nephritic/nephrotic syndromes Flashcards
list the main nephrotic syndromes
list the main nephritic syndromes
-which ones also present with nephropathy?
nephrotic vs nephritic- what is the general etiology that results in either presentation
NEPHROTIC:
- minimal change ds
- focal segmental glomerulosclerosis (FSGS)
- membranous nephropathy
- systemic amyloidosis
- diabetic nephropathy
NEPHRITIC:
- RPGN (rapid proliferative glomerulonephritis) (3 types)
- MPGN (membranous prolif GN, 2 types)
- DPGN (diffuse prolif)
- IgA nephropathy
- Alport Syndrome
MPGN+DPGM = slide into both DMs..
nephrotic= x podocytes in glomerulus–> protein leak
nephritic= inflammed +hypercellular glomerulus draws RBCs through
minimal change ds
nephritic or nephrotic?
- path
- clin
- light microscopy
- immunoflourescence
- EM
nephrotic
- triggered by recent infection/immunization, rarely by lymphoma
- kids: trx a steroids, v responsive
-LM: normal
IF= (-)
EM: effaced foot processes
focal segmental glomerulosclerosis (FSGS)
nephritic or nephrotic?
- path
- clin
- light microscopy
- immunoflourescence
- EM
nephrotic
- idiopathic or 2° to HIV, sickle cell, heroine abuse, severe obesity, IFN trx, or congenital malformations
- AA or Hispanic ppl (focal and segmental aka discrimination??)
LM=segmental sclerosis + hyalinzation
IF= (-)»_space; IC (IgM, C3, C1)
EM= effaced podocytes
what is the purpose of the complement system
complement cascade, is a part of the immune system that enhances (complements) the ability of antibodies and phagocytic cells to ..
- clear out microbes and damaged cells from an organism
- promote inflammation
- attack the pathogen’s cell membrane.
membranous nephropathy aka membranous glomerulonephriits (MGN)
nephritic or nephrotic?
- path
- clin
- light microscopy
- immunoflourescence
- EM
nephrotic
- either primary = anti-phospholipase A2-R Ab (receptor on podocytes)
- or secondary to HBV, HCV, syphilis, solid tumors
LM= diffuse thickening of capillaries + GBM IF= granular IC depo EM= 'spike&dome' subepithelial deposits
**membranous = sub-endo (MGN)/ sub-epi (MPGN) granular deposits, HBV/HCV*
systemic amyloidosis
nephritic or nephrotic?
-path
-light microscopy
nephrotic
- AL/AA amyloid dep
- LM= congo red/apple green depo in mesangium
diabetic glomerulonephropathy
nephritic or nephrotic?
- path
- clin
- light microscopy
nephrotic syndrome
- hyperglycemia -> mesangial expansion + GBM thickening –> inc permeability + inc filtration –> glomerular HTN + inc GFR –> inc glomerular sclerosis
- most common cause of ESRD in US
LM= mesangial expansion + GBM thickening, eosinophilic nodular glomerular sclerosis
aka: Kimmelstiel-Wilson lesions
acute post-strep glomerulonephritis
aka PSGN
nephritic or nephrotic?
- path
- clin
- light microscopy
- immunoflourescence
- EM
nephritic
=2-3 wks post grp a strep TIII hypersensitivity
(+) streptolysin O
=in kids peripheral and periorbital edema, HTN, COLA COLORED URINE dec C3 due to consumption --spont regress in kids, progress in adults
LM= hypercellular glomerulus
starry sky, lumpy bumbpy (taare zameen par)
IF= IC of IgG, IgM, C3 depo along GBM and mesangium
RPGN= rapidly progressive glomerulonephritis
nephritic or nephrotic?
- path
- light microscopy
- immunoflourescence
nephritic
3 types:
- -GOODPASTURE: hematuria/hemoptysis due to TII hypersensitivity vasculitis
IF= linear Abs in GBM + alveolar membrane
trx= plasmapharesis - -Wegener/GPA
IF= pauci-immune,
PR3-ANCA/c-ANCA
C PR3tty girls at GRANd Weddings
- microscopic polyangitis
IF= pauci-immune
MPO-ANCA/ p-ANCA
- microscopic polyangitis
all:
LM= crescents made of fibrin+plasma proteins +glomerular parietal cells, monocytes, Møs
IF= C3b in the crescents
diffuse proliferative glomerulonephritis
DPGN
nephritic or nephrotic?
- clin
- light microscopy
- immunoflourescence
- EM
nephritic
associated w SLE (wire lupus)
MIXED NEPHRITIC + NEPHROTIC (DPGN+MPGN slide into both DMs..)
LM= wiring capillaries (getting your wires cross in his DMS
EM= subendothelial/ intramembranouus IC-IgG + C3 depo
IgA nephropathy
goes by what other names?
nephritic or nephrotic?
- path
- clin
- light microscopy
- immunoflourescence
- EM
aka berger ds aka (the renal manifestations of) (henoch schönlein purpura)
mesa C IgA, Berger, and Henoch episodically
nephritic
sx= episodic hematuria + concurrent respiratory/GI infections
LM= mesangial proliferation IF= IgA-ICs in teh mesangium EM= mesangial IC depo
Alport syndrome
nephritic or nephrotic?
- path
- clin
- light microscopy
- immunoflourescence
- EM
nephritic
x-linked dominant (mc) x T4 collagen -> thin, splitting GBM + retinopathy/lens dislocation, GN, sensory
**“thin Al can’t see, can’t pee, can’t hear a bee-how is he supposed to weave a basket?” **
EM= basket weave membrane
membranous proliferative glomerulonephritis (MPGN)
nephritic or nephrotic?
- path
- clin
- light microscopy
- immunoflourescence
- EM
MIXED NEPHRITIC + NEPHROTIC (DPGN+MPGN slide into both DMs..)
GBM splitting secondary to mesangial ingrowth =
tram track appearance on H&E: PAS+
TYPE 1:
secondary to HBV/HCV or idiopathic
EM=subendothelial IC depo
IF= granular
TYPE 2: “dense deposit ds”
path= C3 nephritic factor (= IgG stabilize C3 convertase –> excessive complement activation –> dec C3 levels, intramembranous depositions
MP: i.e. proliferate tram track in two ways:
- add stuff in-between existing lines
- split the lines themselves (have to be denser to keep the iron apart so need a nephritic factor
