Man Ruzhual K, Gogikar Amaresh, Nanda Ankita, Janga Lakshmi Sai Niharika, Sambe Hembashima G, Yasir Mohamed, Ramphall Shivana
Research, California Institute of Behavioral Neurosciences and Psychology, Fairfield, USA.
Cureus. 2024 Feb 15;16(2):e54268. doi: 10.7759/cureus.54268. eCollection 2024 Feb.
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.
特发性肺纤维化(IPF)与普通间质性肺炎(UIP)具有相同的影像学表现,是一种慢性进行性间质性肺疾病的特殊形式,可导致持续性纤维化和肺功能受损。大多数患者会出现呼吸困难,这对健康相关生活质量(HRQOL)产生不利影响。该病的潜在病因尚不清楚,但对此进行的研究表明,异常的修复机制和失调的免疫反应可能是病因。它可影响任何年龄组,但主要影响50岁以上的患者。据观察,除年龄外,其原因还与吸烟、污染和吸入有害元素有关。由于IPF的病因仍然未知且尚无治愈方法,目前,通过使用抗纤维化药物、尼达尼布和吡非尼酮来治疗,以延缓肺功能丧失。然而,尼达尼布和吡非尼酮都有副作用,这些副作用以不同方式、不同严重程度影响不同患者,从而使治疗对医生来说更具挑战性。本系统评价旨在研究吡非尼酮和尼达尼布在缓解症状和延长患者生存期方面的疗效。对2018年至2023年期间发表在PubMed、ScienceDirect和《新英格兰医学杂志》上的相关文章进行了详细检索。据观察,评估IPF进展最常用的方法是通过评估用力肺活量(FVC)来评估肺功能。多项研究表明,在6至12个月的时间内FVC下降与较高的死亡率直接相关。无论年龄、性别和种族如何,男性和女性的结果相似。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,主要是胃肠道方面的,如腹泻、消化不良和呕吐。就吡非尼酮而言,一些患者还出现了光敏反应和皮疹。在副作用极其严重且比疾病本身更具威胁性的情况下,必须停止治疗。IPF患者的生存率中位数为3至五年,甚至低于许多癌症;因此,一旦发现疾病就应立即开始治疗。然而,需要进一步研究以确定IPF的病因,并建立能够阻止其进展的治疗方法。