目的:评估当代系统性红斑狼疮(SLE)的死亡率和生存率,以人口为基础的设置,并按时间评估潜在的影响,性别,种族,分类标准和诊断年龄。
方法:我们评估了Nor-SLE队列的死亡率和生存率,其中包括1999-2017年居住在挪威东南部(人口290万)的所有图表审查确认的SLE病例。研究结束时死亡,移民,或2022年10月1日。我们根据诊断时年龄<16岁来定义青少年SLE。对于标准化死亡率(SMR)估计,我们每个病例应用了15个人口控制,所有匹配的年龄,性别,residence,和种族。我们通过Kaplan-Meier分析了生存率,并通过cox回归分析了危险因素。
结果:Nor-SLE队列包括1558例SLE病例,其中749人发生事件,符合2019年欧洲风湿病学协会联盟和美国风湿病学会(2019-EA)分类标准.SMR增加到1.8(95%CI1.6-2.2)在成年发病的SLE中,但在女性和男性之间没有差异。生存率在5,10-,15岁和20岁的成年发病SLE低于匹配的对照组。在多变量分析中,与生存率下降相关的狼疮性肾炎,虽然性没有。分开,在整个Nor-SLE队列中的长期死亡率分析显示,SMR在幼年型SLE(n=93)中达到峰值7.2(95%CI3.3-14),并随着SLE诊断年龄的增加而逐渐下降.
结论:这项研究显示,在人群水平上,成年型SLE和对照组之间的死亡率差距持续存在,并提供了青少年型SLE死亡率高令人担忧的迹象。
OBJECTIVE: To estimate mortality and survival rates of SLE in a contemporary, population-based setting and assess potential influences by time, sex, ethnicity, classification criteria and age at diagnosis.
METHODS: We assessed mortality and survival in the Nor-SLE cohort, which includes all chart review-confirmed SLE cases resident in Southeast Norway (population 2.9 million) 1999-2017.
Study end was at death, emigration or 1 October 2022. We defined
juvenile SLE by age <16 years at diagnosis. For standardized mortality rate (SMR) estimates, we applied 15 population controls per case, all matched for age, sex, residency and ethnicity. We analysed survival by Kaplan-Meier and risk factors by Cox regression.
RESULTS: The Nor-SLE cohort included 1558 SLE cases, of whom 749 were incident and met the 2019 EULAR and ACR (2019-EA) classification criteria. SMR was increased to 1.8 (95% CI 1.6-2.2) in incident adult-onset SLE but did not differ between females and males. Survival rates at 5, 10, 15 and 20 years were lower in incident adult-onset SLE than in matched controls. In multivariable analysis, LN associated with decreased survival, while sex did not. Separate, long-term mortality analyses in the total Nor-SLE cohort showed that SMR peaked at 7.2 (95% CI 3.3-14) in
juvenile-onset SLE (n = 93) and fell gradually by increasing age at SLE diagnosis.
CONCLUSIONS: This
study shows persistence of a mortality gap between adult-onset SLE and controls at population level and provides indications of worryingly high mortality in
juvenile-onset SLE.