关键词: 抗肾小球基膜病, 抗肾小球基膜抗体, 免疫复合物抗, 中性粒细胞胞质抗体

Abstract:

Antiglomerular basement membrane(GBM) disease is a rare but severe autoimmune disease.It is characterized by the presence of antiGBM antibodies in circulatory and glomerular basement membrane,and most of the patients have renal involvement,with or without alveolar hemorrhage.It was considered that the clinical manifestations of this disease were typical,the progress was rapid and prognosis was extremely poor.Along with the continuous improvement of diagnostic techniques,it is found that some patients clinical manifestation or laboratory assay results are not completely consistent with  typical antiGBM disease,which brings difficulties to clinical diagnosis and treatment.This paper has discussed about atypical antiGBM disease in pathogenic mechanism,clinical manifestation,and treatments in order to improve the  diagnosis and treatment of atypical antiGBM disease.

Key words: anti-glomerular basement membrane disease, anti-glomerular basement membrane antibody, immune complex, antineutrophilic cytoplasmic antibody