Pathology Flashcards
Name the diameter of the fibrils in amyloid, fibrillary, and immunotactoid, cryoglobulin, collagenofibrotic glomerulonephritis?
Amyloid: ~ 10 nm
Fibrillary: ~ 20 nm
Immunotactoid: ~ 30 nm
Cryoglobulin: 30 nm
Collagenofibrotic: 60 nm
What is the treatment for rapid progressive crescentic IgA nephropathy (> 50% decline in eGFR over 3 months)?
Cyclophosphamide + Steroids
What types of amyloid are associated with the following:
Heroin use?
Senility?
Chronic inflammation?
Malignancy?
Heroin=AA
Senility=ATTR
Chronic inflammation=AA
Malignancy=AL
Name the six classes of lupus nephritis?
I: Minimal mesangial
II: Mesangial proliferative
III: Focal nephritis (< 50% glomeruli)
IV: Diffuse nephritis (> 50% glomeruli)
V: Membranous nephritis
VI: Advanced sclerosing
Crescentric GN IF findings:
Linear pattern?
Negative staining?
Granular pattern?
Linear = Anti-GBM disease
Negative = Pauci immune ANCA
Granular = Immune complex mediated
How does Dense Deposit Disease appear on EM?
Continuous C3 deposits inside GBM (young patients)
How does infection associate GN appear on IF?
Granular IgG, C3, and/or IgA
- IgG disappears leaving C3 behind
- IgA staining indicates Staph infection
How does Fabry Disease appear on electron microscopy?
Lamellar lysosomal inclusions inside podocytes = Zebra bodies
Why do the tubules appear pushed away from each other in ATN?
Interstitial edema
How can you differentiate Alport Syndrome from Thin Basement Membrane Disease on electron microscopy?
Alport Syndrome has a thin GBM which is also irregular. No splitting or irregularity seen in Thin Basement Membrane Disease.
How is Thin Basement Membrane Disease diagnosed?
GBM < 220 nm (normal is 280-350 nm)
What are the three genetic defects seen in Alport’s Disease?
COL4A3 (autosomal recessive)
COL4A4 (autosomal recessive)
COL4A5 (X-linked recessive)
What cells and in what conditions are tubuloreticular inclusions observed in?
Endothelial cells (lupus nephritis, chronic viral infections)
What condition is the fingerprint sign observed in?
Cryoglobulin GN
What is the treatment for TMA associated MPGN?
Eculizumab
What is the latent period for infection associated GN?
1-3 weeks for strep pharyngitis
3-5 weeks for skin infection
How do complement levels differ in infection associated GN and immune complex MPGN?
IAGN: C3 low, C4 variable
IC MPGN: C3 low, C4 low
How does HIVAN differ from HIVICK with regards to microscopy findings?
HIVAN: collapsing FSGS
HIVICK: lupus like immune complexes (full house IF)
Between amyloid, fibrillary, immunotactoid, and collagenofibrotic glomerulopathy, how are the deposits oriented in tissue?
Amyloid: random
Fibrillary: random
Immunotactoid: organized
Collagenofibrotic: organized
Between amyloid, fibrillary, and immunotactoid, which has a rare association with malignancy?
Fibrillary
Which autoantibodies can be seen in fibrillary GN?
Autoantobodies directed against DNAJB9
How does the normal kappa lambda ratio shift in those with CKD?
Upper limit of normal shifts from 1.7 to 3.1 due to kappa being less efficiently excreted than lambda
What does sucrose nephropathy look like on light microscopy, and what drug can cause a similar appearance?
Vacuolization of proximal tubular epithelial cells can be seen with sucrose (ie IVIG) and tacrolimus exposure
Which forms of Alport’s disease can also be diagnosed by skin biopsy?
X linked ONLY
