作者
Antonio Greco, Maria Ida Rizzo, Armando De Virgilio, Andrea Gallo, Massimo Fusconi, Giulio Pagliuca, Salvatore Martellucci, Rosaria Turchetta, Lucia Longo, Marco De Vincentiis
发表日期
2015/3/1
来源
Autoimmunity reviews
卷号
14
期号
3
页码范围
246-253
出版商
Elsevier
简介
Goodpasture's syndrome (GS) is a rare and organ-specific autoimmune disease that is mediated by anti-glomerular basement membrane (anti-GBM) antibodies and has pathology characterized by crescentic glomerulonephritis with linear immunofluorescent staining for IgG on the GBM. It typically presents as acute renal failure caused by a rapidly progressive glomerulonephritis, accompanied by pulmonary hemorrhage that may be life-threatening. It was first described as a distinctive syndrome by Pasture in 1919. Autoimmune Inner Ear Disease (AIED) may be associated. The etiology of GS is unknown. Researchers hypothesized a genetic predisposition HLA-associated. Complex immunological mechanisms are in the pathogenesis. The disease is caused by autoantibodies against the NC1 domain of the alpha 3 chain of type IV collagen. The limited presence of this molecule in the body explains the interest …
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A Greco, MI Rizzo, A De Virgilio, A Gallo, M Fusconi… - Autoimmunity reviews, 2015