Renal: 12.4: Nephritic Syndromes Flashcards

1
Q

What is seen on immunofluorescence in IgA nephropathy?

A

IgA-based immune complex deposits in mesangium

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2
Q

Dx?

  • thinning/splitting of the GBM due to type IV collagen defect
  • isolated hematuria, sensory hearing loss, ocular disturbances
A

Alport Syndrome

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3
Q

What are the s/s of post-strep glomerulonephritis (PSGN)?

A
  • hematuria (Coca-Cola colored urine)
  • oliguria
  • HTN
  • periorbital edema
  • *** 2-3 weeks after a strep infection***
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4
Q

Dx?

  • linear immunofluorescence pattern
A

Goodpasture syndrome

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5
Q

Your pt with nephritic signs has a negative immunofluorescence pattern (pauci-immune). What do you do next?

A

test for ANCA

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6
Q

What is the tx for post-strep glomerulonephritis (PSGN)?

A

supportive care

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7
Q

What is Alport Syndrome?

A
  • thinning/splitting of the GBM due to type IV collagen defect
  • isolated hematuria, sensory hearing loss, ocular disturbances
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8
Q

What is Wegener granulomatosis/granulomatosis with polyangiitis?

A
  • a small vessel vasculitis
  • involves lung, kidneys, and skin WITH nasopharyngeal involvement
  • c-ANCA positive
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9
Q

Dx?

  • crescents in Bowman’s space
A

rapidly progressive glomerulonephritis (RPGN)

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10
Q

In Goodpasture syndrome, an antibody against the collagen and alveolar basement membrane create a _____ immunofluorescence pattern.

A

linear

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11
Q

Dx?

  • hematuria (Coca-Cola colored urine)
  • oliguria
  • HTN
  • periorbital edema
  • 2-3 weeks after a strep infection
A

post-strep glomerulonephritis (PSGN)

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12
Q

This is the most common cause of systemic vasculitis in children and is associated with IgA nephropathy/Berger’s disease.

A

Henoch-Schönlein purpura

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13
Q

What is post-strep glomerulonephritis (PSGN)?

A

nephritic syndrome after a Group A hemolytic strep infection

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14
Q

What is seen on light microscopy in IgA nephropathy?

A

mesangial proliferation

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15
Q

Your pt with nephritic signs has a negative immunofluorescence pattern (pauci-immune) and is postitive for p-ANCA. Dx?

A
  • microscopic polyangiitis
  • Churg-Strauss
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16
Q

Dx?

  • an X-linked type IV collagen defect
A

Alport Syndrome

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17
Q

Dx?

  • negative immunofluorescence pattern (pauci-immune)
A
  • Wegener granulomatosis/granulomatosis with polyangiitis
  • Churg-Strauss
18
Q

Dx?

  • Vasculitis 2° to IgA immune complex deposition
  • triad of palpable purpura on buttocks/legs + arthralgias + ab pain
  • post-URI
A

Henoch-Schönlein purpura

19
Q

In rapidly progressive glomerulonephritis (RPGN), what are the crescents made of?

A

fibrin and macs

20
Q

In Goodpasture syndrome, an antibody against the ____ and _____ create a linear immunofluorescence pattern.

A

collagen alveolar basement membrane

21
Q

What causes nephritic syndrome?

A

GBM disruption

22
Q

Dx?

  • episodic gross hematuria with RBC casts
  • respiratory or GI infection
  • granular mesangial deposition of IgA
A

IgA nephropathy/Berger’s disease

23
Q

What is rapidly progressive glomerulonephritis (RPGN)?

A

nephritic syndrome –> renal failure in weeks to months

24
Q

What is Henoch-Schönlein purpura?

A
  • Vasculitis 2° to IgA immune complex deposition
  • triad of:
  1. palpable purpura on buttocks/legs
    • arthralgias
    • ab pain
  • ***post-URI***
25
Q

In ______, an antibody against the collagen and alveolar basement membrane create a linear immunofluorescence pattern.

A

Goodpasture syndrome

26
Q

What is the tx for Wegener granulomatosis/granulomatosis with polyangiitis?

A
  • cyclophosphamide
  • corticosteroids
27
Q

What is cyclophosphamide? What is it used to treat?

A
  • an alkylating agent that cross-links DNA at guanine
  • tx for CA, vasculitis
28
Q

What causes Alport Syndrome?

A

an X-linked type IV collagen defect

29
Q

What is microscopic polyangiitis?

A
  • a small vessel necrotizing vasculitis involving lung, kidney, and skin
  • NO nasopharyngeal involvement
  • p-ANCA positive
30
Q

Dx?

  • hypercellular, inflamed glomerulus
  • granular immune complex deposition
  • subepithelial humps
A

post-strep glomerulonephritis (PSGN)

31
Q

What is the most common nephropathy?

A

IgA nephropathy/Berger’s disease

32
Q

What does C5a attract?

A

neutrophils

33
Q

What virulence factor of Strep causes postpost-strep glomerulonephritis (PSGN)?

A

M protein

34
Q

What is seen on EM in IgA nephropathy?

A

mesangial immune complex deposits

35
Q

What are the biopsy findings in post-strep glomerulonephritis (PSGN)?

A
  • hypercellular, inflamed glomerulus
  • granular immune complex deposition
  • subepithelial humps
36
Q

Ddx?

  • granular immunofluorescence pattern
A
  • post-strep glomerulonephritis (PSGN)
  • diffuse proliferative glomerulonephritis
  • SLE
37
Q

What is Churg-Strauss?

A
  • a small vessel vasculitis
  • p-ANCA positive
  • s/s include:
    • skin nodules/purpura
    • peripheral neuropathy
    • granulomatous inflammation
    • eosinophilia
    • asthma
    • sinusitis
    • coronary arteritis
38
Q

Dx?

  • skin nodules/purpura
  • peripheral neuropathy
  • granulomatous inflammation
  • eosinophilia
  • asthma
  • sinusitis
  • coronary arteritis
  • p-ANCA positive
A

Churg-Strauss

39
Q

This is a c-ANCA positive small vessel vasculitis involving the lung, kidneys, and skin WITH nasopharyngeal involvement.

A

Wegener granulomatosis/granulomatosis with polyangiitis

40
Q

Dx?

  • URI: sinusitis
  • Lower respiratory tract: hemoptysis, cough, dyspnea
  • Renal: hematuria, red cell casts, RPGN with crescents
  • c-ANCA positive
A

Wegener granulomatosis/granulomatosis with polyangiitis

41
Q

Your pt with nephritic signs has a negative immunofluorescence pattern (pauci-immune) and is postitive for c-ANCA. Dx?

A

Wegener granulomatosis/granulomatosis with polyangiitis

42
Q

Dx?

  • a small vessel necrotizing vasculitis involving lung, kidney, and skin
  • NO nasopharyngeal involvement
  • p-ANCA positive
A

microscopic polyangiitis