Pulmonary arterial hypertension (PAH) is a severe complication of connective tissue disease (CTD), causing death in systemic sclerosis (SSc). The past decade has yielded many scientific insights into microRNA (miRNAs) in PAH and SSc. This growth of knowledge has well-illustrated the complexity of microRNA (miRNA)-based regulation of gene expression in PAH. However, few miRNA-related SSc-PAH were elucidated. This review firstly discusses the role of transforming growth factor-beta (TGF-β) signaling and bone morphogenetic protein receptor type II (BMPR2) in PAH and SSc. Secondly, the miRNAs relating to TGF-β and BMPR2 signaling pathways in PAH and SSc or merely PAH were subsequently summarized. Finally, future studies might develop early diagnostic biomarkers and target-oriented therapeutic strategies for SSc-PAH and PAH treatment.

中文翻译：

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TGF-β或BMPR2信号通路相关miRNA在肺动脉高压和系统性硬化症中的作用

肺动脉高压 (PAH) 是结缔组织病 (CTD) 的严重并发症，可导致系统性硬化症 (SSc) 死亡。在过去的十年中，人们对 PAH 和 SSc 中的 microRNA (miRNA) 产生了许多科学见解。这种知识的增长很好地说明了 PAH 中基于 microRNA (miRNA) 的基因表达调控的复杂性。然而，很少有 miRNA 相关的 SSc-PAH 被阐明。本综述首先讨论了转化生长因子-β (TGF-β) 信号传导和 II 型骨形态发生蛋白受体 (BMPR2) 在 PAH 和 SSc 中的作用。其次，随后总结了与 PAH 和 SSc 或仅 PAH 中 TGF-β 和 BMPR2 信号通路相关的 miRNA。最后，未来的研究可能会为 SSc-PAH 和 PAH 治疗开发早期诊断生物标志物和靶向治疗策略。