Department of Dermatology.
Black Family Stem Cell Institute.
J Clin Invest. 2024 Sep 3;134(17):e183708. doi: 10.1172/JCI183708.
Sarcoidosis is an inflammatory disease characterized by immune cell-rich granulomas that form in multiple organs. In this issue of the JCI, Sati and colleagues used scRNA-seq and spatial transcriptomics of skin samples from patients with sarcoidosis and non-sarcoidosis granulomatous disease to identify upregulation of a stromal-immune CXCL12/CXCR4 axis and accumulation of type 1 innate lymphoid cells (ILC1s) in sarcoidosis. The accumulation of ILC1s in skin and blood was specific to patients with sarcoidosis and not observed in other granulomatous diseases. The authors used a mouse model of lung granuloma to show that ILCs contribute to granuloma formation and that blockade of CXCR4 reduced the formation of granulomas, providing a proof of concept that sarcoidosis may be treated by CXCR4 blockade to prevent the progression of disease in patients. These results suggest ILC1s could serve as a diagnostic biomarker in sarcoidosis and a potential therapeutic target.
结节病是一种炎症性疾病,其特征是在多个器官中形成富含免疫细胞的肉芽肿。在本期 JCI 中,Sati 及其同事利用结节病患者和非结节病肉芽肿性疾病患者的皮肤样本的 scRNA-seq 和空间转录组学,鉴定出基质-免疫 CXCL12/CXCR4 轴的上调和 1 型先天淋巴样细胞 (ILC1) 在结节病中的积累。ILC1 在皮肤和血液中的积累是结节病患者特有的,在其他肉芽肿性疾病中没有观察到。作者使用肺部肉芽肿的小鼠模型表明,ILC 有助于肉芽肿的形成,而阻断 CXCR4 减少了肉芽肿的形成,为 CXCR4 阻断可能通过预防患者疾病进展来治疗结节病的概念提供了证据。这些结果表明,ILC1 可作为结节病的诊断生物标志物和潜在的治疗靶点。