RT Journal Article
SR Electronic
T1 Idiopathic pleuroparenchymal fibroelastosis: 3-D CT assessment of upper lobe lung volume
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 2200637
DO 10.1183/13993003.00637-2022
A1 Atsuki Fukada
A1 Yuzo Suzuki
A1 Kazutaka Mori
A1 Masato Kono
A1 Hirotsugu Hasegawa
A1 Dai Hashimoto
A1 Koshi Yokomura
A1 Shiro Imokawa
A1 Yuko Tanaka
A1 Yusuke Inoue
A1 Hironao Hozumi
A1 Masato Karayama
A1 Kazuki Furuhashi
A1 Noriyuki Enomoto
A1 Tomoyuki Fujisawa
A1 Yutaro Nakamura
A1 Naoki Inui
A1 Yoshihisa Fujino
A1 Hidenori Nakamura
A1 Takafumi Suda
YR 2022
UL http://erj.ersjournals.com/content/early/2022/06/21/13993003.00637-2022.abstract
AB Background Idiopathic pleuroparenchymal fibroelastosis (iPPFE) is a rare interstitial lung disease characterised by predominant upper lobe fibrosis involving the pleura and subpleural lung parenchyma. Despite its poor prognosis, there is no consensus on prognostic determinants of iPPFE to date. Because volume loss in the upper lobe is a distinct feature of iPPFE, we hypothesised that the lung volume of the bilateral upper lobes (upper lobe-volume) accurately indicates disease severity and mortality risk in iPPFE patients.Methods This retrospective study assessed two cohorts of 132 patients with iPPFE (69 in Hamamatsu cohort; 63 in Seirei cohort) and 45 controls. Each lobe volume was quantitatively measured using three-dimensional computed tomography at the time of iPPFE diagnosis and standardised using predicted forced vital capacity.Results The standardised upper lobe-volume was less than twice as small in iPPFE patients versus controls, whereas the lower lobe-volume did not decrease. iPPFE patients with lower standardised upper lobe-volume had significantly shorter survival rates than those with higher volume (median survival: 6.08 versus 2.48 years, p<0.001). In multivariate analysis, the lower standardised upper lobe-volume was significantly associated with increased mortality adjusting for age, sex and forced vital capacity [hazard ratio: 0.939].A composite scoring model, including age, sex and standardised upper lobe volume, was associated with a better risk of death than the gender-age-physiology (GAP) model.Conclusion Assessment of upper lobe-volume provides useful information for managing iPPFE by evaluating disease severity and mortality risk in clinical practice.FootnotesThis manuscript has recently been accepted for publication in the European Respiratory Journal. 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 ERJ online. Please open or download the PDF to view this article.Conflicts of interest: The authors declare that no competing interests exist.