Background Acute exacerbations of interstitial lung diseases (AE-ILD) have a high mortality rate with no effective medical therapies. Lung transplantation is a potentially life-saving option for patients with AE-ILD, but its role is not well established. The aim of this study is to determine if this therapy during AE-ILD significantly affects post-transplant outcomes in comparison to those transplanted with stable disease.
Methods We conducted a retrospective study of consecutive patients with AE-ILD admitted to our institution from 2015 to 2018. The comparison group included patients with stable ILD listed for lung transplant during the same period. The primary end-points were in-hospital mortality for patients admitted with AE-ILD and 1-year survival for the transplanted patients.
Results Of 53 patients admitted for AE-ILD, 28 were treated with medical therapy alone and 25 underwent transplantation. All patients with AE-ILD who underwent transplantation survived to hospital discharge, whereas only 43% of the AE-ILD medically treated did. During the same period, 67 patients with stable ILD underwent transplantation. Survival at 1 year for the transplanted patients was not different for the AE-ILD group versus stable ILD group (96% vs 92.5%). The rates of primary graft dysfunction, post-transplant hospital length-of-stay and acute cellular rejection were similar between the groups.
Conclusion Patients with ILD transplanted during AE-ILD had no meaningful difference in overall survival, rate of primary graft dysfunction or acute rejection compared with those transplanted with stable disease. Our results suggest that lung transplantation can be considered as a therapeutic option for selected patients with AE-ILD.
- interstitial fibrosis
- lung transplantation
Data availability statement
All data relevant to the study are included in the article or uploaded as supplementary information.
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CL and DG-M are joint senior authors.
CL and DG-M contributed equally.
Contributors The authors meet criteria for authorship as recommended by the International Committee of Medical Journal Editors, take responsibility for the integrity of the work as a whole, contributed to the writing and reviewing of the manuscript, and have given final approval for the version to be published. All authors had full access to the data in this study and take complete responsibility for the integrity of the data and accuracy of the data analysis. MC, AI and BA were responsible for data collection, data analysis and manuscript preparation. TM, AS, DP, MP and BM were responsible for data analysis and manuscript preparation. VS was involved in the acquisition of data and data analysis. AP, CL and DG were responsible for database design, data collection, data analysis and manuscript preparation.
Funding B.M NIH U01EB024501, NIH R01AI135128, American Heart Association 18TPA34170486.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.
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