发病机制
肾小球肾炎
肾病
医学
免疫学
免疫系统
免疫球蛋白A
抗体
病理
肾
免疫球蛋白G
内科学
内分泌学
糖尿病
作者
Kazunori Karasawa,Shota Ogura,Yoei Miyabe,Kenichi Akiyama,Kosaku Nitta,Takahito Moriyama
标识
DOI:10.1016/j.clim.2020.108347
摘要
Galactose-deficient immunoglobulin A1 (Gd-IgA1) was recently identified as a critical effector molecule in the pathogenesis of IgA nephropathy (IgAN). Gd-IgA1 is produced by the mucosal immune system. IgAN is thought to develop because of the deposition of a circulating immune-complex containing Gd-IgA1 in the kidney. Multicentric Castleman's disease (MCD) is a rare non-neoplastic lymphoproliferative disorder. As an etiology model, hypercytokinemia, including increased levels of interleukin-6, is the primary pathogenesis of many MCD cases. Here, we present two cases of mesangial proliferative glomerulonephritis with MCD. According to renal biopsy findings, one was diagnosed with non-IgAN and the other with IgAN. Surprisingly, in both cases, Gd-IgA1 was produced by plasma cells in the lymph nodes, suggesting that Gd-IgA1 production alone does not cause IgAN; rather, it may be produced without induction by mucosal immunity. Our findings demonstrate the diversity of the development of IgAN and help to reconsider the onset mechanism of IgAN.
科研通智能强力驱动
Strongly Powered by AbleSci AI