Akgüngör Fehim, Temiz Fatih, Kandur Yaşar
Department of Pediatrics, School of Medicine, Sutcu Imam University, Kahramanmaras, Türkiye.
Division of Nephrology, Department of Pediatrics, School of Medicine, Kirikkale University, Kirikkale, 71450, Türkiye.
Clin Rheumatol. 2025 Aug 23. doi: 10.1007/s10067-025-07659-7.
IgA vasculitis (IGAV) is the most frequently encountered form of vasculitis in the pediatric population and typically follows a self-limiting course. In addition to well known inflammatory markers, several other inflammatory mediators, including Endocan, endothelial nitric oxide synthase (eNOS), and soluble CD89 (sCD89), have not been extensively investigated in the context of IGAV. The aim of this study was to evaluate the association between Endocan, endothelial nitric oxide synthase (eNOS), and soluble CD89 (sCD89) and established inflammatory markers (CRP, erythrocyte sedimentation rate), as well as their relationship with clinical manifestations and laboratory findings in pediatric patients diagnosed with IGAV.
Patients diagnosed with IGAV during the study period (December 2019 to May 2021) were included in the study. These patients were invited for a follow-up visit 3 months after diagnosis. Additionally, 35 healthy children, matched for age and gender and with no known diseases, were included as a control group. At both the time of diagnosis and the 3-month follow-up, blood samples were collected in EDTA tubes in addition to routine hematological and biochemical tests. The samples were centrifuged at 4000 rpm for 10 min and stored at -80 °C. Endocan, sCD89, and eNOS levels were measured using ELISA kits.
The mean sCD89 level in the patient group, both during the acute and remission phases, was significantly lower than in the control group (1.42 ± 0.53 ng/mL and 1.46 ± 0.80 ng/mL vs. 1.83 ± 0.73 ng/mL; p = 0.010 and p = 0.004, respectively). However, there was no statistically significant difference in sCD89 levels between the acute and remission phases within the patient group (p = 0.376). The Endocan level during the remission phase was significantly lower in patients compared to the control group (378.4 ± 238.1 ng/mL vs. 478.9 ± 240.9 ng/mL; p = 0.014). However, no significant difference was found between the control group and the acute phase (p = 0.911). A statistically significant difference in Endocan levels was observed between the acute and remission phases in patients (p = 0.004) (Table 1). The mean eNOS level in the patient group, both during the acute and remission phases, was significantly lower than in the control group (25.29 ± 14.07 U/mL and 30.12 ± 18.10 U/mL vs. 37.71 ± 19.48 U/mL; p = 0.004 and p = 0.031, respectively). However, there was no statistically significant difference in eNOS levels between the acute and remission phases (p = 0.334).
The unexpectedly low levels of these vascular mediators may be due to the fact that endothelial damage in IGAV is triggered by immune complex-mediated mechanisms rather than a proinflammatory cytokine-driven process, which may result in reduced or suppressed endocan release. Secondly IGAV is primarily caused by the deposition of IgA1 immune complexes in the vessel walls. This mechanism differs from other systemic vasculitides. Key Points • This study systematically evaluated the levels of Endocan, Endothelial nitric oxide synthase (eNOS), and soluble CD89 (sCD89) in patients with IGAV. • The level of these mediators was lower than the controls unexpectedly. • It may be due to the fact that endothelial damage in IGAV is triggered by immune complex-mediated mechanisms rather than a proinflammatory cytokine-driven process, which may result in reduced or suppressed release of these mediators.
IgA 血管炎（IGAV）是儿科人群中最常见的血管炎形式，通常呈自限性病程。除了众所周知的炎症标志物外，包括内皮糖蛋白（Endocan）、内皮型一氧化氮合酶（eNOS）和可溶性 CD89（sCD89）在内的其他几种炎症介质，在 IGAV 背景下尚未得到广泛研究。本研究的目的是评估 Endocan、eNOS 和 sCD89 与既定炎症标志物（CRP、红细胞沉降率）之间的关联，以及它们与诊断为 IGAV 的儿科患者临床表现和实验室检查结果的关系。
纳入研究期间（2019 年 12 月至 2021 年 5 月）诊断为 IGAV 的患者。这些患者在诊断后 3 个月被邀请进行随访。此外，纳入 35 名年龄和性别匹配且无已知疾病的健康儿童作为对照组。在诊断时和 3 个月随访时，除了常规血液学和生化检查外，还使用 EDTA 管采集血样。样本以 4000 rpm 离心 10 分钟，并储存在 -80°C。使用 ELISA 试剂盒测量 Endocan、sCD89 和 eNOS 水平。
患者组在急性期和缓解期的平均 sCD89 水平均显著低于对照组（1.42±0.53 ng/mL 和 1.46±0.80 ng/mL 对比 1.83±0.73 ng/mL；p = 0.010 和 p = 0.004，分别）。然而，患者组内急性期和缓解期之间的 sCD89 水平无统计学显著差异（p = 0.376）。与对照组相比，患者缓解期的 Endocan 水平显著降低（378.4±238.1 ng/mL 对比 478.9±240.9 ng/mL；p = 0.014）。然而，对照组与急性期之间未发现显著差异（p = 0.911）。患者急性期和缓解期之间的 Endocan 水平存在统计学显著差异（p = 0.004）（表 1）。患者组在急性期和缓解期的平均 eNOS 水平均显著低于对照组（25.29±14.07 U/mL 和 30.12±18.10 U/mL 对比 37.71±19.48 U/mL；p = 0.004 和 p = 0.031，分别）。然而，急性期和缓解期之间的 eNOS 水平无统计学显著差异（p = 0.334）。
这些血管介质水平意外降低可能是由于 IGAV 中的内皮损伤是由免疫复合物介导机制触发，而非促炎细胞因子驱动过程，这可能导致 Endocan 释放减少或受抑制。其次，IGAV 主要由 IgA1 免疫复合物在血管壁沉积引起。该机制不同于其他系统性血管炎。要点 • 本研究系统评估了 IGAV 患者中 Endocan、内皮型一氧化氮合酶（eNOS）和可溶性 CD89（sCD89）的水平。 • 这些介质的水平意外低于对照组。 • 这可能是由于 IGAV 中的内皮损伤是由免疫复合物介导机制触发，而非促炎细胞因子驱动过程，这可能导致这些介质的释放减少或受抑制。
