目的:探讨伴高球蛋白血症的pSS患者全因死亡风险是否增加。
方法:纳入2016年5月至2021年7月在中国风湿病数据中心注册并符合2002AECG标准或2016ACR/EULARSS分类标准的患者。高球蛋白血症被定义为任何升高的IgG血清水平,IgA,或IgM。主要结果是全因死亡。人口统计学和临床特征的数据,实验室结果,疾病活动,伤害得分,对治疗方法进行了评估。
结果:共有9,527名pSS患者被纳入分析,其中4236例(44.5%)IgG中至少有一种免疫球蛋白水平升高,IgA,和IgM。高球蛋白血症患者的死亡风险显着增加(粗HR2.60;95CI1.91-3.55;校正HR1.90;95CI1.20-3.01)。死亡风险与IgG水平呈正相关(P<0.001)。5-,10-,高球蛋白血症患者的15年生存率为96.9%,92.3%,和87.9%,分别,明显低于相应的98.8%,97.9%,和96.4%的患者没有高球蛋白血症。
结论:高球蛋白血症是pSS患者全因死亡率增加的独立危险因素。死亡风险与IgG水平呈正相关。
OBJECTIVE: To investigate whether primary Sjögren\'s syndrome (pSS) patients with
hyperglobulinemia have an increased risk of all-cause mortality.
METHODS: Patients who registered in the Chinese Rheumatism Data Centre from May 2016 to July 2021 and met the 2002 American European Consensus Group criteria or 2016 American College of Rheumatology /European League Against Rheumatism classification criteria for Sjögren\'s syndrome were included.
Hyperglobulinemia was defined as any elevated serum levels of immunoglobulin G (IgG), immunoglobulin A (IgA), or immunoglobulin M (IgM). The primary outcome was all-cause death. Data for demographic and clinical characteristics, laboratory results, disease activity, damage scores, and treatments were evaluated.
RESULTS: A total of 9527 pSS patients were included in the analysis, of whom 4236 (44.5%) had at least one kind of elevated immunoglobulin level among IgG, IgA, and IgM. Patients with
hyperglobulinemia had a significantly increased risk of death (crude hazard ratio 2.60; 95% confidence interval 1.91-3.55; adjusted hazard ratio 1.90; 95% confidence interval 1.20-3.01). The risk of death was positively correlated with IgG level (P trend <.001). The 5-, 10-, and 15-year survival rates of patients with
hyperglobulinemia were 96.9%, 92.3%, and 87.9%, respectively, and significantly lower than the corresponding rates of 98.8%, 97.9%, and 96.4% in patients without hyperglobulinemia.
CONCLUSIONS: Hyperglobulinemia is an independent risk factor for increased all-cause mortality in pSS patients. The risk of death is positively correlated with IgG level.