Nephrotic Flashcards
5 primary renal nephrotic
Hereditary Nephrotic Syndromes
Minimal change disease
Focal Segmental Glomerulosclerosis
Membranous nephropathy
Membranoprolifeartive GN
3 systemic nephrotic
Diabetes
Amyloid and Light Chain
SLE (membranous)
3 Primary renal Nephritic
Post Infectious
IgA
Rapid Progressive GN
2 systemic Nephritic
Vasculitis (ANCA) Immune Complex (SLE / HSP)
Nephrotic Syndrome - is BV low?
No - edema without low BV because primary problem is with salt excretion
5 SIGNS NEPHROTIC
Prtoeinuria Hypoalbuminemia Lipiduria (oval fat bodies) Hyperlipidemia Edema
Complications of nephrotic
Hypercoagulable
Infection
Decreased VitD
Management nephrotic
ACE-I helps proteinuria (even though drops GFR a tiny bit due to efferent constriction - the drop in proteinuria is worth it)
Low Salt
Diuretics
Most common children
MCD
Most common adults
Membranous and FSGS
Hereditary nephrotic
presentation
infant / child
edema
FtoT
polyhydraminos on ultrasound
Hereditary nephrotic
etiology
mutations in nephrin / podocin
Minimal change disease
presentation
2-8y/o
edema / ascites / weight gain
normal blood pressure
MCD
Labs
renal function normal or slightly depressed
urinalsyis - 4+ protein, hyaline casts, microscopic hematuria (Rare)
MCD associations
allergy/atopy
Hodgkins
NSAIDS
MCD Patho
circulating permeability factor
leads to podocyte injury
(foot process fusion - expression of CD80 in podocytes)
MCD treatment
steroids (prednisone)
short course cytoxan if relapse
MCD
LM
IF
EM
LM - normal
IF - negative
EM - foot process fusion
FSGS
Labs
renal function normal or slightly depressed
4+ protein, hyaline casts, microscopic hematuria
FSGS associations
usually idiopathic
HIV
FSGS pathophsyiology
ciruclating factor (unknown)
Viral factor in HIV
APOL1
FSGS treatment
steroids - does not respond well to treatment
Calcineurin inhibitors / MMF
HIV nephropathy
presents as FSGS - additional characteristics?
collapsed glomerular tuft
cystic tubules
tubuloreticular inclusions (reticuloendothelial)
RESPONDS to antivirals
Membranous nephropathy
associations
cancer / lupus / hepB/ Hg
membranous nephropathy
histology
biopsy shows BM thickening - normocellular - granular immune complex deposits that are subepithelial
Treatment MN?
Steroids and cytoxin (as is immune complex mediated)
GRANULAR DEPOSITS - SUBEPITHEIAL
Membranous
what test should you do on patients with membranous?
cancer screen
pathogeneis membranous?
Antibody to PLA2R on podocyte which is then shed and lodges in subepithelium -
mercury linked to?
membranous
HBV linked to?
membranous
deposits contain HBe antigen
Membranoproliferative GN
presentation
HTN
Mildly reduced renal function
MPGN Type I
associations
HCV
(low C3 and C4 AND + RF)
low grade infection
idiopathic
MPGN pathology
GBM thickening
hypercellularity
granular immune complex deposits (SUBENDOTHELIAL)
MPGN treatment
treat HCV
Steroids
nephrotic that is HTN
MPGN
MPGN type 1
complement?
low c3 and c4
classic pathway
MPGN type 2
complement disorder
low c3 normal c4 (because activates alternate)
usually childhood
nephrotic syndrome blood anomalies?
in addition to the loss of protein we see hypercholesteremia / hypokalemia / hyponatremai
what happens to the glomerulus in nephrotic syndrome?
epithelial cell detachment and BM degradation - loss of polyanion
Pathogenesis of Glomerular injury? MCD/FSGS
Non-inflammatory circulating factors/Ig's bind GEC membranes and or GBM without fixing complement --> loss of polyanion --> GEC detachment (MCD/FSGS)
Pathogenesis of Glomerular injury in membranous
Non-inflammatory
complement fixing anti-GEC Ab’s
alternative complement pathway
may involve oxidants/proteases
minimal change nephropathy clinical morph EM IF Tx/Prog
nephrotic syndrome no change EM - foot process effacment IF negative steroids/good
some circulating factor that we don’t know - CD80 starts to show
FSGS
cause
treatment
unknown CF
can also be due to HIV/Heroine/hyperfiltration in late stage renal disease
steroids - relapse common
Membranous
circulating immunoglobulins attack podocyte PLA2R Subepithelial spikes / bm thickening stain c3/5 - granular BM Steroids
Membranous 85% idio
15% other?
bugs
drugs
tumors
rheum
MPGN
morph
path
treat
GBM thickening - double countour
chronic immune complex subendo deposition
IF - Granular GBM
STeroid / interferon
MPGN common cause
hepB
sle
cryoglobin
what % of amyloidosis cases have renal involvemnet and how does it present?
85%
proteinuria - histology shows amorphous pink material in glomeruli and vessels - birefrigenence green - congo red
EM - Spaghetti
most common cause of esrd
diabetes (38%) HTN/Vascular (25%) GN (19%) Interstitial nephritis (4%) PCKD (5%) Other (9%)
DM
kidney lesions
hyaline arteriolar disease
glomerulosclerosis - diffuse or nodular expansion of mesangium
BM thickening
Kinnelsteil Wilson nodules care characteristic of
glomerular disease in DM
Malignant HTN renal disease
vascular insult –> fibrinoid necrosis and hyperplastic arteriolitis - onion –> kidneys release renin exacerbating problem
HTN renal disease
finely granular surface scarring - scarred glomeruli
hyaline depot in vessels and medial / intimal thickening
