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nephritic Flashcards

1
Q

renal limited GN

A 
IgA
Anti-GBM
MPGN
ANCA
C3
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2
Q

Systemic GN

A

Henoch-Schonlein - associated with IgA

Goospasture - associated with antiGBM

Lupus nephritis - associated with ANA

ANCA -

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3
Q

Nephritic syndrome (4)

A

Hematuria (RBC dysmorphic and RBC casts)
proteinuria
elevated Cr
Elevated BP

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4
Q

Workup

3

A

serum
biopsy
urinalysis

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5
Q

GN associated with aggressive disease? (3)

A

RPGN
Crescentric
PR syndrome

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6
Q

RPGN

Creatinine?

A

serum creatinine doubles or GFR falls by 50% within few days - 3 months

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7
Q

RPGN

Usually associated wtih ?

A

Anti gbm
ANCA
Lupus

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8
Q

RPGN

therapy

A

more aggressive

cytotoxics / plasma exchange

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9
Q

Crescentric GN

capillary wall?

A

rupture

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10
Q

Crescentric GN

Associated wtih

A

Anti gbm
lupus
anca

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11
Q

Crescentric GN

treatment

A

aggressive
cytotoxics
plsama exhcnage

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12
Q

Which Glomerulonephritis are associated with immune complex deposits? (2)

A

lupus

MPGN

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13
Q

Which GNs are associated with antibodies for renal antigens

A

anti gbm

maybe lupus

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14
Q

which GNs are associated wtih other causes of inflammation within the glomeruli?

A

vasculitis (ANCA)
Hemolytic uremic syndrome
C3 glomerulopathy

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15
Q

where can immune complexes deposity?

A

mesangial
subendo
subepi
intramembranous

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16
Q

where do glomerular immune complexes come from?

A

antibodies to glomerular antigens that are capped and shed

antibodies to extraglomerular antigens (infection/cancer.drug)

idiopathic

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17
Q

mesangial immune deposits

clinical picture

A

hematuria - low grade proteinuria - usually not as severe

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18
Q

sub-endo immune complexes

A

hematuria / RBC casts / elevate creatinine / proteinuria

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19
Q

necrotizing lesions / crescentirc disease

A

hematuria / rbc casts / RPRF

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20
Q

IgA nephropathy

cause?

A

mesangial IgA deposits with mesangial expansion - aberrant glycosylation of IgA1
most common cause of GN worldwide

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21
Q

IgA nephropathy presenation

A

hematuria +/- proteinuria

episodes of hematuria may be synphayngetic (w/in days of URI)

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22
Q

IgA course?

A

slowly progressive
30% remit
30% esrd
40% mid

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23
Q

IgA treat?

A

ACE -I

Prednisone

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24
Q

lupus nephritis

affects what % lupus?

A

50%

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25
lupus nephritis | presentation?
variable | between patients and within patient overtime
26
lupus nephritis | cuase?
``` immune complex deposits mesangium (hematuria) I / II subendo (nephritic) III / IV subepi (nephrotic) V scarring (VI) ```
27
ANTI-GBM | CAUSE
Auto antibodies to NC1 domain of alpha3 chain of type IV colagen renal or PR
28
AntiGBM | presentaition
often causes crescentric / necrotizing GN and RPGN
29
AntiGBM | Suspect patients wtih
nephritic syndrome RPGN PR syndrome
30
Anti GBM | Dx
biopsy | detection of antiGBM AB
31
ANTI-GBM | Treatment
cytotoxic steroids plasma exchange
32
ANCA vasculitis | biopsy
pauci immune - few immune compexes seen in kidney but see glomerular inflammation crescentric and necrotizing
33
ANCA mortality without treatment
80% one year
34
ANCA treatment
cytotoxic steroid B cell targeted - retuximab plasma exchange
35
to diagnose | 3
blood urine biopsy
36
As a consequence of acute nephritic syndrome, we see decreased GFR - thus, what do we see happen with the blood?
Na+ and H2O retention --> edema / CHF / HTN Azotemia Hyperkalemia
37
As a consequence of RPGN we see a marked decrease in GFR - what do we see happen with the blood and pee?
excessive fluid retention more azotemia oliguria
38
6 common nephritic diseases?
``` Benign familial hematuria Alport's IgA Post-infectious Focal necrotizing/crescentric Lupus ```
39
Thin basement membrane disease | mutation/prognosis
(benign familial hematuria) excellent prog mutations in gene encoding collagen IV Thin BM
40
Alport disease triad
nephritis deafness ocular lesions
41
Alport disease mutation?
alpha-5 of collagen IV X linked can't form normal BM
42
Basket weave BM
Alport's | usually progresses to ESRD
43
IgA | clinical
usually asymptomatic hematuria / can be nephritic or nephrotic / often coincides with URI or GI infection / liver disease
44
IgA morphology
usually mesangial, focal
45
IgA pathogenesis
Mesangial IgA (abnormal glycosylation) + complement
46
IgA | EM and IF
EM - Mesangial deposits | IF - Mesagangial IgA
47
IgA Therapy and prog
steroids | some progress to esrd
48
IgA systemic association
Henoch Schonlein purpura
49
Henoch Schonlein Purpura =
systemic IgA vasculitis
50
Henoch Schonlein Purpura IgA more likely to see
endocapillary proliferation or crescents | usually
51
in which nephritic disease do we see subepithelial hump-like deposits with starry sky pattern of IF?
Post infectious GN
52
Postinfectious GN | Clinical
usually post-strep infection - | acute nephritic syndrome
53
PI GN | Morphology
diffuse proliferative | exudative GN
54
PI GN | Pathogenesis
exogenous immune complex | SpeB (other cationic exotoxins) can fix complement
55
PI GN EM IF Serology
EM - Subepithelial hump like depsoits IF - Granular GBM - starry sky serology - ASO in strep
56
PI GN | Treatment?
supportive 99% kids recover 60% adults
57
Focal segmental necrotizing crescentric GN | If we see linear staining think?
Goodpastures | AntiGBM (against alpha-3 chain of collagen iv)
58
Focal segmental necrotizing crescentric GN | If we see granular staining think?
SLE (ANA) | c3/c4 depletion
59
Focal segmental necrotizing crescentric GN | If we see no staining think?
ANCA (Wegener's) pauci-immune
60
Focal segmental necrotizing crescentric GN | what is fourth possiblity
endocarditis
61
crescentric GN | Clinical?
usually RPGN
62
Crescentric GN | Path
Variable - immune complex - anti-GBM - systemic disease (pauci-immune vasculitis (ANCA) / SLE (ANA) - idiopathic
63
Crescentric GN | Therapy -
steroids cytotoxics plasma
64
Lupus nephritis | path
immune complex deposition - ANA and anti-dsDNA - 70% have renal involve
65
Lupus nephritis IF
Usually full house granular immune complex pattern | IgG, IgA, IgM, C1Q, C3
66
Lupus nephritis | presentiation?
can present as nephritic, RPGN, nephrotic, mixed | variable morph
67
Renal lupus | diffuse MPGN
class IV - most severe
68
Renal lupus | membranous nephropathy
class V
69
Renal lupus | FSGS
class III
70
Renal lupus | mesangial
class II
71
Renal lupus | MCD
class I

Renal (6 decks)

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