Department of Graduate School, Hebei Medical University, Shijiazhuang, and Department of Rheumatology and Immunology, Hebei General Hospital, Shijiazhuang, China.
Department of Urology, Hebei General Hospital, Shijiazhuang, China.
Clin Exp Rheumatol. 2023 Dec;41(12):2371-2378. doi: 10.55563/clinexprheumatol/o3pxq0. Epub 2023 Mar 16.
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 the 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, who exhibited a lower ESSDAI. In addition, decreased B cells and elevated NK cells were found in ACA-positive patients. 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.
探讨原发性干燥综合征（pSS）患者抗着丝点抗体（ACA）阳性的临床表现、免疫学特征、循环淋巴细胞亚群及相关危险因素。
回顾性收集 333 例新诊断 pSS 患者的临床资料，分析比较 ACA 阳性与 ACA 阴性 pSS 患者的人口学特征、腺体功能障碍、外分泌腺外表现、实验室数据、外周血淋巴细胞谱和血清细胞因子。采用 Logistic 回归分析评估 ACA 与 pSS 特征之间的相关性。
pSS 患者中 ACA 的患病率为 13.5%。ACA 阳性 pSS 患者的诊断年龄较大，病程较长。口干、眼干、腮腺肿大、雷诺现象（RP）、肺和消化系统受累在 ACA 阳性组更为常见，而血液学受累如白细胞减少在 ACA 阴性组更为常见。与 ACA 阴性 pSS 患者相比，ACA 阳性 pSS 患者的类风湿因子、高丙种球蛋白血症、抗 SSA 和抗 SSB 阳性率较低，抗核抗体阳性率较高，且 ESSDAI 较低。此外，ACA 阳性患者的 B 细胞减少，NK 细胞升高。多因素分析发现病程>5 年、腮腺肿大、免疫球蛋白正常及无抗 SSA 抗体是 ACA 阳性 pSS 的危险因素。
ACA 阳性 pSS 患者具有独特的临床表现和免疫特征，疾病活动度较低，体液免疫激活程度较低。医生应注意该部分 pSS 患者出现 RP、肺部和肝脏受累的情况。
