Abstract
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 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 A retrospective analysis was performed on the medical records of all patients at 2 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 2 groups.
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. Moreover, the change in FVC% predicted was significantly greater during the follow-up in patients with IPPFE with UIP compared with those with typical IPF.
Conclusions The efficacy of antifibrotic agents was limited in patients with IPPFE with UIP. Thus, IPPFE with UIP remains a fatal and progressive disease.
Footnotes
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Conflict of interest: Dr. Sugino reports personal fees from Lecture fee from Nippon Boehringer Ingelheim Co.,Ltd., outside the submitted work;.
Conflict of interest: Dr. Ono has nothing to disclose.
Conflict of interest: Dr. Kurosawa has nothing to disclose.
Conflict of interest: Dr. Matsumoto has nothing to disclose.
Conflict of interest: Dr. Ando has nothing to disclose.
Conflict of interest: Dr. Mori has nothing to disclose.
Conflict of interest: Dr. Tsuboi has nothing to disclose.
Conflict of interest: Dr. Homma reports personal fees from Lecture fee from Nippon Boehringer Ingelheim Co.,Ltd., outside the submitted work;. Dr. Homma reports personal fees from Lecture fee from Nippon Boehringer Ingelheim Co.,Ltd., outside the submitted work; and I am 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: Dr. Kishi reports personal fees from Nippon Boehringer Ingelheim Co.,Ltd., outside the submitted work;.
- Received April 19, 2020.
- Accepted November 5, 2020.
- Copyright ©ERS 2020
This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.
