Shenderov Kevin, Collins Samuel L, Powell Jonathan D, Horton Maureen R
Department of Medicine, Division of Pulmonary and Critical Care, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Bloomberg-Kimmel Institute for Cancer Immunotherapy, Sidney Kimmel Comprehensive Cancer Research Center, Department of Oncology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
J Clin Invest. 2021 Jan 19;131(2). doi: 10.1172/JCI143226.
Idiopathic pulmonary fibrosis (IPF) affects hundreds of thousands of people worldwide, reducing their quality of life and leading to death from respiratory failure within years of diagnosis. Treatment options remain limited, with only two FDA-approved drugs available in the United States, neither of which reverse the lung damage caused by the disease or prolong the life of individuals with IPF. The only cure for IPF is lung transplantation. In this review, we discuss recent major advances in our understanding of the role of the immune system in IPF that have revealed immune dysregulation as a critical driver of disease pathophysiology. We also highlight ways in which an improved understanding of the immune system's role in IPF may enable the development of targeted immunomodulatory therapies that successfully halt or potentially even reverse lung fibrosis.
特发性肺纤维化（IPF）影响着全球数十万人，降低了他们的生活质量，并在诊断后的几年内导致呼吸衰竭死亡。治疗选择仍然有限，在美国只有两种获得美国食品药品监督管理局（FDA）批准的药物，这两种药物都无法逆转该疾病造成的肺损伤，也无法延长IPF患者的生命。IPF的唯一治愈方法是肺移植。在本综述中，我们讨论了近期在理解免疫系统在IPF中的作用方面取得的重大进展，这些进展揭示了免疫失调是疾病病理生理学的关键驱动因素。我们还强调了更好地理解免疫系统在IPF中的作用可能如何推动靶向免疫调节疗法的开发，从而成功阻止甚至可能逆转肺纤维化。
