Nephritic Syndrome

foreign engineers in this video today we're going to be talking about nephritic syndrome which is one of those types of glomerulonephritis before we get started I really want us to kind of get into talking a little bit about you know the the anatomy the function of the glomerular filtration barrier I think it's really critical to understanding nephritic syndrome before we actually start getting into that I want you guys to please if you guys find this video beneficial helpful please support us hit that like button comment down the comment section subscribe if you guys have the opportunity also if you guys want some great notes some illustrations to follow along with me during this lecture go down the description box below take you to our website we've got some great notes some great illustrations that I think will really be helpful to follow along with me but let's start talking about nephritic syndrome so first things first we have the anatomy of the glomerular filtration barrier right it's really really critical to understand this because it really describes the pathophysiology the basics behind nephritic syndrome and what really is there's three components of the glomerular filtration barrier and what are these I'm going to abbreviate this as gfb if you will so glomerular filtration barrier there's three components of it the first one is this capillary which is beautifully finished straight what does that mean it needs to get a little pores which allows for plasma and certain things in the plasma filtrate to filter out of the blood and into the Bowman space right that's one a part of the filtration barrier so we have What's called the fenestrated capillaries if you will all right so that's one part of the barrier that controls the blood flow and the certain things within the blood from leaking out into the actual Bowman space really good at that especially big proteins and red blood cells Etc the second component of the glomerular filtration barrier is this big blue molecule here what's that that's called the glomerular basement membrane this sucker is so cool and the reason why is the proteins that make this membrane up give a very heavy negative charge so it's very heavily negatively charged and one of the cool things about that is that proteins most proteins on our bodily are negatively charged and so negative charge negative charges they're the same charge they're going to repel one another and that's really good at preventing very large negatively charged plasma proteins from leaking across the blood plasma into the bone in space the third part of the glomerular filtration barrier my friends is these cute little cells you see these cells right here that are kind of like lining right on top of that that barrier there what are these cells called these are called your podocytes these are called your podocytes and the podocytes if you kind of look you see how they have like these little divots that's called their little foot processes and there's little spaces between those protocytes called filtration slits and that's also supposed to give a little bit of permeability to this gfb but not so much where big things like red blood cells and big proteins and white blood cells leak out so this is your protocytes and again the big thing that I want you to remember that plays a role within the permeability here is the filtration slits now the whole job of the glomerular filtration barrier right is to prevent large things from leaking out we want some things to leak across this barrier we want things like small amino acids glucose sodium electrolytes water certain types of metabolic waste products to filter across here to be peed out right we don't want that stuff in the body and so we're supposed to allow that to happen but what we don't want to cross over is the glomerular filtration barrier is supposed to block the passage of red blood cells white blood cells and proteins here's the problem though and a disease called nephritic syndrome so when someone has nephritic syndrome good old nephritic syndrome what happens in this disease is it causes damage to a very specific thing it damages the glomerular basement membrane which I'm going to abbreviate is GBM so it causes GBM damage or dysfunction now here's the problem when you have glomerular basement membrane damage what happens is you jack up this glomerular filtration barrier it's normally supposed to block the passage of red blood cells white blood cells and proteins but now you introduce a little caveat here in nephritic syndrome you introduce glomerular basement membrane damage or dysfunction and then what ends up happening is you lose the capacity to control what leaves the blood and goes into the Bowman space and then what starts leaking out is you start having the leakage of everything that you could imagine you leak out red blood cells you leak out white blood cells and you leak out proteins and that's the problem that we see in the phretic syndrome and nephrotic syndrome if you guys remember it was damaged to the podocytes and when there was podocyte dysfunction it just caused heavy protein urea and nephritic syndrome where there's glomerular basement membrane damage or dysfunction it's proteinuria subneprotic range hematuria and sterile pyria now let's talk about the next component here which is we have a patient with nephritic syndrome right what we know is is in this disease process we're going to have glomerular basement membrane damage so there's damage this good old GBM this this molecule right here so here is our glomerular basement membrane and this thing is all jacked up so there's glomerular basement membrane damage so there's injury to this glomerular basement membrane right now when you damage the glomerular basement membrane what we said is is you lose the ability to control what things are leaking out and what are some of these things that start leaking out one of them as we said is red blood cells these start leaking out another thing that starts leaking out here is white blood cells and the last thing that starts leaking out here is proteins and that is the big big factor here because whenever you end up actually looking at these patients urine what ends up happening is and one of the big big differences here is that these patients get a lot of red blood cells in their urine what is it called when you get a lot of red blood cells in the urine we call this hematuria we call this hematuria and that's one particular problem so whenever there's glomerular basement membrane damage there is an increase in the loss of particular things this leads to red blood cell loss white blood cell loss and protein loss but here is the big big difference the protein loss is not as much in nephrotics not as much as you compare to a nephrotic syndrome a nephrotic syndrome it was greater than 3.5 grams per day and nephritic syndrome nephritic syndrome it's less than 3.5 grams per day and that is where one of these overarching things come from so you have lots of red blood cell loss a lot of white blood cell loss a lot of protein loss but the protein loss is sub-nephrotic range to add on a little bit here when you get a lot of these red blood cells they get in the urine what happens is they cause the hematuria right but what happens is these red blood cells that get stuck in the kidney tubules and they take on the shape of the kidney tubing they make these long tubes of red blood cells you know what we call that so whenever we have these red blood cells and they take on the shape of the kidney tubules they give you this weird kind of configuration and we call this red blood cell Cass This is highly specific for glomerular disease especially nephritic syndrome another way that these red blood cells can present in the urine is when you look at them they look like they're all spurry like they look like they've just been beaten up and we call this a canthocytes we call this acanthocytes so this is another way that we can see these poor little kind of red blood cells being lost into the urine so hematuria in the form of red blood cell cast or acanthocytes is very common for nephritic syndrome the other thing is you're going to have a lot of white blood cells in the urine all right now the thing is is I really want us to be very specific here this is called sterile pyuria and the reason why it's crucial to mention this is you're going to get a lot of these white blood cells that are going to just be able to fit across you know the glomerular filtration barrier and you're going to have a lot of these all right so you have a lot of red blood cell casts a lot of acantha sites a lot of white blood cells but here's the big difference because you can get a lot of white blood cells and urinary tract infections the big difference is is I'm going to have an increase in white blood cells in the urine but I'm going to have no uh positivity or I'm going to have decreased amounts of what's called I'm going to have less bacteria so I won't have as much of the bacteria that makes me think that it's a urinary tract infection and I'm going to have less leukocyte esterase that's positive and less nitrites which are positive all of these things are suggestive of a urinary tract infection so you're going to have let's actually just so we don't confuse it you'll have decreased bacteria your leukocyte esterase will be negative and your nitrites will be negative but you'll have a lot of white blood cells in the urine this is also suggestive of nephritic syndrome the last one here is you're going to have a lot of protein urea but again this proteinuria is less than 3.5 grams per day and why I want to mention this is that you're not going to have a significant of the hypoalbium anemia the low antithrombin 3 and the low Amino globulins and so what I want to mention in this one here is you see this you see this but what you will not see is there isn't going to be there will be no significant hypoalluminemia no significant antithrombin three deficiency and no significant immunoglobulin loss and that's the big difference here there isn't going to be this very low albumin in the blood there isn't going to be this very low anti-thrombin 3 in the blood and there isn't going to be this very low Amino globulins in the blood the reason why that's important is because these are going to be more suggestive of nephrotic syndrome so therefore in these patients you're not going to be seeing the edema Factor the dvtpe and the infection factor and so that's important there won't be as much edema secondary type of humanemia there won't be a lot of lipids secondary to the hypovium anemia there won't be a lot of dvts and PES and renal vein thrombosis and there won't be a lot of infections so this is why it's really important and helpful to differentiate so because there's no significant loss of these particular things you won't see these particular effects in nephritic syndrome and that is crucial that I need you guys to remember that okay the next thing here that's really helpful that is really really good is that in patients who have nephrotic syndrome we talked about this in that video I just want to quickly recap this that we said that nephrotic syndrome can lead to it can potentially lead to chronic kidney disease and if you guys remember we said that that was with which particular ones focal segmental glomerulosclerosis membranous nephropathy diabetic nephropathy amyloid nephropathy so there is an increased risk of CKD with nephritic syndrome with nephritic syndrome there is a very high risk much higher much much higher of first off acute kidney injury and chronic kidney disease and CKD I mean in nephritic syndrome so you're going to have a much higher incidence of kidney injury renal failure in patients with nephritic syndrome than you would in nephrotic syndrome But realize that there's both the possibility okay the next thing that I want you guys to understand here is that when you cause so much of this GBM damage it leads to a lot of white blood cells proteins so you're going to get a lot of specific molecules in this space here what do we call this space here this is called Bowman's space you're getting a lot of proteins and a lot of white blood cells in there so you need a lot of white blood cells a lot of proteins a lot of cytokines and this is the problem with this disease when these things get out here what they do is they promote a very nasty process where they increase a lot of fibrosis a lot of sclerosis and epithelial cell proliferation so what are the things that they're going to do they're going to increase fibrosis and sclerosis and epithelial cell proliferation let's write that down it's going to cause fibrosis and sclerosis and it's going to cause what's called epithelial cell proliferation now what does that look like all right ready first thing that happens is the epithelial cells these ones here so you know how we have the podocytes which are the visceral layer you know what this layer is called the parietal layer of the Bowman's capsule these start proliferating like crazy because of all of these cytokines and inflammation and white blood cells in the area and look what happens they start just like taking up so much space with inside of the actual Bowman's capsule so there's one thing there's the epithelial cell proliferation let's add on even more problems let's add in all this like scarring the fibrosis sclerosis to this area here oh my gosh this looks absolutely terrible but you know what it kind of takes the shape of which is kind of odd is if you look at it kind of takes the shape if you will of a crescent right looks like a crescent and so what we call this is this leads to this Crescent formation this leads to Crescent formation and this Crescent formation is really really scary you don't want to see it whenever you see a lot of these crescents these glomerular crescents that are starting to form it's suggestive of impending renal failure so this is called glomerular crescents what happens then is that imagine now being able to filter things across this glomerular basement membrane now all of this area here what do you think is going to happen to the glomerular filtration rate suck it's gonna drop man and so you're gonna have a drop in the glomerular filtration rate if you have a drop in the glomerular filtration rate imagine all the particular problems that you're going to have you're not going to be able to get things across this thick barrier here all of this is going to be inhibited or impeded because of these nasty crescents and so what happens with these crescents is this leads to something at least a very scary disease it leads to a disease which is called rapidly Progressive glomerulonephritis and this can lead to renal failure that is the problem and that is the fear of nephritic syndromes is that you can literally cause these crescents to block up the flow of filtrate drop the GFR now here's the thing when you drop off your GFR what's all the negative problems with that well one of them is that a low GFR if I were to bring this one down here a low GFR is kind of problematic man it does a lot of nasty things one is you're supposed to filter out waste products you know these are things that you're supposed to be filtering out you're going to filter them out anymore nope and then all these waste products such as your blood urea nitrogen and your creatinine guess what's going to happen they're going to build up and then as a result now I'm going to have a lot of azotemia but when these levels get really really high they can lead to something they can lead to something called uremia where they can start causing neurological dysfunction platelet dysfunction pericarditis all that great stuff the other thing is is that a low GFR not only will it stimulate this particular process not only can It stimulate this particular process because you don't filter these molecules out but it also can activate the renin Angiotensin aldosterone system if I activate the renin Angiotensin aldosterone system what does that do if I stimulate this doesn't this kind of go and cause Angiotensin II to be increased cause aldosterone ADH all those to be increased yeah if that happens what is that going to do to my blood pressure oh it's going to shoot that blood pressure up and so these patients end up with very high blood pressure so we can end up with high blood pressure as a result what's that called hypertension so let's write that down hyper tension so we can see high blood pressure as a result of this low GFR all right so we can have uremia let's actually make this in a nice black color here so it's the same as all of these so we can have uremia potentially as the result of a low GFR we can have hypertension as a result of a low GFR what's the last thing that we could have as a result of a low GFR the last thing is you don't filter sodium water across the glomerulus if you don't have a good GFR and so you end up building up your sodium and water what happens if you build up sodium and water this will lead to edema and that's the last effect that you could get here so you can get a lot of problems with this low GFR is you can get hypertension edema and uremia and what is this from again this is from a lot of these white blood cells these proteins these cytokines being released over because of what because of GBM damage or dysfunction this thing is damaged it's causing a lot of these things to leak out into the space creating an inflammatory reaction relieves the fibrosis and epithelial cell proliferation you make these nasty crescents they block off the filtrate flow that can lead to rapidly Progressive glomerulonephritis which can lead to renal failure and that renal failure you drop off the GFR you don't filter off these waste products you don't filter off sodium and water and you end up activating your renin Angiotensin aldosterone system these are the negative things that you can see in nephritic syndrome some of these mace found similar though to nephrotic nephrotic syndrome they had hypertension they had edema their edema was more due to what though in nephrotic syndrome low albumin low albumin this is more due to low GFR so a renal failure is going to be in higher incidence in patients with nephritic syndrome as compared to nephrotic syndrome let's now move on to the next part what is the cause of the GBM damage next part here is we got to figure out why is there GBM damage what happened what was the cause of the glomerular basement membrane damage that cause red blood cells and white blood cells and some protein not 3.5 grams plus per day but there's protein loss right what's the reason for this and why are these patients at such high risk of rapidly Progressive glomerulonephritis okay well I didn't tell you which of the types not all of nephritic syndromes cause rapidly Progressive glomerulonephritis I will mention which ones now but I want to go over the causes of each one of those as well so there's a couple of these all right there's a lot of them this is where it gets kind of stressful when you're a student learning all these nephrotic and nephritic syndromes they're they're Sons of Guns but I think we got a good organization system here so the first way that I want you to think about this is is an anti-gbm disease is it an ankovasculitis is it hereditary nephritis or is it an immune complex deposition that's the way I want you to organize it in your head and hopefully we can make sense of it now when you think about anti-gbm disease you're really talking about a disease called good pasture syndrome and some of the things that you want to look for in the the clinical vignette is a patient who has had hemoptysis and hematuria so they have nephritic syndrome and they have hemoptysis that's really what you want to be able to notice is really look for a patient who has hemoptysis and I'll explain why and some type of hematuria usually secondary to their nephritic syndrome the other thing that you want to be able to identify here is that there is a very specific cause it's an anti-gbm antibody and that's really where it's super interesting so these patients have this anti-glomerular basement membrane antibody and it's this antibody and what it does is look at this dang thing it's mean this antibody loves to attack a very specific part of the glomerular basement membrane it likes to attack like this collagenous portion of the glomerular basement membrane so we call the non-collagenous portion and it likes that not just in the glomerular basement membrane but it likes it in the respiratory membrane it has a very strong affinity for it so what happens is when it sees this protein this non-coaginous protein here on the glomerular basement membrane it attacks it and when it sees this protein here on the respiratory membrane it attacks it and what ends up happening is these antibodies they come and bind to a portion of these membranes and they'll activate some inflammation they'll cause some damage to this respiratory membrane and they'll also cause damage to the glomerular basement membrane if you damage the GBM what do you start losing red blood cells proteins white blood cells all of these things start dumping into the urine and that's where you'll get these features that you see in these patients and so one of the biggest things is they're going to have a lot of these anti GBM antibodies that is the particular problem here and you'll see in these patients that they will have features of nephritic syndrome via the red blood cells white blood cells and protein in their urine right and so you'll have red blood cells you'll have white blood cells and you'll have sub-nephrotic range protein in the urine but you'll also cause damage and cause blood to leak into this alveolar capillary membrane and blood will get lost via the respiratory tract when you lose blood via the respiratory tract what is that called it's hemoptysis and that's why we see hemoptysis and features of nephritic syndrome and good pastures due to the anti-gbm antibody all right the next one here is ankle vasculitis and there's three types of anchovasculitis one is called GPA another term that you're aware of just be careful is Wagner's granulomatosis the second one is EPA or eosinophilic polyangiitis this is also known as churg Strauss syndrome and the last one is MPA microscopic polyangiitis that's just the only name for that one so remember GPA Wagner's EPA Church Strauss and MPA and these the name is usually the giveaway there's Incas so there's some type of positive anti-neutrophilic cytoplasmic antibodies that are present all right now here's where it's a little bit confusing these antibodies that you have here these anchas what they do is they do not directly deposit into the GBM at all what they do is is they go and stimulate via their name immune system cells what kind of immune system cells well the primary one would be neutrophils but it can also activate monocytes as well so you're going to get some monocyte activation and you're going to get some neutrophil Activation so you'll get some neutrophil and some monocyte activation right so we're going to stimulate these puppies when we stimulate these what they want to do is is they want to go and infiltrate into small vessels in the body that's where the next little hint comes in they like to deposit into small vessels in the body and they like to cause a lot of cytokines to get released and a lot of inflammatory mediators that then cause glomerular basement membrane damage which leads to the loss of proteins red blood cells white blood cells see what are you going to lose red blood cells white blood cells and protein is it going to be nephrotic range no so that's the big thing for this one but you're going to notice the big difference here is that this is no antibodies it's immune it's immune mediated GPA EPA MPA it's not all of them that cause this we have to have one so these things like to go and attack other areas and so that's where we should actually have a little bit more specificity here and so what we do is we actually look at these anchas we look at the anchors and see which one it is that's actually stimulating these and one what you want to know is was it a c anchor that was stimulating these immune system cells or was it a p anchor that was stimulating these cells right away that's very helpful in differentiating these because in C anchor positive immune reactions this is associated with Wagner's GPA so GPA Panka is associated with MPA and EPA so then you're like oh well dang it I can easily figure out you know if it's GPA but how am I supposed to figure out if it's MP or EPA there's one other difference eosinophilic this one's not eosinophilic so one of the big things for this church Strauss is you want to look for big key buzzwords look for things like asthma and you also want to look for things like an increase in eosinophils so look for an asthma here I'll actually do this two ways one is I want you to look for the increase in eosinophils so we'll draw these like this and then I want you to also associate this with asthma so if you hear EPA which is the pankas look for lots of eosinophils are look for features of asthma all right that's the anchovasculitis all right so we now have an idea here between the angle vasculitis and the anti-gbm are good pasture here's one more thing in the anti-gbm disease or good pasture syndrome and an ankovasculitis both of these have the potential to cause lots of inflammatory mediators and lead to rapidly Progressive glomerulonephritis it's very important to remember so in this particular situation this disease can lead to rapidly Progressive glamelonephritis and this one can lead to rapidly Progressive glamoronephritis what was the end result of these is that this could lead to an early development of renal failure due to those glomerular crescents and so it's important to remember that that rapidly Progressive glomerulonephritis can be seen in both anti-gbm disease and ankovasculitis and they have a very very high incidence in this type of presentation all right so the last one here is just a rando it's often the one that most people get right on the exam just because it's so random and so obvious it's hereditary nephritis so for this one this was immune mediated by white white blood cells this one was antibody mediated this one is genetic all right thus the name hereditary what you want to look for in the hints is you want to look for like a child and you want to look for some other features so another particular thing here is listen for sensory neural deafness because this is kind of some type of loss of the collagen within the cochlear membrane or you want to hear lens problems anterior Linda Kona so there's kind of like the slipping of the lens so they have Visual and hearing problems in a child who also has hematuria the question is how do they get hematuria well the collagen Type 4 collagen so the problem with this disease is there is a mutation where there is a decrease in what's called type 4 collagen and guess where that collagen is actually utilized as well it's used in the lens and it's used in the cochlear membrane guess where else it's utilized in the glomerular basement membrane if you don't have collagen you're going to make a good basement membrane no and so what happens is these patients get a very thin and dysfunctional that's the term dysfunctional GBM and as a result what do they lose a lot of in the urine red blood cells white blood cells and sub-nephrotic range protein so that is what you're going to see here my friends in this one you're going to see this and then you're also going to see a very thin glomerular basement membrane all right that covers hereditary nephritis so so far we've covered three of these types of nephritic syndromes two of them thus far can cause rpgn which can lead to renal failure which is that acute rise in viewing creatinine and you can have potentially some other effects like hypertension and edema now we come to these next three oh actually Four we have the immune complex deposition all right for the immune complex deposition it is thus within the name anti-gbm was an antibody that deposited into the gfb ankovasculitis was immune system cells depositing into the gfb hereditary nephritis is you weren't making a GBM these you have an antibody and antigen complex depositing into the gfb that's the difference so there's a couple of these how do we differentiate things well first off I want you to remember post-shoppedococcal melonephritis IGA nephropathy which can have another presentation of it called Enoch Sean limpura lupus nephritis and the last one is called membrano proliferative glomerulonephritis and post streptococcal glomerulonephritis the key thing that is going to trigger you to know that this is it is if you have a kid who just is one to let's say two weeks one to two weeks status posts strep infection I I think that's the dust within the name but what happens is this strep infection in a child will stimulate very specific so imagine here is the strep bacteria and it's important remember that this can be strep pharyngitis or it can be impetigo from the skin so you can be a skin or strep throat infection either way here's the bacteria here's their strap bacteria what happens is maybe there's a special little antigen special little antigen on the strap bacteria and your body produces antibodies against it and what happens is a piece of that antigen breaks off okay pieces of that antigen from that bacteria break off and you got a bunch of these things now look here's the antibodies that I'm producing against that streptococcal antigens these antibody antigen complexes can go and deposit into the GBM but here's the big thing I need you to remember these have specific names and it helps us diagnostically we can call these anti ASO antibodies or anti dnase B antibodies and these immune complexes that we form as a result of this from the strep antigen can go and deposit into the GBM now look at this here we go we got all these suckers here depositing into the glomerular basement membrane and the Very they deposit very specifically in very specific locations they like to go right between the podocyte and the top layer of the GBM we call that sub-epithelial deposition so what happens is as a result here you get what's called sub ithelial immune complex deposition do you see why we get that immune complex name there and what happens is they cause inflammation you know how they cause inflammation oh shoot son they activate the complement system you guys remember that right like you guys remember whenever you have an antibody that binds to an antigen it activates these proteins I'm going to represent them in pink pink here it'll be this protein we have like what's called C3 C4 C5 C6 all those these are your complement systems they activate the membrane attack complex they opsinize so they make bacteria very tasty for white blood cells that comes in here and they bind to these immune complexes and they try to enhance the inflammation but you know which complement proteins are very very specific C3 so you get what's called C3 complement activation I can't stress this importance of this diagnostically and that leads to GBM damage because of all that inflammation and if you damage the GBM what starts on coming on I'll leak it out my friends what happens you lose red blood cells you lose white blood cells you lose protein is it nephrotic range no and that's the difference here for this one so you're going to get a lot of these antibodies that'll cause some epithelial immune complex deposition cost C3 complement activation and increase GBM damage now here's one quick thing when you activate these complements what you do is you consume them you consume them and if I consume these complement proteins what will happen to their levels after I consume them they will decrease so it causes C3 consumption so C3 consumption that's very important all right so that's post-treptococcal melonephritis another big thing here post streptococcal glamoronephritis from this continuous GBM damage can cause rapidly Progressive glamelonephritis which can lead to early development of renal failure which can lead to renal failure all right so so far we've mentioned which ones are causes anti-gbm we mentioned that it was also the Inca vasculitis and so far post streptococcal okay to make your life easier it's these first three this one does not cause that but we'll get to it our IGA nephropathy plus or minus hinox Sean Lynn prepera this is actually probably the most common I'm going to say this again the most common nephritic syndrome key thing for this one is it's maybe one to two days fit weeks days status post upper respiratory tract infection or some type of git infection and that's really the big kind of cue here and so it's some bacteria virus whatever it may be let's represent this as the same thing it's some type of like bacteria or virus whatever it may be it's got an antigen on it that antigen is immunogenic our body produces antibodies and these antibodies want to stick to it and activate it and cause it to become destroyed what are these antibodies these antibodies that we now kind of like stick to this area here that are sticking to these particular antigens here on the pathogen these are IGA antibodies these are IG a antibodies and these immune complexes what they will do is they will go in and they deposit in a weird area they don't deposit into the GBM believe it or not and what they do is they deposit into this tissue that's nearby it's called the mesangium that purple stuff there it will then go and they will deposit into the mesangium and when they deposit into the mesangium they will cause a lot of inflammation so what are they going to do they're going to cause mesangel deposition so the cosmosangel deposition and what that'll do is that'll kind of activate an inflammatory response it won't activate the complement system though but it will lead to inflammation that causes glomerular basement membrane damage but the key thing is that you see there's no complement activation here so that's the big difference here is that you're going to get mesangel deposition GBM damage and what happens when you damage the GBM what do you lose again um is it is going to be ingrained into your brain red blood cells white blood cells protein is it nephrotic range no and these are going to be lost into the urine there's one other thing that's a little weird and what makes this kind of a little bit more easier to diagnose these IGA antibodies that are bound to these kind of like little immune complex to these antigens from a GI or upper respect infection they can also not only deposit into the mesangium they can deposit into other areas they like to go and deposit into a couple places so that way we remember this it can go and deposit into the skin into the abdomen or into the joints if it deposits into the skin it causes purpuric lesions these like purplish lesions on the baby's butt on the child's butt it also can deposit into the git and this will cause abdominal pain and it can also deposit into the joints and cause joint pain so what you want to look for is a young child who just recently had a respiratory or GI infection who's presenting with hematuria and potentially vasculitic lesions prepared lesions abdominal pain and joint pain that is the big difference here if you see this vasculinity this additional step here this indicates hinock Sean preparera so IGA nephropathy is just this part it's just this part I'm going to put nephropathy but if it's IG nephropathy and these other vasculitic lesions it's hinok seanlin purpura all right does this one cause rapidly Progressive glomerulonephritis no all right does this cause complement activation no all right let's go to the next one lupus nephritis what's the key thing here they have a history of lupus or if it is not diagnostic you have to find features of Lupus look for that Mala rash or the discordial rash or anemias and other types of features of lupus but this is the big thing and lupus there's some type of tissue cell damage their cells are popping open they're being damaged and when these cells are damaged they release out their nuclear material there's nuclear material that are supposed to be present in these cells maybe some DNA maybe some nuclear proteins things like that but when they bust open they release out these antigens and these antigens love to go and bind with specific antibodies that are you know the problematic issue in these diseases guess what these antibodies are called oh it's so beautiful anti-nuclear antibodies anti-double-stranded DNA antibodies if it couldn't get any easier right all right so they will go and they will bind to these particular antigens the problem is that these Sons of Guns will go and deposit into the glomerular basement membrane you know where they like to deposit they don't like to make our lives any easier so with sub-epithelial mesangel they decide to be a little bit of cold and wet there they could do subepithelial and sub-endothelial overachievers I think so you got sub endothelial so between the GBM and endothelium and you got sub epithelia between the podocytes and between the GBM and that's all going to deposit in there so what are you going to get sub ithelial and sub endothelial immune complex deposition and from here this is going to lead to what the same exact thing this did you get those immune complexes in there they're highly desirable for the complement system so it leads to C3 complement activation this will cause C3 consumption so you're going to burn through that and it's going to cause GBM damage because they're going to cause so much inflammation so now look at this you get a lot of these you get a lot of this you get a lot of this you increase your C3 consumption and you cause a lot of GBM damage whoo and as a result of the GBM damage what do you get in the urine again red blood cells white blood cells and protein oh I'm about to mess with you though a little bit is it nephrotic range protein yes this is one of the few that causes nephrotic range proteinuria with nephritic syndrome features so I want you to remember that this will happen to represent this with a lot of up arrows this will have nephrotic range proteinuria it's one of the few nephritic syndromes that can have nephrotic range proteinuria unfortunately this is what Selena Gomez had all right all right we come to this last one here for the immune complexes oh before we do that actually lupus nephritis I already told you I kind of gave you a little hint here that post-treptococcal IGA he knocked Charlotte preparera and lupus nephritis they all can cause what rpgn this will also cause rpgn so you can see rapidly Progressive glomerulonephritis with lupus nephritis and you can see rapidly Progressive glomerulonephritis with IGA nephropathy plus or minus 10 electron and lupus nephritis so again three types of immune complexes that show rpgn is lupus nephritis IGA nephropathy and post streptococcal and then anti-gbm and Ankle vasculitis also can present with rpgn which ones do not present with rpgn hereditary nephritis and membranoproliferative glomerulonephritis okay the last one here is membranoproliferative glomerulonephritis for the immune complex deposition the key thing for this one is that oftentimes it's primary so oftentimes it's primary we don't in other words it's idiopathic we don't really know why it happens it's just it's there okay sometimes it can be secondary and the big things to remember is like Hepatitis B virus hepatitis C virus and a disease called cryoglobulinemia which is where you get a lot of these proteins that precipitate at um less than 37 degrees Celsius but in these particular situations whether it be idiopathic or primary idiopathic which is primary or secondary to these particular diseases what happens we've got to make things a little bit more confusing mpgn has two types type 1 type 2. type 1 what happens is whether it's idiopathic we don't know the reason or it's due to some type of infection some way shape or form our body has an antigen that our antibodies like so we have these antibodies and unfortunately there's no specific name to these antibodies they're just antibodies there's some degree of IGG antibodies but we just don't have a beautiful name for them unfortunate but there is some antigen whether that's idiopathic and we don't know what that antigen is or whether it's due to an infection or cryoglobulin proteins these things forms immune complexes these immune complexes love to go and deposit into the GBM and when they deposit into the GBM these ones here they like to cause a lot of deposition here and these ones cause a lot of that sub-endothelial deposition so what you'll get from this one is you'll get a lot of sub endothelial deposition and this will lead to again what kind of effects here you'll get C3 activation and GBM damage oh so if I activate the C3 system I'm going to increase my consumption of C3 and if I cause a lot of inflammation from these immune complexes I'm going to cause a lot of GBM damage and what does the GBM damage look like in these patients well if you don't know it by now you're better right red blood cell loss white blood cell loss and protein loss but I'm going to mess with you again [Music] you're going to see all of these but the question comes is it nephrotic range protein area yes there's only two diseases that have a nephritic syndrome with a nephrotic range protein area it is lupus nephritis and membranoproliferative glomerulonephritis all right so so far what I've noticed from this one is it's a little bit complicating it's a little tough because I don't have a special antibody that's causing these immune complex depositions I just I just don't have it I just know that they're depositing there they're causing compound activation GBM damage the other one is type 2. type 2 is a little bit odd there is an antibody there is an antibody and we know the name of this antibody you know what this antibody is called it's called nephritic Factor it's called nephritic Factor this is again for type 2 nephritic Factor that's what this thing is what it does is it binds with a special protein that circulates through our body naturally you know what that protein is called C3 convertase it's called C so this antibody is called the phritic factor this blue protein is called C3 convertase once these things bind together they become crazy active and what they do is something really cool they take a molecule so imagine this complex now takes a molecule called C3 oh that sounds familiar let's actually make it black just so we can keep consistent with this we have this molecule here C 3. what it's going to do is is it's going to literally take the C3 convertence when this antibody binds to C3 convertase imagine it causing it to become supercharged and that C3 convertex will tear through the C3 and convert it into c3a and c3b so what I'm going to do is I'm going to represent this by this Arrow here this reaction is highly effective and you are going to massively increase the amount of c3a and c3b proteins and guess where they like to go and deposit yeah you guessed it they like to go and deposit into the glomerular basement membrane and when they deposit into the glomerular basement membrane they make these big dense deposits in the GBM and cause GBM damage so what will you see out of the result of this you'll see glomerular basement membrane damage due to these proteins depositing in here you're going to get a lot of this damage here what happens as a result of uh glomerular basement membrane damage I'm not going to write it down red blood because I don't have any room but red blood cell loss in the urine white blood cell loss in the urine and protein loss in the urine is the protein a product range yes because the only two types that give nephrotic range proteinuria as a nephritic syndrome is remember in a proliferative glamoronephritis and lupus nephritis the differences between these is this is an immune complex which is formed by an unknown antibody and something else could be these could just be we don't know what type two is it is an antibody we know nephritic Factor binding to A protein that we do know C3 convert taste causing lots of these proteins to become hyperactive that's the big difference here so my friends with all of these what I want you to take away is which one was in pure antibody deposition and to GBM which one was Anka associated with immune system deposition andcovasculitis which one was hereditary alpore syndrome which ones were immune complex that caused rapidly Progressive glomerulonephritis boom boom boom which were the ones that actually could potentially lead to in these particular situations 3c3 consumption boom boom boom which one did not cause C3 consumption boom now with these which is the most common type of nephritic syndrome boom that's the big things that I want you guys to take away from this now what we need to do now is say okay I talked about a lot of nephritic syndromes how am I supposed to be able to recognize truly the difference between nephritic and nephrotic and then how am I supposed to be able to determine the exact type of nephritic syndrome let's talk about that all right my friends so now on to the next part which is how do I diagnose nephritic syndrome and truly be able to differentiate that between nephrotic syndrome right because they can kind of be a little confusing well when we talk about these in nephrotic syndrome we said that the biggest thing to start off with is a urinalysis of microscopy because it's going to often give you at least a Prelude to thinking about which one it is so if I look at the urinalysis with microscopy between these two what were the big differentiating features well first off the pathophysiology the basic pathophys nephrotic was podocyte dysfunction all right so this is podocyte dysfunction whereas nephritic syndrome the classic theme is there's GBM dysfunction so there's some type of glomerular basement membrane dysfunction whether this was antibody immune complex immune like white blood cell mediated or hereditary all of it was GBM dysfunction this one led to heavy heavy protein urea and heavy lipiduria and the key kind of big thing between these is that the protein urea here we said can't be truly Quantified with like a urinalysis like the dipstick it can give you like if it's plus one plus two plus three plus four this will be pretty positive it could be like plus three plus four protein urea right but we're not going to actually be able to quantify if it's greater than three and a half grams or more per day we'll save that for the next step what we could see is that there's a lot of lipid urea as well and here's the other thing that can be somewhat helpful is with that lipid urea when you look at this under the microscope you'll see those fat oval bodies that we talked about that's consistent in patients who have what's called nephrotic syndrome in patients with nephritic syndrome where their GBM dysfunction occurs they lose a bunch of different cells they lose red blood cells and I told you there was a very specific thing that when you lose the red blood cells it'll show that they're going to have a lot of red blood cell loss white blood cell loss and protein loss so they'll have red blood cells in the air what do we call that we call that hematuria so that's hematuria and when if we were to actually examine this underneath the microscope it would show those things called the red blood cell Cass or it could even show the acanthocytes and we said that that was some big buzzword terms so watch out for lots of these kind of microscopic hematuria the other thing is we said that we could find lights of lots of white blood cells but we said that this was called a sterile pyuria it's not as diagnostic but it is helpful to know because these patients will have no bacteria right they should have leukoc esteresis are negative nitrites are negative and they shouldn't really have any urinary tract symptoms that's kind of the big feature here so we'll see that and then the last thing here is that we'll have proteinuria but is the protein Uria going to be like plus three plus four proteinuria no it may be like plus one plus two it's sub-nephrotic range with the exception what are the two exceptions so we have to make like a little kind of like disclaimer here that this is also not necessarily the case for which two don't follow this rule this would be the lupus nephritis and membranoproliferative glomerulonephritis for these two this is the I'm going to put a little like exception we'll put ex these are the except or exception exception and these they will have plus three plus four proteinuria all right that's the exception but that's kind of one way of starting off by differentiating between nephrotic and nephritic syndrome now if I come to the next part here which is like okay I gotta go and say is it the urine protein the 24-hour urine protein is the next step so if we come down here what we'll notice is we come to this next part which is being able to quantify the protein loss so how do I know if I've lost more than 3.5 grams per day or less than 3.5 grams per day so in this particular situation here for nephrotic syndrome I said it was podocyte dysfunction right so podocyte dysfunction and if we come over here we say that this is going to be glomerular basement membrane dysfunction now the big thing to remember here is that this will help us to quantify so when we want to quantify the protein loss that's really the key feature and what I can do is I can do a 24-hour urine protein where if a process of 24 hours I collect their urine or I could do a one-time spot urine albumin creatinine ratio this is a great thing you can do it one time done and it's a good representation of the amount of protein loss that they're having so if I look here for nephrotic syndrome I would see proteinuria for both of them right but what's the dead giveaway the protein urea that you're going to notice for nephrotic syndrome will be greater than or equal to how much it'll be so they'll have proteinuria but theirs is going to be greater than or equal to 3.5 grams this will be less than 3.5 grams and that's the big difference between these two is you're going to have lots of proteinuria lots of proteinuria but the difference is is that in the nephrotic syndromes it'll be greater than 3.5 grams per day and the nephritic syndromes it'll be less than 3.5 grams per day with the two exceptions so that's why I want you to remember here what are the two exceptions here the two exceptions are lupus nephritis and membranoproliferative glomerulonephritis these are the exceptions where they will have the actual 3.5 so we'll just put that these will be greater than 3.5 greater than or equal to 3.5 these are the only ones with that particular nephrotic syndrome based proteinuria but they have other features of nephritic syndrome so we kind of call them like a mixed or an intermediate kind of type once we've done this we should have a pretty good idea now if a patient has nephrotic or nephritic syndrome but there's one other thing I told you that really you should be on guard of so when a patient has glomerulonephritis it can be a nephrotic or it can be a nephritic syndrome but what is really helpful is a decline in renal function because I told you right nephrotic syndromes yes nephrotic syndromes have the capacity no doubt about it to cause renal failure but nephritic syndromes can cause a very dangerous and Rapid type of renal failure and that's where I want you guys to understand here the incidence of acute kidney injury and CKD is mountly higher significantly higher in patients who have what nephritic syndromes because of what the risk of rapidly Progressive glomerulonephritis and that is why this is the one that's a little bit more dangerous and this is the one that if you were to test the renal function you did a BMP you would see more evidence of what a reduction in GFR and increase in the bun and increase in the creatinine so these are the things that if you were to test you would see an increase in the bun the creatinine and a decrease and the GFR so an increase in the bun the creatinine in a decrease in the GFR could be indicative of glomerular injury right more specifically seen in nephritic syndrome as compared to nephrotic syndrome remember nephrotic syndromes like focal segmental glomerulosclerosis membranous nephropathy diabetes amyloidosis can cause CKD they can also to some small degree cause Aki but you're more likely going to see this with your nephritic syndromes okay let's now move into the next part which is okay we have now diagnosed a patient pretty clearly with nephritic syndrome how do we know what the specific cause of the nephratic center is an anti-gbm mycovasculitis hereditary immune complex let's go through that next we think we have a patient with the phritic syndrome what do we want to do now okay we want to go ahead and say and think okay I know that it's GBM dysfunction I just don't know how the GBM is getting destroyed and dysfunctional well I know that if I we think about anti-gbm disease that was one particular way well how would I know if it was anti-gbm disease take into consideration the hints that I told you about so in other words what you want to be looking for for this particular patient is is there any features of hemoptysis right so is there any hemoptysis that's one particular thing that could be kind of like a trigger to think about this but then you can check the labs so when I do the labs for these particular patients what's the specific antibody that deposits into the glomerular basement membrane they would have a positive anti-glomerular basement membrane antibody so a positive anti GBM antibodies and that would kind of be like The Telltale sign I wouldn't really need to do much else other than that right well that's the first one pretty straightforward right so look for evidence of hemoptysis and if that's not enough look for that term of having the positive anti-gbm antibody all right what about the Anka vasculitis I think the big things to think about for this one is it's kind of like pretty obvious here you need to test positive for the anchas so look for the actual anchas so if I have a positive C anchor so that's the the C anchor what did I tell you that was suggestive of I told you that was suggestive of GPA which is also known as Wagner's granulomatosis so if I see a positive C anchor I definitely want to go thinking about GPA what's another thing that also can make me think about GPA just to add to the the help of diagnosing it look for evidence of chronic sinusitis that's usually a really really big one so look for chronic sinusitis and whenever you see that in the exam plus them also having features of nephritic syndrome that also adds to the diagnosis of GPA or Wagner's if they are positive for the P anchor what you want to look for here to really help you is if it's p anchor positive I want to know is it eosinophilic polyangiitis or is it microscopic polyangiitis right and this is also known as churg Strauss so how do I differentiate between these two well I told you that in microscopic polyangiitis there's really no eosinophilia there's no asthma whereas and let's actually just so that we have space here we're going to swap these up here this is going to be eosinophilic poly angiitis and microscopic apologies but a microscopic polygienist they'll be p ancha and they'll have no asthma or eosinophilia an eosinophilic polyangiitis they will have positive features of asthma and maybe it's positive four eosinophilia and maybe even other features of allergies that's suggestive of EPA okay so look for the anchas and depending upon which one it is it can kind of stratify out the different types all right hereditary nephritis or alport syndrome it's really key we already said this it's just a constant reminder look for features of sensory neural deafness look for features of what else look for anterior lenticonus so lens problems anterior lenticonus in their exam question look for features of sensory neural deafness which you can determine by that Weber and Renee's test and then you're usually going to see this in some type of child who also has an associated glomerular lymphatic syndrome type of presentation okay and usually this might have to be some type of genetic testing that you'll do all right here's where it gets a little bit more challenging immune complex deposition how am I supposed to differentiate these things all right here's the first thing I want you to do I want you to go ahead and say okay which one which one had the anti ASO antibodies which ones had the anti dnase B antibodies that was post streptococcal glomerulonephritis post-treptococcal glomerulonephritis and that would be one way so if I found positive anti-aso positive anti-as-b dna-sp I'm going to think about post streptococcal myonephritis what are some other big things that would cue you off in the history a recent strep infection so look for also just like an add-on recent and when I say recent we actually should be very because they'll try to trip you up on this in the exam one to two weeks status posts strep infection all right one to two weeks just be careful because they may present like the child had an upper respiratory tract infection which could be strep one to two weeks ago whereas if they say one to two days ago they had a upper respiratory tract infection it could be IGA nephropathy so just be careful all right if I said on top of this all right here's another add-on that I could do here just to make make things even more interesting what happened with the C3 levels do you guys remember which ones were the ones that caused complement pathway consumption psgn lupus nephritis and membranoproliferative glomerulonephritis all consume C3 levels so what would the C3 levels be for this one it would be low so if they have a low C3 level that would be potentially suggestive of post streptococclomerulonephritis all right what's another one all right let's keep going let's say I go to the next one which is I have low C3 levels again okay well that only leaves me with a select few if I have a low C3 level that's only lupus nephritis post-treptococcal and membranoproliferative that makes things a little easy what if I said that they're antibodies that were positive is anti uh neutrophilic or sorry I'm sorry Anka I'm sorry a a anti-nuclear antibodies I apologize anti-nuclear antibodies are positive or anti double-stranded DNA antibodies are positive if I said this what would you think about what would be the one that you would think about lupus nephritis lupus nephritis and look for in their history some degree of a history of SLE or look for features of SLE okay if I said this patient also has a low C3 level so I'm consuming my complement proteins well I already got psgn I got lupus nephritis what's this what's the last one again oh remember no proliferative because ignifrop is the only one that does not have low C3 that's helpful well is there any antibodies that are really positive for this one not really there's not really anything specific because remember I told you the IGG antibodies that bind to some idiopathic or the infections there's not something that we specifically look for with that one and the nephritic factor antibody is not really something as well you can potentially take those into consideration so sometimes if possible you can consider testing for that nephritic factor so you can consider testing for the nephritic factor which was that in type 2. but oftentimes this one's not super obvious it's really not super obvious and often times for this one you may have to progress to a biopsy you may have to progress to a biopsy to confirm the membranoproliferative glomerulonephritis it's not an easy one right sometimes you have to go looking potentially for a biopsy to really really confirm this one because it's a tough one to find the last one here is if that C3 level is completely Stone Cold normal so if the C3 level is normal what could I do could I test any antibodies you can you can test for the IGA antibodies but they're not very specific they're not very good so they're not very good tests to kind of really go off of even though it really makes perfect sense they're not a great one what you would want to look for is is there any evidence of IgA vasculitis is there any evidence of like Enoch Sean Lynn pepera so in other words do they have the skin the prepared lesions the git problems the abdominal pain or the joint problems that's very very helpful okay and another thing that's really really key to kind of like really nailing down the diagnosis of IgA nephropathy he knocked Sean and pepera is looking for that key kind of like buzz term in here which is the child just recently had one to two days status posts upper respiratory tract infection or a git infection so the antibodies aren't super great looking for evidence of IgA vasculitis in the form of phenot Shaolin paper is really helpful and looking for that recent history of infection is even more important so off of this you can kind of get an idea of how to differentiate these things so right away check the C3 levels if the C3 levels C3 levels are low it's these if the C3 level is normal it's likely this if they have very specific antibodies that can point to these if it doesn't really have a specific antibody you may be thinking about mpgn right especially if the history isn't here and oftentimes you really may need a biopsy to confirm that one but if the history is all perfectly there the C3 level is completely Stone Cold normal and you've got that vasculating lesions you got to think about the most common type of nephritic syndrome all right so that leads us to The Next Step here I've done all these tests the serology has been pretty good man it really has kind of led me there may be like one uh maybe two of these that are really kind of tougher to diagnose the only time a renal biopsy is truly necessary is if the cause of nephritic syndrome is super unclear and it's affecting your treatment or if they're developing renal failure like rpgn or they have persistent hematuria that's just not responding to therapy otherwise we can honestly get the diagnosis without a biopsy if you get the point here but if we did do a biopsy we take a chunk of the kidney tissue and we take that tissue and we look on it under three different types of microscopy one is we look at it under what's called light microscopy it gives a general kind of like idea a general change in the glomerulus a general look at the glomerulus electron microscopy gives us a very detailed look at the actual GBM the glomerular basement membrane super super detailed look but what we can actually do is we can do an immunofluorescence and that's going to look for those antibodies or immune complexes that are depositing into the GBM and highlight that make them super green and fluorescent and so what you're going to notice is this is a really helpful test and light microscopy is also actually pretty helpful so let's say that we go to the light microscopy what is it really good for one of the things I can get out of this one is I can get a general look a general detailed change in the glomerulus and that's really helpful because if I notice this what is this crescents if I notice I have some crescents that are present some Crescent some glomerular crescents this was suggestive of r p g n what were the diseases that were associated with rpgn let's see if you guys remember come on anti GBM which is also known as good pastures Anka vasculitis and there was three particular types of immune complex diseases post-treptococcal glomerulonephritis IGA nephropathy and lupus nephritis these all can cause these glomerular crescents which where's the parietal epithelial cell proliferation and then a lot of the fibrotic sclerotic tissue this would lead you to think about these and these are best diagnosed with this all right cool well if they don't have the glomerular crescents that only leaves two that we didn't mention in all of this madness what are we missing we did anti-gbm anchovasculitis hereditary nephritis that one was one that's not mentioned here and what's another one up for the immune complex mpgn all right when you look at the light microscopy for mpgn or even so you can get light microscopy and even electron microscopy is actually pretty good as well so even if you did light microscopy and electron microscopy it actually gives this really cool look at the GBM and what happens is at the GBM both both mpgn one and two both mpgn one and two have this weird thing where here's your glomerular basement membrane and here's what's called your mazanium and what happens is these mesangial cells stick a part of their process into the GBM and they give it this weird type of configuration which when we see this this is that buzzword term and why biopsy is helpful for mpgn you hear the term tram track sign when you hear that term tram track sign I want you to associate that with mpgn type 1 or type 2. it's the big thing my friends so once we've done this we've done either light microscopy electron microscopy and we found tram tracking sign we think about MPG in one MPG and two which is an immune complex deposition the last one is actually the easiest one thank goodness that leaves alport syndrome you're missing Type 4 collagen so it causes a thin very thin and split glomerular basement membrane and when you think about that one I want you to think about alport syndrome alport syndrome all right my friends we moved to the next component here which is I've done the light microscopy what I really can use this for is I can say is it alport done I don't really need to do anything else if I find this on alport I'm done if I see this on mpgn so if I see the tram tracking sign it could be type 1 type 2 I still don't know if it's type 1 or type 2 yet so that's I still got to do some further testing and then if I see glomerular crescents it could be any of these I just don't know which one it is so how do I delineate this all right watch this for the linear it's only one type only one type gives these antibodies that deposit in a linear fashion on immunofluorescence and that's anti-gbm that's anti GBM disease so good pasture done if it's Posse immune what the heck does that mean there's no immune complex you see any immune complexes in here no what's the only one of these that I mentioned besides alport syndrome that has no antibody or any immune complex deposition angle vasculitis so Anka vasculitis will show no immune complex deposition technically if you wanted to be very very particular even you know alport syndrome won't have that as well all right we'll have Posse immune but that's one right there okay that leaves this last one granular the granular one could be post-treptococcal it could be IGA nephropathy it could be what else well generally it's post-treptococcal lupus nephritis and membranoproliferative glomerulonephritis type one and type two IG nephropathy probably won't have to really do this part here but here's what you'll do and I actually put the IG nephropathy over here because it does something a little bit different but when you look at the GBM you're looking for this granular appearance that could be psgn that could be lupus nephritis that could be mpgn1 and that could be mpgn2 how do I know the difference because they're all granular this one takes on what's called a lumpy Village I'm not even kidding the lumpy bumpy appearance all right and it's usually sub-epithelial deposition this one is sub epithelial and so we'll put sub epithelial and sub endothelial deposition mpgn it gives immune complexes immune complex deposition mpgn gives C3 dense deposits that's kind of the big difference here so that's what you're looking for Within These to kind of really highlight the specific type of nephritic syndrome so once we've gone through all of this if we haven't been able to figure it out off of the serology some of the historical factors you can do a biopsy get the light microscopy look for the crescents if you see the crescents it's any of these how do I differentiate immunofluorescence if it's linear a GBM if it's Posse immune andcovasculitis is it one of these you have to go and look at the actual granular appearance and then some specifics to the granular appearance if it's lumpy bumpy subepithelial psgn if it's sub-epithelial and sub-endothelial it's lupus nephritis if it's immune complex deposition in the sub endothelial layer it's mpgn1 and if it's C3 dense deposits it's mpgn2 the only last one that I want to add in here because it doesn't even deposit really into the GBM it deposits into the mesangium you guys remember that one the mesangel deposition that would be IGA nephropathy so if you see this one this would be IGA nephropathy and that would be indicative of mesangel deposition so we'll write that one up here mesangel deposition all right that's the big difference here all right so now we've gone through we've figured out every single type of nephritic syndrome in great detail how do we treat this thing so when we talk about the treatment of nephritic syndrome it's really treating the complications and it's nothing crazy proteinuria what would do we do for that nephrotic syndrome we kind of tried to be careful with the protein diet so maybe just back off a little bit on that protein diet you might have to lose the gains a little bit the other thing I told you which was that cool mechanism was the ACE inhibitors and the arbs these were really cool as well why well you guys remember that ACE inhibitors and arbs what was really cool about these puppies is that they kind of worked on that efferent arterial remember they helped to cause like the efferential vasodilate which helped to allow for more things to leave the glomerulus so it would be these or ACE inhibitors or arbs and these are great for that edema fluid and sodium restriction would be great because you're already going to be retaining a lot of that and then if that doesn't work if the fluid fluid and sodium restriction doesn't work then you can consider diuretics hypertension what's the drug that would inhibit because what's the problem with this one we said it was that low GFR led to renin Angiotensin system activation ACE inhibitors arbs these are the best because they're going to shut down that renin Angiotensin aldosterone system here is treating the cause and the real reason why we have to be able to treat this is because we can't let these patients develop renal failure they have a very high incidence of renal failure if the patient ends up leading to the risk of rapidly Progressive glomerulonephritis which we said was a bunch of these right which ones did we say was the particular scary ones that I want you guys to be able to realize the ones I said that are really important to be able to realize here is anti-gbm anchor vasculitis or post streptococcal glomerulonephritis IGA nephropathy and lupus nephritis all of these have the potential to cause rapidly Progressive glomerulonephritis so what we want to do is in this particular scenario for these is you really want to start off with steroids so we're going to do steroids acutely okay we're going to do steroids acutely and if the patient's renal function does not improve what we would want to consider here right so if the renal function continues to decline so let's put like there's the renal function continues to decline then we will progress to something else and it's only specific to two types of diseases we could do something called plasma phoresis and Plasma Freeze is where you're basically trying to like strip all the immune complexes out of the bloodstream that's causing this problem and the two diseases that this would be good for is going to be anti-gbm and Anka vasculitis that's the only two diseases that this would actually help so anti GBM and Anka vasculitis these are kind of like your emergent therapies to really get this under control if you gain control so if renal function maybe continues to improve then what you want to be able to do from here is is prevent further destruction so you can see this in rpgn so this would be an rpgn and membranoproliferative glomerulonephritis so now we're at this point here where we tried the steroids acutely we got it under control if it was rpgn if it still wasn't under control then we go to plasmapheresis right specifically for these if the renal function is actually stable so let's say stable renal function maybe at some degree stable but we still don't want this disease to progress what we may have to do is if they have rapidly Progressive glamoronephritis or MPGs so we'll put this or mpgn then you may need to do some type of long-term immunosuppressant so then you may need to do a long term immunosuppressant right and the drugs that we usually do in this particular situation would be things like cyclophosphamide and tacrilimus these are generally pretty good drugs for these particular indications so cyclophosphamide and tacro ilimus now sometimes in this particular situation for the rpgns we definitely want to get steroids going and get the plasmapheresis you can consider also giving steroids and the membranoproliferative glomerulonephritis as well all right but you generally will not go to plasmapheresis ever from membranoproliferative glomerulonephritis it's usually only rpgn that is of this particular flavor so RPG and our membrane of proliferative glomerulonephritis with only the rpgns or these you do steroids acutely if they have mpgn you can also do steroids acutely if the patient continues to have deterioration of the renal function and they have anti-gbm or ankle vasculitis you can do a plasmapheresis if the renal function is stable but you want to prevent the continual progression of the rpgn or the mpgn then you can add on a long-term immunosuppressant like cyclophosphamide or tacrolamus all right engineers in this video we talk about nephritic syndrome I hope it made sense I hope that you guys enjoyed it and as always until next time [Music] foreign [Music]

Channel: Ninja Nerd

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Length: 89min 49sec (5389 seconds)

Published: Fri Jun 30 2023