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

Keishi Sugino; Hirotaka Ono; Hiroshige Shimizu; Takeyuki Kurosawa; Keiko Matsumoto; Masahiro Ando; Kiyoshi Mori; Eiyasu Tsuboi; Sakae Homma; Kazuma Kishi

doi:10.1183/23120541.00196-2020

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

ERJ Open Res. 2021 Mar 1;7(1):00196-2020. doi: 10.1183/23120541.00196-2020. eCollection 2021 Jan.

Authors

Affiliations

¹ Dept of Respiratory Medicine, Tsuboi Hospital, Koriyama city, Fukushima, Japan.
² Dept of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan.
³ Dept of Radiology, Toho University Omori Medical Center, Tokyo, Japan.
⁴ Dept of Advanced and Integrated Interstitial Lung Diseases Research, School of Medicine, Toho University, Tokyo, Japan.

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 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.