PDF(Pubmed)
Abstract：
Idiopathic pulmonary fibrosis (IPF), which shares a radiographic pattern with the usual interstitial pneumonia (UIP), is a specific form of chronic and progressive interstitial lung disorder resulting in persistent fibrosis and impaired lung function. Most of the patients suffer from dyspnea which adversely affects health-related quality of life (HRQOL). The underlying etiology of the disease is not yet understood, but research done on the subject reveals that aberrant repair mechanisms and dysregulated immune responses may be the cause. It can affect any age group but predominantly affects patients who are above 50 years of age. It has been observed that in addition to age, the reasons are also related to smoking, pollution, and inhalation of harmful elements. As the cause of IPF is still unknown and there is no cure yet, presently, it is treated to delay lung function loss with antifibrotic medications, nintedanib, and pirfenidone. However, both nintedanib and perfenidone have side effects which affect different patients in different ways and with different levels of severity, thereby making the treatment even more challenging for medical practitioners. The present systematic review aims at studying the efficacy of pirfenidone and nintedanib in relieving symptoms and in extending survival in patients. A detailed search was done in relevant articles listed in PubMed, ScienceDirect, and the New England Journal of Medicine between 2018 and 2023. It was observed that the most accepted way of measuring the progression of IPF is the evaluation of pulmonary function by assessing the forced vital capacity (FVC). Several studies have shown that the decline in FVC over a period of 6-12 months is directly associated with a higher mortality rate. The outcomes were similar in both male and female irrespective of age, gender, and ethnicity. However, some patients being treated with pirfenidone and nintedanib experienced various side-effects which were mainly gastrointestinal like diarrhea, dyspepsia, and vomiting. In the case of pirfenidone, some patients also experienced photosensitivity and skin rashes. In cases where the side-effects are extremely severe and are more threatening than the disease itself, the treatment has to be discontinued. The survival rate in patients with IPF is marked by a median of 3-5 years that is even lower than many cancers; hence, the treatment should be started as soon as the disease is detected. However, further research is needed to establish the etiology of IPF and to establish treatments that can stop its progression.
摘要：
特发性肺纤维化(IPF),与通常的间质性肺炎（UIP）具有放射学特征，是导致持续纤维化和肺功能受损的慢性和进行性间质性肺病的特定形式。大多数患者患有呼吸困难，这会对健康相关的生活质量（HRQOL）产生不利影响。该病的潜在病因尚不清楚,但是对该主题的研究表明，异常的修复机制和失调的免疫反应可能是原因。它可以影响任何年龄组，但主要影响50岁以上的患者。据观察，除了年龄，原因也与吸烟有关，污染,吸入有害元素。由于IPF的病因尚不清楚，目前尚无治愈方法，目前,抗纤维化药物治疗可以延缓肺功能丧失，Nintedanib,还有吡非尼酮.然而,尼达尼布和perfenidone都有副作用，以不同的方式和不同的严重程度影响不同的患者，从而使治疗对医生来说更具挑战性。本系统综述旨在研究吡非尼酮和尼达尼布缓解症状和延长患者生存期的疗效。在PubMed中列出的相关文章中进行了详细的搜索，ScienceDirect,以及2018年至2023年的《新英格兰医学杂志》。观察到测量IPF进展的最可接受的方法是通过评估强迫肺活量（FVC）来评估肺功能。一些研究表明，FVC在6-12个月内的下降与较高的死亡率直接相关。无论年龄大小，男性和女性的结果相似，性别,和种族。然而,一些接受吡非尼酮和尼达尼布治疗的患者出现了各种副作用，主要是胃肠道样腹泻，消化不良,和呕吐。在吡非尼酮的情况下，一些患者还出现了光敏性和皮疹。如果副作用极其严重，比疾病本身更具威胁性，必须停止治疗。IPF患者的生存率中位数为3-5年，甚至低于许多癌症；因此，一旦发现疾病，就应该开始治疗。然而,需要进一步的研究来确定IPF的病因并建立可以阻止其进展的治疗方法.
