3.2.2 Goodpasture's Dz Flashcards Preview

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Flashcards in 3.2.2 Goodpasture's Dz Deck (17)
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1
Q

What are the main clinical signs of Goodpasture’s Disease/Syndrome?

A

Hemoptysis and kidney disfunction

2
Q

What is important about this renal biopsy?

A

This renal biopsy is showing hypercellularity resulting in circumferential crescent formation

3
Q

What is important about this immunofluorescence microscopy of the kidney?

A

Linear deposition of IgG antibody characteristic of Goodpasture’s

4
Q

What is important about this direct immunfluorescence of a kidney?

A

This is IgG staining in linear pattern along the basement membrane.

5
Q

What disease corresponds with these images?

A

Top - Goodpasture’s - Linear deposits of IgG along the GBM

Middle - Lupus Nephritis - Granular deposits of C1q

Bottom - Granulomatosis with polyangitis - Faint deposits of C3

6
Q

What is the underlying cause of Goodpasture’s?

A

Circulating antibodies against an antigen in the glomerular basement membrane

7
Q

What is the allele associated with Goodpasture’s?

A

HLA-D15

8
Q

What is the target of the auto-antibodies in GP?

A

Non-collagenous 1 (NC1) domain of the alpha-3 or alpha-5 chain of type IV collagen

9
Q

What is the pathophysiology associated with GP’s?

A

IgG antibodies bind to the antigen on the basement membrane. Activation of complement and C3 deposition. Neutrophil dependent inflammation leading to obstruction and glomerular scarring and eventual loss.

10
Q

What testing should be done on a person who is suspected of GP’s?

A

Renal biopsy, Serum assay for anti-GBM antibodies, and ANCAs

11
Q

All four of these images can be correlated to?

A

Crescent shaped and linear deposition of IgG characteristic of GP’s

12
Q

What is the importance of plasmapheresis in treating GP’s?

A

It will remove circulating anti-GBM antibodies and also remove complement proteins.

13
Q

What is the idea behind giving high dose steroids when dealing with a patient with GP’s?

A

These will bind the intracellular GC receptor which will go to the nucleus and alter transcription of genes. Ultimately it will slow cytokine production and impair T cells

14
Q

What is the idea behind giving cyclophosphamide to treat GP’s?

A

It is a strong immunosuppressive agent. Will crosslink DNA, which will affect B cell proliferation and Ig production.

15
Q

What is the idea behind giving Rituximab to patients with GP’s?

A

It is an anti-CD20 targeting CD20 B cells. This will deplete patients B cells. Make sure to give after plasmapheresis.

16
Q

What is the idea behind giving Azathioprine (Imuran) to patients with GP’s?

A

This is a maintenance therapy after maximum dose of cyclophosphamide has been reached. This is a nucleotide synthesis inhibitor. It inhibits B and T cell function. It inhibits B cell proliferation. Inhibits antibody synth. Inhibits NK cell activity

17
Q

GOOD JOB!

A

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