Summary Slides and Pics Nephritis Flashcards

1
Q

Membranoproliferative Glomerulonephritis

  • Presentation?
  • Cause
  • Laboratory Marker?
A

Membranoproliferative glomerulonephritis

Presentation
• may present with Hematuria
• non-nephrotic Range Proteinuria
• Nephrotic Syndome
• Acute Nephritic Syndrome
RPGN

Cause
• Often associated with HEPITITIS C in adults (sometime hep B)
• Ideopathic

Laboratory
Associated with low C3

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2
Q

Membranoproliferative Glomerulonephritis

• Changes on LM, IF, and EM

A

LM:
• Hypercellularity duplication of the GBM with double contour appearance

IF:
• Complement Deposition in a rim pattern outlining the glomerular capillary wall

EM:
Type I Thickening of the Glomerular capillary wall

• DDD (type II membranoproliferative) = Ribbon-like appearance of subendothelial and intramembranous material

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3
Q

IgA Nephropathy
• Prevlance
• Pathogensis
• part of glomerulus affected

A

IgA Nephropathy

Prevalence:
• Most common glomerulonephritis worldwide

Location:
MESANGIUM

Pathogenesis:
Aberrent Glycosylation of IgA hinge region leading to auto-antibody formation against IgA

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4
Q

IgA Nephropathy

• what would you expect to see on LM, IF, and EM?

A

LM:
• Variable, may see mesangial Expansion

IF:
Dominant or Co-dominant deposition of IgA

EM:
Mesangial Deposits

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5
Q

Anti-GBM disease (Goodpasture’s)
• pathogenesis
• Key finding on biopsy?

A

Rapid Development of Renal Failure due to AUTOANTIBODIES binding to the noncollagenous portion of the alpha-3 subunit of type IV collagen

• IF = LINEAR PATTERN

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6
Q

Hereditary Nephritis (Alport’s syndrome)
• pathogenesis
• key finding on biopsy

A

Alport’s
• Defects in alpha-5 type IV collagen

Biopsy = EM = Abnormally thin basement membranes with splintering of the lamina desna causing a Basket Weave Appearance

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7
Q

What nephritic disease affects the tissue in blue?
• nephritic disease that affects tissue in green?

A

Blue:
• IgA nephropathy

Green:
• Membranoproliferative glomerulonephritis

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8
Q

What nephritic disease(s) is this image indicative of?

• Key features?

A

• Membranoproliferative Glomerulonephritis and Dense Deposit Disease

Key Features:

  • The glomeruli are large and lobular
  • Cell proliferation – mesangial, endothelial and leukocytes.
  • The GBM is thickened
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9
Q

What changes can be observed between the glomeruli shown here? (normal on the left)

A
  • GBM thickenening
  • Increased Cellularity
  • If this were zoomed out you would also see that these glomeruli are large and lobular
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10
Q

Diagnosis?
• explain the appearance of the glomerulus shown here.
• This disease may be secondary to what other disease?
• Key lab Findings?

A

Membranoproliferative Glomerulonephritis TYPE I

  • Glomerular appearance = TRAM TRACKS, this is due to mesangial cells trying to eat immune complexes in the SUBENDOTHELIUM
  • May be secondary to Hepatitis C infection
  • C3 levels are often LOW/Depleted in these patients
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11
Q

What is happening here?
• what would you expect to see on immunofluorscence of this biopsy?

A

Mesangial Cell has penetrated into Subendothelium to scavenge immune deposits

This is TYPE 1 Glomerulonephritis so patient will have Immunofluorescence for C3 in a GRANULAR PATTERN

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12
Q

What key marker can be used to help differentiate between Type I and Type II (aka Dense deposit disease) MembranoPROLIFERATIVE Glomerulonepthris?

A
  • C3 nephritic factor that STABILIZES C3 convertase = key finding
  • Shown here is dense deposit disease, notice more of a ribbon appearance on the EM
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13
Q

Is Membranoproliferative GN Type I or Type II shown here?

A

This is MPGN type II aka Dense Deposit Disease - notice bright specs.

Below is a picture of MPGN type I - more evenly granular appearance

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14
Q

Describe the major differences between the appearance of the disease shown on the right and the one shown on the left.

A

Left: MPGN type I, you can see splitting of the subendothelium

Right: Dense Deposit Disease, you can see deposits in the subendothelium, but they do not evenly split the membrane like in type I

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15
Q

What disease may have lead to this appearance on H and E considering the patient had low serum C4.

• Mediating antibody type?

A

Hepatitis C may have caused Membranoproliferative Glomerulonephritis. MPGN is associated with high MONOCLONAL IgM levels.

CHRONIC high levels of monoclonal IgM may lead to MULTIPLE MYELOMA

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16
Q

Other than looking for C3 nehpritic factor, how could you differentiate MPGN type I and type II on the basis of complement?

A

Type I - associated with CLASSICAL COMPLEMENT so you would expect C4 depletion

Type II - is associated with alternative complment so you would see a more rapid depletion of C3

17
Q

What disease is shown here? KEY features?
• would you expect there to be elevated levels of inflammatory proteins in the serum? if so which ones?

A

IgA Nephropathy

IgA may or MAY NOT be elevated in the serum so don’t bank on that (its 50% chance)

Key feature here is proliferation of the MESANGIUM this is shown by increased black color on the Silver Stain

Shown below is what a normal silver stain looks like

18
Q

T or F: immune mediated diseases of the glomeruli most often lead to Nephritis rather than nephrotic syndrome.

A

True, INFLAMMATION means its a nephritic condition

19
Q

T or F: increased cellularity is a feature you should look for in all immune mediated nephropathies.

A

True, these will be inflammatory processes so you should definetly see increased cell numbers

20
Q

What is the ONLY antibody that could be mediating that this immunofluorescent dye could be oriented against? why?

A

IgA - the dye is concentrated in the mesangium, IgA nephropathy is the only one where you see Mesangial Antibody deposits

21
Q

What is shown here?

• Risk factor for this pathology?

A

Electron Dense Deposits in the mesangium indicative of IgA nephropathy aka Berger’s disease (associated with smoking)

22
Q

What disease is shown here?
• what antibodies might you look for in this person?
• is this primarily a kidney disease?

A

This is a Rapidly Progressing Glomerulonephritis resulting from Goodpasture’s Syndrome. This is PRIMARILY a kidney disease with some respiratory involvement. In contrast Wegner’s is a respiratory disease with some kidney involvment.

****NOTE: you know that this is a rapidly progressing glomerulonephritis because CRESENT proliferation is pushing down on the glomerulus

23
Q

What is seen here?
• disease most commonly associated with this pattern?

A
  • wire loops are seen here
  • Lupus associated, but you also see cresents starting to form which is suggestive of RPGN
24
Q

What disease PROCESS is shown here?

A

This is how crescent formation looks on Silver stain

25
Q

How is this disease aqcuired?
• Key EM features?

• KEY clinical features to pick up on?

A

Here you see basket weaving indicative of ALPORT syndrome which is acquired via X-LINKED inheritance

KEY clinical features: ASSOCIATED WITH HEARING LOSS and OCULAR DISTURBANCES

26
Q
A