Nephritic syndrome - Anti-GBM disease Flashcards Preview

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Flashcards in Nephritic syndrome - Anti-GBM disease Deck (13)
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1
Q

What characterizes Anti-GBM disease?

A

Characterized by the presence of autoantibodies directed against an epitope in a “non-collagenous” domain of the alpha3 chain of collagen type IV

2
Q

Where is expression of the epitope limited to?

A

Glomerular and pulmonary alveolar basement membranes.

3
Q

What may trigger the formation of the auto anti-bodies?

A
  1. Smoking (most common)
  2. drugs
  3. hydrocarbons
  4. tumors
  5. viruses
4
Q

What sub-set of patients is it most prevalent in?

A

Young white males. There is a genetic predisposition.

5
Q

Histologically how does Anti-GBM disease look?

A

It is a crscentic necrotizing glomerulonephritis with characteristic linear deposits of IgG along the GBM.

6
Q

Are the deposits in Anti-GBM disease visible of EM?

A

No these deposits are not electron dense

7
Q

How is Anti-GBM disease separated from Goodpasture syndrome?

A

With only renal involvement it is referred to as Anti-GBM disease, with renal and pulmonary involvement it is referred to as Goodpasture syndrome

8
Q

What do patients with Anti-GBM disease present with?

A
  1. Prominent hematuria

2. Quickly ensuing renal failure

9
Q

What is the added presentation to Anti-GBM which would turn it into Goodpasture?

A
  1. Pulmonary hemorrhages
  2. Bilateral infiltrates by chest x-ray
  3. Hemoptysis
  4. Pulmonary crackles
10
Q

What other sympoms are common with Anti-GBM/Goodpasture

A
  1. Azotemia
  2. Arthritis or Arthralgia
  3. Dis-proportional Anemia (probably due to pulmonary hemorrhage)
11
Q

Is there a correlation between the titer levels of these antibodies and disease activity?

A

No

12
Q

What are the treatments for Anti-GBM/Goodpasture?

A
  1. Plasmapheresis
  2. Steroids
  3. Cytotoxic agents
13
Q

Who is your typical Anti-GBM patient?

A

Young White Males