This study investigated whether serum syndecan1 at diagnosis reflects activity at diagnosis and predicts poor outcomes during follow-up in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). The study included 79 patients with AAV from the cohort of Korean patients diagnosed with AAV. AAV-specific indices, including the Birmingham vasculitis activity score (BVAS), five-factor score (FFS), 36-item short-form survey (SF-36) physical and mental component summary (PCS and MCS), and vasculitis damage index (VDI), were assessed. Laboratory data including erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels were also collected. The highest tertile and upper half of the BVAS were tentatively defined as having high AAV activity. Serum syndecan1 levels were measured in sera stored at diagnosis. Serum syndecan1 at diagnosis was significantly correlated with AAV activity and functional status, as assessed by BVAS, FFS, SF-36 PCS, MCS, and acute-phase reactants, including ESR and CRP. Patients with serum syndecan1 ≥ 76.1 ng/mL at diagnosis, and those with serum syndecan1 ≥ 60.0 ng/mL at diagnosis showed significantly higher risks for the highest tertile and the upper half of BVAS at diagnosis than those without, respectively. Patients with serum syndecan1 ≥ 120.1 ng/mL at diagnosis had a significantly higher risk for all-cause mortality during follow-up than those without, and further, exhibited a significantly lower cumulative patients’ survival rate than those without. Serum syndecan1 at diagnosis may not only reflect AAV activity at diagnosis but may also be associated with all-cause mortality during follow-up.

中文翻译：

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血清 syndecan1 有可能反映 ANCA 相关血管炎患者诊断时的活动并预测随访期间的死亡


本研究调查了诊断时的血清 syndecan1 是否反映了诊断时的活性并预测抗中性粒细胞胞浆抗体 (ANCA) 相关性血管炎 (AAV) 患者随访期间的不良结果。该研究纳入了韩国确诊 AAV 患者队列中的 79 名 AAV 患者。 AAV 特异性指数，包括伯明翰血管炎活动评分 (BVAS)、五因素评分 (FFS)、36 项简短调查 (SF-36) 身心成分总结（PCS 和 MCS）以及血管炎损伤指数（VDI），进行了评估。还收集了包括红细胞沉降率 (ESR) 和 C 反应蛋白 (CRP) 水平在内的实验室数据。 BVAS 的最高三分位和上半部分被暂时定义为具有高 AAV 活性。测量诊断时储存的血清中的血清 Syndecan1 水平。通过 BVAS、FFS、SF-36 PCS、MCS 和急性期反应物（包括 ESR 和 CRP）评估，诊断时的血清 syndecan1 与 AAV 活性和功能状态显着相关。诊断时血清 Syndecan1 ≥ 76.1 ng/mL 的患者和诊断时血清 Syndecan1 ≥ 60.0 ng/mL 的患者在诊断时表现出最高三分位和上半部分 BVAS 的风险分别显着高于未诊断的患者。诊断时血清 Syndecan1 ≥ 120.1 ng/mL 的患者在随访期间全因死亡的风险显着高于没有血清 Syndecan1 的患者，而且患者的累积生存率显着低于没有血清 Syndecan1 的患者。诊断时的血清 syndecan1 可能不仅反映诊断时的 AAV 活性，还可能与随访期间的全因死亡率相关。