• Ji Eun Park, Song Yee Kim, Joo Han Song, Young Sam Kim, Joon Chang, Jin Gu Lee, Hyo Chae Paik, and Moo Suk Park.
• Division of Pulmonology, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea.
• J Thorac Dis. 2018 Mar 1; 10 (3): 1538-1547.

BackgroundPulmonary involvement is common in connective tissue disease (CTD), and respiratory failure is a major cause of morbidity and mortality in CTD-related interstitial lung disease (CTD-ILD). Lung transplantation is thus important for these patients. However, survival, outcomes, and management of these patients after transplantation have been debated. The aim of this study was to evaluate the outcomes for CTD-ILD compared to those for idiopathic pulmonary fibrosis (IPF) after lung transplantation.MethodsWe performed a single-centre retrospective study of 62 patients with CTD-ILD or IPF who underwent lung transplantation at a tertiary hospital in South Korea between October 2012 and October 2016.ResultsPatients with CTD-ILD (n=15) were younger (46 vs. 60 years, P=0.001) and were less likely to be male (33.3% vs. 76.6%, P=0.004) than were patients with IPF (n=47). The 1-year cumulative survival rate was 80.0% for CTD-ILD and 59.6% for IPF (log-rank P=0.394). There was no difference in the cumulative survival rate (log-rank P=0.613) of age- and sex-matched patients with CTD-ILD (n=15) and IPF (n=15). The incidence of primary graft dysfunction was similar (P=0.154), and 2 (18.2%) patients developed possible CTD flare.ConclusionsPatients with CTD-ILD and those with IPF who underwent lung transplantation had similar survival rates.

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