Pathology

Question 1

Name the diameter of the fibrils in amyloid, fibrillary, and immunotactoid, cryoglobulin, collagenofibrotic glomerulonephritis?

Answer 1

Amyloid: ~ 10 nm
Fibrillary: ~ 20 nm
Immunotactoid: ~ 30 nm
Cryoglobulin: 30 nm
Collagenofibrotic: 60 nm

Question 2

What is the treatment for rapid progressive crescentic IgA nephropathy (> 50% decline in eGFR over 3 months)?

Answer 2

Cyclophosphamide + Steroids

Question 3

What types of amyloid are associated with the following:
Heroin use?
Senility?
Chronic inflammation?
Malignancy?

Answer 3

Heroin=AA
Senility=ATTR
Chronic inflammation=AA
Malignancy=AL

Question 4

Name the six classes of lupus nephritis?

Answer 4

I: Minimal mesangial
II: Mesangial proliferative
III: Focal nephritis (< 50% glomeruli)
IV: Diffuse nephritis (> 50% glomeruli)
V: Membranous nephritis
VI: Advanced sclerosing

Question 5

Crescentric GN IF findings:
Linear pattern?
Negative staining?
Granular pattern?

Answer 5

Linear = Anti-GBM disease
Negative = Pauci immune ANCA
Granular = Immune complex mediated

Question 6

How does Dense Deposit Disease appear on EM?

Answer 6

Continuous C3 deposits inside GBM (young patients)

Question 7

How does infection associate GN appear on IF?

Answer 7

Granular IgG, C3, and/or IgA
- IgG disappears leaving C3 behind
- IgA staining indicates Staph infection

Question 8

How does Fabry Disease appear on electron microscopy?

Answer 8

Lamellar lysosomal inclusions inside podocytes = Zebra bodies

Question 9

Why do the tubules appear pushed away from each other in ATN?

Answer 9

Interstitial edema

Question 10

How can you differentiate Alport Syndrome from Thin Basement Membrane Disease on electron microscopy?

Answer 10

Alport Syndrome has a thin GBM which is also irregular. No splitting or irregularity seen in Thin Basement Membrane Disease.

Question 11

How is Thin Basement Membrane Disease diagnosed?

Answer 11

GBM < 220 nm (normal is 280-350 nm)

Question 12

What are the three genetic defects seen in Alport’s Disease?

Answer 12

COL4A3 (autosomal recessive)
COL4A4 (autosomal recessive)
COL4A5 (X-linked recessive)

Question 13

What cells and in what conditions are tubuloreticular inclusions observed in?

Answer 13

Endothelial cells (lupus nephritis, chronic viral infections)

Question 14

What condition is the fingerprint sign observed in?

Answer 14

Cryoglobulin GN

Question 15

What is the treatment for TMA associated MPGN?

Answer 15

Eculizumab

Question 16

What is the latent period for infection associated GN?

Answer 16

1-3 weeks for strep pharyngitis
3-5 weeks for skin infection

Question 17

How do complement levels differ in infection associated GN and immune complex MPGN?

Answer 17

IAGN: C3 low, C4 variable
IC MPGN: C3 low, C4 low

Question 18

How does HIVAN differ from HIVICK with regards to microscopy findings?

Answer 18

HIVAN: collapsing FSGS
HIVICK: lupus like immune complexes (full house IF)

Question 19

Between amyloid, fibrillary, immunotactoid, and collagenofibrotic glomerulopathy, how are the deposits oriented in tissue?

Answer 19

Amyloid: random
Fibrillary: random
Immunotactoid: organized
Collagenofibrotic: organized

Question 20

Between amyloid, fibrillary, and immunotactoid, which has a rare association with malignancy?

Answer 20

Fibrillary

Question 21

Which autoantibodies can be seen in fibrillary GN?

Answer 21

Autoantobodies directed against DNAJB9

Question 22

How does the normal kappa lambda ratio shift in those with CKD?

Answer 22

Upper limit of normal shifts from 1.7 to 3.1 due to kappa being less efficiently excreted than lambda

Question 23

What does sucrose nephropathy look like on light microscopy, and what drug can cause a similar appearance?

Answer 23

Vacuolization of proximal tubular epithelial cells can be seen with sucrose (ie IVIG) and tacrolimus exposure

Question 24

Which forms of Alport’s disease can also be diagnosed by skin biopsy?

Answer 24

X linked ONLY