Department of Otolaryngology Head and Neck Surgery, Johns Hopkins School of Medicine, Baltimore, Maryland, USA.
Department of Otolaryngology, Stanford University School of Medicine, Stanford, California, USA.
JCI Insight. 2023 Jun 8;8(11):e158456. doi: 10.1172/jci.insight.158456.
Laryngotracheal stenosis (LTS) is pathologic fibrotic narrowing of the larynx and trachea characterized by hypermetabolic fibroblasts and CD4+ T cell-mediated inflammation. However, the role of CD4+ T cells in promoting LTS fibrosis is unknown. The mTOR signaling pathways have been shown to regulate the T cell phenotype. Here we investigated the influence of mTOR signaling in CD4+ T cells on LTS pathogenesis. In this study, human LTS specimens revealed a higher population of CD4+ T cells expressing the activated isoform of mTOR. In a murine LTS model, targeting mTOR with systemic sirolimus and a sirolimus-eluting airway stent reduced fibrosis and Th17 cells. Selective deletion of mTOR in CD4+ cells reduced Th17 cells and attenuated fibrosis, demonstrating CD4+ T cells' pathologic role in LTS. Multispectral immunofluorescence of human LTS revealed increased Th17 cells. In vitro, Th17 cells increased collagen-1 production by LTS fibroblasts, which was prevented with sirolimus pretreatment of Th17 cells. Collectively, mTOR signaling drove pathologic CD4+ T cell phenotypes in LTS, and targeting mTOR with sirolimus was effective at treating LTS through inhibition of profibrotic Th17 cells. Finally, sirolimus may be delivered locally with a drug-eluting stent, transforming clinical therapy for LTS.
喉气管狭窄(LTS)是一种以高代谢成纤维细胞和 CD4+T 细胞介导的炎症为特征的病理性纤维狭窄。然而,CD4+T 细胞在促进 LTS 纤维化中的作用尚不清楚。mTOR 信号通路已被证明可调节 T 细胞表型。在这里,我们研究了 CD4+T 细胞中 mTOR 信号对 LTS 发病机制的影响。在这项研究中,人类 LTS 标本显示出更高比例的表达激活型 mTOR 的 CD4+T 细胞。在小鼠 LTS 模型中,用系统西罗莫司和西罗莫司洗脱气道支架靶向 mTOR 可减少纤维化和 Th17 细胞。CD4+细胞中 mTOR 的选择性缺失减少了 Th17 细胞并减轻了纤维化,表明 CD4+T 细胞在 LTS 中具有病理性作用。人类 LTS 的多光谱免疫荧光显示 Th17 细胞增加。在体外,Th17 细胞增加了 LTS 成纤维细胞的胶原-1 产生,而西罗莫司预处理 Th17 细胞可预防这种情况。总之,mTOR 信号驱动了 LTS 中病理性的 CD4+T 细胞表型,用西罗莫司靶向 mTOR 通过抑制致纤维化的 Th17 细胞治疗 LTS 是有效的。最后,西罗莫司可以通过载药支架局部给药,改变 LTS 的临床治疗方法。