Nephritic syndrome - Anti-GBM disease

Question 1

What characterizes Anti-GBM disease?

Answer 1

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

Question 2

Where is expression of the epitope limited to?

Answer 2

Glomerular and pulmonary alveolar basement membranes.

Question 3

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

Answer 3

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

Question 4

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

Answer 4

Young white males. There is a genetic predisposition.

Question 5

Histologically how does Anti-GBM disease look?

Answer 5

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

Question 6

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

Answer 6

No these deposits are not electron dense

Question 7

How is Anti-GBM disease separated from Goodpasture syndrome?

Answer 7

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

Question 8

What do patients with Anti-GBM disease present with?

Answer 8

1. Prominent hematuria

2. Quickly ensuing renal failure

Question 9

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

Answer 9

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

Question 10

What other sympoms are common with Anti-GBM/Goodpasture

Answer 10

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

Question 11

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

Answer 11

No

Question 12

What are the treatments for Anti-GBM/Goodpasture?

Answer 12

1. Plasmapheresis
2. Steroids
3. Cytotoxic agents

Question 13

Who is your typical Anti-GBM patient?

Answer 13

Young White Males