• Transplant. Proc. · Jul 2019

    Differences Between Patients With Idiopathic Pleuroparenchymal Fibroelastosis and Those With Other Types of Idiopathic Interstitial Pneumonia in Candidates for Lung Transplants.

    • Haruhiko Shiiya, Dong Tian, Masaaki Sato, Takahiro Karasaki, Kentaro Kitano, Kazuhiro Nagayama, Masaki Anraku, Kichizo Kaga, Yoshiro Matsui, and Jun Nakajima.
    • Department of Thoracic Surgery, The University of Tokyo Graduate School of Medicine, Tokyo, Japan; Department of Cardiovascular and Thoracic Surgery, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
    • Transplant. Proc. 2019 Jul 1; 51 (6): 2014-2021.

    IntroductionThe prognostic implications of having patients with idiopathic pleuroparenchymal fibroelastosis (IPPFE) on lung transplantation waiting lists have been unclear. In Japan, where a severe shortage of brain-dead donors remains a major limitation for organ transplantation, it is particularly important to predict the prognoses of patients when they are listed for transplantation. The purpose of this study was to investigate the characteristics of lung transplantation candidates with IPPFE and the influence of those characteristics on prognosis.MethodsThis was a retrospective review of 29 consecutive adult lung transplant candidates with idiopathic interstitial pneumonia between January 2014 and April 2018.ResultsEight patients with IPPFE and 21 with other types of idiopathic interstitial pneumonia were included. Body mass index (median 17.1 kg/m2 vs 23.5 kg/m2, P < .01) and ratio of anteroposterior to transverse diameter of the thoracic cage were significantly lower (0.530 vs 0.583, P = .02) in the IPPFE group. Patients with a body mass index <20.0 kg/m2 (P = .02), 6-minute walk distance <250.0 m (P < .01), ratio of PaO2 to fraction of inspiratory oxygen <300.0 mm Hg (P < .01), and an inability to perform the diffusing capacity of carbon monoxide test (P < .01) had significantly shorter survival times in the other idiopathic interstitial pneumonia, but not in the IPPFE, group. Some patients with IPPFE survived for long enough to undergo transplantation.ConclusionsPatients with IPPFE waiting for transplantation have some distinctive characteristics and should be retained on waiting lists to receive transplants.Copyright © 2019. Published by Elsevier Inc.

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