Department of Internal Medicine, Al-Kindy College of Medicine, University of Baghdad, Baghdad, Iraq.
Department Internal Medicine, B.J. Medical College, Ahmedabad, India.
Immun Inflamm Dis. 2023 Nov;11(11):e1079. doi: 10.1002/iid3.1079.
Idiopathic pulmonary fibrosis (IPF) is a progressive and debilitating lung disease characterized by irreversible scarring of the lungs. The cause of IPF is unknown, but it is thought to involve a combination of genetic and environmental factors. There is no cure for IPF, and treatment is focused on slowing disease progression and relieving symptoms.
We aimed in this review to investigate and provide the latest insights into IPF management modalities, including the potential of Saracatinibas a substitute for current IPF drugs. We also investigated the therapeutic potential of Sotatercept in addressing pulmonary hypertension associated with IPF.
We conducted a comprehensive literature review of relevant studies on IPF management. We searched electronic databases, including PubMed, Scopus, Embase, and Web of science.
The two Food and Drug Administration-approved drugs for IPF, Pirfenidone, and Nintedanib, have been pivotal in slowing disease progression, yet experimental evidence suggests that Saracatinib surpasses their efficacy. Preclinical trials investigating the potential of Saracatinib, a tyrosine kinase inhibitor, have shown to be more effective than current IPF drugs in slowing disease progression in preclinical studies. Also, Sotatercept,a fusion protein, has been shown to reduce pulmonary vascular resistance and improve exercise tolerance in patients with PH associated with IPF in clinical trials.
The advancements discussed in this review hold the promise of improving the quality of life for IPF patients and enhancing our understanding of this condition. There remains a need for further research to confirm the efficacy and safety of new IPF treatments and to develop more effective strategies for managing exacerbations.
特发性肺纤维化（IPF）是一种进行性和使人虚弱的肺部疾病，其特征是肺部不可逆转的瘢痕形成。IPF 的病因不明，但据认为涉及遗传和环境因素的综合作用。目前尚无治愈 IPF 的方法，治疗主要集中在减缓疾病进展和缓解症状上。
在本次综述中，我们旨在调查并提供 IPF 管理方式的最新见解，包括 Saracatinib 作为现有 IPF 药物替代品的潜力。我们还研究了 Sotatercept 在治疗与 IPF 相关的肺动脉高压方面的治疗潜力。
我们对 IPF 管理的相关研究进行了全面的文献综述。我们检索了电子数据库，包括 PubMed、Scopus、Embase 和 Web of Science。
两种经美国食品和药物管理局批准用于 IPF 的药物，Pirfenidone 和 Nintedanib，在减缓疾病进展方面发挥了关键作用，但实验证据表明 Saracatinib 的疗效优于它们。研究 Saracatinib（一种酪氨酸激酶抑制剂）潜力的临床前试验表明，在临床前研究中，它在减缓疾病进展方面比现有的 IPF 药物更有效。此外，Sotatercept，一种融合蛋白，在临床试验中已被证明可降低与 IPF 相关的 PH 患者的肺血管阻力并提高运动耐量。
本综述中讨论的进展有望改善 IPF 患者的生活质量，并增强我们对这种疾病的理解。仍需要进一步的研究来确认新的 IPF 治疗方法的疗效和安全性，并制定更有效的策略来管理加重期。
