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 VO 7 IS 1 A1 Sugino, Keishi A1 Ono, Hirotaka A1 Shimizu, Hiroshige A1 Kurosawa, Takeyuki A1 Matsumoto, Keiko A1 Ando, Masahiro A1 Mori, Kiyoshi A1 Tsuboi, Eiyasu A1 Homma, Sakae A1 Kishi, Kazuma YR 2021 UL http://openres.ersjournals.com/content/7/1/00196-2020.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 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 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 The efficacy of antifibrotic agents was limited in patients with IPPFE with UIP. Thus, IPPFE with UIP remains a fatal and progressive disease.This study demonstrated that patients with IPPFE with UIP have a poorer prognosis than those with typical IPF because these patients were intractable to treatment with antifibrotic agents https://bit.ly/38LbAKD