Flashcards in Nephritis Deck (34):
Membranoproliferative disease tends to have deposits where ?
Immune complex deposits in the subendothelial space
Where does IgA disease have immune complex deposition ?
Lupus nephritis has immune deposits in what location ?
Post - Infectous glomerulonephritis has immune complexes in what location ?
In membranoproliferative gn, IgA nephropathy and lupus where are the immuen complexs formed ?
Outside the glomeruli and trapped there in the process of filtration
In post infectious gn where are the the immune complexes formed ?
They are formed in situ in the glomeruli with antigens and antibodies arriving separately
What happens when the subendothelial immune complex deposits hurt the endothelial cells
Thrombus formation and the release of cytokines and autocoids that will activate the complement system and upregulate adhesion molecules
What is the mechanism of hematuria ?
The immune complexes will cause the glomerular capillaries to rupture and bleed into the urinary space. The injury is aggravated by the activation of the complement system.
What is an uncommon disease that commonly presents with symptoms of nephritic and nephrotic syndrome
What are the basic aspects of Membranoproliferative disease ?
It is a group of three disorders that show thickening of the glomerular membrane and infiltration of immune complexes. On light microscopy they will show an enlargement of the mesangial matrix and a tram track appearance on silver stain
What is type 1 memo-Prolif-glom disease caused by ?
Type one is mediated by immune complexes causing activation of complement by the classical pathway.
What does MGPD type 1 actually do to the glomeruli ?
There is an injury phase featuring an influx of leukocytes which leads to a proliferative phase where damage to the capillary walls will cause the release of blood.
What does mebranoproliferative disease present with ?
It is an immune complex disease that will progress to renal failure. Remember low level of C3 complement because the cascade will remain activated
What is type 2 membranoproliferative disease called ?
Dense Deposite Disease
Who is most often affected by dense deposit disease ?
Older cildren and young adults and it typically follows an upper respiratory infection.
Is dense deposit disease an immune complex disease ?
No it is caused by dysregulation of the complement system. The alternate pathway is inappropriately and excessively activated due to mutations in the regulatory proteins.
What do the antibodies develop against in Dense Deposit Disease ?
They are against Factor H, Factor I, Factor B, C3 convertase (Nephritic factor )
Is Buergers disease an IgA nephropathy ?
No , Berger's disease is but bUERGers disease is not
What happens in IgA nephropahty ?
The deposition of polymeric IgA1 in the mesangial matrix
What is a common cause of Nephritic syndrome ?
IgA nephropathy is a common cause of nephritic syndrome and is most common in children especially east asians.
How does IgA nephropathy differ from post-strep glomerulonephritis ?
It is synpharyngitic because glomerulonephritis with nephritic urinary sediment typically occurs within 1-2 days of the infection.
How is IgA nephropathy differed from other glomerulonephropathis ?
By immunofluorescence of the mesangial IgA deposits often with C3 and peroperdin. Electron microscopy will confirm the presence of the Electron dense deposits but IgA Neph lacks unique characteristics on E- imaging.
Anti-GBM disease is characterized by autoantibodies against ?
The Alpha 3 chain of type 4 collagen.
What are some of the characteristic features of Alports syndrome ?
Slowly progressive nephritis, deafness and lens disease due to mutation in the gene for the alpha 5 chain of type 4 collagen
What is the nephritic factor and what disease is it depleted in ?
C3 convertase and it is depleted in dense deposit disease
What is a thin basement membrane disease ?
Is is a group of autosomal dominant diseases with defects in the alpha-3 or alpha 4 chains of type 4 collagen causing uniform reduction in the glomerular basement membrane to about half its normal thickness.
IgA Nephropathy ?
Common nephritic syndrome with deosits of underglycosylated IgA in the mesangium
What is rapidly progressive Glomerulonephritis ?
Syndrome of hematuria and rapidly progressive renal failure correlated with crescentric glomerulonephritis
Alport Syndrome ?
Deafness, lens disease, and slowly progressice nephritis due to defect in alpha 5 chain of type 4 collagen
What is membranoproliferative disese characterized by ?
-Hypercellularity of the glomerular tuft
-Increased mononuclear cells within the expanded mesangium and within capillary lumens
-Capillary wall changes that result in the double wall contour
***Tram Track appearance
Patient has Hep C Microscopic hematuria and non-nephrotic range proteinuria. What is the problem ?
Low level of C3
MPGN Type 1