Lung transplantation for scleroderma-related lung disease is controversial due to extra-pulmonary organ involvement that may threaten allograft and patient survival after transplant surgery. Despite concerns, several lung transplant programs do offer lung transplantation to patients with scleroderma-related lung disease. In this review, we evaluate the scleroderma-related extra-pulmonary organ involvement that may result in poorer outcomes after lung transplantation 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 reviewed, comprising 226 subjects, survival and freedom from BOS appears 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 appears to be a reasonable intervention for this patient population.
Keywords: GERD; Nissen fundoplication; Scleroderma; acute rejection; bronchiolitis obliterans syndrome; gastroesophageal reflux; idiopathic pulmonary fibrosis; interstitial lung disease; lung transplantation; pulmonary arterial hypertension; scleroderma renal crisis; survival; systemic sclerosis.