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Pathoma - Kidney and Urinary Tract Pathology > Nephritic Syndrome > Flashcards

Nephritic Syndrome Flashcards

1
Q

What characterizes glomerular disorders in nephritic syndrome?

A
  1. Glomerular inflammation

2. Bleeding

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2
Q

What four other symptoms are seen in Nephritic Syndrome?

A
  1. Limited proteinuria (
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3
Q

What does salt retention cause in Nephritic Syndrome?

A

Periorbital edema and hypertension

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4
Q

What causes the red blood cell casts seen in Nephritic Syndrome?

A

Glomerular Bleeding

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5
Q

What does biopsy reveal in Nephritic Syndrome?

A

Hypercellular, inflammed glomeruli

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6
Q

What are RBC casts a sign of?

A

Glomerular bleeding

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7
Q

What causes the hypercellularity seen in Nephritic Syndrome?

A
  1. Immune-complex deposition activates complement

2. C5 attracts neutrophils which mediate damage –> hypercellularity and inflammation

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8
Q

What is Post-Streptococcal Glomerulonephritis (PSGN)?

A

Nephritic syndrome that arises after group A beta hemolytic streptococcal infection of the (1) skin (impetigo) or (2) pharynx

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9
Q

What two things may cause PSGN?

A
  1. Occurs with nephritogenic strains of strep.

2. May occur after infection with non-streptococcal organisms as well

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10
Q

What are nephritogenic strains of strep?

A

They carry M protein virulence factor!!

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11
Q

When does PSGN present?

A

2-3 weeks after infection

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12
Q

What signs and symptoms does PSGN present with?

A
  • Hematuria (cola-colored urine)
  • Oliguria
  • Hypertension
  • Periorbital edema
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13
Q

What populations is PSGN usually seen in?

A

Children, but it may occur in adults

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14
Q

What do you see on H&E with PSGN?

A

Hypercellular, inflamed glomeruli

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15
Q

What mediates PSGN? What do you see on IF?

A

Mediated by immune complex deposition (granular IF)

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16
Q

What do you see on EM of PSGN?

A

Subepithelial ‘humps’

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17
Q

What is the treatment for PSGN?

A

Supportive

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18
Q

How many adults and children with PSGN progress to renal failure?

A
  • Children rarely progress to renal failure (1%)

- Some adults (25%) develop rapidly progressive glomerulonephritis (RPGN)

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19
Q

What is Rapidly Progressive Glomerulonephritis (RPGN)?

A

Nephritic syndrome that progresses to renal failure in weeks to months

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20
Q

What is the first and second thing you do when working up RPGN?

A
  1. Biopsy

2. IF to determine etiology

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21
Q

What are the subepithelial ‘humps’ seen in PSGN?

A

Immune complexes sitting on the basement membrane –> where subepithelial cells would normally sit

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22
Q

What is seen on H&E stain in RPGN?

A

RPGN is characterized by crescents in Bowman’s space (of glomeruli)

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23
Q

What are crescents comprised of?

A

Fibrin and Macrophages

24
Q

What should you use to help figure out the etiology (cause) of RPGN?

A

Clinical picture + IF

25
Q

What nephrotic syndrome is SLE associated with?

A

Membranous Nephropathy

26
Q

What nephritic syndrome is SLE associated with?

A

RPGN

27
Q

What is a p-ANCA?

A

Perinuclear - when antibodies from patient serum bind adjacent to the nucleus

28
Q

What is a c-ANCA?

A

Cytoplasmic - when antibodies bind in the cytoplasm

29
Q

What disease is indicated by a Linear (anti-basement membrane antibody) IF?

A

Goodpasture Syndrome

30
Q

What is the pathogenesis of Goodpasture syndrome?

A

Antibody against collagen in glomerular and alveolar basement membranes

31
Q

What does Goodpasture syndrome present with?

A

Hematuria and hemoptysis

32
Q

What population is Goodpasture syndrome most common in?

A

Young, adult males

33
Q

What diseases are indicated by Granular (immune complex deposition) IF?

A

PSGN (most common)
OR
Diffuse proliferative glomerulonephritis

34
Q

What causes diffuse proliferative glomerulonephritis?

A

Diffuse antigen-antibody complex deposition, usually sub-endothelial

35
Q

What is Diffuse proliferative glomerulonephritis (form of RPGN) common in?

A

Most common type of renal disease in SLE

[And renal disease is the most common cause of death in SLE]

36
Q

What diseases are indicated by Negative IF (pauci-immune)?

A
  • Wegener granulomatosis
  • Microscopic polyangiitis
  • Churg-Strauss Syndrome
37
Q

What test finding is Wegener granulomatosis associated with?

A

+ c-ANCA

38
Q

What systems does Wegener granulomatosis effect?

A
  • Lung
  • Kidney
  • Nasopharyngeal
39
Q

What if a patient presents with RPGN and recurrent sinus infections?

A

Think about Wegener granulomatosis (c-ANCA can be used to separate it from Goodpasture syndrome)

40
Q

What conditions are associated with + p-ANCA?

A
  • Microscopic Polyangiitis

- Churg-Strauss Syndrome

41
Q

What three things distinguish Churg-Strauss Syndrome from Microscopic Polyangiitis?

A
  1. Granulomatous inflammation
  2. Eosinophilia
  3. Asthma
42
Q

What is the most common nephropathy worldwide?

A

IgA Nephropathy (Berger Disease)

43
Q

What causes IgA Nephropathy (Berger disease)?

A

IgA immune complex deposition in mesangium of glomeruli

44
Q

Where does IgA deposit in IgA Nephropathy?

A

Mesangium of glomeruli

45
Q

When do patients usually present with IgA Nephropathy?

A

Childhood

46
Q

What signs and symptoms does IgA Nephropathy usually present with?

A
  • Episodic gross or microscopic hematuria

- RBC casts

47
Q

What does IgA Nephropathy usually follow?

A

Mucosal Infections (e.g. gastroenteritis)

48
Q

What is the pattern of disease in IgA Nephropathy?

A

Relapsing and remitting (symptomatic and asymptomatic) and may lead to renal failure

49
Q

Production of what is increased during infection?

A

IgA production

50
Q

What is seen on IF in IgA Nephropathy (Berger Disease)?

A

IgA immune complex deposition in the mesangium

51
Q

What may IgA Nephropathy slowly progress to?

A

Renal Failure!

52
Q

What is Alport syndrome?

A

Inherited defect in type IV collagen.

53
Q

What is the inheritance associated with Alport syndrome?

A

Most commonly X-linked

54
Q

What does the defect in collagen in Alport syndrome result in?

A

Thinning and splitting or the glomerular basement membrane

55
Q

What does a patient with Alport syndrome present with?

A
  • Isolated hematuria
  • Sensory hearing loss
  • Ocular disturbances

Pathoma - Kidney and Urinary Tract Pathology (9 decks)

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