Acute exacerbation of chronic fibrosing interstitial pneumonia in patients receiving antifibrotic agents: incidence and risk factors from real-world experience

Kodai Kawamura,Keisuke Anan,Yuko Yasuda,Hidenori Ichiyasu,Takuro Sakagami,Kazuya Ichikado,Moritaka Suga

doi:10.1186/s12890-019-0880-0

Abstract

Background and objectiveHere, we present real-world data on the incidence and risk factors of acute exacerbation (AE) in patients with chronic fibrotic interstitial pneumonia (CFIP) treated with antifibrotic agents, which has been previously poorly documented.MethodsWe retrospectively examined clinical characteristics, incidence and risk factors of AE in a cohort of 100 patients with CFIP (n = 75, idiopathic pulmonary fibrosis [IPF]; n = 25, other conditions), all of whom received antifibrotic agents in a real-world setting.ResultsThe median follow-up was 17.4 months (interquartile range [IQR], 6.6 to 26.7 months). During the follow-up periods, 21 patients experienced AE after starting antifibrotic agents. The estimated 1-, 2-, and 3-year AE incidence rates were 11.4% (95% confidence interval [95%CI], 6.2–20.3%), 32% (95%CI, 20.7–47.4%), and 36.3% (95%CI 23.5–53.1%), respectively. Decreased baseline lung function (forced vital capacity and carbon monoxide diffusing capacity of the lung), existence of pulmonary hypertension estimated from an echocardiogram, higher Interstitial Lung Disease-Gender, Age, and Physiology (ILD-GAP) score, supplementary oxygen, and concomitant corticosteroid and proton-pump inhibitor (PPI) use upon starting the antifibrotic agent were risk factors of AE. Concomitant corticosteroid and PPI use and corticosteroid dose were risk factor of AE in a multivariate Cox regression hazard model adjusting for ILD-GAP score.ConclusionAE of CFIP is more common in patients with physiologically and functionally advanced disease under antifibrotic agents. Prudent use of corticosteroids and PPIs when initiating antifibrotic agents may be recommended. Further studies are warranted.

Highlights

Chronic fibrotic interstitial pneumonia (CFIP) is associated with substantially reduced health-related quality of life and survival
Patients Patients with progressive chronic fibrotic interstitial pneumonia (CFIP) treated with antifibrotic agents from 01 Aug 2015 to 31 Aug 2018 with no history of acute exacerbation (AE) were identified at Saiseikai Kumamoto Hospital
Patients who had been diagnosed as not-Idiopathic pulmonary fibrosis (IPF) before commencing anti-fibrotic agents were more frequently prescribed corticosteroids than those with an IPF diagnosis at the time of starting anti-fibrotic agents (IPF n = 15 [20%] vs. non-IPF n = 11 [44%]; p = 0.033)

Summary

Introduction

Chronic fibrotic interstitial pneumonia (CFIP) is associated with substantially reduced health-related quality of life and survival. The disease course of progressive CFIPs, such as IPF, is variable and unpredictable; the median survival time after IPF diagnosis is 3–5 years [1,2,3]. Antifibrotic therapy can slow declining lung function [5, 6] and reduce the risk of death from IPF [6,7,8], but it cannot stop the disease course. International guidelines recommend using antifibrotic agents for IPF patients [9]. We present real-world data on the incidence and risk factors of acute exacerbation (AE) in patients with chronic fibrotic interstitial pneumonia (CFIP) treated with antifibrotic agents, which has been previously poorly documented

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Results

Conclusion