Nephritic Syndrome - classification, pathophysiology, treatment (RPGN, ANCA, Immune complex)

Hello, in this video we are going to talk about glomerulonephritis and the different types. Glomeruli are the functional units of the kidneys. Maryland blue nephritis, which people know as

nephritic

syndrome

, is caused by inflammation of the glomerulus that results in microscopic hematuria or macroscopic site proteinuria. leuco or urea without evidence of infection, so it is sterile, in addition to hypertension, other features include acute kidney injury from inflammation and, depending on the etiology, may have additional kidney involvement, such as rash and alveolar hemorrhage or even alveolar hemorrhage , which can be caused by a variety of conditions including auto

immune

, hereditary, and infectious diseases glomerular nephritis is not a nephrotic

syndrome

information from the glomerulus causes glomerular nephritis

nephritic

syndrome and this damages the glomerular capillary the porous basement membrane allows the Red blood cells leak, causing hematuria.

Red blood cells can join together in the renal tubule forming red blood cell casts. Red blood cells that pass through the costs and tubules can become dysmorphic red blood cells called can Thersites. damaged cells that normally prevent proteins from escaping cause proteinuria typically less than 3.5 grams in 24 hours due to inflammation in the glomerulus white blood cells such as neutrophils are usually recruited to the area and can also leak through the capillaries causing pyuria which is sterile because there is no infection is sterile pyorrhea the information that occurs in the glomerulus reduces glomerular filtration reduces the rate of fluid passing, which causes all the urea and also causes acute kidney injury with elevated creatinine, in addition, the Inflammation causes a retention of urea, so reducing the glomerular filtration rate will stimulate the renin-angiotensin-aldosterone system, which will cause hypertension and this is due to sodium. and water retention by cutting a cross section of the head of the nephron in the glomerulus it is important to appreciate the normal surrounding architecture the parietal cells house Bowman's space which is where we find the glomerular capillaries that are surrounded by the basement membrane around the membrane Basal it is the parasites and the projections of their feet that normally prevent proteins from leaking here is Bowman's space where things leak from the mesangial capillaries.

The cells can be found in the intercapillary spaces and have a role in regulating the glomerular filtration rate as well as phagocytosis and promoting inflammation. There are many causes of glomerular nephritis and it may be part of a spectrum if proteinuria exceeds 3 .5 grams in 24 hours has a nephrotic component different causes of glomerular nephritis and what I mean specifically is nephritic syndrome includes rapidly progressive glomerulonephritis anteed antibody disease of the glomerular basement membrane also known as Goodpasture syndrome associated glomerular nephritis or vasculitis glomerulonephritis mediated by

immune

complex

es that includes IgA nephropathy post-tryptic glomerulonephritis and lupus nephritis and finally another cause is membrane or proliferative glomerulonephritis let's look at each of these in a little more detail starting with the most severe or severe form of glomerular nephritis, which is rapidly progressive glomerulonephritis, now rapidly progressive glomerulonephritis, which is enteric glomerulonephritis Chris.

It is actually not a disease itself, but is caused by other diseases of glomerular nephritis, such as antiglomerular basement membrane. Antibody disease or Goodpasture ANCA-associated glomerular nephritis Immune

complex

-mediated glomerulonephritis Rapidly progressive glomerulonephritis is characterized by a loss of global filtration rate of at least 50 percent and pathological findings of extensive glomerular crescents. Crescents form after the rupture of a glomerular capillary wall leading to the accumulation of macrophages, fibroblasts and epithelial cells and fibrin within Bowman's space, and this leads to the enteric image of Chris seen under the microscope, as You can see, the rupture of the capillary walls will cause the accumulation of many cells in Bowman's space, inflammation in the area, and the release of cytokines. promotes the proliferation of parietal cells this means a serious injury the

treatment

of rapidly progressive glomerulonephritis depends on the disease caused next let's talk about antiglomerular basement membrane disease also known as Goodpasture's disease Goodpasture's disease is a disorder in which the Circulating antibodies are directed against an antigen intrinsic to the glomerular basement membrane in the kidneys as a result, this will present with acute or rapidly progressive glomerulonephritis that is typically associated with crescent formation.

The majority of up to 90% of patients with Goodpasture's disease present clinical features of rapidly progressive glomerulonephritis, the primary target of anti-glomerular basement membrane antibodies is the NC domain of the alpha 3 chain of type 4 collagen that is highly expressed in the glomerular basement membrane of the kidneys, as well as in the alveoli of the lungs. Antibodies can also target the alpha 5 chain, which are also linear deposition of these antibodies across the globe. alveolar hemorrhage and a small proportion of patients may present isolated pulmonary findings. Treatment for antiglomerular basement membrane disease or Goodpasture's disease includes plasmapheresis to remove antibodies after intravenous and then oral cortical steroids, as well as cyclophosphamide, which is an immunosuppressive agent, following glomerulonephritis. is anchor associated vasculitis causing immune necrosis of the horse and enteric guerrillas Chris now these are big words but essentially this category refers to a type of glomerular nephritis in which there are little or no immune deposits by immunofluorescence or electron microscopy interestingly eang Associated vasculitis accounts for the majority of cases of rapidly progressive glomerulonephritis.

Angliss stands for antineutrophil cytoplasmic antibody and these are antibodies produced by plasma cells that target proteins within neutrophil granules and monocyte lysosomes. Two antibodies are produced: P ANCA against Milo peroxidase and see ANCA against neutrophil proteinase. three P ANCA activates neutrophil granules and c-

anca

can activate the lysosomal function of macrophages and monocytes, as well as neutrophils. Activation of macrophages and neutrophils produces renal vasculitis. Most patients with limited renal vasculitis are positive and many have or will develop systemic symptoms. of granulomatosis with polyangiitis or microscopic polyangiitis, anger-associated glomerulonephritis is characterized by a prodrome of malaise, arthralgia, myalgia, and flu-like symptoms, as well as acute kidney injury.

As mentioned, ANCA autoantibodies will activate neutrophils and this will allow extravasation of neutrophils out of the glomerular. capillary, neutrophils will infiltrate the area, causing acute inflammation and necrosis. Monocytes will begin to infiltrate the area as a second line and, over time, the influx of inflammatory processes will cause chronic change involving scarring and fibrosis. An important key point about ANCA-associated glomerular vasculitis or nephritis is that there is no immune deposition in the immunofluorescence

treatment

of ANCA-associated glomerular nephritis that includes induction treatment with steroids and rituximab or cyclophosphamide, followed by maintenance therapy. with azathioprine mycophenolate or rituximab for at least 12 months after stable remission has been achieved, unlike Anka-associated glomerulonephritis, which does have no immune complex deposition or immunofluorescence deposition.

Immune complex-mediated glomerulonephritis does. This is the next type of glomerulonephritis. Immune complex glomerulonephritis refers to the presence of immune deposits in the glomeruli due to any cause. In most cases, serial logic and histological findings will point to the underlying disease, it has three main types, actually IgA nephropathy, where you can see miss angel and glomerular capillary wall immunoglobulin, a stool. The second type is poststreptococcal glomerulonephritis and here there are deposits of immune complexes that form some epithelial humps and, obviously, there is also the presence of anti-shift Akaka antibodies in the blood. The third type of immune complex that mediates terminal nephritis is nephritis. lupus only in lupus nephritis is there the presence of antinuclear antibodies and you have what is called complete immunofluorescence staining and this involves staining basically a large number of immune things such as IgG IgA IgM C 3 and C 1q the deposition can be observed in the Subendothelial and subepithelial mesangium.

In summary, the

pathophysiology

of glomerular immune complex nephritis involves the deposition of immune complexes in the glomerulus and this will activate the resulting classical complement pathway. in inflammation, for this reason, most immune complex-mediated glomerular nephritis is associated with low levels of c3 and c4. IgA is an exception. Immune complex glomerular nephritis will be discussed in more detail in a separate video and you can click the link below to view the final version. The type of glomerulonephritis is a member of non-proliferative glomerular nephritis and remember that non-proliferative bright nephritis is not a disease in itself, but it is actually a pattern of injury seen in glomerulonephritis and product syndrome.

It is important to remember that the membrane of proliferative glomerular nephritis has nephritis and nephrotic symptoms is diagnosed based on renal biopsy with histopathology of the renal kidney demonstrating thick mesangial expansion basement membrane and a characteristic tram track finding with a double contour of the membrane. basal glomerular remember that there is no proliferative glomerular nephritis can be classified into two main types mediated by immune complex fighters prolific membrane or Illumina and membrane-mediated nephritis or proliferative chimera mediated by complement in the immune complex Clemente membrane-mediated or proliferative in the fetus , these types can be from a primary cause, also known as idiopathic cause, or secondary to an infection, autoimmune disease, Alice, as well as paired anemia: the immune complex that is deposited in this area will activate the complement response.

Complement activation occurs via the classical pathway and is usually manifested by a normal or slightly decreased serum c3 concentration and a low serum c4 concentration. Complement-mediated membrane or proliferative glomerulonephritis may also be a primary cause, also known as idiopathic cause, or genetic causes, such as complement deregulation, complement-mediated membrane or proliferative criminal nephritis activates the alternative pathway directly. , which subsequently promotes the inflammatory response resulting in glomerular nephritis, there are generally low serum levels of c3 and normal levels of c4. due to the activation of the alternative pathway, I will have a separate video that will go into more detail about proliferative glomerular nephritis of the limb ganglia to conclude the video on glomerular nephritis, which is a nephritic syndrome, not a nephrotic syndrome, and again the main ones we talked about.

It is rapidly progressive glomerulonephritis, which is the most serious and is not a disease in itself, but is caused by other nephritic conditions. You have Goodpasture syndrome, also known as glomerular basement membrane disease T. You have immune complex-mediated glomerulonephritis. We have Anker-associated glomerulonephritis. nephritis and finally we talk about proliferative glomerulonephritis that shares nephritic and nephrotic symptoms.