Glomerular Disease Flashcards
visceral epithelium
podocytes; also negatively charged. interdigited with foot process
endothelium
fenestrated cytoplasm coated with negatively charged glycoprotein
basement membrane
proteoglycans
laminin
collagen IV
what is BM shaped like
triple helical alpha molcule
hook like non-collagenous domain
mutations in basement membrane lead to
alport syndrome
fx of mesangium
cntracts to regulate glomerular blood flow
tethers to GBM to counteract capillary distentsion pressure
produces GFs and cytokines
phagocytoses debris
two types of glomerular injury due to antibodies
insitu complex deposition
circuling immune complex nephritis
two types of insitu deposition (ab specific to glom)
anti-gbm nephritis
membranous BN
Anti GBM nephritis
Ig against NC1 domain of Collagen IV
IF shows LINEAR immune staining
Goodpasture syndrome is a type of ____ that causes_____
antiGBM nephritis
cross reaction with alveolar basement membrane
Membranous GN
GP330 homolog, Mtype PLA2R–>binding–>C-activation–>subepithelial deposits
circulating immune complex nephritis means that
antigens arent specific to glomeruli but end up on GBM because of its properties and hemodynamic factors
EM will show deposits (exo or endogenous) in subepithelial, mesangial, etc
anionic-subendo
cationic-subepithelial
neutral- mesangium
Three component of any kidney biopsy
light microscopy
immunofloresence
electromicroscopy
way to classify LMs
kidney- focal (some glomeruli) vs difffuse (all glom)
glomerulus: segmental vs global
IF immunocomplex patterns
linear- anti GBM disease, light chain deposition (multiple myeloma)
granular- immune complex
nonsepcific patterns
things to evalute on EM
e- dense deposits
podocyte charges
GBM alterations
ddx of glomerular disease
nephrotic
nephritic
hematuric
rapidly progressive GN
5 types of nephrotic syndrome
minimal change disease FSGS membraneous GN diabetes amyloidosis
3 types nephritic syndrome
post infectious GN
MPGN
lupus nephritis
3 types of hematuric GD
alport syndrome
TBM
IgA nephropathy
clinical features of nephrotic sydnrome
proteinuria
hypoalbuminemia
edema (decreased oncotic and increased Na retneiton)
hypercholesterolemia (inc prod, dec catabolism)
minimal change disease is the MC of nephrotic syndrome in
children
MCD is often secondary to
lymphoma, NSAIDs
what will you see on tests with MCD?
EM: effacement of foot processes
tests for FSGS
LM: segmental collapse ins caring in SOME glomeruli
EM: effacement foot processes, thick BM
IF: - except some IgM/C3 trapping
FSGS etiology
can be either idiopathic (suPAR) or secondary (family, adaptive, viruses (parvo, hiv), drugs (heroin, lithium)
evolution of membranous glomeruopathy
immune complex deposits accumulate—>GBM reacts by forming spike like projects–>grow to spike and dome–>lead to thickened basement membranes
MG is the main cause of nephrotic syndrome in
adults
etiology of MGNopathy
primary and secondary (drugs, tumors, lupus, infx)
tests and MGpathy
LM: suepithelial immune deposits
EM-subepithelial spike like projects of GBM
IF: diffuse GRANULAR pattern–Igg, c3
LM of diabetes early and late
early: glomerular hypertrophy, widened sclerotic mesangium, thickend GBM
advanced progressive GBM thickening with KW spots, microaneurysms, arteriolar hylinization
EM for diabetes
thick GBM
mesangial sclerosis
IF for diabetes
neg for deposits
LM for amyloidosis
mesangial and GBM deposition of b pleated amyloid
congo red!
EM amyloidosis
8-12 fibrils
IF amyloidosis
negative, but lambda chain positive
LM acute post-infectious GN
diffuse endocapi. prolif with LEUKOCYTES
swollen, hypercellular glomerulus
EM APIG
hump like deposits
IF APIG
granular along GBM (c3!)
what is a big etiology of Membrano-proliferative GN?
hep B & C
LM of membrano-proliferative GN
hypercellular, hyperlobulated glomeruli
leukocytes
tran-tracking
GBM deposits (C3)
EM membrane prolif
large subendo deposits
IF membrano proliferative
diffuse C3 deposits corresponding to lg immune complex
progonosis of membranoprolif
poor- end stage renal dx
lupus LM
6 classes–advances to nerotizing wire loop and lesions
EM lupus
extensive mesangial and maybe deposits everywhrre
IF lupus
“full house pattern”-everything, everywhere
3 types of hematuric GNitis
alport syndrome
thin BM disease
IgA Nephropathy (berger disease)
alport syndrome
nephritis with hematuria asociated with nerve deafness and ocular abnormalities
etio alport syndrome
sex-linked heterogenous group of mutations of collagen IV (a3 or 5) chain synthesis–>fragmentation of GBM
EM alport syndrome
fragmentation and splitting of GBM–>basket-weave pattern
thin basement diease
persistent or intemittent micro-hematuria (non-progressive, benign)
EM of thin BM disease
generalized thinning of BM
IgA nephropathy is the
commonest cause of GN worldwide
IgA nephro presentation
gross or microscopic hematuria (+ upper resp infx)
LM IgA
variable, but mainly mesangiprofiliferative
EM IGa and IG IgA
Iga deposits in mesangium
LM cresencitc disease
> 50% of glomerular bowman’s space is crescnet + compresse glomerular tuft + fibrin
IF+ cresenct
linear: anti GBM disease (ex- goodpasture)
granular- immune complex disease
IF- crescent
ANCA -:puaci-immune
ANCA+- systemic vasculatitis (wegners, churg strauss, microscopic polyangitis)
