SSc-ILD（硬皮病相关间质性肺病）是一种复杂的风湿性疾病，其部分特征是免疫失调，导致正常肺结构进行性纤维化替代。由于迫切需要改进的治疗方案，因此对介导这种疾病的纤维增殖机制的进一步研究有可能加速新疗法的开发。先天免疫的贡献是 SSc-ILD 的一个新兴研究领域，因为最近的工作已经证明了 NLRP3 炎性体及其相关细胞因子 TNFα（肿瘤坏死因子 α）、IL-1β（白细胞介素 1 β）的机制和临床意义。 ）和 IL-18 在这种疾病中的作用。在这篇综述中，我们将重点介绍这些研究提供的新颖的病理生理学见解以及利用这种复杂的生物学获得临床益处的潜力。版权所有 © 2023 Woo、Gandhi、Ghincea、Saber、Lee 和 Ryu。

SSc-ILD (scleroderma associated interstitial lung disease) is a complex rheumatic disease characterized in part by immune dysregulation leading to the progressive fibrotic replacement of normal lung architecture. Because improved treatment options are sorely needed, additional study of the fibroproliferative mechanisms mediating this disease has the potential to accelerate development of novel therapies. The contribution of innate immunity is an emerging area of investigation in SSc-ILD as recent work has demonstrated the mechanistic and clinical significance of the NLRP3 inflammasome and its associated cytokines of TNFα (tumor necrosis factor alpha), IL-1β (interleukin-1 beta), and IL-18 in this disease. In this review, we will highlight novel pathophysiologic insights afforded by these studies and the potential of leveraging this complex biology for clinical benefit.Copyright © 2023 Woo, Gandhi, Ghincea, Saber, Lee and Ryu.