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Renal Module > Nephritic Syndrome - MPGN > Flashcards

Nephritic Syndrome - MPGN Flashcards

1
Q

What are the three basic components of Nephritic syndrome?

A
  1. Hematuria
  2. Renal insufficiency
  3. Hypertension
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2
Q

Where is the localization of immune complexes in IgA Nephropathy?

A

Mesangial deposits

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3
Q

Where is the localization of immune deposits in Lupus nephritis?

A

Subendothelial

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4
Q

Where is the localization of immune deposits in post-infectious GN?

A

Subepithelial

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5
Q

What do the immune complexes in MPGN, IgA nephropathy and Lupus Nephritis have in common?

A

The complexes in these diseases are thought to be pre-formed outside the glomeruli and trapped there in the process of filtration.

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6
Q

What effect do subendothelial deposits have on the glomerular capillaries?

A

Subendothelial immune complex deposition is injurious to endothelial cells which can lead to thrombus formation in glomerular capillaries.

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7
Q

What is MPGN?

A

A heterogenous group of uncommon diseases with a shared light microscopic appearance combined thickened glomerular basement with a proliferation of glomerular cells and infiltration of inflammatory cells.

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8
Q

What is the characteristic appearance of the GBM in MPGN?

A

Thickening and splitting causes a Tram Track appearance on Silver stain

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9
Q

What is the most common type of MPGN?

A

Type I MPGN

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10
Q

What are some of the causes of secondary MPGN.

A
  1. HCV (most common)
  2. HBV
  3. Cryoglobulinemia
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11
Q

How does MPGN affect the different age groups?

A

Usually primary in children and secondary in adults.

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12
Q

What is the predominant age range affected by MPGN?

A

7-30

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13
Q

What mediates (causes) type I MPGN?

A

Immune complex deposition causing complement activation by the Classical pathway.

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14
Q

What damages the capillary wall in MPGN Type I?

A

Cytokines and Proteases

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15
Q

What happens to the endothelial cells in MPGN Type I?

A

Loss of fenestration

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16
Q

What would microscopy show in MPGN Type I?

A
  1. Subendothelial deposits

2. Granular deposits of C3 and IgG

17
Q

What would generally be the end result of MPGN?

A

End-stage renal disease

18
Q

What is another name for MPGN Type II?

A

Dense Deposit disease

19
Q

In what subset of patients is type II MPGN most likely to present?

A

Older children and young adults.

20
Q

What usually precedes Type II MPGN?

A

URI

21
Q

Is Type II MPGN / Dense deposit disease caused by immune complexes? d

A

NO NO NO

22
Q

What is the cause of Type II MPGN / Dense deposit disease?

A

Dysregulation of the complement system

23
Q

What pathway is inappropriately and excessively activated in Type II MPGN?

A

The alternative pathway due to congenital mutations of complement regulating proteins or acquired antibodies to them

24
Q

What leads to the injury phase in Type II MPGN?

A

Deposition of complement factors of the alternative pathway and terminal complement complex

25
Q

What are other etiologies of Type II MPGN?

A
  1. Autoantibodies against Complement factor H
  2. Autoantibodies against Complement factor I
  3. Autoantibodies against Complement factor B
  4. Autoantibodies against C3 convertase (C3 nephritic factor.
26
Q

What is the difference on immunoflurescence between Type I and Type II

A

In type II usually there is only granular deposits of C3 along the GBM and in the mesangium but no IgG C1q or C4

27
Q

What differentiates type I from type II on electronmicroscopy?

A

Dense deposits in the basement membrane. Type II features ribbons of dense dark material deposited within the GBM

28
Q

Where else may dense deposits occure in Type II?

A

1.Bruch membrane of eye (drusen)

29
Q

What else differentiates Type II from type I?

A

1.In type II there may b Partial lipodystrophy with loss of subQ fat in the upper half of the body.

30
Q

What is the treatment for MPGN?

A

Prognosis is poor.

Renal Module (29 decks)

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