RT Journal Article SR Electronic T1 Treatment with antifibrotic agents in idiopathic pleuroparenchymal fibroelastosis with usual interstitial pneumonia JF ERJ Open Research JO erjor FD European Respiratory Society SP 00196-2020 DO 10.1183/23120541.00196-2020 A1 Keishi Sugino A1 Hirotaka Ono A1 Shigeru Shimizu A1 Takeyuki Kurosawa A1 Keiko Matsumoto A1 Masahiro Ando A1 Kiyoshi Mori A1 Eiyasu Tsuboi A1 Sakae Homma A1 Kazuma Kishi YR 2020 UL http://openres.ersjournals.com/content/early/2020/11/12/23120541.00196-2020.abstract AB 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.FootnotesThis manuscript has recently been accepted for publication in the ERJ Open Research. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJOR online. Please open or download the PDF to view this article.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;.