point where they're all going to be on it continuously. You of course want to make sure that these patients get all their routine vaccinations, their pneumonia vaccine, their influenza, because this is a bad interstitial lung disease. If they get these pulmonary infections, it can be pretty severe for them. Now, medication, These are the only medications you need to know. They're not going to ask you supportive measures.

It's just hard to ask a question about should they stop smoking. Obviously, they should. These are probably the only two that you should know. So there's a lot of drugs that have been tried in idiopathic pulmonary fibrosis that have shown no clear benefit. You might see anticoagulants, phosphodiasis inhibitors. None of these really showed any real benefit. The only ones that have shown some benefit, the only ones that you need to know, are two

known as nitatinib. and perfenidone. They're antiphobrotic agents. They are by no means a cure, but they slow disease progression. It's really the best thing we have right now outside of a lung transplant. So remember, if they ask you a medication, it's either going to be an atadinib or perfenidone. Those are your antifibrotic agents that slow disease progression. And then your definitive treatment, which of course is a lung transplant. So going over all that, you're going to realize there's a lot of stuff in pulmonary fibrosis that's just very non-specific. So it's unlikely you're going going to get a question on it. The presentation's non-specific. Most of the imaging findings are

generally not specific. It's not much in the way of treatment outside of those two I went over, so I wouldn't waste too much time on that. All right, so let's finish up with our pneumoconiosis. These are also known as occupational or environmental lung diseases. So they're a group of interstitial lung diseases, which are caused by inhalation of dust, minerals. And patients that are commonly exposed are ones that have exposure in the world. workplace. So these are pretty low yield. I'm going to focus on the risk factors for each one, because that's the question you're likely going to get, a description of their job, and then they're going to ask you what job, or they're going to tell you the job they work in, like they're going

to say they work in the textile industry, and then you just need to know that. That's bycinosis. So that's likely what they'll give you. So we'll focus on the risk factors, the workplaces they're at, and then I'll go over a couple other things that I think are important. As far as treatment, there's really nothing specific for these. They're not going to test you on it. It's generally subordinate or steroids. Those are really the main treatment options for these conditions. So I have a couple tricks.

My first trick for memorizing these is that the diseases, some of these diseases are going to have pulmonary nodules with a predilection for a certain part of the lung. So either the upper or lower lobes. And the easy way to remember this is that the lobe the nodule is found in. So the upper or lower lobe is the opposite of the height that the person does their work in. So let me explain this. Give you an example. So if they work in roofing, roofing and attics, they repair insulation high up in attics, like in asbestosis. That's up high.

So where the nodule will be found opposite. So nodules generally found in the lower lobes. If they do work on the ground or deep in the ground like a coal miner, coal miner, nodules are generally found up high in the upper lobes and that works with all of these i'll go over the few um that the because not every condition's going to have a specific lung zone that it's found in but the ones that do actually works so this works out really well um so an easy way to remember all right let's start with co-workers pneumoconiosis um so this is obviously caused from inhalation of coal dust particles so that there's nothing really to memorize there makes sense now patients with co-workers

pneumoconiosis work deep down in the coal mines so where do you think their nodules will be found just like I just said, the opposite. So nodules and co-workers pneumoconiosis, generally found primarily in the upper lobes. There are these small nodule or capacities, generally less than a one centimeter in diameter, and most commonly found in the upper lobes. Now, one other thing I'll mention,

I don't think it's super important, but there's something known as Kaplan's syndrome, and all Kaplan's syndrome is is a combination of co-workers pneumoconiosis and rheumatoid arthritis. One of those things, not super high yield, but maybe your preceptor will ask you it, and you'll sound very smart if you know it.

So Kaplan syndrome, co-workers, pneumocodiosis, combined with rheumatoid arthritis. All right, let's move on to bicinosis or bisinosis. I don't know how you want to pronounce that. But this, the only thing that I remember for this is bisonosis is at risk for people who work in the textile industry, which is where cotton fabrics are made. So they have this cotton mill dust exposure. So people that work in the textile industry, I don't know if you knew this. I didn't know this.

but the textile industry is just people who work with like yarn and making cotton into yarn and things like that. So bycinosis, cotton mill dust exposure from people that work in the textile industry. That's all you need to know for that one. All right. Asbestosis. Inolation of asbestos fibers, which leads to diffuse pulmonary fibrosis. Who are you going to see this in? People who renovate old buildings, insulation like up in attics, shipyards is another one, and then installation of roofing tiles or shingles. In the U.S., asbestos has been limited since the 1970s. The only place you can use this in the U.S.

asbestos is in brake pads and gaskets and cars, roofing products, and fireproof clothing. But the key is that if it's an old building that you're renovating and you're up in the attic, you're in the insulation and this building was built in the 1950s, they can still have exposure. So know that in real life. And for the vignette, if they say something about an old building.

Now chest x-ray, these patients work up with roofing tiles, shingles. They work in insulation in buildings and in attics. Those are all up high. So where are we going to find their pulmonary findings? It's going to be down low. So these aren't so much nodules, but they're plural plaques. And they're commonly in the lower lobes. So patients with asbestosis will have plaques on the parietal plura generally in the lower lung zones.

Remember opposite of where they're working. And then another thing you need to know about chest x-ray. There's something known that's a shaggy heart sign. All it is is this indistinct heart border. So the interstitial and plural involvement caused this hazy, another ground glass appearance to the chest x-ray, and it blurs the diaphragm and the heart border, and that's why they call it the shaggy heart sign. And then finally, these patients obviously have an increased risk of bronchogenic carcinoma, particularly in your smokers because there's a synergistic effect with smoking and people with asbestos.

So people who smoke and have asbestos exposure, super high risk of bronchogenic carcinoma. increases your risk by like 8.7 fold. So really high risk for that. All right, a couple more and then we'll wrap it up. So next one is known as bariliosis. This is chronic beryllium disease. It's a granulomatous disease caused by exposure to beryllium. Barillium, just so you know, it's lighter than aluminum, six times stronger than steel. And for that reason, we kind of see it used in certain work, you know, workplaces. So aerospace, the aeronautics industry is a big one. Electronics, people who manufacture electronics, computers. We also see it using the defense industry dental appliances,

but the two you need to know. The two they'll test you on is the aeronautics industry, aerospace, and electronics. So know those two for berylliosis. Okay, so silicosis is our last one. This is inhalation of silicone dioxide. Risk factors, any job that they mention breaking up silica containing rock or ore, anything they mention rock, stone, moving the earth, digging up the earth, think of silicosis. So people who work with granite, court, sandblasting, which we know is just broken up rock,

masonry work, slate. Again, if it mentions a rock, right away think of silicosis. Chest x-ray. So two things you need to know on chest x-ray that may come up. Now, these patients, they work with stones and rocks and minerals. Where do you find stones and rocks and minerals? Up in the sky? No, it's found in the ground, down low. So where are you going to find their nodules? The opposite, upper lobes. So nodules primarily found in the upper lobes and silicosis. And then the other that you should know, something known as eggshell calcifications. It's this thin layer of calcification around the hyler lymph nodes. They look like eggshells on chest x-ray. It's not only seen in silicosis. You can see it on co-workers pneumoconiosis, longstanding sarcoidosis, but it's commonly seen

in silicosis. It's normally the one they'll mention it in. So be aware of that as well. That's it for silicosis. So just know the patient in the vignette is going to work with rocks, quart, granite, sandblasting, and then on x-ray look for upper lobe nodules and eggshell calcifications. All right. So that's really all I think you need to know. Don't waste your time digging any deeper than this. There's just, it's just not high yield enough to waste your time any further than that. I have a little story to help you memorize the things you need to know in those pneumoconiosies. I like stories for memorization. I think it helps. You kind of create this visual in your head and you can remember for the exam. So hopefully this will help you. It did for me. So I'm going to give you a little story and it'll help you remember the key things you need to know. So here we go. Sylvia was tough as a rock. But this tough.

exterior would shatter like an egg shell every day she'd begrudgingly had to go to work. So Sylvia SILS stands for silicosis. She was tough as a rock. That rock, because remember silicosis risk factors, occupation exposure to rocks, granite slate sandblasting, but her tough exterior would shatter like an eggshell every day she had to go to work. Remember, eggshell and silicosis, those are your calcifications in the hyler lymph node. So again, Sylvia, silicosis was tough as a rock.

That's your risk exposure. But this tough exterior, would shatter like an egg shell, eggshell calcifications, every day she begrudgingly had to go to work. Sylvia longed for the day she could say buy to the textile factory she worked at. So she longed for the day she could say buy. That's bycynosis, buy, and then textile factory. Because remember, bycynosis, increased risk for textile industry workers. Okay.

Her dream was to one day build space shuttles. So each night when she got home from work, she would bury her head in books about aerospace and electronics. So every day she'd get home from work, she would bury B-E-R-Y, Berrileosis, her head in books about aerospace and electronics. Remember, barrileosis, increased risk for individuals working in aerospace and electronics. Okay, last sentence. For now, this was just a dream, and she would have to do her best to get through the long days at the broken down, poorly insulated old building she worked at.

So for now this was just a dream, she would have to do her best as an asbestosis to get through the long days at the broken down, poorly insulated old building, she worked at, remember asbestosis, commonly found in insulation, repair renovation, old buildings. So I'm going to read it one more time. Sylvia was tough as a rock, but this tough exterior would shatter like an eggshell every day she begrudgingly had to go to work. Sylvia long for the day she could say buy to the textile factory she worked at. Her dream was to one day built space shuttles. So each night when she got home from work, she would bury her head in books about aerospace and electronics. For now, this was just a dream and she would have to do her best

to get through the long days at the broken down, poorly insulated old building she worked at. So hopefully you're taking the exam and then all of a sudden you see something that mentions maybe somebody that works in aerospace and you're like, all right, Barry, Barry, Berliosis, she buried her head. I hope that helps and I hope that'll, you know, create some visual in your head so you can remember. And if you need a little bit better of a visual there on my YouTube page, I have some visuals combined with a story, plus you can actually see the words which can sometimes help. All right.

So that's it. Let's do five quick questions and we'll wrap it up. 1.45-year-old African-American female presents to the office today for a persistent, non-productive cough she's had for the last five months. On exam, you note violacious plaques on her nose and cheeks. Bilateral hylar lymphadenopathy is visualized on chest x-ray. First-line medication for the likely diagnosis in this patient is going to be oral corticosteroids. So this is obviously sarcoidosis. Lupus pernial was described by those violacious findings on the face. Bilateral high-lary lymphadenopathy on the chest x-ray and then remember in the vignette it was an african-american

female all signs point to sarcoidosis which oral corticosteroids are your first line if you do need treatment question two a 66 year old male presents to the office with the history of a non-productive cough it works for a construction company that restores and renovates old buildings on chest x-ray bilateral pleural plaques are noted in the lower lobes what diagnosis should be suspected in this patient so that of course is going to be asbestosis plural plaques in the lower lobes works in the construction business repairing old buildings, which remember is one of the risk factors for asbestos. And then, of course, those pluriplax in the lower lobes because remember, it's the opposite of the work that are doing the height. Question three, a patient with sarcoidosis presents with erythema nodocum, bilateral hylar lymphadenopathy, and polyarthralgia's with fever.

The syndrome is known as, remember that, is known as Lofgren syndrome and sarcoidosis. Question four, a patient with co-workers pneumoconiosis would likely have nodules in which point. part of the lungs. So remember, co-workers pneumoconiosis, they're working deep down, so they're going to have nodules in the upper lobes of the lungs. And then question five, last one, what medications are used in patients with idiopathic pulmonary fibrosis to slow progression of disease? These are the only medications you need to know for idiopathic pulmonary fibrosis, and it's going to be peripenidone and it tatinib. All right, so that was it. I hope that was helpful. That is your restrictive pulmonary diseases, and that is following the NCCB.

blueprint the ones that they want you to know please let me know in the comments if it was helpful please check out the YouTube page if you haven't yet and thank you as always for all the really nice comments you have been leaving good luck on your pants your panery your errs and good luck in PA school