| 摘要: |
| C3肾小球病是新提出的肾脏疾病分类, 是指一些罕见的肾炎, 包括致密物沉积病(DDD)、C3肾小球肾炎(C3GN)和CFHR5肾病等。C3肾小球病具有相同的病因, 即补体旁路途径的异常调节, 有时伴基因缺陷或自身抗体异常。C3肾小球病目前尚无持续有效的治疗方法, 但临床正在针对特定的补体成分进行研究治疗, 治疗的时机和持续时间仍有待探索。本文就C3肾小球病的组织学和临床特征、补体检查手段及治疗方法作一综述。 |
| 关键词: C3肾小球病 C3肾小球肾炎 致密物沉积病 补体C3 |
| DOI：10.3724/SP.J.1008.2014.01373 |
| 投稿时间：2014-03-22修订日期：2014-05-13 |
| 基金项目:国家自然科学基金(30900692,81370844), 上海市浦江人才计划(12PJ1403300), 第二军医大学优秀青年学者. |
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| C3 glomerulopathy: advances in diagnosis and treatment |
| SUN Yun-jia1,DAI Bing2* |
(1. Student Brigade, Second Military Medical University, Shanghai 200433, China;
2. Department of Nephrology, Changzheng Hospital, Second Military Medical University, Shanghai 200003, China
*Corresponding author) |
| Abstract: |
| C3 glomerulopathy is a recently defined disease category comprising several rare types of glomerulonephritis (GN), including dense deposit disease (DDD), C3 glomerulonephritis (C3GN), and CFHR5 nephropathy. These disorders share a common etiology involving dysregulation of the complement alternative pathway (AP), with genetic defects and/or autoantibodies seen in a proportion of patients. Currently no consistently effective therapy has been found for C3 glomerulopathy; clinical evaluation of agents targeting specific components of the complement system is undergoing. The appropriate timing and duration of proposed therapies need to be further explored. This review focuses on the clinical and histological features, complement tests and treatments of C3 glomerulopathy. |
| Key words: C3 glomerulopathy C3 glomerulonephritis dense deposit disease complement C3 |
