Pacurari Maricica, Mitra Amal, Turner Timothy
Department of Biology, College of Science, Engineering, and Technology, Jackson State University, Jackson, MS 39217, USA.
Department of Epidemiology and Biostatistics, School of Public Health, Jackson State University, Jackson, MS 39217, USA.
Int J Inflam. 2021 Oct 13;2021:3963659. doi: 10.1155/2021/3963659. eCollection 2021.
Idiopathic pulmonary fibrosis (IPF) is a disease with an unknown etiology mainly characterized by a progressive decline of lung function due to the scarring of the tissue deep in the lungs. The overall survival after diagnosis remains low between 3 and 5 years. IPF is a heterogeneous disease and much progress has been made in the past decade in understanding the disease mechanisms that contributed to the development of two new drugs, pirfenidone and nintedanib, which improved the therapeutic management of the disease. The understanding of the cofactors and comorbidities of IPF also contributed to improved management of the disease outcome. In the present review, we evaluate scientific evidence which indicates IPF as a risk factor for other diseases based on the complexity of molecular and cellular mechanisms involved in the disease development and of comorbidities. We conclude from the existing literature that while much progress has been made in understating the mechanisms involved in IPF development, further studies are still necessary to fully understand IPF pathogenesis which will contribute to the identification of novel therapeutic targets for IPF management as well as other diseases for which IPF is a major risk factor.
特发性肺纤维化（IPF）是一种病因不明的疾病，主要特征是由于肺深部组织瘢痕形成导致肺功能进行性下降。诊断后的总体生存率在3至5年之间仍然很低。IPF是一种异质性疾病，在过去十年中，在理解导致两种新药（吡非尼酮和尼达尼布）研发的疾病机制方面取得了很大进展，这两种药物改善了该疾病的治疗管理。对IPF的辅助因素和合并症的认识也有助于改善疾病结局的管理。在本综述中，基于该疾病发展过程中涉及的分子和细胞机制以及合并症的复杂性，我们评估了表明IPF是其他疾病风险因素的科学证据。我们从现有文献中得出结论，虽然在理解IPF发展所涉及的机制方面已经取得了很大进展，但仍需要进一步研究以充分了解IPF的发病机制，这将有助于确定IPF管理以及IPF是主要风险因素的其他疾病的新治疗靶点。
