Li Yueting, Chen Siyun, Guo Li, Wang Chuhan, Wang Yanhong, Zhao Jiuliang, Xu Dong, Li Mengtao, Zeng Xiaofeng, Zhao Yan
Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital; Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, 100730, China.
National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science and Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Beijing, 100730, China.
Clin Exp Med. 2025 May 25;25(1):174. doi: 10.1007/s10238-025-01715-x.
Hypergammaglobulinemia is prevalent in primary Sjögren's syndrome (pSS) and is considered a marker of disease activity. However, limited data exist regarding its association with disease activity and organ damage. We enrolled patients with pSS from the Chinese Rheumatism Data Centre and categorized them into four groups based on the IgG levels over a 3-year follow-up: consistently high, consistently intermediate, consistently normal, and intermittent. Disease activity and organ damage at the end of the third year were compared across these groups. A total of 351 patients were included in this study, all females with a median age of 45 (IQR 35-53) years. The median baseline IgG was 17.2 (IQR 13.4-21.4) g/L. About 40% of the patients with elevated baseline IgG levels remained in the consistently high or intermediate IgG group. Compared to patients with consistently normal IgG levels, those with consistently high IgG levels had higher median SSDDI scores (2 vs. 1, p = 0.014), higher median ESSPRI scores (2.5 vs. 1.7, p = 0.005), more extra-glandular organ manifestations (IRR 2.34, 95%CI 1.48-3.7, p = < 0.001), and more extra-glandular organ damage (IRR 2.06, 95%CI 1.03-4.12, p = 0.040) at the third-year follow-up. No significant differences in extra-glandular involvement were found between the consistently intermediate IgG group and the consistently normal IgG group. The consistently high IgG level, but not the consistently intermediate level, was associated with a greater number of extra-glandular manifestations and increased extra-glandular organ damage.
高球蛋白血症在原发性干燥综合征（pSS）中很常见，被认为是疾病活动的一个标志物。然而，关于其与疾病活动和器官损害之间关联的数据有限。我们从中国风湿病数据中心招募了pSS患者，并在3年的随访期间根据IgG水平将他们分为四组：持续高、持续中等、持续正常和间歇性。比较了这些组在第三年末的疾病活动和器官损害情况。本研究共纳入351例患者，均为女性，中位年龄为45（四分位间距35 - 53）岁。基线IgG的中位数为17.2（四分位间距13.4 - 21.4）g/L。基线IgG水平升高的患者中约40%仍处于持续高或中等IgG组。与IgG水平持续正常的患者相比，IgG水平持续高的患者在第三年随访时具有更高的中位SSDDI评分（2对1，p = 0.014）、更高的中位ESSPRI评分（2.5对1.7，p = 0.005）、更多的腺外器官表现（发病率比2.34，95%置信区间1.48 - 3.7，p = < 0.001）以及更多的腺外器官损害（发病率比2.06，95%置信区间1.03 - 4.12，p = 0.040）。持续中等IgG组和持续正常IgG组之间在腺外受累方面未发现显著差异。持续高的IgG水平而非持续中等的水平与更多的腺外表现和腺外器官损害增加有关。
