Second Department of Internal Medicine, Jagiellonian University Medical College, Kraków, Poland.
Department of Internal Medicine, Connective Tissue Diseases and Geriatrics, Medical University of Gdansk, Gdańsk, Poland
Pol Arch Intern Med. 2022 Mar 30;132(3). doi: 10.20452/pamw.16187. Epub 2022 Jan 5.
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is characterized by the presence of proteinase‑3 (PR3) or myeloperoxidase (MPO) ANCA. In over 90% of cases, PR3‑ANCA is associated with granulomatosis with polyangiitis (GPA). However, it is also rarely found in microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). On the other hand, MPO‑ANCA being characteristic of MPA (>90% of cases), is also found in about 40% of EGPA and 5% of GPA patients. On the ground of this overlap, clinical importance of ANCA specificity identification has been questioned.
In this study, we analyzed the clinical and demographic characteristics of AAV subgroups identified by ANCA serotype.
We conducted a multicenter study of AAV patients (417 GPA, 106 MPA, 102 EGPA; diagnosed between 1990 and 2016), included in the POLVAS registry. The data were systematically collected according to a standardized protocol.
In the ANCA-positive group (anti‑MPO, anti‑PR3) a male-to-female ratio was 1:1, whereas in the ANCA-negative group it was 1:2, regardless of AAV diagnosis. Anti‑MPO antibodies were present in significantly older patients. Patients with MPO+GPA and MPO+EGPA were older than those with corresponding ANCA‑negative GPA and EGPA as well as PR3+AAV. Moreover, ANCA‑negative AAV was characterized by a low risk of end‑stage kidney disease and death.
The presence and specificity of ANCA in AAV patients are related to sex and age, determine their organ involvement and influence mortality as previously shown. Patients with MPO‑ANCA-positive AAV constitute a clinically homogeneous group, whereas PR3‑ANCA-positive patients are much more clinically heterogeneous. ANCA-negative AAV patients are characterized by better prognosis. Thus, ANCA identification is an indispensable element and should not be omitted in establishing AAV diagnosis.
抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）的特征是存在蛋白酶 3（PR3）或髓过氧化物酶（MPO）ANCA。在超过 90%的病例中，PR3-ANCA 与肉芽肿性多血管炎（GPA）相关。然而，它也很少在显微镜下多血管炎（MPA）和嗜酸性肉芽肿性多血管炎（EGPA）中发现。另一方面，MPO-ANCA 是 MPA 的特征（>90%的病例），也存在于约 40%的 EGPA 和 5%的 GPA 患者中。基于这种重叠，ANCA 特异性鉴定的临床重要性受到了质疑。
本研究分析了根据 ANCA 血清型确定的 AAV 亚组的临床和人口统计学特征。
我们对 1990 年至 2016 年间纳入 POLVAS 登记处的 AAV 患者（417 例 GPA、106 例 MPA、102 例 EGPA）进行了多中心研究。根据标准化方案系统地收集数据。
在 ANCA 阳性组（抗 MPO、抗 PR3）中，男女比例为 1:1，而在 ANCA 阴性组中，无论 AAV 诊断如何，男女比例均为 1:2。抗 MPO 抗体存在于年龄较大的患者中。MPO+GPA 和 MPO+EGPA 患者比相应的 ANCA 阴性 GPA 和 EGPA 患者以及 PR3+AAV 患者年龄更大。此外，ANCA 阴性 AAV 的特征是终末期肾病和死亡的风险较低。
AAV 患者的 ANCA 存在和特异性与性别和年龄有关，如前所述，决定了他们的器官受累情况，并影响了死亡率。MPO-ANCA 阳性 AAV 患者构成了一个临床上同质的群体，而 PR3-ANCA 阳性患者则具有更多的临床异质性。ANCA 阴性 AAV 患者的预后较好。因此，ANCA 的鉴定是不可或缺的元素，在确立 AAV 诊断时不应省略。
