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1

What is the hallmark of Nephritic Syndrome?

Glomerular inflammation and hematuria

2

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

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

3

What will a biopsy of the glomerulus reveal?

Hypercellular and inflammed glomeruli

4

What will an IF show and why?

Granular IF, due to immune deposits

5

What will the EM show? Why?

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.

6

What is the tx for nephritic syndrome?

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

7

What causes nephritic syndrome?

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

8

Why is the glomerulus hypercellular upon biopsy?

The hypercellularity is due to the excess of neutrophilic infiltrates

9

What causes the damage in nephritic syndrome?

Neutrophil mediated damage.

10

What causes PSGN? (Post Strep Glomerular Nephritis)

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

11

Which are the "Nephritic Strains" that causes nephritic syndrome?

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

12

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

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

13

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

2-3 weeks after initial infection.

14

3 general features of PSGN?

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

15

Which age group is more prone to getting PSGN?

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

16

Does PSGN lead to renal failure?

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

17

Generally speaking what is Rapidly Progressing Glomerular Nephritis (RPGN)

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

18

What will a biopsy of Glomerulus reveal if patient has RPGN

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

19

What is the composition of the Crescent Cells?

Inflammatory debris like fibrin and Macrophages, NOT collagen.

20

What will the IF reveal for RPGN? Why?

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

21

What kind of IF pattern do we see in "Good Pasture's Syndrome?"

Linear IF.

22

What is "Good Pasture Syndrome?"

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

23

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

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

24

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

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

25

Which is the most common reason for death in SLE?

Kidney failure, commonly due to Diffuse Proliferative GN.

26

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

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

27

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

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

28

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

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

29

What are the general features of Wegener's Granulomatosis

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).

30

What is the triad of Churg Straus?

Granulomus inflammation, eosinophilia and asthma.

31

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

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

32

How do we treat IgA nephrotic?

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.

33

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

Yes.

34

Lab findings of IgA nephropathy?

Granular IgA deposits.

35

What is Alport's Syndrome?

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

36

Describe the pathophys of Alport's syndrome?

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.