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Renal - pathology > Glomerular disease > Flashcards

Glomerular disease Flashcards

1
Q

What is the broad definition of nephritic syndrome, conceptually?

A

Glomerular disorder characterized by inflammation and bleeding due to disruption of glomerular basement membrane.

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2
Q

What findings are associated with nephritic syndrome?

A
  1. Limited proteinuria (<3.5g/day)
  2. Oliguria
  3. Azotemia
  4. Salt retention with periorbital edema and HTN
  5. RBC casts and dysmorphic RBCs in urine
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3
Q

What are the subtypes of nephritic syndrome?

A
  1. Acute post-streptococcal glomerulonephritis
  2. IgA nephropathy (Berger disease)
  3. Rapidly progressive glomerulonephritis
  4. Diffuse proliferative glomerulonephritits
  5. Membranoproliferative glomerulonephritis
  6. Alport sydrome
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4
Q

What is the classic prelude to PSGN?

A

~2 weeks after group A streptococcal infection involving pharynx or impetigo. Due to nephritogenic strains that carry M protein.

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5
Q

What is the presentation of PSGN?

A

Hematuria (cola colored urine), oliguria, hypertension, and periorbital edema.

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6
Q

What kind of hypersensitivity reaction is PSGN?

A

Type III hypersensitivity, involving immune-complex (antibody+antigen).

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7
Q

What is the appearance of PSGN on light microscopy?

A

Hypercellular, enlarged glomeruli.

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8
Q

What is the appearance of PSGN on immunoflorescence? Why?

A

“Starry sky” granular appearance/lumpy-bumpy appearance. Due to immune complex deposition (IgM, IgG, C3) along GBM and mesangium.

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9
Q

What is the appearance of PSGN on electron microscopy?

A

Subepithelial immune complex humps –> get deposited and pile up.

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10
Q

What is the prognosis of PSGN?

A

Almost always resolves spontaneously in children– <1% children progress to renal failure.
25% of adults progress to renal failure.

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11
Q

What other lab findings are associated with PSGN?

A

Incr anti-DNAse B titers (antibodies to strep) and decreased complement levels (used up and deposited)

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12
Q

What is the classic prelude to IgA nephropathy?

A

2-3 days following mucosal infection (esp gastroenteritis/URI). Common in children, most common nephropathy worldwide.

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13
Q

What is the pathogenesis of IgA nephropathy?

A

IgA immune complex deposition in MESANGIUM of glomeruli.

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14
Q

What is the presentation of IgA nephropathy?

A

Episodic hematuria with RBC casts 2-3 days following mucosal infection - often gastroenteritis. May present as acute renal insufficiency.

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15
Q

What are the findings on light microscopy of IgA nephropathy?

A

Mesangial proliferation

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16
Q

What are the findings on immunoflorescence of IgA nephropathy?

A

IgA based IC deposits in mesangium

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17
Q

What are the findings on electron microscopy of IgA nephropathy?

A

Mesangial IC deposits.

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18
Q

What systemic syndrome can IgA nephropathy be involved in?

A

Henoch-Schonlein purpura.

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19
Q

What organ systems are involved in HSP?

A
  1. Skin - purpuric lesions on extensor surfaces
  2. GI - abdominal pain, vomiting, intestinal bleeding, intuss
  3. Renal - IgA nephropathy
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20
Q

What will biopsy of HSP purpura show on light microscopy?

A

Necrotizing vasculitis of small dermal vessels and subepidermal hemorrhage

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21
Q

What will biopsy of HSP purpura show on immunofluorscence?

A

IgA deposition in dermal capillaries

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22
Q

What is the prognosis of IgA nephropathy?

A

May slowly progress to renal failure.

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23
Q

What is the presentation of Rapidly progressive glomerulonephritis?

A

Very rapidly worsening nephritic syndrome that may progress to renal failure in weeks to months

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24
Q

What is the hallmark of RPGN on light microscopy and IF?

A

Crescents in bowman’s space.

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25
Q

What do the crescents of RPGN consist of?

A

Fibrin and macrophages, + plasma proteins (C3b), some glomerular cellls

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26
Q

What are three disease processes that may result in RPGN?

A
  1. Goodpasture syndrome
  2. Granulomatosis w/ polyangitis
  3. Microscopic polyangitis
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27
Q

What are the associated findings with goodpasture syndrome? (sx, hypersensitivity type, IF findings)

A

Hematuria and hemoptysis.
Type II hypersensitivity, with antibodies to GBM and alveolar basement membrane.
Linear IF.

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28
Q

What are the associated findings with Granulomatosis with polyangitis?

A

Hematuria/hemoptysis, with granulomatous inflammation.
c-ANCA.
Negative IF.

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29
Q

What are the associated findings with Microscopic polyangitis?

A

Necrosis on bx.
p-ANCA, MPO-ANCA.
Negative IF.

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30
Q

What is the presentation of Diffuse proliferative glomerulonephritis?

A

Often presents as nephritic and nephrotic syndromes concurrently

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31
Q

What disease process is diffuse proliferative glomerulonepthritis associated with?

A

SLE - most common cause of death in SLE

32
Q

What are the findings on light microscopy of DPGN?

A

“wire looping” of capillaries

33
Q

What are the findings on immunoflourscnece of DPGN?

A

granular appearance due to deposition

34
Q

What are the findings on EM of DPGN?

A

subendothelial and sometimes intramembranous IgG-based immune complexes, often with C3 deposition.

35
Q

What is the presentation of membranoproliferative glomerulonephritis?

A

Nephritic syndrome that often copresents with nephrotic syndrome.

36
Q

What is “proliferative” about MPGN?

A

Cytoplasm of mesangial cells

37
Q

What are the two types of MPGN? What is the difference between them?

A

Both are due to immune complex deposition with thick GBM membrane - difference is location of deposits.

  1. Subendothelial immune complex deposits
  2. Intramembranous immune complex deposits
38
Q

What are the classic findings of Type I MPGN?

A

EM: Subendothelial IC deposits
IF: granular
Light microscopy: tram track apperance due to GBM splitting caused by mesangial proliferation

39
Q

What are associated diseases with Type I MPGN?

A

HBV and HCV.

40
Q

What are the classic findings of Type II MPGN?

A

Intramembranous “dense deposits” associated with C3 nephritic factor.

41
Q

What is C3 nephritic factor?

A

Stabilizes C3 convertase, leading to decrease serum C3 levels, overactivation of complement and inflammation.

42
Q

What is the prognosis of MPGN?

A

Tends to have a poor response to steroids, progresses to chronic renal failure.

43
Q

What is the pathogensis of Alport syndrome?

A

X-linked mutation in Type IV collagen –> thinning and splitting of glomerular basement membrane.

44
Q

What is the presentation of Alport syndrome?

A

Isolated hematuria, sensorineural hearing loss, and ocular disturbances (retinopathy, lens dislocation)

45
Q

What is the electron microscopy finding of Alport syndrome?

A

Basket-weave appearance.

46
Q

What is the basic principle behind nephrotic syndrome?

A

Charge barrier (basement membrane) disruption: massive proteinuria.

47
Q

What are the resulting sx of nephrotic syndrome?

A

Hypoalbuminemia - pitting edema
Hypogammaglobulinema - increased risk of infection
Hypercoaguable state - loss of antithrombin III
Hyperlipidemia and hypercholesterolemia - may result in fatty casts

48
Q

What are the types of nephrotic syndromes?

A

Podocyte: Minimal Change and FSGS
Membrane: Membranous nephropathy and membranoproliferative
Mesangium: DM, amyloidosis

49
Q

In what populations is minimal change disease most commonly seen?

A

Children - most common cause of nephrotic syndrome

50
Q

What are the causes of minimal change disease?

A

Most often idiopathic
Can be triggered by recent infection/immunization
Rarely can be secondary to hodgkin’s lymphoma

51
Q

What are the findings of minimal change disease on light microscopy?

A

normal glomeruli

52
Q

What are the findings of minimal change disease on IF?

A

No findings

53
Q

What are the findings of minimal change disease on EM?

A

Effacement (fusion) of podocyte foot processes.

54
Q

What is the prognosis of minimal change disease?

A

Very good. Usually responds v well to steroids.

55
Q

What is causing the damage in minimal change disease?

A

Cytokine-mediated damage (from T cells)

56
Q

In what populations is focal segmental glomerulosclerosis most commonly seen?

A

Most common cause of nephrotic syndrome in Hispanics and African Americans.

57
Q

What are the causes of FSGS?

A

Most commonly idiopathic, or secondary to:
HIV, Heroin use,
Sickle Cell Dz, massive obesity,
Interferon treatment, CKD due to congenital malformations

58
Q

What are the findings of FSGS on light microscopy?

A

segmental sclerosis and hyalinosis

59
Q

What are the findings of FSGS on immunoflorescence?

A

non-specific IF

60
Q

What are the findings of FSGS on electron microscopy?

A

effacement of podocytes

61
Q

What does the variant of FSGS associated with HIV consistent of?

A

Collapsing glomerulonephropathy - collapse and sclerosis of whole glomerular tuft, glom epithelial cell proliferation and hypertrophy, with marked tubular injury w/ accompanying microcyst formation

62
Q

What is the prognosis of FSGS?

A

poor response to steroids, progresses to chronic renal failure.

63
Q

What population is membranous nephropathy associated with?

A

Most common cause of nephrotic syndrome in white people

64
Q

What can membranous nephropathy be assocaited with?

A

Most common nephrotic presentation of SLE
Usually idiopathic, may be associated with:
solid tumors
HBV/HCV
drugs (NSAIDs/ penicillamine)
Antibodies to phospholipase A2 receptor.

65
Q

What are the findings of membranous nephropathy on light microscopy?

A

diffiuse capillary and GBM thickening.

66
Q

What are the findings of membranous nephropathy on IF?

A

Granular due to immune complex deposition

67
Q

What are the findings of membranous nephropathy on EM?

A

Spike and dome appearance with subepithelial deposits –> podocytes are trying to lay down addtiional membrane to sit on, dome forms over the deposits.

68
Q

What is the prognosis of membranous nephropathy?

A

Poor response to steroids, progresses to chronic renal failure.

69
Q

Membranous Nephropathy and Membranoproliferative nephropathy both involve immune complex deposition in the GBM membrane. What differentiates them?

A

Membranous: Subepithelial deposits (above membrane - between epithelial cells and basement membrane)
MPN Type 2: Intramembranous.
MPN Type 1: Subendothelial deposits (below the membrane - this is where mesangial proliferation cuts them in half).

70
Q

What is the pathogenesis of renal dysfunction in DM?

A

High serum glucose leads to non-enzymation glycosylation of the vascular basement membrane –> this leads to hyaline arteriosclerosis.
Non-enzymation glycosylation of efferent arterioles preferentially –> Increases GFR, leads to mesangial expansion and sclerosis

71
Q

How does DM cause microalbuminuria?

A

Sclerosis of efferent arteriole increases GFR

Hyperfiltration injury leads to microalbuminuria

72
Q

What is the classic finding of DM nephropathy?

A

Sclerosis of the mesangium; Kimmelsteil-Wilson nodules (eosinophilic nodular glomerular sclerosis).

73
Q

What is the most commonly involved organ in systemic amyloidosis?

A

Kidney.

74
Q

How does amyloidosis cause nephrotic syndrome?

A

Deposits in the mesangium

75
Q

What is the typical finding of amyloidosis?

A

apple-green bireferingence under polarized light after staining w/ congo red.

Renal - pathology (16 decks)

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