膜性肾病合并抗肾小球基底膜病研究进展

Research Progress for Membranous Nephropathy Complicated with Anti-Glomerular Membrane Disease

摘要: 膜性肾病(membranous nephropathy, MN)的典型病理表现是上皮下存在免疫复合物，而在临床中观察到MN患者组织中有新月体的形成，这种情况是十分罕见的。引起新月体型MN的常见原因之一是MN合并了抗肾小球基底膜病(glomerular basement membrane, GBM)。近年来逐渐有MN合并抗GBM病的病例及相关研究报道。在这一类疾病中，MN和抗GBM病可同时出现或先后发生，因此该病例既有MN的临床表现，又有抗GBM病的临床表现，在这些病例的血清中均未发现原发性MN的特征性抗体，但均存在抗GBM抗体阳性。MN合并抗GBM病确切的机制尚不明确，患者的免疫学特点、人类白细胞抗原易感性以及基于动物实验等方面的研究能够为探寻该病的临床表现和发病机制提供一些线索。由于抗GBM抗体的致病性，使得合并抗GBM病的MN患者往往肾脏预后不良，但临床医师对这一类疾病的认识尚不足，本文通过回顾近年来MN合并抗GBM病的病例报道，相关领域的研究进展，对其发病机制、临床病理特征、治疗及预后进行综述。

Abstract: The typical pathology of membranous nephropathy (MN) is the presence of immune complexes un-der the epithelium, while the formation of crescent bodies in the tissues of patients with MN is ob-served clinically, which is very rare. One of the common causes of crescentic MN is the combination of MN with glomerular basement membrane disease (GBM). In recent years, cases of MN combined with anti-GBM disease and related studies have been gradually reported. The exact mechanism of MN combined with anti-GBM disease is not clear, and the immunological characteristics of the pa-tients, their human leukocyte antigen susceptibility, and the animal-based studies are not clear. The exact mechanism of MN combined with anti-GBM disease is not clear, and the immunological characteristics of patients, their susceptibility to human leukocyte antigens, and studies based on animal experiments can provide some clues to explore the clinical presentation and pathogenesis of the disease. Due to the pathogenicity of anti-GBM antibodies, MN patients with combined anti-GBM disease often have a poor renal prognosis, but clinicians are not yet aware of this group of diseases. In this paper, we review the pathogenesis, clinicopathological features, treatment and prognosis of MN combined with anti-GBM disease by reviewing the case reports of MN combined with anti-GBM disease in recent years, and the progress of research in related fields.