为了评估基于人口的研究中系统性红斑狼疮（SLE）的死亡率（MRs）、标准化死亡率比率以及死因。我们对意大利威尼托大区（490万居民）的联合行政卫生数据库进行了研究。SLE的诊断标准为任何医院诊断或医疗补贴豁免。我们从2012年1月1日至2021年12月31日分析了死亡情况。MRs每1000人年龄组分层统计，以年份、性别和年龄分组为划分。通过比较常规区域人口的MRs来计算标准化死亡率比率。死因使用ICD-10编码系统进行编码，并分为：SLE、感染性疾病、心血管疾病（CVD）、癌症或其他疾病。 在4283个SLE现有病例中，发生了603例死亡，对应每年平均标准化MR为18.6（95% CI 17.0-20.2）每1000人。在1092个急性SLE患者中，有90人死亡，发病后第一年呈峰值（每月人均MR 26.5）。总体标准化死亡率比率为2.65（95% CI 2.13-3.26），在年轻患者中最高（<45岁: 5.59, 95% CI 2.05-12.4）。五年和八年的生存率分别为91%和89%。其中约一半死亡的原因为CVD或癌症，而感染病例则较少报道。 虽然自诊断以来的中期生存率很高，但是与普通人群相比，SLE的死亡率仍然较高，尤其是在年轻患者中。如今，在SLE中，CVD似乎是死因的主要原因，而感染病例的死亡比例很低，至少在西方国家是如此。版权©2023欧洲内科学联合会。由Elsevier B.V.出版。保留所有权利。

To assess mortality rates (MRs), standardized mortality ratios, and causes of death in systemic lupus erythematosus (SLE) in a population-based study.We analyzed linked administrative health databases of the Veneto Region (Italy, 4,900,000 residents). SLE was defined by any hospital diagnosis or healthcare copayment exemption for SLE. We analyzed mortality from January 1st, 2012, until December 31st, 2021. MRs per 1000 were stratified by year, sex, and age group. Standardized mortality ratios were derived by comparing MRs of the general regional population. Causes of death were coded using the ICD-10 coding system and they were grouped in: SLE, infectious diseases, cardiovascular diseases (CVD), cancer, or others.Among 4283 SLE prevalent cases, 603 deaths occurred, corresponding to an average annual standardized MR of 18.6 per 1000 person/year (95% CI 17.0-20.2). Out of 1092 incident SLE patients, 90 died with a peak in the first year after diagnosis (MR 26.5 per 10,000 person/month). Standardized mortality ratio was 2.65 (95% CI 2.13-3.26) overall, and highest among younger patients (<45 years: 5.59, 95% CI 2.05-12.4). Five- and 8-year survival were 91% and 89%, respectively. About half of the deaths had CVD or cancer as underlying cause, whereas infections were less frequently reported.Although the medium-term survival since diagnosis is good, SLE mortality is still higher than that of the general population, especially in youngest patients. Nowadays, CVD seems to be the major cause of deaths in SLE, whereas infections account for a low proportion of deaths, at least in Western countries.Copyright © 2023 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.