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Lupus nephritis.
Nature Reviews Disease Primers ( IF 76.9 ) Pub Date : 2020-01-23 , DOI: 10.1038/s41572-019-0141-9
Hans-Joachim Anders 1 , Ramesh Saxena 2 , Ming-Hui Zhao 3, 4 , Ioannis Parodis 5, 6 , Jane E Salmon 7 , Chandra Mohan 8
Affiliation  

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.

中文翻译:

狼疮性肾炎。

狼疮肾炎(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)的最严重器官表现之一。大多数患有LN的SLE患者会在SLE诊断后的5年内出现这种情况,在许多情况下,LN是导致SLE诊断的表现形式。在过去的几十年中,人们对LN的遗传和致病基础的了解已大大提高。LN的治疗通常涉及免疫抑制疗法,通常使用麦考酚酸酯或环磷酰胺以及糖皮质激素,尽管这些疗法并非一律有效。尽管人们对疾病的发病机制有了更多的了解,并且治疗方法得到了改善,但LN仍然是SLE患者发病和死亡的重要原因。在最初的SLE诊断后的10年内,有5-20%的LN患者会发展为终末期肾脏疾病,与免疫抑制治疗相关的多种合并症,包括感染,骨质疏松症以及心血管和生殖作用仍然令人担忧。显然,早期和准确诊断LN并迅速开始治疗对于改善SLE患者的预后至关重要。
更新日期:2020-01-23
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