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Idiopathic pleuroparenchymal fibroelastosis: 3-D CT assessment of upper lobe lung volume

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Jumat, 08 Jul 2022 14:33:23

Atsuki Fukada, Yuzo Suzuki, Kazutaka Mori, Masato Kono, Hirotsugu Hasegawa, Dai Hashimoto, Koshi Yokomura, Shiro Imokawa, Yuko Tanaka, Yusuke Inoue, Hironao Hozumi, Masato Karayama, Kazuki Furuhashi, Noriyuki Enomoto, Tomoyuki Fujisawa, Yutaro Nakamura,Naoki Inui, Yoshihisa Fujino, Hidenori Nakamura, Takafumi Suda

European Respiratory Journal 2022; DOI: 10.1183/13993003.00637-2022

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 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.

Footnotes

This 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.