학술논문

Lung Transplant Outcomes in Systemic Sclerosis with Significant Esophageal Dysfunction. A Comprehensive Single-Center Experience
Document Type
article
Source
Annals of the American Thoracic Society. 13(6)
Subject
Biomedical and Clinical Sciences
Clinical Sciences
Rare Diseases
Scleroderma
Orphan Drug
Transplantation
Organ Transplantation
Digestive Diseases
Lung
Autoimmune Disease
Clinical Research
Respiratory
Aged
Bronchiolitis Obliterans
Esophageal Diseases
Esophagus
Female
Graft Survival
Humans
Lung Transplantation
Male
Middle Aged
Multivariate Analysis
Postoperative Complications
Primary Graft Dysfunction
Retrospective Studies
Scleroderma
Systemic
Survival Analysis
Time Factors
United States
scleroderma
lung transplantation
esophageal dysfunction
gastroesophageal reflux
acute rejection
Cardiovascular medicine and haematology
Clinical sciences
Language
Abstract
RationaleConsideration of lung transplantation in patients with systemic sclerosis (SSc) remains guarded, often due to the concern for esophageal dysfunction and the associated potential for allograft injury and suboptimal post-lung transplantation outcomes.ObjectivesThe purpose of this study was to systematically report our single-center experience regarding lung transplantation in the setting of SSc, with a particular focus on esophageal dysfunction.MethodsWe retrospectively reviewed all lung transplants at our center from January 1, 2000 through August 31, 2012 (n = 562), comparing the SSc group (n = 35) to the following lung transplant diagnostic subsets: all non-SSc (n = 527), non-SSc diffuse fibrotic lung disease (n = 264), and a non-SSc matched group (n = 109). We evaluated post-lung transplant outcomes, including survival, primary graft dysfunction, acute rejection, bronchiolitis obliterans syndrome, and microbiology of respiratory isolates. In addition, we defined severe esophageal dysfunction using esophageal manometry and esophageal morphometry criteria on the basis of chest computed tomography images. For patients with SSc referred for lung transplant but subsequently denied (n = 36), we queried the reason(s) for denial with respect to the concern for esophageal dysfunction.Measurements and main resultsThe 1-, 3-, and 5-year post-lung transplant survival for SSc was 94, 77, and 70%, respectively, and similar to the other groups. The remaining post-lung transplant outcomes evaluated were also similar between SSc and the other groups. Approximately 60% of the SSc group had severe esophageal dysfunction. Pre-lung transplant chest computed tomography imaging demonstrated significantly abnormal esophageal morphometry for SSc when compared with the matched group. Importantly, esophageal dysfunction was the sole reason for lung transplant denial in a single case.ConclusionsRelative to other lung transplant indications, our SSc group experienced comparable survival, primary graft dysfunction, acute rejection, bronchiolitis obliterans syndrome, and microbiology of respiratory isolates, despite the high prevalence of severe esophageal dysfunction. Esophageal dysfunction rarely precluded active listing for lung transplantation.