Department of Rheumatology and Clinical Immunology, University of Lübeck, Germany.
Department of Internal Medicine, Rheumatology and Immunology, Vasculitis Centre South, Medius Kliniken, Teaching Hospital University of Tübingen, Kirchheim unter Teck, Germany.
Clin Exp Rheumatol. 2023 Apr;41(4):936-942. doi: 10.55563/clinexprheumatol/299yme. Epub 2023 Apr 5.
To determine the spectrum of anti-neutrophil cytoplasmic antibody (ANCA) antigen-specificities in eosinophilic granulomatosis with polyangiitis (EGPA), an ANCA-associated vasculitis (AAV) entity.
We conducted a retrospective analysis of 73 EGPA patients from three German tertiary referral centres for vasculitis. In addition to in-house ANCA testing, pentraxin 3 (PTX3)- and olfactomedin 4 (OLM4)-ANCA were determined using a prototype cell-based assay for research (EUROIMMUN, Lübeck, Germany). Patient characteristics and clinical manifestations were evaluated and compared based on ANCA status.
Myeloperoxidase (MPO)-ANCA positive patients (n=8; 11%) significantly more frequently displayed peripheral nervous system (PNS) and pulmonary involvement and less frequently heart involvement compared to MPO-ANCA negative patients. PTX3-ANCA positive patients (n=5; 6.8%) had a significantly higher prevalence of ear, nose and throat, pulmonary, gastrointestinal and PNS involvement, and a lower prevalence of renal and central nervous system involvement compared to PTX3-ANCA negative patients. Proteinase 3 (PR3)-ANCA and OLM4-ANCA were detected in 2 patients (2.7%) each with multiorgan involvement. One PR3-ANCA positive patient was also positive for bactericidal permeability increasing protein (BPI)-ANCA.
In addition to MPO, the spectrum of ANCA antigen specificities includes various other target antigens such as PR3, BPI, PTX3, and OLM4, potentially segregating further EGPA subgroups. A lower prevalence of MPO-ANCA was detected in this study compared with other studies. OLM4 is reported as novel ANCA antigen-specificity in EGPA, and thus AAV.
确定嗜酸性肉芽肿伴多血管炎（EGPA），一种抗中性粒细胞胞质抗体（ANCA）相关性血管炎（AAV）实体中，抗中性粒细胞胞质抗体（ANCA）抗原特异性的谱。
我们对来自德国三个血管炎三级转诊中心的 73 名 EGPA 患者进行了回顾性分析。除了内部 ANCA 检测外，还使用基于细胞的研究原型检测试剂盒（EUROIMMUN，吕贝克，德国）测定五聚素 3（PTX3）-和嗅觉素 4（OLM4）-ANCA。根据 ANCA 状态评估和比较患者特征和临床表现。
髓过氧化物酶（MPO）-ANCA 阳性患者（n=8；11%）与 MPO-ANCA 阴性患者相比，更频繁地出现周围神经系统（PNS）和肺部受累，而较少出现心脏受累。PTX3-ANCA 阳性患者（n=5；6.8%）与 PTX3-ANCA 阴性患者相比，更频繁地出现耳鼻喉、肺、胃肠道和 PNS 受累，而肾脏和中枢神经系统受累的发生率较低。蛋白酶 3（PR3）-ANCA 和 OLM4-ANCA 分别在 2 名患者（2.7%）中检测到，这 2 名患者均有多器官受累。一名 PR3-ANCA 阳性患者还对杀菌通透性增加蛋白（BPI）-ANCA 呈阳性。
除 MPO 外，抗中性粒细胞胞质抗体（ANCA）抗原特异性谱还包括其他各种靶抗原，如 PR3、BPI、PTX3 和 OLM4，可能进一步分离出 EGPA 亚组。与其他研究相比，本研究中检测到的 MPO-ANCA 患病率较低。OLM4 被报道为 EGPA 中新型抗中性粒细胞胞质抗体（ANCA）抗原特异性，因此也是 AAV。
