Choi Hyun Joon, Ha Jang Woo, Song Jason Jungsik, Park Yong-Beom, Lee Sang-Won
Department of Medicine, Yonsei University College of Medicine, Seoul 03722, Republic of Korea.
Division of Rheumatology, Department of Internal Medicine, Yongin Severance Hospital, Yonsei University College of Medicine, Yongin 16995, Republic of Korea.
Diagnostics (Basel). 2025 Apr 25;15(9):1099. doi: 10.3390/diagnostics15091099.
: The overlap syndrome of primary Sjögren syndrome (pSS) with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) (OvSD/pSS/AAV) has been reported in other studies. This study applied the new criteria for AAV proposed by the American College of Rheumatology/European Alliance of Associations for Rheumatology in 2022 (the ACR/EULAR criteria) to patients with pSS presenting signs and symptoms suggestive of small- and medium-vessel vasculitis. It also investigated the overall frequency of OvSD/pSS/AAV and the major contributing factors to its reclassification. : This study included 116 patients with pSS from March 2005 to December 2020, according to the inclusion criteria, and defined signs and symptoms suggestive of small- or medium-vessel vasculitides as lung parenchymal lesions supporting AAV, peripheral neuropathy, and suspected renal vasculitis. The classification could be made when the total scores for microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) are ≥5 points and the eosinophilic GPA (EGPA) score is ≥6 points. : The median age of the patients was 56.0 years, and 101 patients (87.1%) were women. In total, 95, 12, and 37 patients had lung parenchymal lesions supporting AAV, peripheral neuropathy, and suspected renal vasculitis, respectively. According to the ACR/EULAR criteria for AAV, 35 of 116 (30.2%) patients were reclassified as having OvSD/pSS/AAV. Among these 35 patients, 4 were reclassified as having both OvSD/pSS/MPA and OvSD/pSS/GPA and 1 as having both OvSD/pSS/MPA and OvSD/pSS/EGPA simultaneously. The major contributing factor to the reclassification of OvSD/pSS/AAV was ANCA positivity. : The overall frequency of the reclassification of OvSD/pSS/AAV was 30.2% in pSS patients presenting signs and symptoms suggestive of small- and medium-vessel vasculitis. Its likelihood increased according to ANCA positivity.
其他研究已报道了原发性干燥综合征（pSS）与抗中性粒细胞胞浆抗体（ANCA）相关血管炎（AAV）的重叠综合征（OvSD/pSS/AAV）。本研究将美国风湿病学会/欧洲抗风湿病联盟于2022年提出的AAV新诊断标准（ACR/EULAR标准）应用于出现中小血管炎体征和症状的pSS患者。本研究还调查了OvSD/pSS/AAV的总体发生率及其重新分类的主要影响因素。
本研究纳入了2005年3月至2020年12月期间符合纳入标准的116例pSS患者，并将提示中小血管炎的体征和症状定义为支持AAV的肺实质病变、周围神经病变和疑似肾血管炎。当显微镜下多血管炎（MPA）和肉芽肿性多血管炎（GPA）的总分≥5分且嗜酸性肉芽肿性多血管炎（EGPA）评分≥6分时可做出诊断。
患者的中位年龄为56.0岁，101例（87.1%）为女性。共有95例、12例和37例患者分别有支持AAV的肺实质病变、周围神经病变和疑似肾血管炎。根据AAV的ACR/EULAR标准，116例患者中有35例（30.2%）被重新分类为患有OvSD/pSS/AAV。在这35例患者中，4例被重新分类为同时患有OvSD/pSS/MPA和OvSD/pSS/GPA，1例被重新分类为同时患有OvSD/pSS/MPA和OvSD/pSS/EGPA。OvSD/pSS/AAV重新分类的主要影响因素是ANCA阳性。
在出现中小血管炎体征和症状的pSS患者中，OvSD/pSS/AAV重新分类的总体发生率为30.2%。其发生可能性随ANCA阳性而增加。
