Renal: 12.3: Nephrotic Syndromes Flashcards
Why does Hodgkin lymphoma cause minimal change disease?
- Hodgkin lymphoma = huge amounts of cytokines
- m.c.d. = effacement (flattening) of the foot processes (podocytes) due to cytokines
What is C3 nephritic factor?
an autoantibody that stabilizes C3 convertase –> overactivation of complement –> inflammation, damage to the glomerulus
What kind of nephrotic syndrome do pts with hepatitis B or C get?
membranous nephropathy
What usually causes membranous nephropathy?
idiopathic
What is the most commonly involved organ in systemic amyloidosis?
the kidney
What is minimal change disease?
effacement (flattening) of the foot processes (podocytes) due to cytokines
What is the key finding on immunofluorescence in membranous nephropathy?
granular immune complex deposition
What can focal segmental glomerulonephritis (FSGS) progress to?
chronic renal failure
What is penicillamine? What is it used to treat?
- a chelating agent
- tx for heavy metal intox or deposition
What kind of nephrotic syndrome do pts with solid tumors get?
membranous nephropathy
What is focal segmental glomerulonephritis (FSGS) sometimes associated with?
- HIV
- heroin
- sickle cell disease
Why do pts with nephrotic syndrome have a hypercoaguable state?
preferential loss of antithrombin III (AT3) –> can’t break up thrombin
What is Type I membranoproliferative glomerulonephritis (MPGN)?
Type I = subendothelial deposits, assoc. with HBV and HCV, tram-tracking more often seen
Why do pts with nephrotic syndrome have hyperlipidemia and hypercholesterolemia?
the liver tries to compensate for the thinned blood
Dx?
- thickened glomerular basement membranes
- granular immune complex depositions
- subendothelial deposits with “spike and dome” appearance
membranous nephropathy
What can membranous nephropathy be associated with?
- hepatitis B or C
- solid tumors
- SLE
- drugs (NSAIDs, penicillamine)
What findings are seen on EM in focal segmental glomerulonephritis (FSGS)?
effacement of the foot processes
What does membranoproliferative glomerulonephritis (MPGN) look like on H&E?
thick capillary membranes with “tram-track” appearance
Dx?
- effacement (flattening) of the foot processes (podocytes) due to cytokines
- normal glomeruli
- lipid in proximal tubules
minimal change disease
What is hyaline arteriolosclerosis assoc. with?
- diabetes
- HTN
What kind of nephrotic syndrome to HIV pts get?
focal segmental glomerulonephritis (FSGS)
What is the usual cause of focal segmental glomerulonephritis (FSGS)?
idiopathic
What is the key finding on EM in membranous nephropathy?
subendothelial deposits with “spike and dome” appearance
What can minimal change disease progress to if the steroids don’t work?
focal segmental glomerulonephritis (FSGS)
Dx?
- amyloid deposits in the mesangium
systemic amyloidosis
Dx?
- pink sclerosis within some portions of the glomerulus
- effacement of the foot processes
focal segmental glomerulonephritis (FSGS)
What is the key finding on H&E in membranous nephropathy?
thickened glomerular basement membranes
What causes thickening of the membrane in “membranous” diseases?
immune complex depositions
What does “proliferative” indicate in nephrotic syndromes?
- the cytoplasm in the mesangial cell proliferates to separate the immune deposits
- creates a tram-track appearance
This is effacement (flattening) of the foot processes (podocytes) due to cytokines.
minimal change disease
Which part of the kidney is more affected by hyaline arteriolosclerosis? What does this cause?
- the efferent arteriole
- high glomerular filtration pressure
What imaging findings are classic for systemic amyloidosis?
apple green birefringence under polarized light
What are the 2 types of membranoproliferative glomerulonephritis (MPGN)? What makes them different?
- Type I = subendothelial deposits, assoc. with HBV and HCV
- Type II = intramembranous (basement membrane) deposits, assoc. with C3 nephritic factor
What usually causes minimal change disease? What can cause it on rare occasions?
- usu. idiopathic
- rare = Hodgkin lymphoma
What are the hallmarks of nephrotic syndrome?
- proteinuria >3.5g/day
- hypoalbuminemia –> edema
- hypogammaglobulinemia –> increased infections
- hypercoaguable state
- hyperlipidemia
- hypercholesterolemia
What will the immunofluorescence show in minimal change disease?
no findings (no immune complex deposits)
What causes the thick capillary membranes with “tram-track” appearance in membranoproliferative glomerulonephritis (MPGN)?
immune complex deposition
What is the most common nephrotic syndrome among Blacks and Hispanics?
focal segmental glomerulonephritis (FSGS)
This drug is a chelating agent used to treat heavy metal intox or deposition.
penicillamine
High serum glucose leads to non-enzymatic glycosylation of vascular basement membranes, including in the kidney, resulting in _______.
hyaline arteriolosclerosis
What happens microscopically in minimal change disease?
- effacement (flattening) of the foot processes (podocytes) due to cytokines
- normal glomeruli
- lipid in proximal tubules
What is a Kimmelsteil-Wilson nodule assoc. with?
hyaline arteriolosclerosis in diabetes
Which nephrotic syndrome can be treated with steroids?
minimal change disease
Why do systemic amyloidosis pts get nephrotic syndromes?
amyloid deposits in the mesangium
What is the tx for minimal change disease? Why?
- steroids
- damage is mediated by cytokines from T cells; knock out T cells = cure
What kind of nephrotic syndrome do SLE pts get?
membranous nephropathy
What kind of nephrotic syndrome do NSAIDs, penicillamine cause?
membranous nephropathy
What is the most common nephrotic syndrome among white patients?
membranous nephropathy
What proteins are lost in minimal change disease?
albumin only = selective proteinuria
What is the most common cause of nephrotic syndrome in children?
minimal change disease
What is the most common renal disorder in SLE pts?
diffuse proliferative glomerulonephritis
What kind of nephrotic syndrome to heroin abusing pts get?
focal segmental glomerulonephritis (FSGS)
Dx?
- kidney appears apple green birefringence under polarized light
systemic amyloidosis
What kind of nephrotic syndrome to sickle cell pts get?
focal segmental glomerulonephritis (FSGS)
What findings are seen on immunofluorescence in focal segmental glomerulonephritis (FSGS)?
none- no immune complex deposits in FSGS
What is the most common cause of death in SLE pts?
renal failure
What findings are seen on H&E in focal segmental glomerulonephritis (FSGS)?
pink sclerosis within the glomerulus (only part of it, though- hence focal and segmental)
Dx?
- thick capillary membranes with “tram-track” appearance
membranoproliferative glomerulonephritis (MPGN)
Which type of membranoproliferative glomerulonephritis (MPGN) is subendothelial, assoc. with HBV and HCV?
Type I
What is Type II membranoproliferative glomerulonephritis (MPGN)?
Type II = intramembranous (basement membrane) deposits, assoc. with C3 nephritic factor
Which type of membranoproliferative glomerulonephritis (MPGN) is intramembranous, assoc. with C3 nephritic factor?
Type II
