Nephritis

Question 1

Membranoproliferative disease tends to have deposits where ?

Answer 1

Immune complex deposits in the subendothelial space

Question 2

Where does IgA disease have immune complex deposition ?

Answer 2

Mesangial location

Question 3

Lupus nephritis has immune deposits in what location ?

Answer 3

Subendothelial

Question 4

Post - Infectous glomerulonephritis has immune complexes in what location ?

Answer 4

Subepithelial

Question 5

In membranoproliferative gn, IgA nephropathy and lupus where are the immuen complexs formed ?

Answer 5

Outside the glomeruli and trapped there in the process of filtration

Question 6

In post infectious gn where are the the immune complexes formed ?

Answer 6

They are formed in situ in the glomeruli with antigens and antibodies arriving separately

Question 7

What happens when the subendothelial immune complex deposits hurt the endothelial cells

Answer 7

Thrombus formation and the release of cytokines and autocoids that will activate the complement system and upregulate adhesion molecules

Question 8

What is the mechanism of hematuria ?

Answer 8

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.

Question 9

What is an uncommon disease that commonly presents with symptoms of nephritic and nephrotic syndrome

Answer 9

membranoproliferative disease

Question 10

What are the basic aspects of Membranoproliferative disease ?

Answer 10

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

Question 11

What is type 1 memo-Prolif-glom disease caused by ?

Answer 11

Type one is mediated by immune complexes causing activation of complement by the classical pathway.

Question 12

What does MGPD type 1 actually do to the glomeruli ?

Answer 12

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.

Question 13

What does mebranoproliferative disease present with ?

Answer 13

It is an immune complex disease that will progress to renal failure. Remember low level of C3 complement because the cascade will remain activated

Question 14

What is type 2 membranoproliferative disease called ?

Answer 14

Dense Deposite Disease

Question 15

Who is most often affected by dense deposit disease ?

Answer 15

Older cildren and young adults and it typically follows an upper respiratory infection.

Question 16

Is dense deposit disease an immune complex disease ?

Answer 16

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.

Question 17

What do the antibodies develop against in Dense Deposit Disease ?

Answer 17

They are against Factor H, Factor I, Factor B, C3 convertase (Nephritic factor )

Question 18

Is Buergers disease an IgA nephropathy ?

Answer 18

No , Berger’s disease is but bUERGers disease is not

Question 19

What happens in IgA nephropahty ?

Answer 19

The deposition of polymeric IgA1 in the mesangial matrix

Question 20

What is a common cause of Nephritic syndrome ?

Answer 20

IgA nephropathy is a common cause of nephritic syndrome and is most common in children especially east asians.

Question 21

How does IgA nephropathy differ from post-strep glomerulonephritis ?

Answer 21

It is synpharyngitic because glomerulonephritis with nephritic urinary sediment typically occurs within 1-2 days of the infection.

Question 22

How is IgA nephropathy differed from other glomerulonephropathis ?

Answer 22

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.

Question 23

Anti-GBM disease is characterized by autoantibodies against ?

Answer 23

The Alpha 3 chain of type 4 collagen.

Question 24

Basket Weave

Answer 24

Alport.

Question 25

What are some of the characteristic features of Alports syndrome ?

Answer 25

Slowly progressive nephritis, deafness and lens disease due to mutation in the gene for the alpha 5 chain of type 4 collagen

Question 26

What is the nephritic factor and what disease is it depleted in ?

Answer 26

C3 convertase and it is depleted in dense deposit disease

Question 27

What is a thin basement membrane disease ?

Answer 27

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.

Question 28

IgA Nephropathy ?

Answer 28

Common nephritic syndrome with deosits of underglycosylated IgA in the mesangium

Question 29

What is rapidly progressive Glomerulonephritis ?

Answer 29

Syndrome of hematuria and rapidly progressive renal failure correlated with crescentric glomerulonephritis

Question 30

Alport Syndrome ?

Answer 30

Deafness, lens disease, and slowly progressice nephritis due to defect in alpha 5 chain of type 4 collagen

Question 31

What is membranoproliferative disese characterized by ?

Answer 31

• 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

Question 32

Tram Tracks

Answer 32

Membranoproliferative

Question 33

Patient has Hep C Microscopic hematuria and non-nephrotic range proteinuria. What is the problem ?
Low level of C3

Answer 33

MPGN Type 1

Question 34

C3 nephritic factor ?

Answer 34

Dense Deposit Disease