Cheung Chee Kay, Rajasekaran Arun, Barratt Jonathan, Rizk Dana V
Department of Cardiovascular Sciences, University of Leicester, Leicester LE1 7RH, UK.
John Walls Renal Unit, University Hospitals of Leicester NHS Trust, Leicester LE5 4PW, UK.
J Clin Med. 2021 Jun 4;10(11):2493. doi: 10.3390/jcm10112493.
IgA nephropathy remains the most common primary glomerular disease worldwide. It affects children and adults of all ages, and is a leading cause of end-stage kidney disease, making it a considerable public health issue in many countries. Despite being initially described over 50 years ago, there are still no disease specific treatments, with current management for most patients being focused on lifestyle measures and renin-angiotensin-aldosterone system blockade. However, significant advances in the understanding of its pathogenesis have been made particularly over the past decade, leading to great interest in developing new therapeutic strategies, and a significant rise in the number of interventional clinical trials being performed. In this review, we will summarise the current state of management of IgAN, and then describe major areas of interest where new therapies are at their most advanced stages of development, that include the gut mucosal immune system, B cell signalling, the complement system and non-immune modulators. Finally, we describe clinical trials that are taking place in each area and explore future directions for translational research.
IgA肾病仍然是全球最常见的原发性肾小球疾病。它影响各个年龄段的儿童和成人，是终末期肾病的主要病因，在许多国家成为一个相当严重的公共卫生问题。尽管在50多年前就首次被描述，但目前仍没有针对该疾病的特异性治疗方法，大多数患者目前的治疗重点是生活方式措施和肾素-血管紧张素-醛固酮系统阻断。然而，特别是在过去十年中，对其发病机制的理解取得了重大进展，引发了对开发新治疗策略的极大兴趣，并且正在进行的干预性临床试验数量显著增加。在这篇综述中，我们将总结IgA肾病的当前管理状况，然后描述新疗法处于最先进发展阶段的主要关注领域，包括肠道黏膜免疫系统、B细胞信号传导、补体系统和非免疫调节剂。最后，我们描述每个领域正在进行的临床试验，并探索转化研究的未来方向。
