Sketchy Path: The Nephritic Syndrome

Question 1

Many cases of nephritic syndrome are caused by ______________.

Answer 1

immune deposits in the subendothelial space or mesangium (like how the green mace on the Mongol’s back is between the endothelial camp and the basement membrane wall)

Question 2

Immune deposits lead to _____________ activation.

Answer 2

complement (like the guys high fiving and complimenting each other)

This is why serum C3 levels will be decreased and neutrophils will be attracted to C5a.

Question 3

Inflammatory infiltrate leads to ______________.

Answer 3

destruction of the endothelium and basement membrane (like how the neutrophil first responders are carrying away the injured endothelium Mongol)

This is what causes hematuria.

Question 4

Like nephrotic syndrome, nephritic syndrome is defined by clinical signs and symptoms. List the features.

Answer 4

• Hematuria (like the red river leaking under the wall)
• Azotemia (like the BUN and credit card near the baby Ghengis Khan)
• Oliguria (like the spilled soda cup by the Mongol camp)
• HTN (like the steam coming out of little Ghengis’s ears)
• Periorbital edema (like Ghengis’s squished face)
• Proteinuria, but less than in nephrotic syndrome (like the meat in the river by the dead Mongol)

Question 5

In the urine of someone with nephritic syndrome, you’ll see _____________.

Answer 5

dysmorphic RBCs and RBC casts (like the beat up Mongol and the group of red helmets floating in the river) as well as WBC casts (like the guy dressed in white floating in the river)

These both tell you that the bleeding occurred in the glomerulus. A bleed in the ureter, for instance, will not have these.

Question 6

The most common nephritic syndrome is ____________.

Answer 6

IgA nephropathy (like the IgA-evoking dummies that the young Mongol prince is attacking in the mesangium green field)

Question 7

Why does IgA deposit in the mesangium?

Answer 7

It’s thought that in some people IgA is incorrectly glycosylated and gets secreted into the serum instead of the mucosa.

Question 8

In addition to IgA nephropathy, GI pain, and purpura, Henoch-Schonlein purpura can cause _____________.

Answer 8

arthritis (like the Mongol captain’s sore ankle)

Question 9

In IF analysis of Berger disease, look for ______________.

Answer 9

deposits in the mesangium (like the green dummies in the grass)

Question 10

IgA nephropathy usually responds to ______________.

Answer 10

steroids (like the prince’s moon shield)

Question 11

Where do the immune deposits accumulate in PSGN?

Answer 11

They start out in the mesangium (like the kid eating the pie in the field), but then move to the basement membrane and subepithelial space (like the other kid throwing pies on the foot soldier).

Question 12

Light microscopy of a glomerulus with PSGN will show _________________.

Answer 12

hypercellularity of the mesangium with inflammatory infiltrate (like the first responders carrying the berry pie in the mesangial field)

Question 13

IF of PSGN will show _______________.

Answer 13

granular deposits (like the green dots on the pie tins)

Question 14

Although the S. pyogenes organisms will be gone by the time of PSGN, you can test for _______________.

Answer 14

anti-streptolysin O and anti-DNAse B (like the donuts)

Note: ASO is degraded by oils in the skin, so in a PSGN from impetigo you will only detect DNAse.

Question 15

PSGN in adults can lead to ______________.

Answer 15

RPGN (crescentic glomerulus degeneration)

Question 16

Describe the most common renal symptom of lupus.

Answer 16

Diffuse proliferative glomerulonephritis presents with expanded mesangium and endothelium (like the pack of wolves and the group of Mongols in the ring surrounded by antibodies to the DNA-shaped wire).

Question 17

_________________ is a common light microscopy finding of lupus nephritis.

Answer 17

Wire-looping of the capillaries (like the looped wires around the endothelium camp)

Question 18

IF of lupus nephritis will show _______________.

Answer 18

granular deposition (like the dotted green wire)

Question 19

Lupus nephritis usually progresses to kidney failure, but ________________ can slow the progression.

Answer 19

steroids and cyclophosphamide (like the Mongol’s moon shield in the ring)

Question 20

Describe the pathogenesis of MPGN.

Answer 20

Mesangial proliferation leads to membrane proliferation leading to a nephritic/nephrotic syndrome.

(Think of the Mongol guy digging into the wall from the mesangial side.)

Question 21

What causes the “tram-tracking” seen in MPGN?

Answer 21

Growth of mesangium into the basement membrane!

Think of the guy digging from the mesangium into the wall and causing a track through it.

Question 22

In MPGN, immune deposits (from HBV, HCV, monoclonal gammopathies, etc.) stick in the ____________ space.

Answer 22

subendothelial (like the string of green lanterns behind the guy digging into the wall – it’s between the wall and the endothelial camp!)

Question 23

On light microscopy, MPGN will show _____________.

Answer 23

a lobular glomerulus (like the wall guy’s lobular shovel)

Question 24

What is dense deposit disease?

Answer 24

A mysterious disorder in which an unknown dense substance deposits in the mesangium and leads to ribbon-like degradation of the membrane (like the mysterious gray stones in the wall put by the other Mongols)

Question 25

MPGN is considered a ______________ disease.

Answer 25

mixed nephritic/nephrotic (like how the proteinuria meat cart is on the wall by the MPGN guy)

Question 26

DDD seems to be caused by _____________.

Answer 26

the alternative complement pathway (like the three-way compliments being dealt out by the dudes in the wall) with C3 nephritic factor (like the bracelets)

Question 27

What is RPGN?

Answer 27

Rapidly progressive glomerulonephritis is a clinical diagnosis that results from breaking of the basement membrane. It is defined as rapid-onset renal failure with crescent formation in the glomerulus.

(Think of the Mongols rapidly crossing the wall through the break with the crescent-shaped weapon.)

Question 28

What can cause RPGN?

Answer 28

• Immune complex disorders –all of them (like the green mace held by the Mongol captain)
• Pauci-immune disorders (like the archer targeting the first responders –ANCA –without any green on him)
• Goodpasture’s (like the archer shooting arrows at the wall from the mesangial space)

Question 29

What forms the crescent in RPGN?

Answer 29

• Fibrin and plasma proteins (like the twigs leaking through the cracked wall)
• Proliferating epithelium and inflammatory cells (like the Chinese army responding to the break in the wall)

Question 30

The immune deposits in diffuse proliferative glomerulonephritis are in the _________ space.

Answer 30

sub-endothelial (like how the green fence is around the endothelial camp)