Centre for Nephrology, Division of Medicine, University College London, Royal Free Hospital, London, UK.
Nephrol Dial Transplant. 2018 Feb 1;33(2):196-202. doi: 10.1093/ndt/gfx057.
Goodpasture's or anti-glomerular basement membrane (GBM) disease is classically characterized by the presence of circulating autoantibodies directed against the non-collagenous domain of the α3 chain of type IV collagen, targeting glomerular and alveolar basement membranes, and associated with rapidly progressive crescentic glomerulonephritis, with alveolar haemorrhage in over half the patients. However, there are increasing examples of variants or atypical presentations of this disease, and novel therapeutic options have been proposed, which nephrologists should be aware of. The pathophysiology of this condition has been understood through molecular analysis of the antibody-antigen interactions and the use of human leucocyte antigen-transgenic animals, while the association of anti-GBM antibodies with anti-neutrophil cytoplasm antibodies and their combined impact on disease phenotype is increasingly recognized, providing some insights into the basis of glomerular damage and autoimmunity.
Goodpasture 病或抗肾小球基底膜（GBM）病的特征通常为循环自身抗体的存在，这些自身抗体针对 IV 型胶原α3 链的非胶原结构域，靶向肾小球和肺泡基底膜，并与新月体性肾小球肾炎快速进展相关，超过一半的患者伴有肺泡出血。然而，这种疾病的变异型或非典型表现的病例越来越多，并且提出了新的治疗选择，肾病学家应该了解这些选择。通过对抗体-抗原相互作用的分子分析以及使用人类白细胞抗原转基因动物，已经了解了这种疾病的病理生理学，同时，抗 GBM 抗体与抗中性粒细胞胞质抗体的关联及其对疾病表型的综合影响也越来越受到重视，这为肾小球损伤和自身免疫的基础提供了一些见解。
