Summary Slides and Pics Nephritis Flashcards
Membranoproliferative Glomerulonephritis
- Presentation?
- Cause
- Laboratory Marker?
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
Membranoproliferative Glomerulonephritis
• Changes on LM, IF, and EM
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
IgA Nephropathy
• Prevlance
• Pathogensis
• part of glomerulus affected
IgA Nephropathy
Prevalence:
• Most common glomerulonephritis worldwide
Location:
• MESANGIUM
Pathogenesis:
• Aberrent Glycosylation of IgA hinge region leading to auto-antibody formation against IgA
IgA Nephropathy
• what would you expect to see on LM, IF, and EM?
LM:
• Variable, may see mesangial Expansion
IF:
• Dominant or Co-dominant deposition of IgA
EM:
• Mesangial Deposits
Anti-GBM disease (Goodpasture’s)
• pathogenesis
• Key finding on biopsy?
• 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
Hereditary Nephritis (Alport’s syndrome)
• pathogenesis
• key finding on biopsy
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
What nephritic disease affects the tissue in blue?
• nephritic disease that affects tissue in green?
Blue:
• IgA nephropathy
Green:
• Membranoproliferative glomerulonephritis
What nephritic disease(s) is this image indicative of?
• Key features?
• Membranoproliferative Glomerulonephritis and Dense Deposit Disease
Key Features:
- The glomeruli are large and lobular
- Cell proliferation – mesangial, endothelial and leukocytes.
- The GBM is thickened
What changes can be observed between the glomeruli shown here? (normal on the left)
- GBM thickenening
- Increased Cellularity
- If this were zoomed out you would also see that these glomeruli are large and lobular
Diagnosis?
• explain the appearance of the glomerulus shown here.
• This disease may be secondary to what other disease?
• Key lab Findings?
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
What is happening here?
• what would you expect to see on immunofluorscence of this biopsy?
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
What key marker can be used to help differentiate between Type I and Type II (aka Dense deposit disease) MembranoPROLIFERATIVE Glomerulonepthris?
- C3 nephritic factor that STABILIZES C3 convertase = key finding
- Shown here is dense deposit disease, notice more of a ribbon appearance on the EM
Is Membranoproliferative GN Type I or Type II shown here?
This is MPGN type II aka Dense Deposit Disease - notice bright specs.
Below is a picture of MPGN type I - more evenly granular appearance
Describe the major differences between the appearance of the disease shown on the right and the one shown on the left.
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
What disease may have lead to this appearance on H and E considering the patient had low serum C4.
• Mediating antibody type?
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