Systemic autoimmune rheumatic diseases (SARDs) are a heterogeneous group of chronic multisystem inflammatory disorders that are thought to have a complex pathophysiology, which is not yet fully understood. Recently, the role of non-coding RNAs, including long non-coding RNA (lncRNA), has been of particular interest in the pathogenesis of SARDs. We aimed to summarize the potential roles of lncRNA in SARDs affecting the skin including, systemic sclerosis (SSc), dermatomyositis (DM) and cutaneous lupus erythematosus (CLE). We conducted a narrative review summarizing original articles published until July 19, 2021, regarding lncRNA associated with SSc, DM, and CLE. Several lncRNAs were hypothesized to play an important role in disease pathogenesis of SSc, DM and CLE. In SSc, Negative Regulator of IFN Response (NRIR) was thought to modulate Interferon (IFN) response in monocytes, anti-sense gene to X-inactivation specific transcript (TSIX) to regulate increased collagen stability, HOX transcript antisense RNA (HOTAIR) to increase numbers of myofibroblasts, OTUD6B-Anti-Sense RNA 1 to decrease fibroblast apoptosis, ncRNA00201 to regulate pathways in SSc pathogenesis and carcinogenesis, H19X potentiating TGF-β-driven extracellular matrix production, and finally PSMB8-AS1 potentiates IFN response. In DM, linc-DGCR6-1 expression was hypothesized to target the USP18 protein, a type 1 IFN-inducible protein that is considered a key regulator of IFN signaling. Additionally, AL136018.1 is suggested to regulate the expression Cathepsin G, which increases the permeability of vascular endothelial cells and the chemotaxis of inflammatory cells in peripheral blood and muscle tissue in DM. Lastly, lnc-MIPOL1-6 and lnc-DDX47-3 in discoid CLE were thought to be associated with the expression of chemokines, which are significant in Th1 mediated disease. In this review, we summarize the key lncRNAs that may drive pathogenesis of these connective tissue diseases and could potentially serve as therapeutic targets in the future.

系统性自身免疫性风湿病 (SARD) 是一组异质性的慢性多系统炎症性疾病，被认为具有复杂的病理生理学机制，目前尚不完全清楚。最近，非编码 RNA，包括长链非编码 RNA (lncRNA)，在 SARD 的发病机制中的作用引起了特别的关注。我们旨在总结 lncRNA 在影响皮肤的 SARD 中的潜在作用，包括系统性硬化症 (SSc)、皮肌炎 (DM) 和皮肤红斑狼疮 (CLE)。我们对 2021 年 7 月 19 日前发表的关于与 SSc、DM 和 CLE 相关的 lncRNA 的原始文章进行了叙述性审查。假设几种 lncRNA 在 SSc、DM 和 CLE 的疾病发病机制中发挥重要作用。在 SSc 中，IFN 反应的负调控因子 (NRIR) 被认为调节单核细胞中的干扰素 (IFN) 反应，反义基因对 X 失活特异性转录物 (TSIX)调节增加的胶原蛋白稳定性，HOX 转录物反义 RNA (HOTAIR)增加肌成纤维细胞的数量，OTUD6B-Anti - 感应 RNA 1可减少成纤维细胞凋亡，ncRNA00201可调节 SSc 发病机制和致癌作用中的通路，H19X可增强 TGF-β 驱动的细胞外基质产生，最后PSMB8-AS1可增强 IFN 反应。在 DM 中，linc-DGCR6-1假设表达以 USP18 蛋白为靶点，USP18 蛋白是一种 1 型 IFN 诱导蛋白，被认为是 IFN 信号传导的关键调节因子。此外，建议 AL136018.1 调节 Cathepsin G 的表达，从而增加 DM 中血管内皮细胞的通透性和外周血和肌肉组织中炎症细胞的趋化性。最后，盘状 CLE 中的lnc-MIPOL1-6和lnc-DDX47-3被认为与趋化因子的表达有关，这在 Th1 介导的疾病中具有重要意义。在这篇综述中，我们总结了可能驱动这些结缔组织疾病发病机制的关键 lncRNA，并可能在未来作为治疗靶点。