Fcγ Receptor Iib–Deficient Mice Develop Goodpasture's Syndrome upon Immunization with Type IV Collagen

• DOI: 10.1084/jem.191.5.899
• Publication Date: 2002-07-26T16:48:33Z
• Authors: Akira Nakamura; Takae Yuasa; Azusa Ujike; Masao Ono; Toshihiro Nukiwa; Jeffrey V. Ravetch; Toshiyuki Takai
• Keywords: Goodpasture syndrome; Immune complex

The combination of hemorrhagic pneumonitis and rapidly progressive glomerulonephritis is a characteristic feature Goodpasture's syndrome (GPS), an autoimmune disease resulting from the interaction pathogenic anti–collagen type IV (C-IV) antibodies with alveolar glomerular basement membranes. Lack suitable animal model for this fatal has hampered both basic understanding its etiology development therapeutic strategies. We now report novel GPS using mice deficient in central regulatory receptor immunoglobulin (Ig)G antibody expression function, IIB Fc IgG (FcγRIIB). Mutant immunized bovine C-IV reproducibly develop massive pulmonary hemorrhage neutrophil macrophage infiltration crescentic glomerulonephritis. distinctive linear, ribbon-like deposition immune complex seen was observed along tubulointerstitial membranes diseased animals. These results highlight role FcγRIIB maintaining tolerance suggest that it may play pathogenesis human GPS.