Efficacy of early antifibrotic treatment for idiopathic pulmonary fibrosis

Keishi Sugino,Natsumi Watanabe,Eiyasu Tsuboi,Kazuma Kishi,Masahiro Ando,Sakae Homma,Hirotaka Ono

doi:10.1186/s12890-021-01595-3

Keishi Sugino, Natsumi Watanabe + Show 5 more

Open Access

https://doi.org/10.1186/s12890-021-01595-3

Copy DOI

Abstract

BackgroundAlthough antifibrotic drugs, including nintedanib and pirfenidone, slow the progression of idiopathic pulmonary fibrosis (IPF), there is little data about the timing of start of antifibrotic treatment in real-world clinical practice. The present study aimed to clarify the efficacy of nintedanib and pirfenidone in patients with early-stage IPF.MethodsWe compared survival and disease progression between patients with IPF with Japanese Respiratory Society (JRS) disease severity system stage I with and without oxygen desaturation on the 6-min walk test (6MWT) and increased the gender–age–physiology (GAP) staging. We examined the efficacy of antifibrotic drugs in patients with early-stage IPF.ResultsThe severity of stage I IPF (n = 179) according to the JRS criteria consisted of the following GAP staging criteria: stage I, 111 cases; stage II, 58 cases; stage III, 10 cases. The duration from the initial visit to disease progression and survival time was significantly shorter in JRS stage I patients with oxygen desaturation on the 6MWT or with increased GAP staging (unfavorable group) compared with patients without those factors. In the unfavorable group, the relative decline in percentage predicted forced vital capacity (%FVC) over 6 months was significantly lower in patients undergoing antifibrotic treatment compared with non-treated patients.ConclusionAntifibrotic drugs have a beneficial effect on the decline in %FVC in Japanese patients with early-stage IPF who have oxygen desaturation on the 6MWT or increased GAP staging.

Highlights

Idiopathic pulmonary fibrosis (IPF) is a fatal and progressive lung disease of undetermined etiology that has a heterogeneous clinical course
In Japan, idiopathic pulmonary fibrosis (IPF) disease severity is assessed using the Japanese Respiratory Society (JRS) disease severity system, which is based on the partial pressure of oxygen in arterial blood (PaO2) at rest and the presence of oxygen
Combined pulmonary fibrosis and emphysema (CPFE) was defined as concomitant IPF and ≥ 10% emphysema on chest high-resolution computed tomography (HRCT) by criteria proposed by Ryerson, et al [15]

Summary

Introduction

Idiopathic pulmonary fibrosis (IPF) is a fatal and progressive lung disease of undetermined etiology that has a heterogeneous clinical course. There are no cures and few treatment options for patients with IPF, nintedanib and pirfenidone significantly reduce the decline in forced vital capacity (FVC) and have been approved for the treatment of IPF [3, 4]. A post-hoc analysis of pooled date from the INPULSIS trials suggested that nintedanib had a similar beneficial effect on the rate of decline in FVC in patients with GAP stage I versus patients with GAP stage II/III at baseline [13]. Antifibrotic drugs, including nintedanib and pirfenidone, slow the progression of idiopathic pulmonary fibrosis (IPF), there is little data about the timing of start of antifibrotic treatment in real-world clinical practice.

Objectives

Methods

Results

Conclusion