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狼疮性肾炎。

Lupus nephritis.

机构信息

Division of Nephrology, Department of Medicine IV, University Hospital, LMU Munich, Munich, Germany.

Division of Nephrology, Department of Medicine, UT Southwestern Medical Center, Dallas, TX, USA.

出版信息

Nat Rev Dis Primers. 2020 Jan 23;6(1):7. doi: 10.1038/s41572-019-0141-9.

Abstract

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.

摘要

狼疮性肾炎(LN)是一种肾小球肾炎,是系统性红斑狼疮(SLE)这一自身免疫性疾病最严重的器官表现之一。大多数发生 LN 的 SLE 患者在 SLE 诊断后 5 年内发病,而且在许多情况下,LN 是导致 SLE 诊断的首发表现。过去几十年中,对 LN 的遗传和发病基础的认识有了很大提高。LN 的治疗通常涉及免疫抑制治疗,通常采用霉酚酸酯或环磷酰胺和糖皮质激素,但这些治疗并非都有效。尽管对疾病发病机制的认识有所提高,治疗选择也有所改善,但 LN 仍然是 SLE 患者发病率和死亡率的主要原因。在首次 SLE 诊断后 10 年内,5-20%的 LN 患者会发展为终末期肾病,而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影响,仍然令人担忧。显然,早期和准确诊断 LN 并及时开始治疗对改善 SLE 患者的预后至关重要。

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