Safety and efficacy of pirfenidone in severe Idiopathic Pulmonary Fibrosis: A real-world observational study

Tzouvelekis, Argyrios; Ntolios, Paschalis; Karampitsakos, Theodoros; Tzilas, Vasilios; Anevlavis, Stavros; Bouros, Evangelos; Steiropoulos, Paschalis; Koulouris, Nikolaos; Stratakos, Grigoris; Froudarakis, Marios

doi:10.1016/j.pupt.2017.08.011

Cited by 44 publications

(39 citation statements)

References 31 publications

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“…However, in these studies, the results of the advanced group were not analyzed separately or the definition of advanced stage was not based on lung function. A recent study suggested that in advanced IPF, defined as poor lung function (FVC < 50% predicted and/or DLco < 35% predicted), pirfenidone could improve lung function decline after treatment (ΔFVC [6 months]: –3.3 ± 4.6 [before] vs. 0.49 ± 11.4% predicted [after]; p = 0.06), which was consistent with our findings [22]. …”

Section: Discussionsupporting

confidence: 92%

“…Our study showed that pirfenidone could stabilize the lung function decline in patients with advanced IPF, similar to non-advanced patients. Previous studies support our findings [10-14, 22, 23]. In an open-label phase 2 study ( n = 54), Raghu et al[10] demonstrated that pirfenidone could stabilize or improve lung function (in 70.7% of patients at 6 months) in IPF patients, some of whom were at an advanced stage (FVC range, 26–108% predicted; DLco range, 8–104% predicted).…”

Section: Discussionsupporting

confidence: 89%

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Efficacy and Safety of Pirfenidone in Advanced Idiopathic Pulmonary Fibrosis

Yoon

Kim

Song³

2018

Respiration

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Background: Although phase 3 trials showed significant efficacy and acceptable safety profiles for pirfenidone in mild-to-moderate idiopathic pulmonary fibrosis (IPF), data on advanced IPF are limited. Objectives: The study aimed to evaluate the efficacy and safety of pirfenidone in advanced IPF patients. Methods: The clinical data of 138 IPF patients (advanced group: 27%) treated with pirfenidone were retrospectively reviewed and compared between advanced and non-advanced groups. Advanced IPF was defined as (1) forced vital capacity (FVC) < 50% predicted or (2) diffusing capacity for carbon monoxide < 30% predicted. Results: The mean treatment duration was 51.3 weeks, and lung function analysis was performed in 81 patients (17 in the advanced group). Changes in FVC and total lung capacity (TLC) were significantly reduced at 6 months after treatment in both the advanced (ΔFVC [6 months]: –6.3 [before] vs. 0.7% predicted [after]; ΔTLC: –5.3 vs. 0.8) and non-advanced (ΔFVC: –3.4 vs. 0.5; ΔTLC: –3.1 vs. –0.9) groups. The rate of decline in FVC and TLC was significant before treatment, but not after treatment in the advanced (FVC: –1.27 [before] vs. 0.21% predicted/month [after]; TLC: –0.89 vs. –0.15) and non-advanced (FVC: –0.60 vs. –0.20; TLC: –0.54 vs. –0.17) groups. The advanced group showed a similar rate of adverse events (AEs) (78.4 vs. 88.1%, p = 0.270), but more serious AEs (40.5 vs. 10.9%, p < 0.001) including death (24.3 vs. 5.0%, p = 0.002). Conclusions: In advanced IPF, pirfenidone showed similar efficacy and safety to non-advanced IPF except for serious AEs, which may be due to the advanced status itself.

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Section: Discussionsupporting

confidence: 92%

Section: Discussionsupporting

confidence: 89%

Efficacy and Safety of Pirfenidone in Advanced Idiopathic Pulmonary Fibrosis

Yoon

Kim

Song³

2018

Respiration

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“…Despite the results of RCTs and the published guidelines, the existing financial crisis and the shortage of resources in several public health systems worldwide pose obstacles to the clinical use of both novel agents. Furthermore, these agents have been recently approved, and there are few data available regarding their clinical use in the real-world setting [32-34]. Since cost has been recognized as a major issue in the application of public health policies, more data are needed regarding the clinical use of these agents [15].…”

Section: Introductionmentioning

confidence: 99%

“…In this setting, only sparse data regarding IPF epidemiology and its management in Greece are available [19, 32]. No registry for IPF has been conducted in Greece.…”

Section: Introductionmentioning

confidence: 99%

Design, Rationale, Methodology, and Aims of a Greek Prospective Idiopathic Pulmonary Fibrosis Registry: Investigating Idiopathic Pulmonary Fibrosis in Greece (INDULGE IPF)

et al. 2018

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Background: Idiopathic pulmonary fibrosis (IPF) is a chronic, fibrosing interstitial pneumonia of unknown origin. Despite the fact that the guidelines on the diagnosis and management of the disease were updated in 2015, incorporating novel agents recently introduced in the therapeutic approach of IPF, there is a lack of data on the epidemiology, disease status, and treatment in clinical practice. Contemporary data provided by national registries in IPF provide valuable information to guide clinical management of the disease in the real-world setting, adjusted to the local needs. Objective: Investigating Idiopathic Pulmonary Fibrosis in Greece (INDULGE IPF) is a Greek observational registry aiming at gaining further knowledge on the characteristics, management, progression, and outcomes of patients with IPF treated under real-world, clinical practice conditions in Greece. Methods: Approximately 300 patients will be enrolled consecutively in seven reference centers, constituting the largest IPF registry ever established in Greece. Conclusion: This registry is expected to provide data on the characteristics of IPF patients in Greece and the entire clinical management during the course of the disease.

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“…It was approved by the European Medicines Agency (EMA) in February 2011. In the last years, a lot of studies [1-6] were published reporting the efficacy and safety profile of pirfenidone encountered in several countries. Each study deserves particular attention since it can provide valuable information on the efficacy and safety of pirfenidone in everyday clinical practice, evolution in diagnostic procedures, and practical issues that may emerge.…”

mentioning

confidence: 99%