Abstract
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 in iPPFE patients was less than half that of 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 (HR 0.939). A composite scoring model, including age, sex and standardised upper-lobe volume, better predicted risk of death than the gender-age-physiology model.
Conclusion Assessment of upper-lobe volume provides useful information for managing iPPFE by evaluating disease severity and mortality risk in clinical practice.
Abstract
This novel method for quantitative measurements of upper lung lobe volume using standardised three-dimensional computed tomography is useful for assessing disease severity and mortality risk in patients with idiopathic pleuroparenchymal fibroelastosis https://bit.ly/3bHlOPD
Footnotes
Data availability statement: The data that support the findings of this study are available from the corresponding authors upon reasonable request.
This article has an editorial commentary: https://doi.org/10.1183/13993003.01798-2022
Author contributions: A. Fukada: conception and design, data collection, data analysis and interpretation, manuscript writing. Y. Suzuki: conception and design, data collection, data analysis and interpretation, manuscript writing, final approval of the manuscript. K. Mori and Y. Fujino: statistical analysis and interpretation, manuscript writing. M. Kono, H. Hasegawa, D. Hashimoto, K. Yokomura and S. Imokawa: conception and design, data collection, data analysis. Y. Tanaka, Y. Inoue, H. Hozumi, M. Karayama, K. Furuhashi, N. Enomoto, T. Fujisawa, Y. Nakamura, N. Inui and H. Nakamura: data collection, data analysis, supervision. T. Suda: conception and design, manuscript writing, administrative support.
Conflicts of interest: The authors declare that no competing interests exist.
Support statement: This work was supported by a grant-in-aid for scientific research from the Japan Society for the Promotion of Science (grant numbers 19K17632 and 22K08279, received by Y. Suzuki). Funding information for this article has been deposited with the Crossref Funder Registry.
- Received December 20, 2021.
- Accepted June 28, 2022.
- Copyright ©The authors 2022. For reproduction rights and permissions contact permissions{at}ersnet.org