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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.
a simple case introducing clincial presentation and calling for a differential diagnosis to get students thinking.
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.
Anti-GBM GN appears to be linked to development of autoimmunity to type IV collagen.
Biopsy
Anti-GBM GN is characterized by linear capillary staining with IgG and C3
Therapy consists of high-dose oral prednosine, cytotoxic agents such as cyclophosphamide, and plasma exchange.
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