Department of Clinical Laboratory, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.
Department of Rheumatology, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.
Scand J Immunol. 2022 Jul;96(1):e13166. doi: 10.1111/sji.13166. Epub 2022 Apr 3.
Innate lymphoid cells (ILCs), a novel group of innate immune cells, play a key role in the early immune response via rapidly reacting to signals expressed by tissue-resident cells. ILCs contribute to some autoimmune diseases. We aim to investigate the proportions of circulating ILC subgroups in patients with primary biliary cholangitis (PBC). Overall, 48 patients with PBC and 24 healthy controls (HCs) were enrolled. Circulating ILCs and cytokine production were detected by flow cytometry. The proportions of total ILCs, ILC precursors (ILCPs) and ILCP/ILC2 ratio increased and that of ILC2s decreased in patients with PBC. ILC2 proportion was negatively correlated with gamma-glutamyl transpeptidase (GGT), alanine aminotransferase (ALT) and aspartate aminotransferase (AST). The proportion of ILCPs and ILCP/ILC2 ratio were positively correlated with alkaline phosphatase, GGT, ALT and AST. ILC2 proportion was significantly decreased in the ursodeoxycholic acid (UDCA) -non-responder group compared with the UDCA-responder group, whereas the proportion of ILCPs and ILCP/ILC2 were ratio significantly increased. The proportions of CD38 ILC2s, CD38 ILCPs, CD45RO ILC2s and CD45RO ILCPs were significantly higher in patients with PBC than in HCs. Levels of IL-17A producing ILCs were higher in patients with PBC than in HCs. PBC is accompanied by alterations in circulating ILCs. The proportions of ILC2s, ILCPs, and ILCP/ILC2 ratio were associated with the PBC disease activity. The proportions of ILCPs and ILCP/ILC2 ratio may reflect the UDCA treatment failure in patients with PBC. ILC2s and ILCPs from patients with PBC get activated, these cells may be involved in the pathogenesis of PBC.
固有淋巴细胞（ILC）是一类新型的固有免疫细胞，通过快速响应组织驻留细胞表达的信号，在早期免疫反应中发挥关键作用。ILC 参与了一些自身免疫性疾病的发生。本研究旨在探讨原发性胆汁性胆管炎（PBC）患者循环 ILC 亚群的比例。共纳入 48 例 PBC 患者和 24 例健康对照（HC）。采用流式细胞术检测循环 ILC 及细胞因子产生情况。结果显示，与 HC 相比，PBC 患者总 ILC、ILC 前体细胞（ILCP）和 ILCP/ILC2 比值增加，ILC2 比例降低。ILC2 比例与γ-谷氨酰转肽酶（GGT）、丙氨酸氨基转移酶（ALT）和天冬氨酸氨基转移酶（AST）呈负相关。ILCP 比例和 ILCP/ILC2 比值与碱性磷酸酶、GGT、ALT 和 AST 呈正相关。与 UDCA 应答组相比，UDCA 无应答组患者的 ILC2 比例显著降低，而 ILCP 比例和 ILCP/ILC2 比值显著增加。PBC 患者 CD38+ ILC2、CD38+ ILCP、CD45RO+ ILC2 和 CD45RO+ ILCP 比例明显高于 HC。PBC 患者产生 IL-17A 的 ILC 水平高于 HC。PBC 患者循环 ILC 发生改变。ILC2 比例、ILCP 比例和 ILCP/ILC2 比值与 PBC 疾病活动度相关。ILCP 比例和 ILCP/ILC2 比值可能反映了 PBC 患者 UDCA 治疗失败。PBC 患者的 ILC2 和 ILCP 被激活，这些细胞可能参与了 PBC 的发病机制。
