Clinical manifestations
It usually presents with rapidly progressive renal failure with or without pulmonary hemorrhage.
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.
