Nephritic Disorders Flashcards

1
Q

Nephritic disorders with NORMAL complement levels

A
  • IgA nephropathy/HSP (henoch schonlein purpura)
  • Alport’s
  • SLE I, II, V
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2
Q

Nephritic disorders with LOW complement levels

A
  • PSGN
  • membranoproliferative glomerulonephritis (MPGN)
  • SLE III, IV
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3
Q

Episodes of gross hematuria associated with viral respiratory illness or GI illness, is most likely due to ________

A

IgA nephropathy

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4
Q

To suspect IgA nephropathy, look for gross hematuria associated with _________

A

Upper respiratory tract infection or GI illness

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5
Q

Describe the presentation of someone with HSP (henoch Schonlein purpura)

A

Child with purpuric rash that starts at feet and goes upward and also has hematuria; it is a type of vasculitis

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6
Q

What conditions are associated with IgA nephropathy

A
  • hepatic cirrhosis
  • gluten enteropathy
  • HIV
  • minimal change disease
  • ankylosing spondylitis
  • malignancy
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7
Q

The damage of the glomeruli in IgA nephropathy is due to the IgA immune complexes that activate complement found in _______________

A

Glomerular mesangium

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8
Q

What are you most likely to see in IF in someone with IgA nephropathy?

A

Dominantly IgA deposits in the mesangium (can also see C3 and can also be seen in subendothelium)

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9
Q

PSGN manifests usually 10 days following _______ and 3 weeks following _______

A

Pharyngitis; impetigo

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10
Q

What are the complement levels in PSGN?

A

LOW

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11
Q

Elevated titers of _____________ and __________ and __________ complement are indicative of PSGN

A

Anti-streptolysin O; anti-DNAase B; LOW

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12
Q

↑ antistreptolysin O antibody or ↑ anti DNAase B with low complement is most likely indicative of what renal disease?

A

PSGN

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13
Q

Initially in PSGN, there are __________ IC deposits leading to activation of complement with resulting in _________

A

subendothelial; ↓ GFR

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14
Q

Subepithelial humps are characteristics for ________

A

PSGN

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15
Q

What is the rate of resolution of proteinuria in PSGN and why?

A

SLOW; bc IC deposits are cleared very slowly

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16
Q

What are some features of PSGN you’d see on light microscopy?

A
  • hypercellular glomeruli
  • proliferation of mesangial, endothelial and epithelial
  • closure of capillarity loops due to proliferation and swelling of endothelial cells +leukocyte infiltration
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17
Q

closure of capillary loops is something you see on LM in what renal disease?

A

PSGN

18
Q

In IF of someone with PSGN, you will see granular deposits of _______ in the ____________

A

IgG and C3; mesangium and capillary walls

19
Q

MPGN is characterized by: (3)

MPGN is a pattern under light microscopy NOT a diagnosis

A
  • thickening of BM/ reduplicaiton of capillary BM
  • mesangial proliferation
  • infiltration of inflammatory cells
20
Q

“Tram track” appearance

A

MPGN

21
Q

What is the difference between type I and type II MPGN?

A

Type I: subendothelial deposits

Type II: dense material deposition along GBM (C3 GN = dense deposit disease only in kids)

22
Q

A patients renal biopsy shows splitting of BM and a “tram track” appearance. What are some underlying diseases that they can have?

A
  • lupus
  • hepC
  • cryoglubinemia
  • endocarditis
  • parasitic infection
  • malignancy
23
Q

Type I MPGN will have complement activation via the _____ pathway and Type II via the ________ pathway

A

Classical; alternative

24
Q

What are some lab indications that would make you think of the diagnosis as dense deposit disease?

A
  • normal C4 levels
  • ↓ C3 elves due to C3 nephritic factor which stabilizes C3bBb convertase allowing for constant degradation of C3
  • child
  • MPGN like pattern (dense deposits of C3 in the GBM)
25
Q

What is the typical presentation of someone with RPGN

A

Rapid decline in renal function with severe oliguria and pain. (One day normal, next day renal symptoms)
MEDICAL EMERGENCY

26
Q

What would you see histologically (LM) in someone with RPGN?

A

Proliferative GN with prominent “crescent” formation in glomeruli w/ or w/o segmental necrosis

27
Q

Discuss the different type of crescents seen in _________

A

RPGN;

New to old: cellular → fibrocellular → fibrous (cant treat fibrous because glomeruli already dead)

28
Q

Type I RPGN has ______ staining on IF

A

Linear;

Anti GBM disease: good pastures or idiopathic

29
Q

type 2 RPGN has _____ staining on IF

A

Granular;

Caused by immune complex diseases such as: SLE, post infectious, IgA, HSP

30
Q

Type III has ______ pattern of staining on IF

A

NO STAINING;

Caused by pauci-immune GN which is a form of vasculitis with minimal evidence of HS upon IF staining: wegeners, microscopic PAN, Chung Strauss

31
Q

Which diseases are associated with Type I RPGN?

A

Anti GBM disease: Goodpastures (linear)

32
Q

What type of disease is associated with Type II RPGN?

A

Immune complex diseases such as:

  • SLE
  • post infectious
  • IgA
  • HSP
33
Q

What disease are associated with Type 3 RPGN?

A

Pauci immune :

  • Wegners
  • Chung Strauss
  • Microscopic PAN
34
Q

SLE (lupus) is an autoimmune disorder of unknown etiology characterized by production of _________ and subsequent immune complex formation leading to chronic inflammation and tissue destruction

A

Autoantibodies

35
Q

In lupus nephritis, there is an abnormal excess production of antibodies against “endogenous” nuclear antigens such as:

A

DsDNA, ANA (antinuclear antibody), Sm (anti-Smith), Ro, La

36
Q

What MUST you be able to stain for and see in IF in order to diagnose lupus in a patient?

A

For all classes of lupus nephritis will have a full house (IgG, IgA, IgM, C1q, C3)

MUST HAVE C1Q

37
Q

What is the difference between Class III lupus nephritis and Class IV lupus nephritis?

A

Class III: FOCAL (< 50% affected)

Class IV: DIFFUSE (> 50% affected)

38
Q

What characterizes classs V lupus nephritis?

A

Class V is also called membranous lupus nephritis → SUBEPITHELIAL IMMUNE COMPLEX DEPOSITS with diffuse thinking of GBM (presents same as idiopathic membranous)

39
Q

How is the disease activity of a patient with lupus monitored?

A

Measure:

  • complement
  • anti dsDNA antibodies
  • ESR
  • C-reactive protein
40
Q

Quickly describe the classes of lupus (6) based on histology and EM:

A
Type I: minimal mesangial 
Type II: mesangial proliferative
Type III: focal segmental proliferative
Type IV: diffuse proliferative 
Type V: membranous 
Type VI: advanced sclerosing