Anticentromere antibody positive patients with primary Sjögren's syndrome have distinctive clinical and immunological characteristics

医学 类风湿因子 内科学 抗体 干眼症 胃肠病学 类风湿性关节炎 免疫学 疾病 淋巴细胞 维生素 维生素A缺乏 视黄醇
作者
Yixuan Liu,Liuxiong Guo,Wei Lin,Xiaoran Ning,Meilu Liu,Jingjing Cao,Yashuang Su,Xiao Zheng,Shuo Li,Fang Li,Luping Ren,Guangyao Song
出处
期刊:Clinical and Experimental Rheumatology
标识
DOI:10.55563/clinexprheumatol/o3pxq0
摘要

To investigate the clinical manifestations, immunological characteristics, circulating lymphocyte subsets and risk factors of anticentromere antibody (ACA) positive patients with primary Sjögren's syndrome (pSS).Data of 333 patients with newly diagnosed pSS were collected and analysed retrospectively. The demographic features, glandular dysfunction, extraglandular manifestations, laboratory data, peripheral blood lymphocyte profiles and serum cytokines were compared between ACA-positive and ACA-negative pSS patients. Logistic regression analysis was used to evaluate the association between ACA and pSS characteristics.The prevalence of ACA among pSS patients was 13.5%. ACA-positive pSS patients were older at diagnosis and had longer disease duration. Xerostomia, xerophthalmia, parotid enlargement, Raynaud's phenomenon (RP), lung and digestive system involvement were more common in ACA-positive group, whereas haematological involvement such as leukopenia was more common in ACA-negative group. Less frequency of rheumatoid factor, hypergammaglobulinaemia, anti-SSA and anti-SSB positivity, as well as higher positivity rate of ANA were observed in ACA-positive pSS patients, which exhibited a lower ESSDAI. In addition, decreased B cells and elevated NK cells were found in patients with ACA-positive. Multivariate analysis identified that disease duration longer than 5 years, parotid enlargement, normal immunoglobulin and the absence of anti-SSA antibody were risk factors of ACA-positive pSS.ACA positive pSS patients have distinctive clinical manifestations and less severe immunological features, present a lower disease activity and lower activation of the humoral immune system. Physicians should pay attention to RP, lung and liver involvement in this subset of pSS.

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