B5-046 Renal Pathology II Flashcards
dominant features of nephritis
3
- hematuria
- declining GFR
- “active” urine sediment
subset of nephritis with relatively rapid onset
rapidly progressive glomerulonephritis
morphologic correlate of RPGN
glomerular crescents
3 causes of RPGN
- anti-GBM (type II HSR)
- immune complex (type III HSR)
- ANCA (type II HSR)
classically associated with group A strep
post-streptococcal glomerulonephritis
when do post-streptococcal glomerulonephritis symptoms begin?
delay, usually about 2 weeks post infections
symptoms of post-streptococcal glomerulonephritis
- gross hematuria
- hypocomplementemia
- decline in GFR
pathology of post-streptococcal glomerulonephritis
neutrophils, crescents
LM: glomeruli enlarged and hypercellular
IF: granular “starry sky” appearance due to IgG, C3 deposition along GBM and mesangium
EM: subepithelial immune complex humps
post-streptococcal glomerulonephritis
IF: IgG, C3
EM: subepithelial humps
post-streptococcal glomerulonephritis
- LM: crescent moon shape
- crescents consist of fibrin and plasma proteins with glomerular parietal cells, monocytes, and macrophages
RPGN
linear IF due to antibodies to GBM and alveolar basement membrane
Goodpasture
hematuria associated with simultaneous URTI
IgA nephropathy
- most common in young males
- normal complement levels
IgA nephropathy
episodic hematuria that occurs with RTI or GI infection
IgA nephropathy
LM: mesangial proliferation
IF: IgA based immune complex deposits in mesangium
EM: mesangial immune complex deposition
IgA nephropathy
IF: mesangial IgA deposition
EM: mesangial electron dense deposits
IgA nephropathy
Henoch-Schonlein tetrad
- nephritis
- rash
- arthritis
- purpura
- patient under 10 years old
- pathology more likely to have crescents but otherwise indistinguishable from IgA nephropathy
Henoch-Schonlein
multi-system autoimmune disorder common in young females
lupus nephritis
- variable presentation including both nephritic and nephrotic syndromes
- 6 different morphologic patterns
- proliferative lupus nephritis (class III and IV)
lupus nephritis
which classes of lupus nephritis are considered proliferative?
III and IV
IF: “full house”
EM: deposits in all glomerular compartments, esp subendothelial
lupus nephritis
nephritic syndrome that often copresents with nephrotic syndrome
membrano-proliferative glomerulonephritis
Ig+ associated with antigen excess due to infection like HCV or HBV
membrano-proliferative glomerulonephritis Type 1
Ig- associated with abnormal alternative complement activation
membrano-proliferative glomerulonephritis Type 2
can be secondary to hepatitis B or C, may also be idiopathic
membrano-proliferative glomerulonephritis Type I
associated with low C3 nephritic factor
membrano-proliferative glomerulonephritis Type II
- mesangial ingrowth causes GBM splitting
- “tram track” on H&E and PAS stains
membrano-proliferative glomerulonephritis
which type of membrano-proliferative glomerulonephritis has subendothelial immune complex deposits with granular IF?
Type I membrano-proliferative glomerulonephritis
which type of membrano-proliferative glomerulonephritis causes intramembranous deposits (also called dense deposit disease)?
Type II membrano-proliferative glomerulonephritis
LM: lobular hypercellularity, double contours
IF: IgG and C3
Type I membrano-proliferative glomerulonephritis
LM: lobular hypercellularity, double contours
IF: C3
Type II membrano-proliferative glomerulonephritis
auto-antibody to alpha 3 chain of type IV collagen
Goodpasture
- pulmonary-renal involement
- typically young adult males
Goodpasture
LM: glomerular crescents, no arteritis
IF: linear IgG and C3 in GBM
Goodpasture
associated with anti-neutrophil cytoplasmic antibodies
c-ANCA or p-ANCA
ANCA
causes systemic vasculitis affecting small arteries and glomeruli
ANCA
4 different ANCA syndromes
- GPA
- MPA
- EGPA
- renal-limited
may result in RPGN
2
ANCA
Goodpasture
IF is “pauci-immune”, meaning only minimal, non-specific staining
ANCA
negative IF with no IgC3 deposition
ANCA
pauci immune
inherited mutation in type IV collagen causing irregular thinning and thickening and splitting of GBM
Alport
- hematuria
- deafness
- chronic progressive renal decline
Alport
mutations associated with Alport
- COL4A5 (x linked)
- COL4A3 (AD)
- COL4A4 (AR)
- eye problems
- glomerulonephritis
- sensorineural deafness
Alport
cant see, cant pee, cant hear a bee
“basket” weave appearance due to irregular thickening of GBM
Alport
EM shows variable splitting, thinning of GBM
Alport
- indolent hematuria
- AD inheritance
- some patients found to have mutations in COL4A3 or 4
- EM show thin GBM
thin basement membrane disease
nephritic syndromes
6
- PSGN
- IgA nephropathy
- RPGN
- lupus nephritis
- Alport
- MPGN
Goodpasture
auto-antibody to COL4A3
PSGN
Alport
ANCA
lupus nephritis
Henoch-Shonlein or IgA nephropathy depending on age of patient
sinusitis that does not respond to therapy
GPA (ANCA)
ANCA vasculitis
nephrin and TRPC6 are mutated in
FSGS
variant alleles of APOL1 increase the risk of
FSGS
and HIVAN
auto-antibodies to PLA2R
primary membranous glomeruolpathy
MPGN
IgM, IgG, IgA, C3, an C1q deposits
lupus nephritis
IgA immune deposits
IgA nephropathy
shows IgG and C3 immune depositis
PSGN
- presents commonly in children
- nephrotic and nephritic
- C3 levels are low
dense deposit disease
MPGN Type II
membranoproliferative pattern with “sausage-like” dense deposits in GBM
dense deposit disease
MPGN Type II
Ig+ staining and subendothelial deposits
Ig+ MPGN
linear staining of GBM by IF
Goodpasture
synpharyngitic nephritis
IgA nephropathy