Betensley Alan, Sharif Rabab, Karamichos Dimitrios
Nazih Zuhdi Transplant Institute, Integris Baptist Medical Center, Oklahoma City, OK 73112, USA.
Department of Cell Biology, University of Oklahoma Health Sciences Center, Oklahoma City, OK 73104, USA.
J Clin Med. 2016 Dec 26;6(1):2. doi: 10.3390/jcm6010002.
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive interstitial lung disorder showcasing an interaction between genetic predisposition and environmental risks. This usually involves the coaction of a mixture of cell types associated with abnormal wound healing, leading to structural distortion and loss of gas exchange function. IPF bears fatal prognosis due to respiratory failure, revealing a median survival of approximately 2 to 3 years. This review showcases the ongoing progress in understanding the complex pathophysiology of IPF and it highlights the latest potential clinical treatments. In IPF, various components of the immune system, particularly clotting cascade and shortened telomeres, are highly involved in disease pathobiology and progression. This review also illustrates two US Food and Drug Administration (FDA)-approved drugs, nintedanib (OFEV, Boehringer Ingelheim, Ingelheim am Rhein, Germany) and pirfenidone (Esbriet, Roche, Basel, Switzerland), that slow IPF progression, but unfortunately neither drug can reverse the course of the disease. Although the mechanisms underlying IPF remain poorly understood, this review unveils the past and current advances that encourage the detection of new IPF pathogenic pathways and the development of effective treatment methods for the near future.
特发性肺纤维化（IPF）是一种慢性进行性间质性肺疾病，表现出遗传易感性与环境风险之间的相互作用。这通常涉及与异常伤口愈合相关的多种细胞类型的共同作用，导致结构扭曲和气体交换功能丧失。由于呼吸衰竭，IPF预后不良，中位生存期约为2至3年。本综述展示了在理解IPF复杂病理生理学方面的最新进展，并强调了最新的潜在临床治疗方法。在IPF中，免疫系统的各种成分，特别是凝血级联反应和缩短的端粒，高度参与疾病的病理生物学和进展。本综述还阐述了两种美国食品药品监督管理局（FDA）批准的药物，尼达尼布（Ofev，勃林格殷格翰公司，德国莱茵河畔英格尔海姆）和吡非尼酮（Esbriet，罗氏公司，瑞士巴塞尔），它们可减缓IPF的进展，但遗憾的是，这两种药物都无法逆转疾病进程。尽管IPF的潜在机制仍知之甚少，但本综述揭示了过去和当前的进展，这些进展有助于发现新的IPF致病途径，并在不久的将来开发出有效的治疗方法。
