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
In \_\_\_\_\_\_, an antibody against the collagen and alveolar basement membrane create a linear immunofluorescence pattern.
Goodpasture syndrome
26
What is the tx for Wegener granulomatosis/granulomatosis with polyangiitis?
* cyclophosphamide * corticosteroids
27
What is cyclophosphamide? What is it used to treat?
* an alkylating agent that cross-links DNA at guanine * tx for CA, vasculitis
28
What causes Alport Syndrome?
an X-linked type IV collagen defect
29
What is microscopic polyangiitis?
* a small vessel necrotizing vasculitis involving lung, kidney, and skin * NO nasopharyngeal involvement * p-ANCA positive
30
Dx? * hypercellular, inflamed glomerulus * granular immune complex deposition * subepithelial humps
post-strep glomerulonephritis (PSGN)
31
What is the most common nephropathy?
IgA nephropathy/Berger's disease
32
What does C5a attract?
neutrophils
33
What virulence factor of Strep causes postpost-strep glomerulonephritis (PSGN)?
M protein
34
What is seen on EM in IgA nephropathy?
mesangial immune complex deposits
35
What are the biopsy findings in post-strep glomerulonephritis (PSGN)?
* hypercellular, inflamed glomerulus * granular immune complex deposition * subepithelial humps
36
Ddx? * granular immunofluorescence pattern
* post-strep glomerulonephritis (PSGN) * diffuse proliferative glomerulonephritis * SLE
37
What is Churg-Strauss?
* a small vessel vasculitis * p-ANCA positive * s/s include: * skin nodules/purpura * peripheral neuropathy * granulomatous inflammation * eosinophilia * asthma * sinusitis * coronary arteritis
38
Dx? * skin nodules/purpura * peripheral neuropathy * granulomatous inflammation * eosinophilia * asthma * sinusitis * coronary arteritis * p-ANCA positive
Churg-Strauss
39
This is a c-ANCA positive small vessel vasculitis involving the lung, kidneys, and skin WITH nasopharyngeal involvement.
Wegener granulomatosis/granulomatosis with polyangiitis
40
Dx? * URI: sinusitis * Lower respiratory tract: hemoptysis, cough, dyspnea * Renal: hematuria, red cell casts, RPGN with crescents * c-ANCA positive
Wegener granulomatosis/granulomatosis with polyangiitis
41
Your pt with nephritic signs has a negative immunofluorescence pattern (pauci-immune) and is postitive for c-ANCA. Dx?
Wegener granulomatosis/granulomatosis with polyangiitis
42
Dx? * a small vessel necrotizing vasculitis involving lung, kidney, and skin * NO nasopharyngeal involvement * p-ANCA positive
microscopic polyangiitis