Treatment with antifibrotic agents in idiopathic pleuroparenchymal fibroelastosis with usual interstitial pneumonia.
Journal
ERJ open research
ISSN: 2312-0541
Titre abrégé: ERJ Open Res
Pays: England
ID NLM: 101671641
Informations de publication
Date de publication:
Jan 2021
Jan 2021
Historique:
received:
19
04
2020
accepted:
05
11
2020
entrez:
8
3
2021
pubmed:
9
3
2021
medline:
9
3
2021
Statut:
epublish
Résumé
There are no established therapeutic options available for idiopathic pleuroparenchymal fibroelastosis (IPPFE) apart from supportive care and lung transplantation. Furthermore, it is known that IPPFE with a usual interstitial pneumonia (UIP) pattern and lower lobe predominance is a disease entity distinct from idiopathic pulmonary fibrosis (IPF). To our knowledge, few studies are available that report on the efficacy of antifibrotic agents for IPPFE with UIP. The aim of this study was to compare the efficacy of antifibrotic agents between IPPFE with UIP and typical IPF in real-world clinical practice. A retrospective analysis was performed on the medical records of all patients at two interstitial lung disease centres. Sixty-four patients were diagnosed as having IPPFE with UIP and 195 patients were diagnosed with typical IPF. We compared the efficacy of antifibrotic agents between these two groups. Survival time was significantly shorter in the patients with IPPFE with UIP. Some 125 patients were administered antifibrotic agents for over 6 months (34 with IPPFE with UIP and 91 with typical IPF). Reduced forced vital capacity (FVC) 6 months after treatment with antifibrotic agents was significantly greater in the IPPFE with UIP group than in those in the typical IPF group. Moreover, the change in % predicted FVC was significantly greater during the follow-up in patients with IPPFE with UIP compared with those with typical IPF. The efficacy of antifibrotic agents was limited in patients with IPPFE with UIP. Thus, IPPFE with UIP remains a fatal and progressive disease.
Sections du résumé
BACKGROUND
BACKGROUND
There are no established therapeutic options available for idiopathic pleuroparenchymal fibroelastosis (IPPFE) apart from supportive care and lung transplantation. Furthermore, it is known that IPPFE with a usual interstitial pneumonia (UIP) pattern and lower lobe predominance is a disease entity distinct from idiopathic pulmonary fibrosis (IPF). To our knowledge, few studies are available that report on the efficacy of antifibrotic agents for IPPFE with UIP.
AIM
OBJECTIVE
The aim of this study was to compare the efficacy of antifibrotic agents between IPPFE with UIP and typical IPF in real-world clinical practice.
PATIENTS AND METHODS
METHODS
A retrospective analysis was performed on the medical records of all patients at two interstitial lung disease centres. Sixty-four patients were diagnosed as having IPPFE with UIP and 195 patients were diagnosed with typical IPF. We compared the efficacy of antifibrotic agents between these two groups.
RESULTS
RESULTS
Survival time was significantly shorter in the patients with IPPFE with UIP. Some 125 patients were administered antifibrotic agents for over 6 months (34 with IPPFE with UIP and 91 with typical IPF). Reduced forced vital capacity (FVC) 6 months after treatment with antifibrotic agents was significantly greater in the IPPFE with UIP group than in those in the typical IPF group. Moreover, the change in % predicted FVC was significantly greater during the follow-up in patients with IPPFE with UIP compared with those with typical IPF.
CONCLUSIONS
CONCLUSIONS
The efficacy of antifibrotic agents was limited in patients with IPPFE with UIP. Thus, IPPFE with UIP remains a fatal and progressive disease.
Identifiants
pubmed: 33681342
doi: 10.1183/23120541.00196-2020
pii: 00196-2020
pmc: PMC7917230
pii:
doi:
Types de publication
Journal Article
Langues
eng
Informations de copyright
Copyright ©ERS 2021.
Déclaration de conflit d'intérêts
Conflict of interest: K. Sugino reports a lecture fee from Nippon Boehringer Ingelheim Co., Ltd., outside the submitted work. Conflict of interest: H. Ono has nothing to disclose. Conflict of interest: H. Shimizu has nothing to disclose. Conflict of interest: T. Kurosawa has nothing to disclose. Conflict of interest: K. Matsumoto has nothing to disclose. Conflict of interest: M. Ando has nothing to disclose. Conflict of interest: K. Mori has nothing to disclose. Conflict of interest: E. Tsuboi has nothing to disclose. Conflict of interest: S. Homma reports a lecture fee from Nippon Boehringer Ingelheim Co., Ltd., outside the submitted work; and is a member of an endowed department sponsored by Teijin Pharma, Co., Ltd, Nippon Boehringer Ingelheim, Co., Ltd, Shionogi & Co., Ltd, Chugai Pharmaceutical Co., Ltd, and Asahi Kasei Pharma Co., Ltd. Conflict of interest: K. Kishi reports a lecture fee from Nippon Boehringer Ingelheim Co., Ltd., outside the submitted work.
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