Glomerulonephritis Flashcards
define glomerulonephritis
signs…
inflamm injury of glomeruli
infiltration of leukocytes
deposition of immune proteins
glomerulonephritis may involve (4)
1) mesangium
2) podocytes
3) capillaries/endothelium
4) parietal epithelial cells
if you see mesangial proliferative with IgA deposits…
If you see mesangial proliferative with lupus
IgA nephropathy
lupus class 2
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
how does nephritic syndrome affect glomerular capillaries
1) damages wall
2) allows protein into urine and red cells and white cells
“Glomerular” RBCs due to …
damaged with blebs as pass thru capillary wall
RBC cast means…
protein stuck together in shape of tubule
GLOMERULONEPHRITIS
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
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
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
definitive diagnosis of GN requires…
kidney biopsy
make sure patient hold breath or kidney moves
syndromes assoc with aggressive disease
1) RPGN
2) crescentic GN
3) pulm-renal syndrome
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
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
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
causes of GN
1) deposited immune complexes (lupus, MPGN)
2) antibodies for renal antigens
(goodpasture
3) ANCA vascultiis
HUS
C3 glomerulopathy
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
