Pathology of Glomerular Disease Flashcards
What is the location of the immunecomplex deposition?
Is circulating or formed in situ?
what is this a hallmark for?
The location of the immune complexs get trapped from the circulation in the subendothelial location of the cappillary leading to an in situ formation of immune complexes in the subendothelium and subsequent inflammatory response.
Later, the complexes dissociate and migrate across the BM and reform on the subepithelial side of the GBM.
The complexes then activate the compliment system and reform in the hallmark “hump-like” deposits of post streptococcal GN
What type of pattern is this?
What does this pattern imply?
Granular patten of immunoflorescence
a granular pattern on immunoflorescence is a characteristic of immune complex deposition
What is this?
what cells are proliferating?
what is it characteristic of?
This is crescentic glomerulonephritis
the parietal epithelial cells (on the bowman capsule) that are proliferating in the extracapilary space.
This is characteristic of rapidly progressie glomerulonephritis (RPGN)
Rapidly Progressive Glomerulonephritis
is a complication of _______?
what pattern will we see on IF?
where do the cells proliferate?
Rapidly Progressive Glomerulonephritis
is a complication of ANY of the immune complex nephritides
we will see a granular staining pattern on IF
The proliferating cells are extrapillary
What type of pattern is shown?
What type of nephritis does it suggest?
What is the antibody?
This is a linear pattern of staining on IF because the antigens are intrinsic to the basement membrane and are therefore diffused and fixed
This is suggestive of Anti-GBM antibody induced glomerulonephritis
the antigen is a component of the noncollagenous domain of the alpha 3 chain of the type IV collagen
How would this patient present?
What does this patient have?
kidney only?
lung involvement?
how would we treat the pulmonary hemorrhage?
This patient would present with a rapid onset of HTN, oliguria (small amounts of urine) and will have renal failure within weeks to months if not treated.
If only kidney’s are involved this is Anti-GBM
if there is lung involvement = goodpasture’s syndrome
pulmonary hemorrhage is a medical emergency and requires plasmapheresis
What type of glomerulonephritis is this?
what type of immune complexes are present?
- if the pt presents with:
- granulomatous inflammation (sinuses involved)?
- granulomatous inflammation and asthma?
- Skin, lung, and kidney involvement?
This is type III RPGN (ANCA associated glomerulonephritis or Pauci Immune crescentic GN)
ANCA stands for antineutrophil cytoplasmic antibodies
there is a lack of detectable anti-GBM or immune complexes by immunoflorescence and EM
- If associated with inflammation/sinus = granulomatosis with polyangiitis (Wegener’s) C-ANCA
- + asthma involvement= Eosinophilic granulomatosis ith polyangiitis (Churg-Strauss)
- dermato-pulmonary-renal syndrome (P-ANCA)
What is this pattern of injury?
what structures are involved?
what are their changes?
this is membranoproliferative glomerulonephritis.
This pattern of injury shows thickening of the capillary wall (tram-track appearance) or a double contour, and a hypercellularity
mesangiocapillary involvement
Dense Deposit Disease:
- subcategory of what?
- etiology?
Dense Deposit Disease is a type of Membranoproliferative Glomerulonephritis. It is associated with excessive activation of the alternative complement pathway
Associated factors are C3 nephritic factor and a mutation in Factor H
Where are the immune complexes located in MPGN?
immune complexes in MPGN are subendothelial
what is this?
what causes it?
Double contours of the duplicated basement membrane in MPGN pattern
The double contour formation is due to the remodeling of the GBM after endothelial cell injury
what is this?
where is the accumulation of immunoglobulin deposits?
what is the cause of proteinuria?
this is membranous nephropathy- a diffuse thickening of the capillary membrane wall.
the thickening is due to immunoglobulin deposits along the subepithelial side of the membrand (near the podocytes) this causes the capillary wall to become leaky causing massive proteinuria
What is this?
what happens to the podocytes?
what would we see on silver stain?
on IF?
immune complexes between the basement membrane and overlying podocytes with effacement of foot processes
the BM membrane material is laid down between deposits- seen as irregular spikes on a silver stain
granular deposits
Primary or idoipathic membranous neuropathy
what is the causative antigen?
where does it form:
in situ/circulation
sub endo/epithelium
Primary MN is caused by he renal autoantigen Phospholipase A2 receptor present in teh visceral epithelial cell (podocyte) membrane
deposits form in situ along the subepithelial aspect of the BM
Secondary MG
etiology:
endogenous antigens:
exogenous:
other causes:
Secondary MG
injury results from deposition of circulating immune complex that dissociates and reforms in the subepithelial region
it is a chronic immune mediated disease
endogenous antigens: Autoantibodies in SLE
exogenous: Hep B or C
other causes: drugs (penicillamine and NSAIDs)
what is this?
what causes it?
pathology?
what will we see under light microscopy?
are immune complex deposits present?
Minimal change disease
caused by a diffuse effacement of foot processes of podocytes
unknown pathology
will be normal on light microscopy
NO immune complex deposits
Focal:
Segmental:
genetic involvement:
Focal: <50% of glomeruli are involved
Segmental: only a portion of the affected glomerulus shows a lesion
Apolipoprotein A1 gene (APOL1) on chromosome 22
What is this?
what stain?
what is the prognosis?
This is collapsing glomerulopathy
it is a silver stain
shows proliferation and hypertrophy of the visceral epithelial cells and a collapse of the glomerular tuft
collapsing glomerulopathy has the worst prognosis of all FSGS
