Abstract Background Scleroderma is a multisystem disorder of which the primary cause of mortality is progressive respiratory failure. For these patients, lung transplantation has been shown in recent years to have similar rates of mortality and rejection in non-scleroderma patients. One of the barriers to lung transplantation in this population is the presence of esophago-gastric disease and its proposed association with the risk of rejection in the post-transplant period. The purpose of this study is to outline specific classes of esophageal-gastric disorders that may potentially affect the risk of graft dysfunction and post-transplant complications in lung transplant recipients with scleroderma. Methods A retrospective chart review analysis was performed on patients with scleroderma who underwent lung transplant and gastro-esophageal testing between 2020 and 2024. Chicago classification was used to classify the underlying manometric disorder as either a disorder of the esophagogastric junction outflow obstruction (EGJOO) or a disorder of peristalsis. 24-hour ph-impedance results was used to classify patients into groups either positive or negative for gastroesophageal reflux disease (GERD). Additionally, survival rates and post-lung transplantation complications were assessed over the 1st year of transplantation. Results 17 patients with history of scleroderma underwent lung transplant and had both 24-hour ph-impedance, and esophageal manometry performed peri-transplant. Given the history of scleroderma, all patients were restricted to enteral meals via gastric-jejunal-tube placement post transplantation. In this cohort, the overall one-year survival rate was 82%. The overall incidence of primary graft dysfunction and acute cellular rejection within the first year was 1 (5.9%) and 3 (17.6%) respectively. All 3 episodes of ACR were recorded A1 rejection by transbronchial biopsy. 13 (77%) patients had impaired manometry signifying disease of peristalsis. 4 (24%) patients had findings of EGJOO. Combined, 14 (82%) had findings of either peristalsis disease or EGJOO. In the group of patients with impaired manometry and or EGJOO, the incidence of ACR was 2/14 (14%%) vs 1/3 (33%) in patients who had normal manometry testing. In the group of patients with abnormal pH- impedance testing, the incidence of ACR was 2/11 (18%) compared to ¼ (25%) in patients who had normal pH impedance testing. Average amount of time with gastric-jejunal-tube was 6 months. Conclusions We have shown relatively low rates of first-year occurrence of ACR in this cohort of patients undergoing lung transplantation for scleroderma despite high rates of esophago-gastric dysfunction. This abstract is funded by: N/A
Bien-Aime et al. (2026) studied this question.