Department of Personalised Medicine, State Research Institute Center for Innovative Medicine, Santariskiu st. 5, LT-08405 Vilnius, Lithuania.
Medicina (Kaunas). 2024 Nov 18;60(11):1894. doi: 10.3390/medicina60111894.
Sjögren's disease (SjD), or primary Sjögren's syndrome (pSS), is a heterogeneous chronic autoimmune disorder with multiple clinical manifestations that can develop into non-Hodgkin's lymphoma in mucosa-associated lymphoid tissue. SjD is one of the autoimmune diseases with the maximum delayed diagnosis due to its insidious onset, heterogeneous clinical features and varied course. It is increasingly recognized that extraglandular manifestations represent a clinical challenge for patients with SjD. The European League Against Rheumatism (EULAR) Sjögren's Syndrome (SS) Disease Activity Index (ESSDAI) is a systemic disease activity index designed to measure disease activity in patients with primary Sjogren's syndrome. It consists of 12 domains: cutaneous, pulmonary, renal, articular, muscular, peripheral nervous system, central nervous system, hematological, glandular, constitutional, lymphadenopathy and lymphoma, biological. More than a quarter of patients with pSS may have systemic features that are not included in the ESSDAI classification, i.e., various cardiovascular, ophthalmic, ENT, and other systemic or organ involvement that increase the magnitude of the systemic phenotype in the disease. The ESSDAI also excludes the gastrointestinal (GI) tract, and unfortunately, GI manifestations are not routinely assessed. Gastroesophageal reflux disease (GERD) is one of the most prevalent gastrointestinal disorders, impairing quality of life and consuming a large volume of medical resources. Recently carried out the Mendelian randomized trial confirmed the causal link between SjD and gastroesophageal reflux disease (GERD) and showed that GERD is a risk factor for SjD. This review aims to provide an overview of the research describing evidenced based links between Sjögren's disease and gastroesophageal reflux disease, with the intention of ensuring that any systemic pathology in Sjögren's disease is properly assessed and that management of the disease is directed towards the patient. A comprehensive literature search was carried out on PubMed, Web of Science, Scopus and the Cochrane Library databases. Two researchers searched for published studies indexed from inception to 1 September 2024 using the keywords 'Sjögren's syndrome' OR 'Sjögren's disease' AND 'gastroesophageal reflux disease' AND 'microbiota' OR microbiota dysbiosis'. We limited our search for scientific articles to human studies, and only included articles in English. Overall, there is a lack of evidence-based studies assessing the association between GERD and Sjögren's disease and the changes in the microbiota associated with GERD in a multidisciplinary setting. Such studies are needed for the future, as this will improve the early diagnosis of Sjögren's disease and the personalized management of the disease.
干燥综合征(SjD)或原发性干燥综合征(pSS)是一种异质性慢性自身免疫性疾病,具有多种临床表现,可在黏膜相关淋巴组织中发展为非霍奇金淋巴瘤。由于其隐匿性发病、异质性临床特征和多变的病程,SjD 是自身免疫性疾病中最大程度延迟诊断的疾病之一。人们越来越认识到,腺外表现是 SjD 患者面临的临床挑战。欧洲抗风湿病联盟(EULAR)干燥综合征疾病活动指数(ESSDAI)是一种旨在测量原发性干燥综合征患者疾病活动度的系统性疾病活动指数。它由 12 个领域组成:皮肤、肺、肾、关节、肌肉、周围神经系统、中枢神经系统、血液学、腺体、全身症状、淋巴结病和淋巴瘤、生物学。超过四分之一的 pSS 患者可能有 ESSDAI 分类中未包含的全身特征,即各种心血管、眼科、耳鼻喉科和其他全身或器官受累,这些特征增加了疾病中全身表型的严重程度。ESSDAI 还排除了胃肠道(GI),不幸的是,GI 表现未被常规评估。胃食管反流病(GERD)是最常见的胃肠道疾病之一,它会降低生活质量并消耗大量医疗资源。最近进行的孟德尔随机试验证实了 SjD 和胃食管反流病(GERD)之间的因果关系,并表明 GERD 是 SjD 的一个危险因素。本综述旨在概述描述 SjD 和胃食管反流病之间基于证据的关联的研究,目的是确保对 SjD 中的任何系统性病理学进行适当评估,并针对患者对疾病进行管理。对 PubMed、Web of Science、Scopus 和 Cochrane 图书馆数据库进行了全面的文献检索。两名研究人员使用关键词“干燥综合征”或“干燥综合征”和“胃食管反流病”和“微生物组”或微生物组失调,从成立到 2024 年 9 月 1 日对已发表的研究进行了搜索。我们将科学文章的搜索范围限制在人类研究上,并且只包括英语文章。总的来说,缺乏评估 GERD 和 SjD 之间关联以及多学科环境中与 GERD 相关的微生物群变化的基于证据的研究。未来需要进行此类研究,因为这将改善 SjD 的早期诊断和疾病的个体化管理。