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纯膜性狼疮性肾炎:150例患者队列描述及文献综述

Pure Membranous Lupus Nephritis: Description of a Cohort of 150 Patients and Review of the Literature.

作者信息

Silva-Fernández Lucía, Otón Teresa, Askanase Anca, Carreira Patricia, López-Longo Francisco Javier, Olivé Alejandro, Rúa-Figueroa Íñigo, Narváez Javier, Ruiz-Lucea Esther, Andrés Mariano, Calvo Enrique, Toyos Francisco, Alegre-Sancho Juan José, Tomero Eva, Montilla Carlos, Zea Antonio, Uriarte Esther, Calvo-Alén Jaime, Marras Carlos, Martínez-Taboada Víctor M, Belmonte-López María Ángeles, Rosas José, Raya Enrique, Bonilla Gema, Freire Mercedes, Pego-Reigosa José María, Millán Isabel, Hughes-Morley Adwoa, Andreu José Luis

机构信息

Servicio de Reumatología, Complexo Hospitalario Universitario de Ferrol, Ferrol, A Coruña, Spain.

Servicio de Reumatología, Instituto de Salud Musculoesquelética, Madrid, Spain.

出版信息

Reumatol Clin (Engl Ed). 2019 Jan-Feb;15(1):34-42. doi: 10.1016/j.reuma.2017.04.005. Epub 2017 May 18.

Abstract

OBJECTIVES

The course and long-term outcome of pure membranous lupus nephritis (MLN) are little understood. The aims of this study are to evaluate the clinical features, course, outcome and prognostic indicators in pure MLN and to determine the impact of ethnicity and the type of health insurance on the course and prognosis of pure MLN.

METHODS

We conducted a retrospective review of medical records of 150 patients with pure MLN from Spain and the USA.

RESULTS

Mean age was 34.2±12.5 and 80% were women. Sixty-eight percent of patients had nephrotic syndrome at diagnosis. The average serum creatinine was 0.98±0.78mg/dl. Six percent of patients died and 5.3% developed end-stage renal disease (ESRD). ESRD was predicted by male sex, hypertension, dyslipidemia, high basal 24h-proteinuria, high basal serum creatinine and a low basal creatinine clearance. Age, cardiac insufficiency, peripheral artheriopathy, hemodialysis and not having received mycophenolate mofetil or antimalarials for MLN predicted death.

CONCLUSIONS

Pure MLN frequently presents with nephrotic syndrome, high proteinuria and normal serum creatinine. Its prognosis is favourable in maintaining renal function although proteinuria usually persists over time. Baseline cardiovascular disease and not having a health insurance are related with poor prognosis.

摘要

目的

对单纯膜性狼疮性肾炎(MLN)的病程及长期预后了解甚少。本研究旨在评估单纯MLN的临床特征、病程、结局及预后指标,并确定种族和医疗保险类型对单纯MLN病程及预后的影响。

方法

我们对来自西班牙和美国的150例单纯MLN患者的病历进行了回顾性分析。

结果

平均年龄为34.2±12.5岁,80%为女性。68%的患者在诊断时患有肾病综合征。平均血清肌酐为0.98±0.78mg/dl。6%的患者死亡,5.3%发展为终末期肾病(ESRD)。男性、高血压、血脂异常、基础24小时蛋白尿高、基础血清肌酐高及基础肌酐清除率低可预测ESRD。年龄、心脏功能不全、外周动脉病变、血液透析以及未接受用于MLN的霉酚酸酯或抗疟药可预测死亡。

结论

单纯MLN常表现为肾病综合征、高蛋白尿及血清肌酐正常。尽管蛋白尿通常会随时间持续存在,但在维持肾功能方面其预后良好。基线心血管疾病和未参加医疗保险与预后不良有关。

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