Lecture 14: Goodpasture's Flashcards
what is goodpasture’s disease also called?
anti-glomerular basement membrane antibody disease (anti-GBM)
what does anti-GBM cause
pulmonary and renal disease
what is anti-GBM caused by
autoantibody to the alpha 3 chain of type IV collagen
what is a genetic marker for anti-GBM
human leukocyte antigen HLA-DRB1*1501 & HLA-DR4
which antibodies do patients also have with this disease
positive anti-neutrophil cytoplasmic antibodies
what are the age peaks?
20-30yrs & 60-70yrs
who is it more prevalent in?
men 60-40 women
what are the symptoms (8)
cough
SOB
heamoptysis
fatigue
malaise
dark urine
decreased urine output
leg oedema
what are used for assessment of kidney function (3)
urea
electrolytes
creatinine levels
what are some of the other lab tests (7)
hepatitis panel
cryoglobulins
Anti-nuclear Antibody
Anti-streptolysin O
Anti-glomerular Basement Membrane antibodies
clotting screen
renal biopsy
what are the differentials (7)
systemic lupus erythematosus
granulomatosis (Wegner’s)
microscopic polyangiitis
churg-strauss syndrome
glomerulonephritis
cryoglobulinemia
hepatitis B/C
explain ANCA with myeloperoxidase (MPO)
indirect-immunofluorescence
MPO is a protein found in azurophilic granules of neutrophils and monocytes
found in 30-40% patients
explain ANCA with proteinase-3 (PR3)
indirect-immunofluorescence
protein at the site of inflammation
found in 90% Wegener’s granulomatosis
levels correlate with disease activity and disappear after treatment
what is seen in the histology for goodpasture’s (2)
a thickened and rigid glomeraular basement membrane
in a silver stain spikes and holes can be seen
what is the gold standard test for amyloid
congo red
