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Abstract：
To establish refined risk prediction models for mortality in patients with microscopic polyangiitis (MPA) by using comprehensive clinical characteristics.
Data from the multicentre Japanese registry of patients with vasculitis (REVEAL cohort) were used in our analysis. In total, 194 patients with newly diagnosed MPA were included, and baseline demographic, clinical, laboratory, and treatment details were collected. Univariate and multivariate analyses were conducted to identify the significant risk factors predictive of mortality.
Over a median follow-up of 202.5 (84-352) weeks, 60 (30.9%) of 194 patients died. The causes of death included MPA-related vasculitis (18.3%), infection (50.0%), and others (31.7%). Deceased patients were older (median age 76.2 years) than survivors (72.3 years) (P < 0.0001). The death group had shorter observation periods (median 128.5 [35.3-248] weeks) than the survivor group (229 [112-392] weeks). Compared to survivors, the death group exhibited a higher smoking index, lower serum albumin levels, higher serum C-reactive protein levels, higher Birmingham Vasculitis Activity Score (BVAS), higher Five-Factor Score, and a more severe European Vasculitis Study Group (EUVAS) categorization system. Multivariate analysis revealed that higher BVAS and severe EUVAS independently predicted mortality. Kaplan-Meier survival curves demonstrated lower survival rates for BVAS ≥20 and severe EUVAS, and a risk prediction model (RPM) based on these stratified patients into low, moderate, and high-risk mortality groups.
The developed RPM is promising to predict mortality in patients with MPA and provides clinicians with a valuable tool for risk assessment and informed clinical decision-making.
Data from the multicentre Japanese registry of patients with vasculitis (REVEAL cohort) were used in our analysis. In total, 194 patients with newly diagnosed MPA were included, and baseline demographic, clinical, laboratory, and treatment details were collected. Univariate and multivariate analyses were conducted to identify the significant risk factors predictive of mortality.
Over a median follow-up of 202.5 (84-352) weeks, 60 (30.9%) of 194 patients died. The causes of death included MPA-related vasculitis (18.3%), infection (50.0%), and others (31.7%). Deceased patients were older (median age 76.2 years) than survivors (72.3 years) (P < 0.0001). The death group had shorter observation periods (median 128.5 [35.3-248] weeks) than the survivor group (229 [112-392] weeks). Compared to survivors, the death group exhibited a higher smoking index, lower serum albumin levels, higher serum C-reactive protein levels, higher Birmingham Vasculitis Activity Score (BVAS), higher Five-Factor Score, and a more severe European Vasculitis Study Group (EUVAS) categorization system. Multivariate analysis revealed that higher BVAS and severe EUVAS independently predicted mortality. Kaplan-Meier survival curves demonstrated lower survival rates for BVAS ≥20 and severe EUVAS, and a risk prediction model (RPM) based on these stratified patients into low, moderate, and high-risk mortality groups.
The developed RPM is promising to predict mortality in patients with MPA and provides clinicians with a valuable tool for risk assessment and informed clinical decision-making.
摘要：
背景：通过使用综合临床特征，为显微镜下多血管炎（MPA）患者的死亡率建立完善的风险预测模型。
方法：我们的分析使用了日本多中心血管炎患者注册（REVEAL队列）的数据。总的来说,纳入194例新诊断的MPA患者，和基线人口统计，临床,实验室,并收集治疗细节。进行单变量和多变量分析以确定预测死亡率的重要危险因素。
结果：在202.5（84-352）周的中位随访期间，194例患者中有60例（30.9%）死亡。死亡原因包括MPA相关性血管炎（18.3%），感染（50.0%），和其他(31.7%)。死亡患者年龄（中位年龄76.2岁）比幸存者（72.3岁）大（P<0.0001）。死亡组的观察期（中位数为128.5[35.3-248]周）短于幸存者组（229[112-392]周）。与幸存者相比，死亡组显示出较高的吸烟指数，降低血清白蛋白水平，血清C反应蛋白水平较高，较高的伯明翰血管炎活动评分（BVAS），较高的五因素得分，和更严重的欧洲血管炎研究组（EUVAS）分类系统。多因素分析显示，较高的BVAS和严重的EUVAS独立预测死亡率。Kaplan-Meier生存曲线显示BVAS≥20和严重EUVAS的生存率较低，以及基于这些分层患者的风险预测模型(RPM)，中度,和高危死亡人群。
结论：开发的RPM有望预测MPA患者的死亡率，并为临床医生提供了风险评估和明智的临床决策的有价值的工具。
方法：我们的分析使用了日本多中心血管炎患者注册（REVEAL队列）的数据。总的来说,纳入194例新诊断的MPA患者，和基线人口统计，临床,实验室,并收集治疗细节。进行单变量和多变量分析以确定预测死亡率的重要危险因素。
结果：在202.5（84-352）周的中位随访期间，194例患者中有60例（30.9%）死亡。死亡原因包括MPA相关性血管炎（18.3%），感染（50.0%），和其他(31.7%)。死亡患者年龄（中位年龄76.2岁）比幸存者（72.3岁）大（P<0.0001）。死亡组的观察期（中位数为128.5[35.3-248]周）短于幸存者组（229[112-392]周）。与幸存者相比，死亡组显示出较高的吸烟指数，降低血清白蛋白水平，血清C反应蛋白水平较高，较高的伯明翰血管炎活动评分（BVAS），较高的五因素得分，和更严重的欧洲血管炎研究组（EUVAS）分类系统。多因素分析显示，较高的BVAS和严重的EUVAS独立预测死亡率。Kaplan-Meier生存曲线显示BVAS≥20和严重EUVAS的生存率较低，以及基于这些分层患者的风险预测模型(RPM)，中度,和高危死亡人群。
结论：开发的RPM有望预测MPA患者的死亡率，并为临床医生提供了风险评估和明智的临床决策的有价值的工具。
