IRCCS Humanitas Research Hospital, Rozzano, Italy.
Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Italy.
Front Immunol. 2021 Sep 10;12:714174. doi: 10.3389/fimmu.2021.714174. eCollection 2021.
Antineutrophil cytoplasmic antibodies (ANCA) are primarily involved in the pathogenesis of ANCA-associated vasculitides (AAV). However, ANCA may also be present in healthy subjects and in patients with autoimmune disorders different from AAV. We hypothesized that serum ANCA are associated with a worse prognosis in disorders other than AAV.
We investigated the association between the overall survival and the presence of serum ANCA in 1,024 Italian subjects with various testing indications in a 10-year interval.
In this retrospective cohort study, a population of 6,285 patients (many of whom were subsequently excluded due to our criteria) who tested for ANCA at a single center in 10 years was considered, and life status and comorbidities of subjects were collected. We compared the overall survival of ANCA-positive and ANCA-negative patients by means of Kaplan-Meier curves, while a multivariable adjusted Cox regression was used to evaluate the association between the ANCA status and the outcome (death) in terms of hazard ratios (HR) with 95% confidence intervals (CI).
The positivity of perinuclear ANCA (pANCA) increased significantly mortality (HR, 1.60; 95% CI, 1.10-2.32), while cytoplasmic ANCA (cANCA) positivity failed to show a significant association (HR, 1.43; 95% CI, 0.77-2.68). The increased mortality rate was observed for both pANCA and cANCA in patients suffering from rheumatic disorders. No association was found between mortality and anti-MPO (HR, 0.63; 95% CI, 0.20-2.00) or anti-PR3 (HR, 0.98; 95% CI, 0.24-3.96) after adjusting for confounders.
Serum pANCA and cANCA are independent negative prognostic factors in patients with concurrent autoimmune diseases.
抗中性粒细胞胞浆抗体（ANCA）主要参与 ANCA 相关性血管炎（AAV）的发病机制。然而，ANCA 也可能存在于健康受试者和与 AAV 不同的自身免疫性疾病患者中。我们假设血清 ANCA 与 AAV 以外的疾病的预后较差有关。
我们在 10 年的时间间隔内，对意大利的 1024 名具有各种检测指征的受试者进行了研究，以调查血清 ANCA 与总生存率之间的关系。
在这项回顾性队列研究中，考虑了在 10 年内对单个中心进行 ANCA 检测的 6285 名患者（由于我们的标准，其中许多患者后来被排除在外），并收集了受试者的生存状态和合并症。我们通过 Kaplan-Meier 曲线比较了 ANCA 阳性和 ANCA 阴性患者的总生存率，同时使用多变量调整 Cox 回归来评估 ANCA 状态与结局（死亡）之间的关联，即危险比（HR）及其 95%置信区间（CI）。
核周型抗中性粒细胞胞浆抗体（pANCA）的阳性率显著增加了死亡率（HR，1.60；95%CI，1.10-2.32），而细胞质型抗中性粒细胞胞浆抗体（cANCA）的阳性率未显示出显著相关性（HR，1.43；95%CI，0.77-2.68）。pANCA 和 cANCA 阳性的患者均观察到死亡率升高。在调整混杂因素后，抗髓过氧化物酶（MPO）（HR，0.63；95%CI，0.20-2.00）或抗蛋白酶 3（PR3）（HR，0.98；95%CI，0.24-3.96）与死亡率之间未发现相关性。
血清 pANCA 和 cANCA 是伴有自身免疫性疾病患者的独立负预后因素。
