Interstitial Lung Diseases Center of Excellence, Department of Pulmonology, St. Antonius Hospital, 3435 CM Nieuwegein, The Netherlands.
Interstitial Lung Diseases Center of Excellence, Pathologie DNA, Department of Pathology, St. Antonius Hospital, 3435 CM Nieuwegein, The Netherlands.
Int J Mol Sci. 2023 Aug 14;24(16):12792. doi: 10.3390/ijms241612792.
The unknown etiology of sarcoidosis, along with the variability in organ involvement and disease course, complicates the effective treatment of this disease. Based on recent studies, the cellular inflammatory pathways involved in granuloma formation are of interest regarding possible new treatment options, such as the mechanistic (formerly mammalian) target of rapamycin complex 1 (mTORC1) pathway, the Janus kinase/signal transducers and activators of transcription (JAK/STAT) pathway, and the nucleotide-binding domain, leucine-rich-containing family, pyrin domain-containing-3 (NLRP3) inflammasome pathway. The aim of this study was to explore the potential coexpression of these three inflammatory pathways in patients with sarcoidosis and see whether possible differences were related to disease outcome. The tissue of 60 patients with sarcoidosis was used to determine the activity of these three signaling pathways using immunohistochemistry. The activation of NLRP3 was present in 85% of all patients, and the activation of mTORC1 and JAK/STAT was present in 49% and 50% of patients, respectively. Furthermore, the presence of NLRP3 activation at diagnosis was associated with a chronic disease course of sarcoidosis. Our finding of different new conceptual inflammatory tissue phenotypes in sarcoidosis could possibly guide future treatment studies using the available inhibitors of either NLRP3, JAK-STAT, and mTORC1 inhibitors in a more personalized medicine approach.
结节病的病因不明,且其器官受累和疾病进程存在差异,这使得该病的有效治疗变得复杂。基于最近的研究,在探讨新的治疗选择时,肉芽肿形成所涉及的细胞炎症途径引起了关注,如机械性(原哺乳动物)雷帕霉素靶蛋白复合体 1(mTORC1)途径、Janus 激酶/信号转导和转录激活因子(JAK/STAT)途径以及核苷酸结合域、富含亮氨酸重复家族、pyrin 结构域包含蛋白 3(NLRP3)炎症小体途径。本研究旨在探讨这三种炎症途径在结节病患者中的潜在共表达情况,并观察其与疾病结局的可能相关性。本研究使用免疫组织化学方法检测了 60 例结节病患者的组织中这三种炎症通路的活性。结果显示,所有患者中有 85%存在 NLRP3 激活,有 49%和 50%的患者存在 mTORC1 和 JAK/STAT 的激活。此外,NLRP3 激活的存在与结节病的慢性病程相关。我们在结节病中发现了不同的新概念炎症组织表型,这可能有助于未来的治疗研究,以更个体化的医学方法使用现有的 NLRP3、JAK-STAT 和 mTORC1 抑制剂。