Letter to the Editor Open Access
Copyright ©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Oct 6, 2024; 12(28): 6247-6249
Published online Oct 6, 2024. doi: 10.12998/wjcc.v12.i28.6247
Early pirfenidone treatment enhances lung function in idiopathic pulmonary fibrosis patients
Jin-Wei Zhang, Medical School, University of Exeter, Exeter EX4 4PS, United Kingdom
ORCID number: Jin-Wei Zhang (0000-0001-8683-509X).
Author contributions: Zhang JW designed the overall concept and outline of the manuscript; Zhang JW contributed to the discussion and design of the manuscript; Zhang JW contributed to the writing and editing of the manuscript, illustrations, and review of the literature.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: Https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Jin-Wei Zhang, BSc, MSc, PhD, Academic Editor, Professor, Senior Editor, Medical School, University of Exeter, Stocker Rd, Exeter EX4 4PS, United Kingdom. j.zhang5@exeter.ac.uk
Received: June 29, 2024
Revised: July 25, 2024
Accepted: July 30, 2024
Published online: October 6, 2024
Processing time: 44 Days and 23.5 Hours

Abstract

This editorial comments on the study by Lei et al investigating the efficacy of early treatment with pirfenidone on the lung function of patients with idiopathic pulmonary fibrosis (IPF) published. This study evaluates the efficacy of early treatment with pirfenidone on lung function in patients with IPF. The early and advanced stages of IPF are defined, highlighting the drug's benefits. While prior research indicates pirfenidone's effectiveness in advanced IPF, this study focuses on its advantages in early stages. The study emphasizes the importance of computed tomography imaging alongside biochemical data and lung function tests for a comprehensive analysis of symptom relief. Results show that early intervention with pirfenidone significantly reduces disease progression and preserves lung function, underscoring its potential as a critical treatment strategy in early IPF.

Key Words: Idiopathic pulmonary fibrosis; Pirfenidone; Early intervention; Pulmonary function; Inflammatory markers

Core Tip: Early intervention with pirfenidone in idiopathic pulmonary fibrosis (IPF) patients significantly improves lung function, reduces inflammation, enhances physical endurance, and decreases adverse reactions. These findings underscore the potential of early pirfenidone treatment to alter the disease course and improve patient outcomes, emphasizing its importance in clinical management strategies for IPF.



TO THE EDITOR

Idiopathic pulmonary fibrosis (IPF) is a debilitating lung disease characterized by progressive scarring of lung tissue, leading to a decline in lung function and eventual respiratory failure[1]. Managing IPF remains challenging due to its unpredictable course and limited treatment options[2]. Pirfenidone, an antifibrotic agent, has shown promise in slowing disease progression[3]. Early-stage IPF is typically defined by a mild to moderate decline in lung function [forced vital capacity (FVC) ≥ 50% and diffusion capacity of the lung for carbon monoxide (DLCO) ≥ 35% predicted], while advanced-stage IPF involves more severe impairment (FVC < 50% and/or DLCO < 35% predicted). In the recent issue of the World Journal of Clinical Cases, Lei et al‘s study titled "Study on the Efficacy of Early Treatment with Pirfenidone on Lung Function of Idiopathic Pulmonary Fibrosis Patients" aims to assess the effects of early pirfenidone intervention on lung function and other clinical outcomes in IPF patients[4].

Methods

This retrospective study involved 113 IPF patients, divided into a control group and an observation group. The control group received routine therapy with methylprednisolone, while the observation group received routine therapy plus pirfenidone. The researchers assessed various parameters, including pulmonary function (FVC, DLCO), inflammatory markers [C-reactive protein (CRP), interleukin-2 (IL-2), interleukin-8 (IL-8)], adverse reactions, and the 6-minute walk distance (6MWD), as previously reported[5]. Data were analyzed using statistical methods to compare pre- and post-treatment values within and between groups.

Key findings

(1) Pulmonary function: Patients in the observation group showed a significant improvement in FVC and DLCO compared to the control group (P < 0.05); (2) Inflammatory markers: Levels of CRP, IL-2, and IL-8 were significantly lower in the observation group post-treatment, indicating reduced inflammation (P < 0.05); (3) Physical endurance: The 6MWD increased significantly in the observation group, suggesting enhanced physical endurance and functional capacity (P < 0.05); and (4) Adverse reactions: The occurrence of adverse reactions was lower in the observation group, highlighting the safety and tolerability of pirfenidone when administered early.

Comparisons with relevant studies

These findings align with previous studies demonstrating the benefits of pirfenidone in IPF treatment. For instance, the ASCEND and CAPACITY trials showed that pirfenidone reduces disease progression and preserves lung function[6-8]. However, these studies primarily focused on later stages of IPF. Whereas this current study uniquely emphasizes the benefits of early intervention, potentially offering a more substantial impact on patient outcomes.

In-depth discussion

The significant improvement in pulmonary function observed in the early intervention group underscores the potential of pirfenidone to alter the disease course when administered at the onset of IPF. Early treatment may prevent irreversible lung damage, thus preserving respiratory function longer[9]. The reduction in inflammatory markers suggests that pirfenidone not only slows fibrosis but also modulates underlying inflammatory processes. This dual action could be crucial in managing IPF, which involves both fibrotic and inflammatory components[10]. Additionally, computed tomography (CT) imaging data showed a reduction in fibrotic changes, supporting the biochemical and functional improvements observed. The increase in 6MWD reflects enhanced physical endurance and quality of life, crucial metrics in chronic respiratory diseases. This improvement indicates that patients can maintain better physical activity levels, which correlates with overall health and well-being[11]. Lei et al’s study also highlights the safety profile of pirfenidone in early-stage IPF[4]. Fewer adverse reactions in the observation group suggest that early administration might be better tolerated, potentially increasing patient adherence to the treatment regimen.

Despite these promising findings, the study's retrospective nature warrants cautious interpretation. Future prospective, randomized controlled trials are necessary to confirm these results and explore long-term outcomes of early pirfenidone intervention.

Future perspectives

The results of this study pave the way for further research into early intervention strategies for IPF. Future studies should focus on: (1) Long-term efficacy and safety of early pirfenidone treatment; (2) Comparative effectiveness of pirfenidone vs other antifibrotic agents in early-stage IPF; (3) Mechanistic studies to understand how pirfenidone modulates fibrosis and inflammation at the molecular level; and (4) Integration of biomarkers to identify patients who would benefit most from early intervention.

This study demonstrates that early intervention with pirfenidone in IPF patients significantly improves pulmonary function, reduces inflammation, enhances physical endurance, decreases adverse reactions, and is supported by favorable CT imaging changes. These findings suggest that early pirfenidone treatment could be a critical component in the management strategy for IPF, potentially altering the disease trajectory and improving patient outcomes. Further research is needed to confirm these findings and optimize early intervention protocols.

Footnotes

Provenance and peer review: Invited article; Externally peer reviewed.

Peer-review model: Single blind

Specialty type: Medicine, research and experimental

Country of origin: United Kingdom

Peer-review report’s classification

Scientific Quality: Grade C

Novelty: Grade C

Creativity or Innovation: Grade C

Scientific Significance: Grade C

P-Reviewer: Wang M S-Editor: Qu XL L-Editor: A P-Editor: Chen YX

References
1.  Lederer DJ, Martinez FJ. Idiopathic Pulmonary Fibrosis. N Engl J Med. 2018;378:1811-1823.  [PubMed]  [DOI]  [Cited in This Article: ]  [Cited by in Crossref: 849]  [Cited by in F6Publishing: 1098]  [Article Influence: 183.0]  [Reference Citation Analysis (0)]
2.  Bonella F, Spagnolo P, Ryerson C. Current and Future Treatment Landscape for Idiopathic Pulmonary Fibrosis. Drugs. 2023;83:1581-1593.  [PubMed]  [DOI]  [Cited in This Article: ]  [Cited by in F6Publishing: 13]  [Reference Citation Analysis (0)]
3.  Meyer KC, Decker CA. Role of pirfenidone in the management of pulmonary fibrosis. Ther Clin Risk Manag. 2017;13:427-437.  [PubMed]  [DOI]  [Cited in This Article: ]  [Cited by in Crossref: 27]  [Cited by in F6Publishing: 34]  [Article Influence: 4.9]  [Reference Citation Analysis (0)]
4.  Lei Y, Sheng J, Jin X, Liu X, Zheng X, Xu X. Study on the efficacy of early treatment with pirfenidone on the lung function of patients with idiopathic pulmonary fibrosis. World J Clin Cases. 2024;12:4913-4923.  [PubMed]  [DOI]  [Cited in This Article: ]  [Reference Citation Analysis (0)]
5.  Bonella F, Stowasser S, Wollin L. Idiopathic pulmonary fibrosis: current treatment options and critical appraisal of nintedanib. Drug Des Devel Ther. 2015;9:6407-6419.  [PubMed]  [DOI]  [Cited in This Article: ]  [Cited by in Crossref: 13]  [Cited by in F6Publishing: 28]  [Article Influence: 3.1]  [Reference Citation Analysis (0)]
6.  Noble PW, Albera C, Bradford WZ, Costabel U, Glassberg MK, Kardatzke D, King TE Jr, Lancaster L, Sahn SA, Szwarcberg J, Valeyre D, du Bois RM; CAPACITY Study Group. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011;377:1760-1769.  [PubMed]  [DOI]  [Cited in This Article: ]  [Cited by in Crossref: 1375]  [Cited by in F6Publishing: 1443]  [Article Influence: 111.0]  [Reference Citation Analysis (0)]
7.  King TE Jr, Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK, Gorina E, Hopkins PM, Kardatzke D, Lancaster L, Lederer DJ, Nathan SD, Pereira CA, Sahn SA, Sussman R, Swigris JJ, Noble PW; ASCEND Study Group. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370:2083-2092.  [PubMed]  [DOI]  [Cited in This Article: ]  [Cited by in Crossref: 2418]  [Cited by in F6Publishing: 2676]  [Article Influence: 267.6]  [Reference Citation Analysis (0)]
8.  Lancaster L, Albera C, Bradford WZ, Costabel U, du Bois RM, Fagan EA, Fishman RS, Glaspole I, Glassberg MK, King TE Jr, Lederer DJ, Lin Z, Nathan SD, Pereira CA, Swigris JJ, Valeyre D, Noble PW. Safety of pirfenidone in patients with idiopathic pulmonary fibrosis: integrated analysis of cumulative data from 5 clinical trials. BMJ Open Respir Res. 2016;3:e000105.  [PubMed]  [DOI]  [Cited in This Article: ]  [Cited by in Crossref: 70]  [Cited by in F6Publishing: 81]  [Article Influence: 10.1]  [Reference Citation Analysis (0)]
9.  Sgalla G, Iovene B, Calvello M, Ori M, Varone F, Richeldi L. Idiopathic pulmonary fibrosis: pathogenesis and management. Respir Res. 2018;19:32.  [PubMed]  [DOI]  [Cited in This Article: ]  [Cited by in Crossref: 212]  [Cited by in F6Publishing: 345]  [Article Influence: 57.5]  [Reference Citation Analysis (0)]
10.  Aimo A, Spitaleri G, Nieri D, Tavanti LM, Meschi C, Panichella G, Lupón J, Pistelli F, Carrozzi L, Bayes-Genis A, Emdin M. Pirfenidone for Idiopathic Pulmonary Fibrosis and Beyond. Card Fail Rev. 2022;8:e12.  [PubMed]  [DOI]  [Cited in This Article: ]  [Cited by in Crossref: 3]  [Cited by in F6Publishing: 17]  [Article Influence: 8.5]  [Reference Citation Analysis (0)]
11.  Li J, Li X, Deng M, Liang X, Wei H, Wu X. Features and predictive value of 6-min walk test outcomes in interstitial lung disease: an observation study using wearable monitors. BMJ Open. 2022;12:e055077.  [PubMed]  [DOI]  [Cited in This Article: ]  [Cited by in Crossref: 1]  [Reference Citation Analysis (0)]