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12 Kidney Pathology > 12.4 Nephritic Syndrome > Flashcards

12.4 Nephritic Syndrome Flashcards

1
Q
  1. If pt presents with hemoptysis and hematuria, what is first thought at diagnosis?
  2. However, if pt has nasopharyngeal symptoms as well (eg sinusitis), consider what else and why?
  3. use what lab tests to distinguish
A
  1. Goodpasture’s syndrome
  2. Wegener’s granulomatosis (nasopharyngeal symptoms not seen in Goodpasture’s)
  3. use ANCA and IF:

goodpasture’s: linear IF, no ANCA

Wegener’s: no IF, c-ANCA

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2
Q

Goodpasture syndrome

  • mech
  • symptoms
  • classic population
A
  • Linear IF stain, RPGN
  • Ab against collagen in glomerular BM (hematuria )and alveolar BM (hemoptysis)
  • young, adult males
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3
Q

Diffuse proliferative glomerulonephritis

  • mech
  • what is it associated with
A
  • Granular IF, causes RPGN
  • diffuse antigen-Ab complex deposition (usu subendothelial)
  • most common type of renal disease in SLE
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3
Q

Pt with hematuria, hearing loss, and vision loss–suspect what? and ask what questions?

A

Alport syndrome.

-ask if others in family have same symptoms. (usu X linked)

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4
Q

RPGN (rapidly progressive glomerulonephritis)

-diseases that cause it, based on IF pattern (3 patterns)

A
  1. Linear (anti-BM Ab)
    - Goodpasture syndrome
  2. Granular (immune complex deposition)
    - PSGN, diffuse proliferative glomerulonephritis
  3. Negative IF (pauci-immune)
    - Wegener’s granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome
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5
Q

IgA nephropathy (Berger’s disease)

  • what to see on histology
  • progression of disease
A
  • IF can show IgA immune complex deposits in mesangium
  • can progress to renal failure, slowly
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6
Q

PSGN (post streptococcal glomerulonephritis)

  • what kind of strep infection can cause it
  • what is a nephritogenic strain
  • can PSGN occur with non-strep bacteria?
A
  • nephritic syndrome
  • arises after group A B-hemolytic strep infection of skin (impetigo) or pharynx (pharyngitis)
  • requires nephritogenic strains–strep that carries M protein virulence factor
  • Yes, may occur after infection with nonstrep
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7
Q

PSGN (post streptococcal glomerulonephritis)

-what do you see on histology (H&E, EM, IF)

A
  • H&E: hypercellular, inflamed glomeruli (typical for nephritic syndrome)
  • EM: subepithelial humps (immune complexes first deposit in subendothelial space, then to subepithelial, then exit)
  • IF: granular IF
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8
Q

what is feared complication of PSGN (poststrep glomerulonephritis)

A

25% of adults develop RPGN–rapidly progressive glomerulonephritis

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9
Q

IgA nephropathy (Berger’s disease)

  • mech
  • how does it present
A
  • IgA immune complex deposition in mesangium
  • Presents during childhood as episodic hematuria (gross/microscopic) with RBC casts, usu following mucosal infections (eg gastroenteritis). Episodic b/c IgA increases during each infection.
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10
Q

PSGN (post streptococcal glomerulonephritis)

-tx

A
  • supportive
  • complexes (subepithelial humps) eventually disappear
  • children 1% to CRF, adults 25%
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12
Q

RPGN (rapidly progressive glomerulonephritis)

  • what is it, timeframe
  • if you see pt with signs of RPGN, do what?
A
  • nephritic syndrome that progresses to renal failure in weeks to months
  • do biopsy to confirm
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13
Q

what is the most common nephropathy worldwide?

A

IgA nephropathy (Berger’s disease)

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14
Q

Alport syndrome

-mech

A
  • inherited defect in type 4 collagen, most commonly X-linked
  • results in thinning and splitting of glomerular BM
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15
Q

PSGN (post streptococcal glomerulonephritis)

  • clinical presentation (4 symptoms)
  • time after infection?
A
  • hematuria (cola-colored)
  • oliguria
  • HTN
  • periorbital edema
  • 2-3 weeks after infection
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16
Q

RPGN (rapidly progressive glomerulonephritis)

  • what you do see on biopsy?
  • what does biopsy tell you or not tell you
A
  • crescents in Bowman space of glomeruli (made of fibrin and macrophages)
  • biopsy confirms RPGN, but still need to use clinical picture and IF to diagnose etiology
17
Q

Nephritic syndrome

-what do you see on histology

A

-hypercellular (too many cells), inflammed glomerulus

18
Q

Nephritic syndrome

-what is it characterized by?

A
  1. glomerular inflammation, bleeding
  2. limited proteinuira (
  3. oliguria, azotemia
  4. Na retention: periorbital edema, HTN
  5. RBC casts, dysmorphic RBCs in urine
19
Q

PSGN (post streptococcal glomerulonephritis)

-difference btwn how it occurs in children and adults

A
  • children: rarely progress to renal failure (1%)
  • adults: 25% progress to rapidly progressive glomerulonephritis (RPGN)
20
Q

Alport syndrome

-clinical presentation (3 symptoms)

A
  • remember, Type 4 collagen defect means thin, split BM
    1. hematuria
    2. hearing loss
    3. ocular disturbances

12 Kidney Pathology (12 decks)

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