Goodpasture Syndrome - rapidly progressive GN/ crescent Flashcards
what is good pasture disease ?
rare type of small-vessel vasculitis
Anti-glomerular basement membrane (GBM) disease
associated with both pulmonary haemorrhage and rapidly progressive glomerulonephritis.
pathophysiology of good pasture syndrome ?
nti-glomerular basement membrane (anti-GBM) antibodies against type IV collagen.
risk factor of GP syndrome ?
more common in men
bimodal age distribution (peaks in 20-30 and 60-70 age bracket)
HLA DR2
clinical features of GP syndrome ?
pulmonary haemorrhage
rapidly progressive glomerulonephritis = results in a rapid onset acute kidney injury
nephritis → proteinuria + haematuria
diagnosis of GP syndrome ?
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
management of GP syndrome ?
plasma exchange (plasmapheresis)
cyclophosphamide
steroids -prednisone ,
rituximab
does GP have low compliment levels ?
nO - THEY HAVE NORMAL COMPLEMENT LEVELS