Flashcards in Renal Deck (115)
What is the response to Tx of membranous nephropathy?
poor response to steroids causing chronic renal failure
How does membranoproliferative glomerulonephritis present on H&E? EM? IF?
HE: thick glomerular BM often w/ "tram-track" appearance
EM: 2 types based on location of deposits (sub endothelial or intramembranous)
IF: IC deposition = granular IF
What is the response to Tx of membranoproliferative GN?
poor response to steroids leads to chronic renal failure
Describe the pathophys of renal disease in diabetes mellitus
high glucose leads to NEG of vascular BM causing HYALINE ARTERIOSCLEROSIS (thick wall damages lumen) =>
glomerular EFFERENT arteriole constriction leads to hyperfiltration causing microalbuminuria =>
nephrotic syndrome w/ sclerosis of mesangium => formation of Kimmelstiel Wilson nodules (dense sclerosis)
What is the response to Tx of diabetic nephrotic syndrome? why?
ACE like hyaline arteriosclerosis causes efferent constriction so ACE inhibitors slow dz progression
How does amyloidosis affect the kidney? How can it be proven to be the cause?
MC systemic organ in systemic amyloidosis=>
amyloid deposits in mesangium causing nephrotic syndrome
Apple-green birefringence under polarized light after Congo red stain
If minimal change disease does not respond to steroids, what is the progression?
Based on the location of any IC deposition, differentiate the the types of nephrotic syndrome
Membranous = sub epithelial deposits (S&D)
Type I MPGN = subendothelial
Type II MPGN = dense deposits in intramembranous portion of glomerulus
Type I MPGN is assoc with what?
HBV & HCV
Differentiate how HBV & HCV can cause nephrotic syndrome
Membranous nephropathy has sub epithelial deposits w/ spike & dome
type I MPGN has subendothelial deposits
Type II MPGN is assoc w/ what? Describe how it gets its appearance on EM
assoc w/ C3 nephritic factor => autoAb stabilizes C3 convertase leading to over activation of complement, inflammation, & LOW circulating C3
binds to intramembranous portion of BM in kidney causing tram-track appearance
Define nephritic syndrome
glomerular inflammation & bleeding =>
limited proteinuria (<3.5g/day);
oliguria & azotemia;
salt retention w/ periorbital edema progressing to HTN;
RBC casts & dysmorphic RBC in urine
If nephritic syndrome is suspected, how can it be confirmed?
Bx showing hyper cellular & inflamed glomeruli
What causes the damage in nephritic syndrome?
IC deposition activates complement leading to C5a attracting neutrophils which cause the damage
Differentiate the 2 types of nephritic syndrome that occurs after infections
both occur after infection
post-strept GN arises after GAS of skin or pharynx but can be occur after nonstrept organism as well
IgA nephropathy (Berger dz) occurs after mucosal infection such as gastroenteritis
What is the virulence factor that must be present in organism to cause PSGN?
nephritogenic strains must carry M protein virulence factor
When & how will patient w/ PSGN present?
2-3wks after infxn w/ hematuria (cola colored), oliguria, HTN & periorbital edema
usually kids but can be in adults
What is the appearance of PSGN on HE? EM? IF?
HE: hypercellular, inflamed glomeruli
EM: subepithelial humps
IF: granular = IC deposits
What is the Tx for PSGN?
supportive only bc progression is self limited w/ only 1% of kids going to renal failure
25% of adults develop rapidly progressive GN (RPGN)
What is the timeline for RPGN?
nephritic syndrome to renal failure in wks to months
How does RPGN appear on HE? IF?
HE: crescents in Bowman space
IF is variable depending on underlying cause
What does a linear pattern on IF assoc w/ RPGN mean? How will it present? why? who is most likely to have this dz?
Goodpasture syndrome => anti-BM Ab
Ab against collagen in glomerular (hematuria) & alveolar (hemoptysis) BM
classically is young, adult males
What does a granular pattern on IF in presentation of RPGN?
PSGN or diffuse proliferative GN
What is significant about diffuse proliferative GN?
antigen-Ab complex deposition usually sub endothelial
MC type of renal disease in SLE
differentiate the types of renal dz seen in SLE. What is significant about renal dz in SLE?
MC COD in SLE
membranous nephropathy is nephrotic w/ granular IF but SUBEPITHELIAL deposits (S&D)
DPGN is nephritic w/ granular IF w/ antigen-Ab complexes but are SUBENDOTHELIAL deposits
If a pt presents w/ crescents in Bowman space & negative IF w/ hematuria & slight proteinuria, what should the next test be? What is the Ddx?
C-ANCA => Wegener granulomatosis
P-ANCA => microscopic polyangiitis & Churg Strauss
What differentiates the P-ANCA assoc nephritic syndromes?
Churg Strauss has 3 things:
What is the MC nephropathy worldwide? present on IF? profession
IgA IC deposits in mesangium of glomeruli
may slowly progress to renal failure
How does the IgA nephropathy present typically?
kid w/ episodic gross or microscopic hematuria w/ RBC casts following mucosal infection (gastroenteritis) causing IgA to increase then deposit in mesangium