Horai Yoshiro, Shimizu Toshimasa, Umeda Masataka, Nishihata Shin-Ya, Nakamura Hideki, Kawakami Atsushi
Department of Rheumatology, Sasebo City General Hospital, Sasebo 857-8511, Japan.
Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki 852-8523, Japan.
J Clin Med. 2023 Sep 10;12(18):5873. doi: 10.3390/jcm12185873.
Sjögren's syndrome (SS) is a rheumatic disease characterized by sicca and extraglandular symptoms, such as interstitial lung disease and renal tubular acidosis. SS potentially affects the prognosis of patients, especially in cases of complicated extraglandular symptoms; however, only symptomatic therapies against xerophthalmia and xerostomia are currently included in the practice guidelines as recommended therapies for SS. Considering that SS is presumed to be a multifactorial entity caused by genetic and environmental factors, a multidisciplinary approach is necessary to clarify the whole picture of its pathogenesis and to develop disease-specific therapies for SS. This review discusses past achievements and future prospects for pursuing the pathophysiology and therapeutic targets for SS, especially from the perspectives of viral infections, toll-like receptors (TLRs), long-noncoding RNAs (lncRNAs), and related signals. Based on the emerging roles of viral infections, TLRs, long-noncoding RNAs and related signals, antiviral therapy, hydroxychloroquine, and vitamin D may lower the risk of or mitigate SS. Janus-kinase (JAK) inhibitors are also potential novel therapeutic options for several rheumatic diseases involving the JAK-signal transducer and activator of transcription pathways, which are yet to be ascertained in a randomized controlled study targeting SS.
干燥综合征(SS)是一种以口干、眼干及腺体外症状为特征的风湿性疾病,如间质性肺病和肾小管酸中毒。SS可能影响患者的预后,尤其是在合并腺体外症状的情况下;然而,目前实践指南中仅将针对干眼症和口干症的对症治疗作为SS的推荐治疗方法。鉴于SS被认为是一种由遗传和环境因素引起的多因素疾病,需要采用多学科方法来阐明其发病机制的全貌,并开发针对SS的特异性疾病治疗方法。本综述讨论了探索SS病理生理学和治疗靶点的过去成就和未来前景,特别是从病毒感染、Toll样受体(TLR)、长链非编码RNA(lncRNA)及相关信号的角度。基于病毒感染、TLR、长链非编码RNA及相关信号的新作用,抗病毒治疗、羟氯喹和维生素D可能会降低SS的风险或减轻其症状。Janus激酶(JAK)抑制剂也是涉及JAK-信号转导子和转录激活子途径的几种风湿性疾病的潜在新型治疗选择,但在针对SS的随机对照研究中尚未得到证实。