This study aimed to identify new characteristics of elderly onset large-vessel vasculitis (EOLVV) by focusing on human leucocyte antigen (HLA) genotype, polymyalgia rheumatica (PMR), and affected vascular lesions observed on positron emission tomography/computed tomography (PET/CT) imaging. We retrospectively studied 65 consecutive Japanese patients with large-vessel vasculitis (LVV) who had extracranial vasculitis lesions and underwent PET/CT imaging. PET/CT images were assessed using the semi-quantitative PET visual score of each affected vessel, and the PET vascular activity score (PETVAS) and number of affected vessels were calculated. Subjects were subsequently grouped based on age at onset, superficial temporal artery (STA) involvement, and presence of PMR and compared each group according to HLA genotype. Unsupervised hierarchical cluster analysis was used to identify the patients with similar characteristics in terms of affected vascular lesions detected through PET/CT imaging. The clinical characteristics and PET/CT findings of the population newly identified in this study were examined. Twenty-seven patients with EOLVV did not meet the American College of Rheumatology 1990 criteria for giant cell arteritis (GCA) and Takayasu arteritis and were considered as unclassified EOLVV (UEOLVV). The unsupervised hierarchical cluster analysis revealed that UEOLVV with PMR and large-vessel GCA (LV-GCA) formed a cluster of LVV with GCA features (i.e., PMR and/or STA involvement) when restricted to patients who were HLA-B52-positive. Patients who were HLA-B52-positive with LVV and GCA features had similar clinical characteristics and patterns of affected vessels and presented with diffuse LVV lesions. HLA-B52-positive patients who had LVV with GCA features also presented with higher PETVAS, more affected vessels, and lower rates of biologics usage and relapse compared to HLA-B52-positive patients with TAK. Patients who had UEOLVV with PMR had similar characteristics to patients with LV-GCA. Patients who were HLA-B52-positive and had LVV with GCA features presented with diffuse vascular lesions and may comprise a core population of Japanese patients with EOLVV. The findings of HLA-B52 positivity and diffusely affected vessels in patients with EOLVV can be considered as suspicious findings of LV-GCA.

中文翻译：

---

巨细胞动脉炎中的 HLA-B52 等位基因可能表明弥漫性大血管炎的形成：一项回顾性研究

本研究旨在通过关注人类白细胞抗原 (HLA) 基因型、风湿性多肌痛 (PMR) 和正电子发射断层扫描/计算机断层扫描 (PET/CT) 上观察到的受累血管病变，确定老年大血管炎 (EOLVV) 的新特征) 成像。我们回顾性研究了 65 名日本大血管炎 (LVV) 患者，这些患者有颅外血管炎病变并接受了 PET/CT 成像。使用每个受影响血管的半定量 PET 视觉评分评估 PET/CT 图像，并计算 PET 血管活动评分 (PETVAS) 和受影响血管的数量。随后根据发病年龄、颞浅动脉 (STA) 受累和 PMR 的存在对受试者进行分组，并根据 HLA 基因型比较各组。在通过 PET/CT 成像检测到的受影响的血管病变方面，使用无监督的层次聚类分析来识别具有相似特征的患者。检查了本研究中新发现的人群的临床特征和 PET/CT 结果。27 名 EOLVV 患者不符合美国风湿病学会 1990 年关于巨细胞动脉炎 (GCA) 和 Takayasu 动脉炎的标准，被认为是未分类的 EOLVV (UEOLVV)。无监督层次聚类分析显示，当仅限于 HLA-B52 阳性患者时，具有 PMR 和大血管 GCA (LV-GCA) 的 UEOLVV 形成了具有 GCA 特征（即 PMR 和/或 STA 受累）的 LVV 集群。具有 LVV 和 GCA 特征的 HLA-B52 阳性患者具有相似的临床特征和受累血管模式，并表现为弥漫性 LVV 病变。与具有 TAK 的 HLA-B52 阳性患者相比，具有 GCA 特征的 LVV 的 HLA-B52 阳性患者的 PETVAS 更高，受影响的血管更多，生物制剂使用率和复发率更低。具有 PMR 的 UEOLVV 患者与 LV-GCA 患者具有相似的特征。HLA-B52 阳性且 LVV 具有 GCA 特征的患者表现为弥漫性血管病变，可能包括日本 EOLVV 患者的核心人群。EOLVV 患者 HLA-B52 阳性和弥漫性受累血管的发现可被视为 LV-GCA 的可疑发现。与具有 TAK 的 HLA-B52 阳性患者相比，具有 GCA 特征的 LVV 的 HLA-B52 阳性患者的 PETVAS 更高，受影响的血管更多，生物制剂使用率和复发率更低。具有 PMR 的 UEOLVV 患者与 LV-GCA 患者具有相似的特征。HLA-B52 阳性且 LVV 具有 GCA 特征的患者表现为弥漫性血管病变，可能包括日本 EOLVV 患者的核心人群。EOLVV 患者 HLA-B52 阳性和弥漫性受累血管的发现可被视为 LV-GCA 的可疑发现。与具有 TAK 的 HLA-B52 阳性患者相比，具有 GCA 特征的 LVV 的 HLA-B52 阳性患者的 PETVAS 更高，受影响的血管更多，生物制剂使用率和复发率更低。具有 PMR 的 UEOLVV 患者与 LV-GCA 患者具有相似的特征。HLA-B52 阳性且 LVV 具有 GCA 特征的患者表现为弥漫性血管病变，可能包括日本 EOLVV 患者的核心人群。EOLVV 患者 HLA-B52 阳性和弥漫性受累血管的发现可被视为 LV-GCA 的可疑发现。具有 PMR 的 UEOLVV 患者与 LV-GCA 患者具有相似的特征。HLA-B52 阳性且 LVV 具有 GCA 特征的患者表现为弥漫性血管病变，可能包括日本 EOLVV 患者的核心人群。EOLVV 患者 HLA-B52 阳性和弥漫性受累血管的发现可被视为 LV-GCA 的可疑发现。具有 PMR 的 UEOLVV 患者与 LV-GCA 患者具有相似的特征。HLA-B52 阳性且 LVV 具有 GCA 特征的患者表现为弥漫性血管病变，可能包括日本 EOLVV 患者的核心人群。EOLVV 患者 HLA-B52 阳性和弥漫性受累血管的发现可被视为 LV-GCA 的可疑发现。