Department of Internal Medicine, CHU de Caen Normandie, Caen, France.
Laboratoire de Biochimie, CHU de Caen Normandie, Caen, France.
PLoS One. 2024 Oct 10;19(10):e0311678. doi: 10.1371/journal.pone.0311678. eCollection 2024.
Ceruloplasmin is an inhibitor of myeloperoxidase (MPO) activity that plays an important role in the pathophysiology of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). This study aimed to evaluate the prognostic impact of serum level of ceruloplasmin at diagnosis in patients with anti-MPO antibody-positive AAV.
This retrospective monocentric study in Caen University Hospital involved all consecutive adult anti-MPO antibody-positive patients with microscopic polyangiitis or granulomatosis with polyangiitis, diagnosed between January 2010 and January 2022 with available serum sample at inclusion. Patients outcomes were analyzed from two subgroups constituted according to the median serum level of ceruloplasmin. The same analyses were then performed in anti-proteinase 3 (PR3) antibody-positive patients.
Within the 92 patients analyzed, 50 patients had anti-MPO antibodies with a median ceruloplasmin level of 0.44 [quartiles 1-3, 0.40-0.49] g/L and a median Birmingham Vasculitis Activity Score of 19 [14-22]. After a median follow-up period of 40 [22-86] months, 13 (26%) patients had died: 10 (40%) in the low ceruloplasmin group and 3 (12%) in the high ceruloplasmin group (p = 0.03), with a significantly worse survival rate in the low ceruloplasmin group (p = 0.021). No significant differences in relapse rate or renal failure was observed between the two groups. The same analyses performed in the group of AAV patients with anti-PR3 antibody did not show any differences.
In anti-MPO AAV patients, serum level of ceruloplasmin at diagnosis seems to be associated with a significant impact on survival.
铜蓝蛋白是髓过氧化物酶（MPO）活性的抑制剂，在抗中性粒细胞胞质抗体相关性血管炎（AAV）的病理生理学中发挥重要作用。本研究旨在评估抗髓过氧化物酶抗体阳性 AAV 患者诊断时血清铜蓝蛋白水平对预后的影响。
本研究为回顾性单中心研究，纳入了 2010 年 1 月至 2022 年 1 月期间在卡昂大学医院连续就诊的所有抗髓过氧化物酶抗体阳性的显微镜下多血管炎或肉芽肿性多血管炎成年患者，并在纳入时检测了血清样本。根据血清铜蓝蛋白中位数将患者分为两个亚组进行分析。然后在抗蛋白酶 3（PR3）抗体阳性患者中进行了相同的分析。
在 92 例分析的患者中，50 例患者存在抗 MPO 抗体，铜蓝蛋白中位数为 0.44 [四分位数 1-3，0.40-0.49] g/L，Birmingham 血管炎活动评分中位数为 19 [14-22]。中位随访时间为 40 [22-86] 个月后，13 例（26%）患者死亡：低铜蓝蛋白组 10 例（40%），高铜蓝蛋白组 3 例（12%）（p = 0.03），低铜蓝蛋白组的生存率明显更差（p = 0.021）。两组间复发率或肾功能衰竭无显著差异。在抗 PR3 抗体阳性的 AAV 患者组中进行的相同分析未显示出任何差异。
在抗 MPO AAV 患者中，诊断时血清铜蓝蛋白水平似乎与生存显著相关。
