Prognostic factors and outcomes in Japanese lung transplant candidates with interstitial lung disease.

Kohei Ikezoe,Takeshi Kubo,Toyohiro Hirai,Yuko Yamamoto,Toyofumi F Chen-Yoshikawa,Yoshinari Nakatsuka,Sonoko Nagai,Kazuo Chin,Hideki Motoyama,Kiminobu Tanizawa,Akihiro Aoyama,Tomohiro Handa,Kyoko Hijiya,Shinsaku Tokuda,Shin-Ichi Harashima,Ayako Oshima,Hiroshi Date,Vinicio A De Jesus Perez

doi:10.1371/journal.pone.0183171

Kohei Ikezoe, Takeshi Kubo + Show 16 more

Open Access

https://doi.org/10.1371/journal.pone.0183171

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Abstract

ObjectiveYoung patients with advanced interstitial lung disease (ILD) are potential candidates for cadaveric lung transplantation. This study aimed to examine clinical features, outcomes, and prognostic factors in Japanese ILD patients awaiting lung transplantation.MethodsWe investigated the clinical features and outcomes of 77 consecutive candidates with ILD who were referred to Kyoto University Hospital and subsequently actively listed for lung transplant in the Japan Organ Transplant Network between 2010 and 2014.ResultsOf the 77 candidates, 33 had idiopathic pulmonary fibrosis (IPF) and 15 had unclassifiable ILD. During the observational period, 23 patients (30%) received lung transplantations and 49 patients (64%) died before transplantation. Of the 33 patients with IPF, 13 (39%) had a family history of ILD and 13 (39%) had an “inconsistent with usual interstitial pneumonia pattern” on high-resolution computed tomography (HRCT). The median survival time from registration was 16.7 months, and mortality was similar among patients with IPF, unclassifiable ILD, and other ILDs. Using a multivariate stepwise Cox proportional hazards model, 6-min walking distance was shown to be an independent prognostic factor in candidates with ILD (per 10 m, hazard ratio (HR): 0.97; 95% confidence interval (CI): 0.95–0.99, p<0.01), while lower body mass index (HR: 0.83; 95% CI: 0.72–0.95, p < 0.01) independently contributed to mortality in patients with IPF.ConclusionsJapanese patients with ILD awaiting transplantation had very poor outcomes regardless of their specific diagnosis. A substantial percentage of IPF patients had an atypical HRCT pattern. 6-min walking distance in ILD patients and body mass index in IPF patients were independent predictors of mortality.

Highlights

Interstitial lung disease (ILD) is difficult to cure and its prognosis is often poor
The median survival time from registration was 16.7 months, and mortality was similar among patients with idiopathic pulmonary fibrosis (IPF), unclassifiable ILD, and other ILDs
Using a multivariate stepwise Cox proportional hazards model, 6-min walking distance was shown to be an independent prognostic factor in candidates with ILD (per 10 m, hazard ratio (HR): 0.97; 95% confidence interval (CI): 0.95–0.99, p

Summary

Introduction

Interstitial lung disease (ILD) is difficult to cure and its prognosis is often poor. Patients with other ILDs, such as idiopathic nonspecific interstitial pneumonia (NSIP), connective tissue disease-associated ILD (CTDILD), and chronic hypersensitivity pneumonitis (CHP), sometimes have progressive disease, their prognosis is usually more favorable than that of IPF [2,3,4]. As the number of cadaveric lung transplantation in Japan is limited, the average waiting time is more than 800 days [7, 8]. Lung allocation is performed based on accrued time on the waiting list, and there is no rule to prioritize patients with more rapidly progressing diseases such as IPF. That Japanese lung transplant candidates with ILD may have poor outcome

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Conclusion