Clinical Glomerulonephritis

Question 1

PIGN complement levels

Answer 1

low C3, can have low C4

Question 2

PIGN serologies

Answer 2

ASO (post strep pharyngitis), anti Dnase B (cellulitis)

Question 3

patho for PIGN (LM, IF, EM)

Answer 3

LM- large, hypercellular glomeruli

IF- granular, lumpy bumpy or starry sky from IgG, IgM, C3 deposits

EM- subepithelial immune complex humps

Question 4

disease association w/ PIGN, type of reaction

Answer 4

commonly in kids 2-4 weeks after group A strep infection of pharynx or skin

will resolve spontaneously

type III hypersensitivity

Question 5

presentation of PIGN

Answer 5

periorbital edema, cola colored urine, HTN

Question 6

proliferative lupus nephritis (PLN) complement

Answer 6

low C3 and C4

Question 7

serology of PLN

Answer 7

ANA, dsDNA, anti-Smith

Question 8

patho of PLN

Answer 8

LM- wire looping of capillariies

EM- subendothelial or intramembranous IgG w/ C3 deposits

IF- granular

Question 9

disease associations w/ PLN

Answer 9

SLE manifestations: ulcers, malar rash, photosensitive, arthritis

LN 1-4 are nephritic
LN 5 are nephrotic

Question 10

complement in MPGN

Answer 10

low C3, maybe low C4

Question 11

serology of MPGN

Answer 11

hep C Ab, cryoglobulins

Question 12

patho of MPGN

Answer 12

EM- subendothelial complex deposists

IF- granular

LM- hypercellular and thickened BM (tram track on PAS stain)

Question 13

disease associations w/ MPGN (concurrent syndrome, infections, malignancies)

Answer 13

-often concurrent w/ nephrotic syndrome

associated w/ HBV, HCV, multiple myeloma, waldenstroms, osteomyolitis, syphilis

can present w/ cryoglobulinemic vasculitis

Question 14

C3 glomerulopathy (C3G) complement levels

Answer 14

very low C3, C4 is normal

Question 15

serology/etiology of C3G

Answer 15

antibodies or mutations to complement regulatory factors

caused by dysfn of alternate complement pathway, excess activation

Question 16

patho of C3G

Answer 16

IF- stains for C3 deposits but no Ig

Question 17

IgA nephropathy (IgAN) complement levels

Answer 17

normal

Question 18

GN disorders w/ reduced complement, esp C3

Answer 18

most immune complex disorders- PIGN, PLN, MPGN (not IgA!)

also C3G

Question 19

IgAN patho

Answer 19

LM- mesangial proliferation

EM- mesangial immune complex deposits

IF- IgA staining, granular mesangial deposits

Question 20

disease associations w/ IgA (concurrent processes, presentations)

Answer 20

• inflammatory bowel disease
• ankylosing spondylitis
• IgA vasculitis (henoch scholein purpura)
• episodic gross hematuria

**often concurrent w/ pharyngeal infection (synpharyngitic hematuria) or GI infection

Question 21

alport syndrome patho (EM)

Answer 21

EM- basket weave appearance, split and laminated GBM

Question 22

alport syndrome pathogenesis

Answer 22

mutation in type IV collagen in GBM, leads to thinning and splitting

most commonly X linked

Question 23

other manifestations of alport

Answer 23

eye problems and senserineural deafness

Question 24

pauci immune GN complement

Answer 24

normal

Question 25

serology for pauci immune

Answer 25

ANCA (can be negative on test but still have ANCA)- PR3 and MPO

Question 26

patho of ANCA

Answer 26

LM: crescentic with or without fibrinoid necrosis

negative IF!

Question 27

clinical correlates to ANCA

Answer 27

GPA, MPA, EGPA (could also be limited to kidney)

can have RPGN

Question 28

anti GBM disease complement and serology

Answer 28

normal complement

anti-GBM antibodies can be seen, type II hypersensitivity

Question 29

patho of anti GBM

Answer 29

IF- linear staining

LM- crescentic

Question 30

clinical correlate of anti GBM

Answer 30

w/ pulm hemorrhage= Goodpasture

can present w/ RPGN

Question 31

tx for anti GBM

Answer 31

emergent plasmapharesis