Nephritic Syndrome - Pathoma Flashcards Preview

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

Flashcards in Nephritic Syndrome - Pathoma Deck (23):
1

What is the hallmark of Nephritic Syndrome?

  • Glomerular inflammation
  • Bleeding from glomerulus

2

What are the common signs and symptoms of Nephritic Syndrome?

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

3

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

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

4

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

Poststreptococcal Glomerulonephritis

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

5

What bacterial strains cause Poststreptococcal Glomerulonephritis?

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

6

What is the classic presentation of Poststreptococcal Glomerulonephritis?

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

7

What would you see on kidney biopsy in Poststreptococcal Glomerulonephritis?

  • Hypercellular, inflamed glomeruli on H&E
    • Mediated by immune complex deposition
      • granular IF
  • Subepithelial "humps" on EM
    • deposits pile up on BM

8

What is the treatment for Poststreptococcal Glomerulonephritis?

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

9

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

Weeks to Months

10

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

  • Crescents in Bowman's Space on H&E
    • comprised of fibrin and macrophages (inflammatory debris)

11

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

Immunofluorescence

12

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

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

13

What is the classic presentation of Goodpasture Syndrome?

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

14

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

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

15

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

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

16

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

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

17

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

  • Churg-Strauss has three things MP does not:
    • 1. granulomatous inflammation
    • 2. eosinophilia
    • 3. asthma

18

What is the most common cause of Nephropathy worldwide?

IgA Nephropathy

19

Where does IgA deposit in IgA nephropathy?

Mesangium of glomeruli

20

What is the common presentation of IgA Nephropathy?

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

21

What is the histological hallmark of IgA nephropathy?

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

22

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?

Alport Syndrome

23

How does Alport Syndrome classically present?

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