Nephritic Syndrome - Pathoma Flashcards Preview

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

Flashcards in Nephritic Syndrome - Pathoma Deck (23):

What is the hallmark of Nephritic Syndrome?

  • Glomerular inflammation
  • Bleeding from glomerulus


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


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


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

Poststreptococcal Glomerulonephritis

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


What bacterial strains cause Poststreptococcal Glomerulonephritis?

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


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


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


What is the treatment for Poststreptococcal Glomerulonephritis?

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


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

Weeks to Months


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)


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



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


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


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


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


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)


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


What is the most common cause of Nephropathy worldwide?

IgA Nephropathy


Where does IgA deposit in IgA nephropathy?

Mesangium of glomeruli


What is the common presentation of IgA Nephropathy?

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


What is the histological hallmark of IgA nephropathy?

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


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


How does Alport Syndrome classically present?

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