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狼疮性肾炎:最新进展

Lupus nephritis: an update.

作者信息

Imran Tasnim F, Yick Frederick, Verma Suneet, Estiverne Christopher, Ogbonnaya-Odor Chinonye, Thiruvarudsothy Srikanth, Reddi Alluru S, Kothari Neil

机构信息

Department of Medicine, New Jersey Medical School, Rutgers, The State University of New Jersey, 185 South Orange Avenue, Newark, NJ, 07103, USA.

Division of Nephrology, New Jersey Medical School, Rutgers, The State University of New Jersey, 185 South Orange Avenue, Newark, NJ, 07103, USA.

出版信息

Clin Exp Nephrol. 2016 Feb;20(1):1-13. doi: 10.1007/s10157-015-1179-y. Epub 2015 Oct 16.

Abstract

Lupus nephritis (LN) is an inflammatory condition of the kidneys that encompasses various patterns of renal disease including glomerular and tubulointerstitial pathology. It is a major predictor of poor prognosis in patients with systemic lupus erythematosus (SLE). Genetic factors, including several predisposing loci, and environmental factors, such as EBV and ultraviolet light, have been implicated in the pathogenesis. It carries a high morbidity and mortality if left untreated. Renal biopsy findings are utilized to guide treatment. Optimizing risk factors such as proteinuria and hypertension with renin-angiotensin receptor blockade is crucial. Immunosuppressive therapy is recommended for patients with focal or diffuse proliferative lupus nephritis (Class III or IV) disease, and certain patients with membranous LN (Class V) disease. Over the past decade, immunosuppressive therapies have significantly improved long-term outcomes, but the optimal therapy for LN remains to be elucidated. Cyclophosphamide-based regimens, given concomitantly with corticosteroids, have improved survival significantly. Even though many patients achieve remission, the risk of relapse remains considerably high. Other treatments include hydroxychloroquine, mycofenolate mofetil, and biologic therapies such as Belimumab, Rituximab, and Abatacept. In this paper, we provide a review of LN, including pathogenesis, classification, and clinical manifestations. We will focus, though, on discussion of the established as well as emerging therapies for patients with proliferative and membranous lupus nephritis.

摘要

狼疮性肾炎(LN)是一种肾脏炎症性疾病,涵盖多种肾脏疾病模式,包括肾小球和肾小管间质病变。它是系统性红斑狼疮(SLE)患者预后不良的主要预测指标。遗传因素,包括多个易感基因座,以及环境因素,如EB病毒和紫外线,都与发病机制有关。如果不治疗,其发病率和死亡率都很高。肾活检结果用于指导治疗。通过肾素 - 血管紧张素受体阻滞剂优化蛋白尿和高血压等危险因素至关重要。对于局灶性或弥漫性增殖性狼疮性肾炎(III级或IV级)患者以及某些膜性LN(V级)患者,建议进行免疫抑制治疗。在过去十年中,免疫抑制疗法显著改善了长期预后,但LN的最佳治疗方案仍有待阐明。与皮质类固醇联合使用的基于环磷酰胺的方案显著提高了生存率。尽管许多患者实现缓解,但复发风险仍然相当高。其他治疗方法包括羟氯喹、霉酚酸酯以及贝利尤单抗、利妥昔单抗和阿巴西普等生物疗法。在本文中,我们对LN进行综述,包括发病机制、分类和临床表现。不过,我们将重点讨论针对增殖性和膜性狼疮性肾炎患者的既定疗法以及新兴疗法。

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