Goodpasture Syndrome - rapidly progressive GN/ crescent Flashcards

1
Q

what is good pasture disease ?

A

rare type of small-vessel vasculitis
Anti-glomerular basement membrane (GBM) disease
associated with both pulmonary haemorrhage and rapidly progressive glomerulonephritis.

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2
Q

pathophysiology of good pasture syndrome ?

A

nti-glomerular basement membrane (anti-GBM) antibodies against type IV collagen.

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3
Q

risk factor of GP syndrome ?

A

more common in men
bimodal age distribution (peaks in 20-30 and 60-70 age bracket)
HLA DR2

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4
Q

clinical features of GP syndrome ?

A

pulmonary haemorrhage
rapidly progressive glomerulonephritis = results in a rapid onset acute kidney injury
nephritis → proteinuria + haematuria

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5
Q

diagnosis of GP syndrome ?

A

crescentic glomerulonephritis. Glomeruli appear like a crescent moon on light microscopy and immunofluorescence.

renal biopsy
IgG deposits
immunofluorescence
prescence of anti GBM antibodies

specifically showing a strong linear ribbon-like appearance

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6
Q

management of GP syndrome ?

A

plasma exchange (plasmapheresis)
cyclophosphamide
steroids -prednisone ,
rituximab

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7
Q

does GP have low compliment levels ?

A

nO - THEY HAVE NORMAL COMPLEMENT LEVELS

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