Treatment with antifibrotic agents in idiopathic pleuroparenchymal fibroelastosis with usual interstitial pneumonia

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 diseas...

Full description

Bibliographic Details
Main Authors: Keishi Sugino, Hirotaka Ono, Hiroshige Shimizu, Takeyuki Kurosawa, Keiko Matsumoto, Masahiro Ando, Kiyoshi Mori, Eiyasu Tsuboi, Sakae Homma, Kazuma Kishi
Format: Article
Language:English
Published: European Respiratory Society 2021-03-01
Series:ERJ Open Research
Online Access:http://openres.ersjournals.com/content/7/1/00196-2020.full