Abstract
Abstract
Aim
To examine the esophageal motility of lung transplant recipients diagnosed with systemic connective tissue disorder, and to evaluate the changes
Backgrounds
The decision to offer lung transplantation to patients diagnosed with a systemic connective tissue disorder (CTD) is frequently complicated by associated esophageal dysmotility. Absent or ineffective esophageal motility and associated gastroesophageal reflux disease are serious risk factors of posttransplant bronchiolitis obliterans syndrome and subsequent allograft dysfunction. The aim of our study was to evaluate esophageal motility of lung transplant recipients with systemic CTDs, focusing on 1-year survival.
Methods
After IRB approval of this retrospective cohort study, we reviewed patients who underwent lung transplant at our institution between January 2012 and December 2017. Patients with systemic CTDs such as scleroderma, rheumatoid arthritis, systemic lupus erythematosus, polymyositis, dermatomyositis, mixed connective tissue disorder, and Sjögren syndrome were included. Endoscopy studies, high-resolution manometry (HRM), and ambulatory pH studies were re-evaluated.
Results
A total of 545 patients underwent lung transplant during study period; of these, 34 met study criteria. Detailed demographic characteristics of the study cohort are shown in Table 1. The overall 1-year survival was 85.3%. Of the 34 patients diagnosed with a systemic CTD (mean age: 59.24 ± 10.44 years; 24/34 women [70.6%]), 27 had both pre- and posttransplant esophageal function testing data available. 7 patients were diagnosed with complete aperistalsis, 5 patients had ineffective esophageal motility, and the remaining 15 patients had normal or hypercontractile esophageal motility (Figure 1). Pathologic distal acid exposure in each of these groups was 57.1%, 40.0%, and 26.7%, respectively. Seven patients with aperistalsis (5 with scleroderma, 1 with polymyositis, and 1 with dermatomyositis) showed no improvement in esophageal body motility, regardless of underlying CTD. 1-year survival in these patients was 55.5%. Five patients with ineffective esophageal motility (3 with rheumatoid arthritis, 1 with scleroderma, 1 with mixed connective tissue disorder) showed significant peristaltic improvement (mean positive change in distal contractile integral: 1288 ± 391.3 mmHg*cm*s and in the number of normal swallows 6.4 ± 1.4 out of 10). All 5 patients with ineffective esophageal motility were alive 1 year posttransplant.
Conclusion
In our experience, preexisting absent esophageal body motility (i.e., peristalsis) is unlikely to improve after transplantation in patients with systemic CTDs. This cohort of patients appeared to have a significantly decreased 1-year survival. In contrast, ineffective esophageal body motility improved significantly after transplant and was associated with excellent 1-year survival. Detailed esophageal evaluation is necessary in patients with a systemic connective tissue disorder to minimize the risk of future allograft injury.