Clinical Glomerulonephritis Flashcards
PIGN complement levels
low C3, can have low C4
PIGN serologies
ASO (post strep pharyngitis), anti Dnase B (cellulitis)
patho for PIGN (LM, IF, EM)
LM- large, hypercellular glomeruli
IF- granular, lumpy bumpy or starry sky from IgG, IgM, C3 deposits
EM- subepithelial immune complex humps
disease association w/ PIGN, type of reaction
commonly in kids 2-4 weeks after group A strep infection of pharynx or skin
will resolve spontaneously
type III hypersensitivity
presentation of PIGN
periorbital edema, cola colored urine, HTN
proliferative lupus nephritis (PLN) complement
low C3 and C4
serology of PLN
ANA, dsDNA, anti-Smith
patho of PLN
LM- wire looping of capillariies
EM- subendothelial or intramembranous IgG w/ C3 deposits
IF- granular
disease associations w/ PLN
SLE manifestations: ulcers, malar rash, photosensitive, arthritis
LN 1-4 are nephritic
LN 5 are nephrotic
complement in MPGN
low C3, maybe low C4
serology of MPGN
hep C Ab, cryoglobulins
patho of MPGN
EM- subendothelial complex deposists
IF- granular
LM- hypercellular and thickened BM (tram track on PAS stain)
disease associations w/ MPGN (concurrent syndrome, infections, malignancies)
-often concurrent w/ nephrotic syndrome
associated w/ HBV, HCV, multiple myeloma, waldenstroms, osteomyolitis, syphilis
can present w/ cryoglobulinemic vasculitis
C3 glomerulopathy (C3G) complement levels
very low C3, C4 is normal
serology/etiology of C3G
antibodies or mutations to complement regulatory factors
caused by dysfn of alternate complement pathway, excess activation
patho of C3G
IF- stains for C3 deposits but no Ig
IgA nephropathy (IgAN) complement levels
normal
GN disorders w/ reduced complement, esp C3
most immune complex disorders- PIGN, PLN, MPGN (not IgA!)
also C3G
IgAN patho
LM- mesangial proliferation
EM- mesangial immune complex deposits
IF- IgA staining, granular mesangial deposits
disease associations w/ IgA (concurrent processes, presentations)
- inflammatory bowel disease
- ankylosing spondylitis
- IgA vasculitis (henoch scholein purpura)
- episodic gross hematuria
**often concurrent w/ pharyngeal infection (synpharyngitic hematuria) or GI infection
alport syndrome patho (EM)
EM- basket weave appearance, split and laminated GBM
alport syndrome pathogenesis
mutation in type IV collagen in GBM, leads to thinning and splitting
most commonly X linked
other manifestations of alport
eye problems and senserineural deafness
pauci immune GN complement
normal
serology for pauci immune
ANCA (can be negative on test but still have ANCA)- PR3 and MPO
patho of ANCA
LM: crescentic with or without fibrinoid necrosis
negative IF!
clinical correlates to ANCA
GPA, MPA, EGPA (could also be limited to kidney)
can have RPGN
anti GBM disease complement and serology
normal complement
anti-GBM antibodies can be seen, type II hypersensitivity
patho of anti GBM
IF- linear staining
LM- crescentic
clinical correlate of anti GBM
w/ pulm hemorrhage= Goodpasture
can present w/ RPGN
tx for anti GBM
emergent plasmapharesis
