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Renal > nephritic > Flashcards

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
Q

lupus nephritis

presentation?

A

variable

between patients and within patient overtime

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26
Q

lupus nephritis

cuase?

A 
immune complex deposits 
mesangium (hematuria) I / II
subendo (nephritic) III / IV
subepi (nephrotic) V
scarring (VI)
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27
Q

ANTI-GBM

CAUSE

A

Auto antibodies to NC1 domain of alpha3 chain of type IV colagen
renal or PR

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28
Q

AntiGBM

presentaition

A

often causes crescentric / necrotizing GN and RPGN

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29
Q

AntiGBM

Suspect patients wtih

A

nephritic syndrome
RPGN
PR syndrome

30
Q

Anti GBM

Dx

A

biopsy

detection of antiGBM AB

31
Q

ANTI-GBM

Treatment

A

cytotoxic
steroids
plasma exchange

32
Q

ANCA vasculitis

biopsy

A

pauci immune - few immune compexes seen in kidney

but see glomerular inflammation crescentric and necrotizing

33
Q

ANCA mortality without treatment

A

80% one year

34
Q

ANCA treatment

A

cytotoxic
steroid
B cell targeted - retuximab
plasma exchange

35
Q

to diagnose

3

A

blood
urine
biopsy

36
Q

As a consequence of acute nephritic syndrome, we see decreased GFR - thus, what do we see happen with the blood?

A

Na+ and H2O retention
–> edema / CHF / HTN
Azotemia
Hyperkalemia

37
Q

As a consequence of RPGN we see a marked decrease in GFR - what do we see happen with the blood and pee?

A

excessive fluid retention
more azotemia
oliguria

38
Q

6 common nephritic diseases?

A 
Benign familial hematuria
Alport's 
IgA
Post-infectious
Focal necrotizing/crescentric 
Lupus
39
Q

Thin basement membrane disease

mutation/prognosis

A

(benign familial hematuria)
excellent prog
mutations in gene encoding collagen IV
Thin BM

40
Q

Alport disease triad

A

nephritis
deafness
ocular lesions

41
Q

Alport disease mutation?

A

alpha-5 of collagen IV
X linked
can’t form normal BM

42
Q

Basket weave BM

A

Alport’s

usually progresses to ESRD

43
Q

IgA

clinical

A

usually asymptomatic hematuria / can be nephritic or nephrotic / often coincides with URI or GI infection / liver disease

44
Q

IgA morphology

A

usually mesangial, focal

45
Q

IgA pathogenesis

A

Mesangial IgA (abnormal glycosylation) + complement

46
Q

IgA

EM and IF

A

EM - Mesangial deposits

IF - Mesagangial IgA

47
Q

IgA Therapy and prog

A

steroids

some progress to esrd

48
Q

IgA systemic association

A

Henoch Schonlein purpura

49
Q

Henoch Schonlein Purpura =

A

systemic IgA vasculitis

50
Q

Henoch Schonlein Purpura IgA more likely to see

A

endocapillary proliferation or crescents

usually

51
Q

in which nephritic disease do we see subepithelial hump-like deposits with starry sky pattern of IF?

A

Post infectious GN

52
Q

Postinfectious GN

Clinical

A

usually post-strep infection -

acute nephritic syndrome

53
Q

PI GN

Morphology

A

diffuse proliferative

exudative GN

54
Q

PI GN

Pathogenesis

A

exogenous immune complex

SpeB (other cationic exotoxins) can fix complement

55
Q

PI GN
EM
IF
Serology

A

EM - Subepithelial hump like depsoits
IF - Granular GBM - starry sky
serology - ASO in strep

56
Q

PI GN

Treatment?

A

supportive
99% kids recover
60% adults

57
Q

Focal segmental necrotizing crescentric GN

If we see linear staining think?

A

Goodpastures

AntiGBM (against alpha-3 chain of collagen iv)

58
Q

Focal segmental necrotizing crescentric GN

If we see granular staining think?

A

SLE (ANA)

c3/c4 depletion

59
Q

Focal segmental necrotizing crescentric GN

If we see no staining think?

A

ANCA
(Wegener’s)
pauci-immune

60
Q

Focal segmental necrotizing crescentric GN

what is fourth possiblity

A

endocarditis

61
Q

crescentric GN

Clinical?

A

usually RPGN

62
Q

Crescentric GN

Path

A

Variable

  • immune complex
  • anti-GBM
  • systemic disease (pauci-immune vasculitis (ANCA) / SLE (ANA)
  • idiopathic
63
Q

Crescentric GN

Therapy -

A

steroids
cytotoxics
plasma

64
Q

Lupus nephritis

path

A

immune complex deposition

  • ANA and anti-dsDNA
  • 70% have renal involve
65
Q

Lupus nephritis IF

A

Usually full house granular immune complex pattern

IgG, IgA, IgM, C1Q, C3

66
Q

Lupus nephritis

presentiation?

A

can present as nephritic, RPGN, nephrotic, mixed

variable morph

67
Q

Renal lupus

diffuse MPGN

A

class IV - most severe

68
Q

Renal lupus

membranous nephropathy

69
Q

Renal lupus

FSGS

70
Q

Renal lupus

mesangial

71
Q

Renal lupus

MCD

Renal (6 decks)

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