B5-046 Renal Pathology II Flashcards

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1
Q

dominant features of nephritis

3

A
  • hematuria
  • declining GFR
  • “active” urine sediment
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2
Q

subset of nephritis with relatively rapid onset

A

rapidly progressive glomerulonephritis

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3
Q

morphologic correlate of RPGN

A

glomerular crescents

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4
Q

3 causes of RPGN

A
  1. anti-GBM (type II HSR)
  2. immune complex (type III HSR)
  3. ANCA (type II HSR)
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5
Q

classically associated with group A strep

A

post-streptococcal glomerulonephritis

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6
Q

when do post-streptococcal glomerulonephritis symptoms begin?

A

delay, usually about 2 weeks post infections

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7
Q

symptoms of post-streptococcal glomerulonephritis

A
  • gross hematuria
  • hypocomplementemia
  • decline in GFR
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8
Q

pathology of post-streptococcal glomerulonephritis

A

neutrophils, crescents

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9
Q

LM: glomeruli enlarged and hypercellular
IF: granular “starry sky” appearance due to IgG, C3 deposition along GBM and mesangium
EM: subepithelial immune complex humps

A

post-streptococcal glomerulonephritis

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10
Q

IF: IgG, C3
EM: subepithelial humps

A

post-streptococcal glomerulonephritis

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11
Q
  • LM: crescent moon shape
  • crescents consist of fibrin and plasma proteins with glomerular parietal cells, monocytes, and macrophages
A

RPGN

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12
Q

linear IF due to antibodies to GBM and alveolar basement membrane

A

Goodpasture

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13
Q

hematuria associated with simultaneous URTI

A

IgA nephropathy

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14
Q
  • most common in young males
  • normal complement levels
A

IgA nephropathy

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15
Q

episodic hematuria that occurs with RTI or GI infection

A

IgA nephropathy

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16
Q

LM: mesangial proliferation
IF: IgA based immune complex deposits in mesangium
EM: mesangial immune complex deposition

A

IgA nephropathy

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17
Q

IF: mesangial IgA deposition
EM: mesangial electron dense deposits

A

IgA nephropathy

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18
Q

Henoch-Schonlein tetrad

A
  • nephritis
  • rash
  • arthritis
  • purpura
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19
Q
  • patient under 10 years old
  • pathology more likely to have crescents but otherwise indistinguishable from IgA nephropathy
A

Henoch-Schonlein

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20
Q

multi-system autoimmune disorder common in young females

A

lupus nephritis

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21
Q
  • variable presentation including both nephritic and nephrotic syndromes
  • 6 different morphologic patterns
  • proliferative lupus nephritis (class III and IV)
A

lupus nephritis

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22
Q

which classes of lupus nephritis are considered proliferative?

A

III and IV

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23
Q

IF: “full house”
EM: deposits in all glomerular compartments, esp subendothelial

A

lupus nephritis

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24
Q

nephritic syndrome that often copresents with nephrotic syndrome

A

membrano-proliferative glomerulonephritis

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25
Q

Ig+ associated with antigen excess due to infection like HCV or HBV

A

membrano-proliferative glomerulonephritis Type 1

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26
Q

Ig- associated with abnormal alternative complement activation

A

membrano-proliferative glomerulonephritis Type 2

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27
Q

can be secondary to hepatitis B or C, may also be idiopathic

A

membrano-proliferative glomerulonephritis Type I

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28
Q

associated with low C3 nephritic factor

A

membrano-proliferative glomerulonephritis Type II

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29
Q
  • mesangial ingrowth causes GBM splitting
  • “tram track” on H&E and PAS stains
A

membrano-proliferative glomerulonephritis

30
Q

which type of membrano-proliferative glomerulonephritis has subendothelial immune complex deposits with granular IF?

A

Type I membrano-proliferative glomerulonephritis

31
Q

which type of membrano-proliferative glomerulonephritis causes intramembranous deposits (also called dense deposit disease)?

A

Type II membrano-proliferative glomerulonephritis

32
Q

LM: lobular hypercellularity, double contours
IF: IgG and C3

A

Type I membrano-proliferative glomerulonephritis

33
Q

LM: lobular hypercellularity, double contours
IF: C3

A

Type II membrano-proliferative glomerulonephritis

34
Q

auto-antibody to alpha 3 chain of type IV collagen

A

Goodpasture

35
Q
  • pulmonary-renal involement
  • typically young adult males
A

Goodpasture

36
Q

LM: glomerular crescents, no arteritis
IF: linear IgG and C3 in GBM

A

Goodpasture

37
Q

associated with anti-neutrophil cytoplasmic antibodies

c-ANCA or p-ANCA

A

ANCA

38
Q

causes systemic vasculitis affecting small arteries and glomeruli

A

ANCA

39
Q

4 different ANCA syndromes

A
  • GPA
  • MPA
  • EGPA
  • renal-limited
40
Q

may result in RPGN

2

A

ANCA
Goodpasture

41
Q

IF is “pauci-immune”, meaning only minimal, non-specific staining

A

ANCA

42
Q

negative IF with no IgC3 deposition

A

ANCA

pauci immune

43
Q

inherited mutation in type IV collagen causing irregular thinning and thickening and splitting of GBM

A

Alport

44
Q
  • hematuria
  • deafness
  • chronic progressive renal decline
A

Alport

45
Q

mutations associated with Alport

A
  • COL4A5 (x linked)
  • COL4A3 (AD)
  • COL4A4 (AR)
46
Q
  • eye problems
  • glomerulonephritis
  • sensorineural deafness
A

Alport

cant see, cant pee, cant hear a bee

47
Q

“basket” weave appearance due to irregular thickening of GBM

A

Alport

48
Q

EM shows variable splitting, thinning of GBM

A

Alport

49
Q
  • indolent hematuria
  • AD inheritance
  • some patients found to have mutations in COL4A3 or 4
  • EM show thin GBM
A

thin basement membrane disease

50
Q

nephritic syndromes

6

A
  • PSGN
  • IgA nephropathy
  • RPGN
  • lupus nephritis
  • Alport
  • MPGN
51
Q
A

Goodpasture

auto-antibody to COL4A3

52
Q
A

PSGN

53
Q
A

Alport

54
Q
A

ANCA

55
Q
A

lupus nephritis

56
Q
A

Henoch-Shonlein or IgA nephropathy depending on age of patient

57
Q

sinusitis that does not respond to therapy

A

GPA (ANCA)

58
Q
A

ANCA vasculitis

59
Q

nephrin and TRPC6 are mutated in

A

FSGS

60
Q

variant alleles of APOL1 increase the risk of

A

FSGS

and HIVAN

61
Q

auto-antibodies to PLA2R

A

primary membranous glomeruolpathy

62
Q
A

MPGN

63
Q

IgM, IgG, IgA, C3, an C1q deposits

A

lupus nephritis

64
Q

IgA immune deposits

A

IgA nephropathy

65
Q

shows IgG and C3 immune depositis

A

PSGN

66
Q
  • presents commonly in children
  • nephrotic and nephritic
  • C3 levels are low
A

dense deposit disease

MPGN Type II

67
Q

membranoproliferative pattern with “sausage-like” dense deposits in GBM

A

dense deposit disease

MPGN Type II

68
Q

Ig+ staining and subendothelial deposits

A

Ig+ MPGN

69
Q

linear staining of GBM by IF

A

Goodpasture

70
Q

synpharyngitic nephritis

A

IgA nephropathy