14 year old girl w/ acute onset of hematuria, proteinuria, oliguria, mild HTN, RBC cast,

IF positive w/ IgG n C3 in coarse granular pattern along glomerular BM.

low serum C3 initially

cola colored urine, puffy eyes, 4 weeks ago had ear infection.

PIGN

nephritic syndrome: hematuria, proteinuria, oliguria, mild HTN, RBC cast

Immune complex disease: IgG, C3 deposit in coarse granular pattern. (lumpy-bumpy)

micro: all glomeruli show lumen capillary lumen obliteration + diffuse endocapillary proliferation.

no changes in blood vessel and tubules

PMN: exudates in capillary lumen, also crescents, capsular adhesion.

IF: IgG, C3 in all G - coarsely granular

EM: few, large subepithelial EDD

no mesengial changes - not chronic

9 yo kid w/ repeated episode of massive proteinuria

effacement of epithelial cell foot processes

IF: minimal IgM rxn

Normal tubules-back to back

Normal glomeruli: open urinary lumen + capillary

normal mesengium

normal open tuft:

BM uniform thickness

MCD

possibly due to pathologic release of permeable factor from CD8 cell,

enhance albumin movmenet

Tx:

MCD

ideopathic FSGS

membranous glomerulopathy

alport

PIGN

MPGN

steroid

alkylating agent therapy: cyclophosphamide/chlorambucil

cyclosporin

poorly to steroid. Renal transplant w/ 30% recurrence.

reversal with cytotoxics + steroids before BM thickened.

renal transplant only.

self-limiting

resistant to steroid.

2 DD:

mesangila proliferative GN

defined by

see 3+ nuclei per mesangial matrix

late stage PIGN

IgA nephropathy

4 DD:

GN w/ mesangial IgA

IgA nephropathy

Henoch-Schonlein purpura

cirrhotic glomerulosclerosis

diffuse proliferative or mesangioproliferative, lupus nephritis

most common primary GN worldwide

most common 2ndary GN worldwide

most common cuase of nephrotic syndrome in kids

most common cause of nephrotic syndrome in AA

most common cause of nephrotic syndrome inwhite adults

IgA nephropathy

DM

MCD

FSGS

Membranous glomerulonephritis

20 y.o male, repeated episode of macroscopic hematuria following URI, mild proteinuri.

chronic RF in 10-15 yr.

IF: IgA1 (3+), C3

IgA nephropathy

more common: adolescence, male

IF: IgA1 in mesangium, not IgA2

Lab: serum IgA increase,

normal complement level

UA: RBC, protein

LM: can be mesangoproliferative appearing or FSGS appearing depending on stage

IF: IgA1 in mesangium, IgG, C3.

EM: EDD in mesengium

IgA nephropathy / Berger's disease

IgA nephropathy

Px:

Dx

30% to RF in 10-15 yr.

renal transplant. IgA deposit common.

DD nephrotic syndrome

nephrotic syndrome + hematuria + decreased GFR + HTN

MCD

FSGS

19 y.o. w/ nephrotic syndrome

IF: segmental positive glomerular rxn w/ C3 and IgM

Pathogenesis

MCD

idiopathic FSGS

MGN

Renal amyloidosis

membranous type Lupus Nephritis

HIV-related FSGS

T cell mediated release of permeability factor enhancing albumin mnt across wall.

defect in visceral epi structural protein

immune complex disease 2ndary to lupus, syphilis, malaria, viral hep, gold therapy, malignancy causing irreversible thickened BM.

AL amyloid deposits secondary to chronic inflammatory condition: TB, rheumatic, IBD, osteomyelitis, bronchiectasis, leprosy,

LM: hyalinosis, and foam cell seen in parts of glomerular tufts

with increased masangial matrix and collapse/obliteration of capillary lumens and compression of BM

hypereosinophilia in some glomeruli

initially in Juxtamedullary glomeruli

tubular atrophy

interstitial fibrosis

EM: effacement of epithelial cell foot processes.

lifting/detachment of epithelial cell from BM

hyperplastic epi w/ increased cytoplasmicc vesicle.

no EDD

thickened BM

increased mesangial matrix, absence of capillary lumen

LM: hyalinosis, and foam cell seen in parts of glomerular tufts

with increased masangial matrix and collapse/obliteration of capillary lumens and compression of BM

hypereosinophilia in some glomeruli

collapse of glomerular capillary loop

prominent visceral epi cell proliferation

initially in Juxtamedullary glomeruli

tubular atrophy

interstitial fibrosis

EM: effacement of epithelial cell foot processes.

lifting/detachment of epithelial cell from BM

hyperplastic epi w/ increased cytoplasmicc vesicle/foam cell

no EDD

thickened BM

increased mesangial matrix, absence of capillary lumen

collapsing FSGS

segmental lesion

focal lesion

35 y.o. F w/ lupus, slow indolent nephrotic syndrome, hematuria, HTN.

IF: intense positive finely granular IgG + C3 on all glomerular BM uniformly.

EM: thickened BM, subepithelial EDD with spikes, intramembranous EDD in BM in advanced stage. 

diffuse effacement of epi cell foot process.

mesengail deposit + mesengial cell proliferation

membranous type Lupus Nephritis

R/o membranous GN by lupus.

DD

few, large subepithelial EDD

numerous, small subepithelial EDD

PIGN

membranous type Lupus Nephritis

15 y.o. M

acute onset of hematuria, proteinuria, RBC cast, persistent low C3

IF: positive IgG + C3 on GBM and mesangium

MPGN type 1

nephritic syndrome

persistent hypocomplementemia -> MPGN, r/o PIGN

IgG + C3 -> type 1

young adults ->primary MPGN

but clinically it can also be prototypical PIGN

LM: all glomerular has increased matrix and hypercellularity, majority of capillary is collapsed.

cellular crescent is observed

splitting of BM

accentuation of lobulation of glomerular tuft

irregular thickening of capillary wall

IF: C3 + IgG

EM: subendothelial EDD

extension of mesangial cell in capillary BM

MPGN type 1

Immune complex disease

LM: all glomerular has increased matrix and hypercellularity, majority of capillary is collapsed.

cellular crescent is observed

splitting of BM

no accentuation of lobulation of glomerular tuft b/c less mesengial proliferation

IF: C3

serum: C3 nephritic factor, lack of IgG and early complment.

EM: BM entirely replaced by EDD.

duplication of BM

MPGN type 2

Dense Deposit Disease

Px:

PIGN

MPGN

few rapid: -> severe diffuse proliferation GN + crescents -> RF

recovery:

self limiting.

chronic: slght proteinuria + HTN -> ESRD later. biopsy show MPGN

kids recover better than adults.

both type has hypocomplementemia, tend to progress to renal failure, type 1 more common, and is a disease of kids and young adult. Hep C + lupus are 2ndary causes

MGN

27 y.o ->adult onset

nephrotic syndrome -> MCD, FSGS, MGN

insidious onset of edema -> MGN or lupus

LM: all glomeruli, uniform diffuse thickening of capillary wall/BM

mild mesangial cellularity

eventially sclerosed glomeruli

tubules cocntain lipid droplets appearing as foamy cell.

IF:IgG, C3 on GBM, finely granular

EM: numerous, small EDD on subepithelial location all along GBM.

become intramembranous in advanced stage.

Px:

MCD

ideopathic FSGS

membranous glomerulopathy

good prognosis, adult more intense, premature withdrawal incrase early relapse. Renal function is good, no HTN

bad prognosis, worse when have collapsing FSGS

bad prognosis, progressive and slow deterioration to ESRD.

6 y.o. M, hematuria, proteinuria, persistently low C3.

IF: IgM + C3 on GBM

MPGN type 2

hematuria + proteinuria -> nephritic + nephrotic

persistently low C3 -> lupus + MPGN

C3 ->type 2

7 y o F, lupus

heavy proteinuria, arteritis

low C3, + ANA

cellular crescent

IF: positive in GBM, mesangium , and vesel wall with C3, IgG

lupus nephritis, diffuse proliferative type

diffuse proliferative

5 year prognosis = 38% - 83 %

IM: hematoxylin body

cell crescent

wire loop lesion

bright pink hyaline thrombi

widespread sclerosis

IF: IgG, IgM, IgA, C3

EM: subendo + subepi, in vessel wall + tubular BM

Diffuse proliferative, lupus nephritis

vasculitis + interstitial nephritis

9 y o female with lupus, normal GFR, hematuria, mild proteinuria

IF: + on mesangium, C3, IgM, IgA. no deposits on BM

suspect mild disease

BM is well preserved.

mesangioproliferative, lupus nephritis

42 y. o. lupus, nephrotic syndrome,

IM: tubular atrophy, prominent thickening of all GBM

cell crescent

IF: + finely granular rxn on GBM, IgG, C3

EM: supepithelial + intermembranous EDD

tubular atrophy = chronic change

GBM change only, think membranous GN

membranous, lupus nephritis

11 y.o., hematuria,

mother w/ renal disease

IF: segmental + rxn on glomeruli w/ IgM

EM: thinning of BM but foot process normal. diffuse alteration of GBM, irregular lamination of lamina densa.

think hereditary

hematuria always, nephrotic lateron

has basket-weave pattern on BM

Alport's disease

hematuria, proteinuria, deafness, cataract

M

Fm

LM: mesangioproliferative, FSGS, interstitial foam

IF: nonspecific

EM: thinning + thickening of GBM, splintered/basket-weave apearancec of lamina densa

triad of sex-linked alport syndrome

alpha 5 chain of type 4 collagen fucked.

EM show diffuse alteration of GBM and multilayered laminated lamina densa

28 y.o. w/ hemolytic anemia, thrombocytopenia, renal failure

IF: IgM, fibrinogen

EM: subendothelial electron-lucent widening

not immune complex disease

has fibrin inside capilary lumen

thrombotic disease

HUS

Dx:

thrombotic disease

DIC

HUS

TTP

Dx:

electron lucency in subendothelial area

transplant glomerulopathy

DIC

HUS

TTP

50 y.o nephrotic syndrome

IF: nonspecific

EM: thickening BM, no EDD

nephrotic syndrome: MCD, FSGS, membranous, transplant, amyloidosis, lupus, HIV related

bad BM: r/o MCD, transplant

no sclerosis: r/o FSGS

nonspecific IF: r/o immun complex disease

no HIV...

smooth, homogenous, eosinophilic fibrils present in subendothelial and mesangial space

Renal amyloidosis

3 primary:

alport,

Thin basement membrane disease,

IgA nephropathy

3 secondary:

lupus, mesangioproliferative

DM

HTN

isolated hematuria, proteinuria

fx: AD

UA: RBC

LM: normal

IF: negative

EM: thinning of GBM

absence of alport syndrome

Thin BM disease

proteinuria of more than 3g / 24 hr

hypoalbuminemia

edema

hypercholesterolemia

primary:

MCD

FSGS

membranous

transplant

secondary:

amyloidosis

lupus, membranous type

HIV-nephropathy

form of renal amyloidosis

primary

secondary

AL amyloid, CT structures, kidney 35%

AA amyloid from SerumAmyloidAssocicated protein in chronic inflammation situation, affect parenchyma, kidney 80-90%

DD:

glomerular deposition of fibrils

use congo red

+: renal amyloidosis

-: fibrillary glomerulopathy: fibrils larger than amyloid

pirmary:

PIGN

MPGN type 1, type 2

IgA nephropathy

secondary:

diffuse proliferative

focal proliferative

Henoch-Schonlein purpura

microscopic Polyarteritis

Wegener's

Anti-GBM disease

most common cause of nephrotic syndrome in renal transplant patient

most common IC glomerulonephritis in transplant patient

transplant glomerulopathy

membranous glomerulonephritis

what do u think of when u see increae in glomerular cellularity and obliteration of capillary loop

hematuria, RBC cast, HTN + fluid overload, renal deficiency

children

upper respiratory infection, then see GN, urpura, abdominal symptom, arthralgia

IF: IgA

P-ANCA

LM: fibrinoid necrosis of medium sized arteries,

show granulation in repairing stage

show fibrosis in healed stage

P-ANCA

LM: fibrinoid necrosis of small artery, arteriole,

focal necrotizing GN

crescentic GN

C-ANCA

LM: cellular proliferation, fibrinoid necrosis,

crescent-GN

anti-GBM disease:

goodpasture's syndrome

LM: focal crescentic GN

IF GBM + alveolar BM: diffuse fine, linear pattern of IgG, C3

crescent stain for fibrinogen

EM: void of EDD