Original article
General thoracic
Long-Term Survival in Bilateral Lung Transplantation for Scleroderma-Related Lung Disease

Presented at the Fifty-third Annual Meeting of The Society of Thoracic Surgeons, Houston, TX, Jan 21–25, 2017.
https://doi.org/10.1016/j.athoracsur.2017.09.038Get rights and content

Background

Lung disease is the leading cause of morbidity and death in scleroderma patients, but scleroderma is often considered a contraindication to lung transplantation because of concerns for worse outcomes. We evaluated whether 5-year survival in scleroderma patients after lung transplantation differed from other patients with restrictive lung disease.

Methods

This was a single-center, retrospective cohort study of all patients undergoing bilateral lung transplantation for scleroderma-related pulmonary disease between January 2006 and December 2014. This cohort was compared with patients undergoing bilateral lung transplantation for nonscleroderma group D restrictive disease. Primary outcomes reported were 1-year and 5-year survival. Diagnoses were identified by United Network of Organ Sharing listing and were confirmed by clinical examination and prelisting workup.

Results

We compared 26 patients who underwent BLT for scleroderma and 155 patients who underwent BLT for group D restrictive disease. Overall, the nonscleroderma cohort was younger, with lower lung allocation score but no difference in functional status. Donor characteristics were not different between the cohorts. Survival at 1 year was not different (73.1% vs 80.0%, p = 0.323). Long-term survival at 5 years was also not significantly different (65.4% vs 66.5%, p = 0.608). Multivariate Cox proportional hazards analysis found no differences in survival between scleroderma and nonscleroderma group D restrictive disease (hazard ratio, 2.19; p = 0.122).

Conclusions

Despite being at high risk for extrapulmonary complications, patients undergoing bilateral lung transplantation for scleroderma have similar 1-year and 5-year survival as those with restrictive lung disease. Transplantation is a reasonable treatment option for a carefully selected population of candidates.

Section snippets

Study Design

We retrospectively reviewed our clinical database, including all lung transplant recipients who underwent transplant between January 1, 2008, and December 31, 2014, at the Houston Methodist Hospital. We identified all patients who underwent a bilateral lung transplant. We included only those recipients with a diagnosis of group D restrictive lung disease. For classification purposes, all scleroderma patients were listed in our database as group D restrictive disease, including those with

Results

Demographic data are reported in Table 1. From 2008 to 2014, our institution performed 684 lung transplants, including 394 bilateral transplants. Of that cohort, 181 met our criteria for inclusion into the analysis, with 26 scleroderma patients and 155 nonscleroderma group D restrictive disease patients (Fig 1).

Several differences were noted between the cohorts at baseline. The scleroderma patients tended to be younger, and the percentages of women and African-American patients were higher.

Comment

Although lung transplantation has been established as a safe and effective treatment for a variety of etiologies of end-stage lung disease, patients with scleroderma are still frequently declined for fear of their overall risk profile. This patient population has been a challenging one to study for several reasons. Scleroderma is a rare disease process that accounts for 1.4% of those undergoing lung transplantation [11], so previous research has mostly involved small case series from

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