Abstract
Lung transplantation for scleroderma-related lung disease is considered a controversial procedure due to extrapulmonary organ involvement that may threaten allograft and patient survival after transplant surgery. Despite concerns, however, several lung transplant programs do offer this option for patients with scleroderma-related lung disease. In this review, we evaluate the scleroderma-related extrapulmonary organ involvement that may result in poorer outcomes as well as the existing evidence on survival, freedom from bronchiolitis obliterans syndrome (BOS), and other important clinical outcomes after lung transplantation. Among the nine studies comprising 226 subjects included in this review, survival and freedom from BOS appear to be similar for subjects undergoing lung transplantation for scleroderma compared to non-scleroderma lung diseases. Although scleroderma is a systemic disease with several unique potential threats to allograft and patient survival, lung transplantation seems to be a reasonable intervention for this patient population.
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Claire B. Richardson and Jonathan P Singer have reported no conflicts of interest.
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Richardson, C.B., Singer, J.P. Lung transplantation for scleroderma-related lung disease. Curr Respir Care Rep 3, 79–87 (2014). https://doi.org/10.1007/s13665-014-0080-6
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DOI: https://doi.org/10.1007/s13665-014-0080-6