Steblay R W, Rudofsky U H
Clin Immunol Immunopathol. 1983 Apr;27(1):65-80. doi: 10.1016/0090-1229(83)90057-0.
We compared the effects of injecting human, homologous or autologous lung basement membrane (LBM) and complete Freund's adjuvant into 6-month-old sheep and injecting the same antigens into guinea pigs. All sheep receiving human LBM died of antiglomerular basement membrane (GBM) nephritis by Day 126. They had autoantibodies to antigenic determinants shared by fetal or adult sheep and human LBM and GBM and certain nonvascular basement membranes, but these autoantibodies did not localize in the lung in vivo or cause lung hemorrhages. No sheep injected with homologous or autologous LBM had any evidence of anti-GBM autoantibodies or nephritis or specific lung lesions. Their kidneys are indistinguishable by histologic, immunohistologic, and functional studies from kidneys of age-matched controls. Thus, sheep are very tolerant to their own LBM. Although all guinea pigs developed anti-GBM autoantibodies, they did not get anti-GBM nephritis or specific lung lesions. A hypothesis for the pathogenesis of Goodpasture's syndrome is presented.
我们比较了向6月龄绵羊注射人源、同源或自体肺基底膜（LBM）及完全弗氏佐剂，与向豚鼠注射相同抗原的效果。所有接受人源LBM注射的绵羊在第126天时均死于抗肾小球基底膜（GBM）肾炎。它们具有针对胎儿或成年绵羊与人源LBM、GBM以及某些非血管基底膜共有的抗原决定簇的自身抗体，但这些自身抗体在体内并未定位于肺部，也未导致肺出血。注射同源或自体LBM的绵羊没有任何抗GBM自身抗体、肾炎或特定肺部病变的证据。通过组织学、免疫组织学和功能研究，它们的肾脏与年龄匹配的对照羊的肾脏无法区分。因此，绵羊对自身的LBM具有很强的耐受性。尽管所有豚鼠都产生了抗GBM自身抗体，但它们并未患上抗GBM肾炎或特定肺部病变。本文提出了关于Goodpasture综合征发病机制的假说。
