Lupus nephritis
Lupus nephritis
复制标题
狼疮性肾炎
DOI：
10.1038/s41572-019-0141-9
复制
发表时间：
2020-01-23
影响因子：
81.5
通讯作者：
Mohan, Chandra
中科院分区：
文献类型：
--
作者：
Anders, Hans-Joachim;Saxena, Ramesh;Mohan, Chandra
狼疮性肾炎是由自身免疫性疾病系统性红斑狼疮引起的肾脏炎症。本文综述了狼疮性肾炎的流行病学、发病机制及其治疗进展。狼疮性肾炎(LN)是一种肾小球肾炎，是自身免疫性疾病系统性红斑狼疮(SLE)最严重的器官表现之一。大多数SLE患者在确诊后5年内发展为LN，在许多情况下，LN是导致SLE诊断的主要表现。在过去的几十年里，对LN的遗传和致病基础的了解已经有了很大的进步。LN的治疗通常包括免疫抑制治疗，典型的是霉酚酸酯或环磷酰胺和糖皮质激素，尽管这些治疗并不是一致有效的。尽管对疾病发病机制的了解不断增加，治疗方法也有所改进，但LN仍然是SLE患者发病和死亡的主要原因。在最初诊断为系统性红斑狼疮的10年内，5%-20%的LN患者发展为终末期肾脏疾病，与免疫抑制治疗相关的多种并发症，包括感染、骨质疏松症以及心血管和生殖影响，仍然是一个令人担忧的问题。显然，早期、准确地诊断LN并及时开始治疗对于改善SLE患者的预后至关重要。
Lupus nephritis is an inflammation of the kidneys caused by the autoimmune disease systemic lupus erythematosus. This Primer reviews the epidemiology of lupus nephritis, its pathogenesis and advances in its treatment.Lupus nephritis (LN) is a form of glomerulonephritis that constitutes one of the most severe organ manifestations of the autoimmune disease systemic lupus erythematosus (SLE). Most patients with SLE who develop LN do so within 5 years of an SLE diagnosis and, in many cases, LN is the presenting manifestation resulting in the diagnosis of SLE. Understanding of the genetic and pathogenetic basis of LN has improved substantially over the past few decades. Treatment of LN usually involves immunosuppressive therapy, typically with mycophenolate mofetil or cyclophosphamide and with glucocorticoids, although these treatments are not uniformly effective. Despite increased knowledge of disease pathogenesis and improved treatment options, LN remains a substantial cause of morbidity and death among patients with SLE. Within 10 years of an initial SLE diagnosis, 5-20% of patients with LN develop end-stage kidney disease, and the multiple comorbidities associated with immunosuppressive treatment, including infections, osteoporosis and cardiovascular and reproductive effects, remain a concern. Clearly, early and accurate diagnosis of LN and prompt initiation of therapy are of vital importance to improve outcomes in patients with SLE.