Rapidly Progessive Glomerulonephritis (RPGN) is characterized by glomerular hematuria and renal failure developing over weeks to months, along with epithelial proliferation in Bowman's space leading to crecent formation.
RPGN can occur with immune deposits, either anti-GBM or immune-complex type.
Goodpasture's syndrome is RPGN with accompanying pulmonary hemorrhage.
Immune complex RPGN is almost always seen with other conditions, includiing SLE, cryoglobulinemia, post-nfectious glomerulonephritis, and others
Non-immune-mediated PRGN can be due to small vessel vasculidites such as Wegener's granulomatosis or microscopic polyarteritis.
Biopsy
Anti-GBM GN is characterized by linear capillary staining with IgG and C3
Anti-GBM GN appears to be linked to development of autoimmunity to type IV collagen.
Therapy consists of high-dose oral prednosine, cytotoxic agents such as cyclophosphamide, and plasma exchange.