Glomerulonephritis Flashcards by A B

define glomerulonephritis

signs…

inflamm injury of glomeruli

infiltration of leukocytes
deposition of immune proteins

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glomerulonephritis may involve (4)

1) mesangium
2) podocytes
3) capillaries/endothelium
4) parietal epithelial cells

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if you see mesangial proliferative with IgA deposits…

If you see mesangial proliferative with lupus

IgA nephropathy

lupus class 2

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nephritic syndrome

signs

1) hematuria (dysmorphic RBCs + casts)
2) proteinuria (200 mg–> 10g)
3) elev creatinine
4) HTN with loss of kidney function holding on to Na+ and H2O

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how does nephritic syndrome affect glomerular capillaries

1) damages wall

2) allows protein into urine and red cells and white cells

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“Glomerular” RBCs due to …

damaged with blebs as pass thru capillary wall

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RBC cast means…

protein stuck together in shape of tubule

GLOMERULONEPHRITIS

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If ANA or anti-dsDNA positive, …

2) if blood culture positive
3) if ASO psoitive
4) if Hep C positive
5) if hep B positive
6) if ANCA positive
7) if anti-GBM positive

1) lupus
2) bacterial endocarditis
3) post infectious GN
4) cryoglobulinemia
5) membranoproliferative glomerulonephritis
6) pauci-immune vasculitis
7) goodpasture’s

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if patients have low serum complement, what systemic and what renal limited disease

systemic

1) SLE
2) cryoglobulinemia
3) HUS

renal

1) acute post strep GN
2) MPGN

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if patients have normal serum complement, what systemic and what renal limited

systemic =

1) wegener’s
2) henoch-schonlein purpura
3) goodpasture’s
4) polyarteritis nodosa

renal =

1) IgA nephropathy
2) idiopathic RPGN
3) anti-GBM disease

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definitive diagnosis of GN requires…

kidney biopsy

make sure patient hold breath or kidney moves

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syndromes assoc with aggressive disease

1) RPGN
2) crescentic GN
3) pulm-renal syndrome

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RPGN
1) serum creatinine change?
GFR change?

2) assoc with ..
3) treatment

1) creatinine doubles
GFR falls by 50%
within few days to 3 mo

2) anti-GBM
ANCA vasculitis
lupus

3) cytotoxic drugs
plasmapharesis

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crescentic GN
1) signs

1) assoc with…
2) treatment

1) rupture of capillary wall = severe= capillary loops squeezed down with crescents

2) anti-GBM
ANCA vasculitis
lupus

3) cytotoxic drugs
plasmapharesis

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pulm renal syndrome
1) GN with ___

2) presents with ___
3) assoc with __
4) treatment

1) pulm capillaritis

2) nephritic syndrome
hemoptysis

3) anti-GBM
ANCA vasculitis
lupus

4) cytotoxic drugs
plasmapharesis

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causes of GN

1) deposited immune complexes (lupus, MPGN)

2) antibodies for renal antigens
(goodpasture

3) ANCA vascultiis
HUS
C3 glomerulopathy

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types of immune complex deposition

1) mesangial
2) subendothelial
3) subepithelial
4) intra-membranous deposition

glomerular immune complexes may caused by

treatment?

antibodies to ..

1) glomerular antigens
2) extraglomerular antigens (infection, cancer, drugs)
3) idiopathic

treat with plasmapharesis to remove antibodies

mechanism of injury with immune complex

1) immune complex dposit
2) activ complement –> proinflamm
3) injure nearby cells –> affects adaptive immune system

how does immune complex injury change between subendo space vs. subepi space

1) subendo = activ complement –> inflamm capillary wall
2) subepi space –> flux of urine across wall sweeps complex away so less inflamm (membranous disease = antibodies to podocytes- no hematuria or renal failure)

if you see hematuria, low grade proteinuria what find on histology

if you see hematuria, RBC casts, elev creatinine, proteinuria what find on histology

if you see hematuria, RBC casts, rapidly progressive renal faiure what find on histology

mesangial immune complexes

subendo immune complexes

necrotizing lesions or crescentic disease

purpose of plasma exchange

1) remove patient plasma replace with healthy plasma, albumin
2) decr auto-antibodies
3) replace missing factors

how do you treat
anti-GBM disease
anca vasculitis
cryoglobulinemia

plasma exchange

IgA nephropathy
1) significance

2) pathology
3) presents with …
4) assoc with
5) treatment
6) prognosis

1) most common GN
2) mesangial IgA and mesangial expansion
3) hematuria +/- subnephrotic proteinuria, gross hematuria, assoc with URI
4) assoc with aberrant glycosylation of IgA (immune complex)

5) immunosuppressive (prednisone)
blood pressure, ACE inhib

6) 30% ESRD, 30% spont remission

IgA glycosylaton in IgA neuropathy | how to form immune complex

1) Gal-deficient o-glycans bound to IgA | 2) autoantibodies bind O-glycans --> immune complex --> stuck in mesangium

Lupus nephritis 1) affects ___% of lupus patients 2) ___ presentation 3) caused by immune complex deposits ___ if hematuria ___ if nephritic ___ if nephrotic 4) on IF

1) 50% 2) variable 3) mesangium subendothelail subepithelial if subendo --> high dose chemo 4) granular/patchy

lupus nephritis class 1 and 2 1) deposits 2) presentation 3) immunosuppression?

1) mesangial 2) hematuria 3) no

lupus nephritis class 3 and 4 1) deposits 2) presentation 3) immunosuppression?

1) subendothelial 2) hematuria, nephritic syndrome 3) yes

lupus nephritis class 5 1) deposits 2) presentation 3) immunosuppression?

1) subepithelial 2) proteinuria 3) yes

lupus nephritis class 6 1) deposits 2) presentation 3) immunosuppression?

1) scarring > 90% glomeruli 2) nephritic syndrome, progressive renal failure 3) no

Anti-GBM disease 1) caused by... 2) presents as 3) often causes ... 4) on IF on EM

1) autoantibodies to NC1 domain of alpha 3 chain of type 4 collagen 2) renal limited or pulm-renal syndrome 3) crescentic, necrotizing GN and RPGN 4) linear staining = entire basement membrane unlike lupus = granular EM = crescents

anti-gbm suspect in patients with ... diagnosis by...

1) nephritic syndrome RPGN pulm renal syndrome 2) biopsy, detect anti-GBM antibody

treatment of anti-GBM disease if left untreated...

1) cytotoxic drugs (cyclophosphamide) corticosteroids plasma exchange 2) ESRD

ANCA assoc vasculitis 1) type of vasculitis 2) ANCA is ____ 3) on EM 4) treatment

1) small vessel 2) pathogeneic 3) crescents + necrotizing lesions ``` 4) 80% die without treatment cytotoxic drugs (cyclophos) corticosteroids Rituximab Plasma exchange ```

ANCA vasculitis suspect in patients with ... diagnosis by...

1) nephritic syndrome RPGN pulm renal syndrome 2) ANCA (not specific) BIOPSY = PAUCI IMMUNE = FEW IMMUNE COMPLEX

CLINICAL FINDINGS 1) nephritic syndrome (extra-renal involvement (airways) C-ANCA (PR3)

Granulomatosis with polyangiitis

Clinical findings nephritic syndrome with extra-renal involvement P-ANCA (MPO

microscopic polyangitis

lung and skin vasculitis, asthma, eosinophilia P-ANCA (MPO) > C-ANCA (PR3)

Churg-Strauss

C-ANCA

antibodies to PR3 but can be MPO ANCA

P-ANCA

> 90% antibodies to MPO