Respiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova, Padova, Italy.
Laboratory of Cell Therapies and Respiratory Medicine, Department of Medical and Surgical Sciences for Children & Adults, University Hospital of Modena and Reggio Emilia, Modena, Italy.
Expert Opin Ther Targets. 2022 Jul;26(7):617-631. doi: 10.1080/14728222.2022.2114897. Epub 2022 Aug 30.
Idiopathic pulmonary fibrosis (IPF) is a chronic disease of unknown origin characterized by progressive scarring of the lung leading to irreversible loss of function. Despite the availability of two drugs that are able to slow down disease progression, IPF remains a deadly disease. The pathogenesis of IPF is poorly understood, but a dysregulated wound healing response following recurrent alveolar epithelial injury is thought to be crucial.
In the last few years, the role of the immune system in IPF pathobiology has been reconsidered; indeed, recent data suggest that a dysfunctional immune system may promote and unfavorable interplay with pro-fibrotic pathways thus acting as a cofactor in disease development and progression. In this article, we review and critically discuss the role of T cells in the pathogenesis and progression of IPF in the attempt to highlight ways in which further research in this area may enable the development of targeted immunomodulatory therapies for this dreadful disease.
A better understanding of T cell interactions has the potential to facilitate the development of immune modulators targeting multiple T cell-mediated pathways, thus halting disease initiation and progression.
特发性肺纤维化（IPF）是一种病因不明的慢性疾病，其特征是肺部进行性瘢痕形成，导致肺功能不可逆转的丧失。尽管有两种能够减缓疾病进展的药物，但 IPF 仍然是一种致命的疾病。IPF 的发病机制尚不清楚，但反复的肺泡上皮损伤后的失调性愈合反应被认为是关键。
在过去的几年中，免疫系统在 IPF 病理生物学中的作用已经被重新考虑；事实上，最近的数据表明，功能失调的免疫系统可能促进和不利地与促纤维化途径相互作用，从而作为疾病发展和进展的协同因素。在本文中，我们回顾和批判性地讨论了 T 细胞在 IPF 的发病机制和进展中的作用，试图强调在这一领域进一步研究可能使针对这种可怕疾病的靶向免疫调节疗法的发展成为可能的方式。
更好地了解 T 细胞相互作用有可能促进针对多种 T 细胞介导途径的免疫调节剂的开发，从而阻止疾病的发生和进展。
