A Comparison of the Effectiveness of Nintedanib and Pirfenidone in Treating Idiopathic Pulmonary Fibrosis: A Systematic Review

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.

Keywords: dlco; dyspnoea; dysregulated immune response; forced vital capacity; idiopathic pulmonary fibrosis; interstitial lung disease; lung repair mechanism; nintedanib; pirfenidone.

Figure 1. Keyword frequency across all databases collected between 2018 and 2023

ScienceDirect is the world's leading source for scientific, technical, and medical research; idiopathic pulmonary fibrosis (IPF); the New England Journal of Medicine (NEJM); PubMed comprises more than 36 million citations for biomedical literature, life science journals, and online books.

Figure 2. Displays the PRISMA flowchart of the literature and the study's search methodology

Number (n); Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA); Cochrane Collaboration Risk of Bias Tool (CCRBT); ScienceDirect is the world's leading source for scientific, technical, and medical research; idiopathic pulmonary fibrosis (IPF); the New England Journal of Medicine (NEJM); PubMed comprises more than 36 million citations for biomedical literature, life science journals, and online books.