Zhu Xiaofang, Lu Saisai, Zhu Lixia, Yu Mengjiao, Wei Tingting, Zhu Xiaochun, Chen Dan, Chen Chengshui
Department of Rheumatology, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, China.
Department of Pneumology, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, China.
Front Mol Biosci. 2021 May 13;8:640779. doi: 10.3389/fmolb.2021.640779. eCollection 2021.
Primary Sjögren's syndrome (pSS) is a chronic systemic autoimmune disease characterized by typical autoantibody production and lymphocytic-mediated exocrine gland damage. Interstitial lung disease (ILD) is a common complication of pSS and can be associated with a poor prognosis. However, the pathogenesis of ILD in pSS is still unclear. In this study, we used RNA sequencing to investigate the gene-expression profile of the minor salivary glands (MSGs) from 36 patients with ILD-pSS and 128 patients with non-ILD-pSS. In the remarkably enriched chemokine-mediated signaling pathway, C-X-C motif chemokine receptor 2 (), a receptor for interleukin-8, which participates in the activation of neutrophils, was found to be significantly elevated in both MSG and plasma from pSS patients with vs. without ILD ( < 0.001). Furthermore, the expression level in MSG and plasma was significantly associated with the diffusing capacity of the lungs for carbon monoxide, erythrocyte sedimentation rate, and EULAR Sjögren's Syndrome disease Activity Index in ILD-pSS. Therefore, with its potential role in ILD progression in patients with pSS and its strong association with clinical manifestations of the disease, may serve as a useful index for disease activity in ILD associated with pSS.
原发性干燥综合征（pSS）是一种慢性全身性自身免疫性疾病，其特征为典型自身抗体产生及淋巴细胞介导的外分泌腺损伤。间质性肺疾病（ILD）是pSS的常见并发症，且可能与预后不良相关。然而，pSS中ILD的发病机制仍不清楚。在本研究中，我们使用RNA测序来研究36例合并ILD的pSS患者及128例未合并ILD的pSS患者的小唾液腺（MSG）的基因表达谱。在显著富集的趋化因子介导的信号通路中，发现参与中性粒细胞激活的白细胞介素-8受体C-X-C基序趋化因子受体2（）在合并ILD与未合并ILD的pSS患者的MSG及血浆中均显著升高（<0.001）。此外，在合并ILD的pSS中，MSG和血浆中的表达水平与肺一氧化碳弥散量、红细胞沉降率及欧洲抗风湿病联盟干燥综合征疾病活动指数显著相关。因此，鉴于其在pSS患者ILD进展中的潜在作用及其与疾病临床表现的密切关联，可能作为合并pSS的ILD疾病活动的有用指标。
