A. D’Angelo1, D. Odell1, N. Shigemura1, C. Bermudez1, T. Richards1, M. Crespo1, J. Pilewski1, J. Luketich1, J. D’Cunha1 1University Of Pittsburg,Pittsburgh, PA, USA
Introduction: End-stage lung disease is the leading cause of death in people with systemic sclerosis. Lung transplantation (LTx) is often the only viable therapy available when drug treatment fails to suppress progression of pulmonary disease. Patients are often declined for LTx because of the associated morbidity related to esophageal dysmotility. Because of the lack of viable options for their end-stage lung disease, we have considered these patients for LTx and followed a unique post-operative nutritional management algorithm because of their esophageal disease. The aim of this study was to review our approach and the associated outcomes.
Methods: Between 2008 and June 2013, our center transplanted 48 patients with systemic sclerosis. An IRB-approved retrospective analysis was performed to examine the nutritional management and subsequent complications in these patients. Patients were categorized based on route of enteral access (percutaneous endoscopic gastrojejunostomy (PEG-J) or nasojejunal (NJ) tube). The incidence, timing, and types of complications were analyzed. Student's t-test was used to compare groups (p<0.05 was significant).
Results: In our 48 patients with systemic sclerosis receiving LTx, 47 received bilateral grafts. 33 received PEG-J tube nutritional support, while the remaining 15 received NJ tube feeding alone. In patients receiving a PEG-J feeding, an average of 16.75 days (median = 12 days) elapsed between transplant and PEG-J tube placement, during which time nutrition/medications were provided by NJ tube only. There was one complication (localized infection) in the PEG-J. There were no major complications in the NJ treatment group. An average of 68 days elapsed from transplantation to first oral intake in patients receiving a PEG-J tube, compared to 35.5 days for patients with a NJ tube (p = 0.001). Average hospital length of stay was 40 days for patients with PEG-J feeding tube and 42.6 days for patients with NJ tube feeding (p = 0.75). Overall survival was 85% at one year and there was no difference between the groups (NJ 86%, PEG-J 84%).
Conclusion: End-stage lung disease secondary to systemic sclerosis is a highly morbid condition for which LTx is a life-saving intervention. Although many centers will not consider these patients for LTx, we have developed a low morbidity nutritional management algorithm with acceptable short-term outcomes.