狼疮肾炎。

IF 76.9 1区 医学 Q1 MEDICINE, GENERAL & INTERNAL
Ioannis Parodis, Brad H Rovin, Maria G Tektonidou, Hans-Joachim Anders, Ana Malvar, Chi Chiu Mok, Chandra Mohan
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引用次数: 0

摘要

狼疮肾炎(LN)是一种肾小球肾炎,是系统性红斑狼疮(SLE)最严重的并发症之一。LN影响25-60%的SLE患者,其发病率和患病率因年龄、性别、种族和社会经济因素而异。LN主要发生在SLE诊断后的5年内,对许多患者来说,它是导致SLE识别的最初表现。在一些患者中,LN可能在病程晚期出现,这突出了持续意识到其症状和体征的重要性。尽管人们对疾病生物学的了解越来越多,治疗方案也越来越有效,但LN仍然是发病率和死亡率的重要原因,因为它可导致不可逆的肾衰竭和相关并发症。进展为肾衰竭的危险因素包括持续性蛋白尿、肾小球滤过率低、诊断时高血压和频繁的疾病发作。LN的发病机制涉及复杂的免疫失调,关键途径包括I型干扰素信号传导、钙调磷酸酶激活、B细胞和T细胞功能障碍。几种免疫调节药物被用于LN的治疗,治疗模式正日益转向多药方案。除了适当的药物治疗外,针对患者个人需求量身定制的多学科护理,包括风湿病学家、肾病学家、社会工作者和其他卫生专业人员,对于全面解决进行性肾功能丧失的免疫和非免疫危险因素以及最大限度地延长LN患者的肾脏寿命至关重要。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Lupus nephritis.

Lupus nephritis (LN) is a type of glomerulonephritis and one of the most serious complications of systemic lupus erythematosus (SLE). LN affects 25-60% of patients with SLE, with incidence and prevalence varying by age, sex, ethnicity and socioeconomic factors. LN predominantly develops within 5 years of an SLE diagnosis and, for many patients, it is the initial manifestation that leads to the recognition of SLE. In some patients, LN may develop late in the disease course, highlighting the importance of persistent awareness of its symptoms and signs. Despite an increasing understanding of disease biology and more effective treatment options, LN remains a substantial cause of morbidity and mortality as it can lead to irreversible kidney failure and associated complications. Risk factors for progression to kidney failure include persistent proteinuria, low glomerular filtration rate, hypertension at diagnosis and frequent disease flares. LN pathogenesis involves complex immune dysregulation, with key pathways including type I interferon signalling, calcineurin activation, and B and T cell dysfunction. Several immunomodulatory drugs are used for the management of LN, and treatment paradigms are increasingly shifting towards multi-agent regimens. Along with appropriate pharmacotherapy, multidisciplinary care tailored to the patient's individual needs, involving rheumatologists, nephrologists, social workers and other health professionals, is crucial for holistically addressing both the immune and non-immune risk factors for progressive kidney function loss and for maximizing kidney lifespan in LN.

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来源期刊
Nature Reviews Disease Primers
Nature Reviews Disease Primers Medicine-General Medicine
CiteScore
76.70
自引率
0.20%
发文量
75
期刊介绍: Nature Reviews Disease Primers, a part of the Nature Reviews journal portfolio, features sections on epidemiology, mechanisms, diagnosis, management, and patient quality of life. The editorial team commissions top researchers — comprising basic scientists and clinical researchers — to write the Primers, which are designed for use by early career researchers, medical students and principal investigators. Each Primer concludes with an Outlook section, highlighting future research directions. Covered medical specialties include Cardiology, Dermatology, Ear, Nose and Throat, Emergency Medicine, Endocrinology, Gastroenterology, Genetic Conditions, Gynaecology and Obstetrics, Hepatology, Haematology, Infectious Diseases, Maxillofacial and Oral Medicine, Nephrology, Neurology, Nutrition, Oncology, Ophthalmology, Orthopaedics, Psychiatry, Respiratory Medicine, Rheumatology, Sleep Medicine, and Urology.
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