Anti-glomerular basement membrane (GBM) antibody disease
This is an automimmune disease in which autoantibodies are directed against type IV collagen. About 60% have concomittent affection of both glomerular basement membrane and the alveolar capillary membrane which share the same target antigen. This association is known as Goodpasture's syndrome. The binding of antigen to antibody results in an inflammatory reaction.
It usually presents with rapidly progressive renal failure with or without pulmonary hemorrhage.
Serum Anti-GBM, renal biopsy in addition to other biopsies from the skin, lungs and nasal mucosa which maybe required to establish the diagnosis.
The idea is to remove the anti-GBM from serum while at the same time suppressing the new formation of antibodies.
High dose oral prednisone (60mg PO qd tapered over 8 weeks) + CPX 2mg/kg PO qd for 8 weeks in concert with total volume plasma exchange.
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