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CRRAB II Week 4 - WLB > Nephritic Syndrome - Pathoma > Flashcards

Nephritic Syndrome - Pathoma Flashcards

1
Q

What is the hallmark of Nephritic Syndrome?

A
  • Glomerular inflammation
  • Bleeding from glomerulus
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2
Q

What are the common signs and symptoms of Nephritic Syndrome?

A
  • Hematuria
  • Oliguria (small urine production)
  • Azotemia (high BUN)
  • Salt retention with periorbital edema
    • fluid retention
  • HTN
  • RBC casts
  • Dysmorphic RBCs in urine
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3
Q

What would be seen in general on a biopsy of glomeruli in Nephritic Syndrome?

A
  • Hypercellular, inflamed glomeruli
  • Immune-complex deposition → activates complement
    • C5a attracts neutrophils → mediate damage
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4
Q

What nephritic syndrome occurs mostly in children after impetigo or strep?

A

Poststreptococcal Glomerulonephritis

(arises after a Beta-hemolytic Strep infection of skin/pharynx)

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5
Q

What bacterial strains cause Poststreptococcal Glomerulonephritis?

A
  • Nephritogenic strains
    • Carry M protein virulence factor
    • may occur after infection with non-strep organisms as well
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6
Q

What is the classic presentation of Poststreptococcal Glomerulonephritis?

A
  • Presentation 2-3 weeks after infection
    • usually child, but may occur in adults
  • Hematuria
    • “coca-cola” colored urine
  • Oliguria
  • Hypertension
  • Periorbital edema
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7
Q

What would you see on kidney biopsy in Poststreptococcal Glomerulonephritis?

A
  • Hypercellular, inflamed glomeruli on H&E
    • Mediated by immune complex deposition
      • granular IF
  • Subepithelial “humps” on EM
    • ​deposits pile up on BM
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8
Q

What is the treatment for Poststreptococcal Glomerulonephritis?

A
  • SUPPORTIVE
    • ​children rarely progress to renal failure
    • some adults develop Rapidly Progressive Glomerulonephritis → renal failure in weeks-months
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9
Q

How soon do patients with Rapidly Progressive Glomerulonephritis progress to renal failure?

A

Weeks to Months

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10
Q

What findings are characteristic of Rapidly Progressive Glomerulonephritis in a kidney biopsy ?

A
  • Crescents in Bowman’s Space on H&E
    • comprised of fibrin and macrophages (inflammatory debris)
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11
Q

What test after renal biopsy will help you figure out the etiology of Rapidly Progressive Glomerulonephritis?

A

Immunofluorescence

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12
Q

What does a finding of Linear immunofluorescence suggest as the etiology of Rapidly Progressive Glomerulonephritis?

A
  • Antibody against the collagen in basement membrane
    • binds basement membrane → creates sharp line = linear immunofluorescence
      • SUGGESTS: Goodpasture Syndrome
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13
Q

What is the classic presentation of Goodpasture Syndrome?

A
  • Hemoptysis
    • antibody against collagen in alveolar basement membrane
  • Hematuria
    • antibody against collagen in glomerular basement membrane
  • Young, adult male
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14
Q

What does a finding of Granular immunofluorescence suggest as the etiology of Rapidly Progressive Glomerulonephritis?

A
  • Immune complex deposition
    • Poststreptococcus Glomerulonephritis
      • most common
    • Diffuse Proliferative Glomerulonephritis
      • diffuse deposition of antibody-antigen complexes subendothelially
        • common in SLE with renal disease
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15
Q

What does a finding of Negative immunofluorescence suggest as the etiology of Rapidly Progressive Glomerulonephritis?

A
  • Pauci-Immune (limited immune)
    • ​Could be:
      • Wegener granulomatosis
      • Microscopic polyangiitis
      • Churg-Strauss Syndrome
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16
Q

After finding a Negative immunofluorescence in the setting of Rapidly Progressive Glomerulonephritis, what is your next step in differentiating between the possible etiologies?

A
  • Perform ANCA test
    • Wegener granulomatosis (c-ANCA)
      • nasopharynx, lung, kidney
    • Microscopic polyangiitis (p-ANCA)
    • Churg-Strauss Syndrome (p-ANCA)
17
Q

How do you differentiate between Churg-Strauss and Microscopic Polyangiitis when you get a p-ANCA result?

A
  • Churg-Strauss has three things MP does not:
      1. granulomatous inflammation
      1. eosinophilia
      1. asthma
18
Q

What is the most common cause of Nephropathy worldwide?

A

IgA Nephropathy

19
Q

Where does IgA deposit in IgA nephropathy?

A

Mesangium of glomeruli

20
Q

What is the common presentation of IgA Nephropathy?

A
  • Episodic gross or microscopic hematuria
  • RBC casts
  • Following mucosal infections
    • produce too much IgA
21
Q

What is the histological hallmark of IgA nephropathy?

A
  • IgA immune complex deposition in the mesangium
    • seen in Immunofluorescence (granular)
22
Q

What syndrome is characterized by an inherited defect in Type IV collagen (most commonly X-linked) that results in thinning and splitting of the glomerular basement membrane?

A

Alport Syndrome

23
Q

How does Alport Syndrome classically present?

A
  • Isolated hematuria
  • Sensory hearing loss
  • Ocular disturbances
    • lens dislocation
    • cataracts
    • corneal dystrophy

CRRAB II Week 4 - WLB (9 decks)

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