Nephritic Syndrome

Question 1

What is the hallmark of Nephritic Syndrome?

Answer 1

Glomerular inflammation and hematuria

Question 2

What are the 7 features you expect to see clinically in a patient with nephritic syndrome?

Answer 2

1. Hematuria, 4+ on urine dipstick
2. Proteinuria, but less than 3.5 mg/day
3. Salt and water retention due to increased aldosterone secretion, leading to:
   a. HTN
   b. Periorbital edema
4. Glomerular inflammation
5. Azotemia
6. Oliguria
7. Renal RBC casts and dysmorphic RBC’s

Question 3

What will a biopsy of the glomerulus reveal?

Answer 3

Hypercellular and inflammed glomeruli

Question 4

What will an IF show and why?

Answer 4

Granular IF, due to immune deposits

Question 5

What will the EM show? Why?

Answer 5

Sub-epithelial humps, granular deposits begins to pule up on the endothelial cells and works its way to the epithelial cells, seen as “humps.” Eventually this pops off and goes back to normal.

Question 6

What is the tx for nephritic syndrome?

Answer 6

It is more or less self limiting so just supportive care is enough.

Question 7

What causes nephritic syndrome?

Answer 7

Immune-complex deposits itself into the glomeruli, starting from the endothelium and works its way up to the epithelium. This deposit activates the compliment.
1. C5a from the compliment will recruit neutrophils –> extensive damage to the glomeruli

Question 8

Why is the glomerulus hypercellular upon biopsy?

Answer 8

The hypercellularity is due to the excess of neutrophilic infiltrates

Question 9

What causes the damage in nephritic syndrome?

Answer 9

Neutrophil mediated damage.

Question 10

What causes PSGN? (Post Strep Glomerular Nephritis)

Answer 10

Can be others but usually Group A Beta hemolytic Strep infection of skin (impetigo) or esophagus. Generally Strep Pyogenes.

Question 11

Which are the “Nephritic Strains” that causes nephritic syndrome?

Answer 11

Strep that produces a specific type of M proteins, specifically the 12, 4, and 1 varients of the M proteins.

Question 12

What about the nephritic strains of Strep that makes you prone to nephritic syndrome?

Answer 12

The virulence factor found in M proteins. Disease is perpetuated by the presence of M proteins which the immune cells respond to.

Question 13

When do patient present with Nephritic Syndrome generally due to PSGN?

Answer 13

2-3 weeks after initial infection.

Question 14

3 general features of PSGN?

Answer 14

1. Hematuria
2. Oliguria
3. HTN and periorbital edema

Question 15

Which age group is more prone to getting PSGN?

Answer 15

Between ages 6-10 usually although adults can also get it.

Question 16

Does PSGN lead to renal failure?

Answer 16

Kids almost always fully recovers, adults can progress into Rapidly Proliferating Glomerular nephritis and then KF.

Question 17

Generally speaking what is Rapidly Progressing Glomerular Nephritis (RPGN)

Answer 17

A more serious complication of Nephritic Syndrome that can lead to renal failure within weeks or months if left untreated.

Question 18

What will a biopsy of Glomerulus reveal if patient has RPGN

Answer 18

It will reveal crescent cells in the bowman’s space, obliterating the capillary space that were normally thin walled to allow for easy diffusion.

Question 19

What is the composition of the Crescent Cells?

Answer 19

Inflammatory debris like fibrin and Macrophages, NOT collagen.

Question 20

What will the IF reveal for RPGN? Why?

Answer 20

Linear Immunofluoresence, because the AB’s line up in the GBM.

Question 21

What kind of IF pattern do we see in “Good Pasture’s Syndrome?”

Answer 21

Linear IF.

Question 22

What is “Good Pasture Syndrome?”

Answer 22

AB’s attacking the GBM and aveolar BM in the lungs, due to the presence of similar type of collagen.

Question 23

Who generally gets Good Pasture Syndrome and how do they present clinically?

Answer 23

Young adult males generally get Good Pasture Syndrome, they present with hematuira and hemeoptisis due to kidney and lung involvement, respectively.

Question 24

What syndromes will show a granular IF? Why is this the case?

Answer 24

PSGN most commonly, also Diffuse Proliferative GN, diffuse AB deposition found in the sub-epithelium.

Question 25

Which is the most common reason for death in SLE?

Answer 25

Kidney failure, commonly due to Diffuse Proliferative GN.

Question 26

What happens if IF comes out to be negative or inconclusive? What is this called?

Answer 26

Pauci-Immune, and we have to run the ANCA test (Anti-Nuclear Cytoplasmic Antibodies).

Question 27

What is c-ANCA and what can we conclude if it comes out to be positive?

Answer 27

Cytoplasmic ANCA, this means that the patient has Wegener’s Granulomatosis.

Question 28

What is p-ANCA and what does being positive for p-ANCA reveal?

Answer 28

Peri-nuclear ANCA, being positive for this means you can have Churg Strauss disease or Microscopic Polyangitis.

Question 29

What are the general features of Wegener’s Granulomatosis

Answer 29

Disease that involves both the lungs and the kidney, similar to Good Pasture’s Syndrome. It involves the infection of the nasopharynx (sinus infections, sinusitis, etc).

Question 30

What is the triad of Churg Straus?

Answer 30

Granulomus inflammation, eosinophilia and asthma.

Question 31

What is IgA nephropathy? Describe the pathophys and age group involved.

Answer 31

Seen most often in kids, sinus infection leads to production of IgA, which deposits in the mesangium of the glomeruli –> glomerular bleeding.

Question 32

How do we treat IgA nephrotic?

Answer 32

Secondary, tx the causative agent first. Symptoms goes away after sinus infection is taken care of, and returns if another sinus or mucosal infection occurs.

Question 33

Can IgA nephropathy progress to RF? (KF, Renal or kidney failure)

Answer 33

Yes.

Question 34

Lab findings of IgA nephropathy?

Answer 34

Granular IgA deposits.

Question 35

What is Alport’s Syndrome?

Answer 35

An x-linked disease that affects the type IV collagen.

Question 36

Describe the pathophys of Alport’s syndrome?

Answer 36

ABs produced against type 4 collagen which makes up the GBM as well as parts of the eyes and ears. As a result, GBM integrity is comprimised (visual and hearing disturbances are also seen) –> episodes of isolated hematuria.