12.4: Nephritic syndrome Flashcards

1
Q

What are the two hallmark findings of nephritic syndrome?

A

Glomerular inflammation and bleeding, leading to oliguria and azotemia

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2
Q

What is the amount of proteinuria in nephritic syndrome? Nephrotic syndrome?

A

Nephritic = less than 3.5 g /day

Nephrotic = more than 3.5 g /day

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3
Q

Where does edema present with nephritic syndrome, if at all? Why does this occur?

A

around the eyes d/t salt retention

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4
Q

What are the urinary findings with nephritic syndrome?

A

RBC casts and dysmorphic RBCs in urine

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5
Q

Why is there HTN with nephritic syndrome?

A

Salt retention

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6
Q

Why are there casts with nephritic syndrome?

A

Necrotic RBCs get stuck in the tubules, forming the shape (cast) of the tubule

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7
Q

A bx of the glomerulus with nephritic syndrome will reveal what?

A

Inflamed, hypercellular glomeruli

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8
Q

What is the underlying pathophysiology of nephritic syndrome?

A

Immune complex deposition, which activates complement (C5a) and attracts PMNs

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9
Q

What complement is activated by the immune complexes deposited in nephritic syndrome, and mediates the PMN damage?

A

C5a

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10
Q

What causes the hypercellularity within the glomerulus with nephritic syndrome?

A

PMNs are attracted to the C5a produced by immune complex deposition

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11
Q

What is post-streptococcal glomerulonephritis?

A

Nephritic syndrome that arises after group A, beta-hemolytic strep infection of the SKIN or PHARYNX

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12
Q

What is the virulence factor carried by the GAS species that mediate PSGN? What is the function of this protein, and how does this cause PSGN?

A
  • M protein

- Antigen variation/mimicry

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13
Q

True or false: PSGN only occurs with GAS

A

False- can occur with other, non-strep organisms as well

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14
Q

What are the s/sx of PSGN (4), and when do these occur, relative to the initial strep infection?

A
  • 2-3 weeks after infx
  • Hematuria (cola-urine)
  • Oliguria
  • HTN
  • Periorbital edema
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15
Q

In whom is PSGN usually seen in?

A

Children

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16
Q

What are the findings of PSGN in LM, EM, and IF?

A
LM = hypercellular, inflamed glomeruli
EM = Subepithelial humps
IF = granular IF
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17
Q

What causes the subepithelial humps seen with PSGN?

A

immune complexes build up in the BM, translocate to the epithelium, and aggregate

18
Q

What is the treatment and prognosis for PSGN?

A

Supportive and benign, but rarely progresses to renal failure

19
Q

What is RPGN?

A

Nephritic syndrome that progresses to renal failure within weeks to months

20
Q

What are the LM findings of RPGN?

A

Crescents in bowman’s space

21
Q

What are the crescents comprised of that fill Bowman’s space with RPGN? (2)

A

Fibrin and macrophages

22
Q

What RPGN disease has a linear immunofluorescence? Why is it linear?

A

Goodpasture syndrome.

Linear because there is a line of antibodies against the BM

23
Q

What is Goodpasture syndrome? S/sx? Who does this usually occur in?

A

Antibody against collagen IV, leading to hematuria and hemoptysis

Young male adults affected

24
Q

What are the two causes of granular appearance of immune complexes on IF, in order of incidence?

A
  1. PSGN

2. Diffuse proliferative glomerulonephritis

25
Q

What causes the granular appearance on IF of PSGN and diffuse proliferative glomerulonephritis?

A

Subendothelial deposits of antigen complexes

26
Q

What is the most common renal disease found in SLE?

A

Diffuse proliferative glomerulonephritis

27
Q

What is the cause of nephritic and nephrotic syndrome with SLE pts? Which is more commonly seen?

A

Nephritic = diffuse proliferative glomerulonephritis

Nephrotic = Membranous nephropathy

28
Q

What is pauci-immune pattern of immune complexes? In which nephritic syndromes (3) is this seen?

A

Negative IF:

  • Wegener’s granulomatosis
  • Microscopic polyangiitis
  • Churg-Strauss syndrome
29
Q

What is the next step in the workup of a pt with a pauci-immune IF?

A

ANCA

30
Q

What is the basis of the ANCA test? Results (2)

A

Take a PMN on a slide, and apply ab.

If ab binds to nucleus = pANCA

If ab binds in cytoplasm - cANCA

31
Q

cANCA is associated with what disease? pANCA?

A

cANCA = Wegener’s granulomatosis

pANCA = Churg-strauss syndrome

32
Q

What are the three locations that are involved with Wegener’s granulomatosis?

A
  • Lungs
  • Kidneys
  • Nasopharynx
33
Q

Pt with hematuria, nephritic crescent syndrome, and hemoptysis = ? What about if they also have pharynx associated problems?

A

Goodpasture

Wegener’s with pharynx

34
Q

What are the three things that will be present in Churg-Strauss syndrome that are not present in microscopic polyangiitis?

A
  1. Granulomatous inflammation
  2. Eosinophilia
  3. Asthma
35
Q

What is the most common cause of nephropathy worldwide?

A

IgA nephropathy

36
Q

What is the underlying pathophysiology of IgA nephropathy?

A

IgA immune complex deposition in the mesangium of the glomeruli

37
Q

When does IgA nephropathy usually present? What are the s/sx?

A
  • Childhood, following mucosal infx

- Episodic gross or microscopic hematuria with RBC casts

38
Q

What is the prognosis for IgA nephropathy?

A

May slowly progress to renal failure

39
Q

Where does IgA deposit in the glomerulus?

A

In the mesangium

40
Q

What is Alport syndrome? What is the inheritance pattern? How does it present (3)?

A

XLR defect in type IV collagen, resulting in thinning and splitting of the glomerular BM

Presents as isolated hematuria, sensory hearing loss, and ocular disturbances

41
Q

What causes the hearing loss and ocular disturbances with Alport syndrome?

A

Damage to BM in both cases, caused by crappy type IV collagen