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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.
Clinical manifestations
It usually presents with rapidly progressive renal failure with or without
pulmonary hemorrhage.
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Diagnosis
Serum Anti-GBM, renal biopsy in addition to other biopsies from the skin,
lungs and nasal mucosa which maybe required to establish the diagnosis.
Treatment
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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