非典型抗肾小球基膜病

Atypical anti-glomerular basement membrane disease

抗肾小球基膜(GBM)病是一种较少见但严重的自身免疫性疾病。其特点为血液循环及肾小球基膜存在抗GBM抗体,多数患者有肾脏损害,伴(或)不伴肺累及。既往认为该病临床表现典型,进展迅速,预后极差。现随着诊断技术的提高,临床工作中发现部分患者临床表现或实验室检查结果不完全符合典型的抗GBM病,给临床诊断及治疗带来困难。本文就非典型抗GBM病的发病机制、临床表现及实验室检查等作一综述,旨在提高对非典型抗GBM病的进一步认识,减少误诊及漏诊。

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.