Patients with scleroderma (SSC) associated restrictive lung disease (RD) and pulmonary hypertension (PH) are often turned down for lung transplant due to associated co-morbidities, particularly gastro-esophageal reflux. Our Lung Transplant Center's policy of aggressively managing associated gastrointestinal (GI) complications has allowed us to accept many SSC patients, following transplant denial elsewhere. We contrast here the demographics, length of stay (LOS), 1-year survival and co-morbidities between SSC lung transplant patients at our center and those in the United Network for Organ Sharing (UNOS) database. This is a part of an ongoing study examining the frequency of pneumonia and bronchiolitis obliterans syndrome (BOS) in these high risk SSC patients.

Method: Our center and UNOS databases were queried for diagnoses of SSC-RD and SSC-PH from 2008-2014, to indentify 28 and 180 patients respectively. Demographics, LOS and risk factors were compared between the two databases. Risk of mortality was assessed by univariable and multivariable analyses, with p-value < 0.05 considered significant. Kaplan Meier curves for 1-year survival were also plotted.

Results: Consistent with our center's commitment to transplant these patients, SSC patients represented 4.1% of all transplanted patients at our center compared to 1.6% of UNOS lung transplant recipients. Analysis also demonstrated a greater female preponderance for our center's SSC lung transplant patient population (82% vs UNOS 58.3%, p=0.016). LOS and 6 and 12 months survival (log-rank test p=0.278), were however not statically significant between the two patient cohorts. Of note, estimated glomerular filtration rate < 60 mL/min/1.73 m², was significantly associated with mortality on multivariate analyses (HR 2.37, p = 0.043, CI 1.03-5.49).

Conclusion: Expanding the SSC lung transplant recipient pool to include SSC patients often denied transplant elsewhere, has not adversely impacted survival of these patients at our center. The impact of our specific GI interventions to mitigate aspiration, BOS and pneumonia hence warrants further evaluation.

CITATION INFORMATION: Memon H, Frost A, Graviss E, Nguyen D, Goodarzi A, Sinha N. The Impact of Scleroderma Diagnosis on Lung Transplant Outcomes: A Single Center's Experience. Am J Transplant. 2017;17 (suppl 3).

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