Interstitial lung disease (ILD) is a common cause of end-stage lung disease and a frequent cause of morbidity and mortality among patients with connective tissue disorders (CTD). Although lung transplantation is a viable option for many patients with ILD, very few patients with ILD related to CTD have been transplanted due to concerns about systemic disease activity. Recent studies have suggested that lung transplant outcomes for selected patients with scleroderma are similar to those transplanted with other diseases. Our hypothesis is that lung transplant outcomes in patients with CTD other than scleroderma are comparable to those of patients transplanted for other indications.
We retrospectively reviewed lung transplants performed for CTD related ILD that were reported in the UNOS database from 1987 and 2008. Patients listed as having scleroderma, idiopathic pulmonary fibrosis (IPF), and primary pulmonary hypertension (PPH) were selected as the control groups. Either the unpaired t-test or Mann-Whitney test was used to compare continuous variables, the chi-square test to compare categorical variables, and Kaplan-Meier analysis to evaluate post-transplant survival. A p value <0.05 was considered statistically significant.
Of the 55 patients who underwent lung transplantation for CTD, 20 (36%) had rheumatoid arthritis, 14 (25%) polymyositis or dermatomyositis, 11 (20%) mixed connective tissue disease, 3 (5%) Sjögren's syndrome, 3 systemic lupus erythematosus (5%), and 4 (7%) had unspecified CTD. Survival at 30 days post-transplantation was 92.3% in the CTD group, compared with 94.1% in the scleroderma group (p=0.98), 92.1% in the IPF group (p=0.95), and 80.9% in the PPH group (p = 0.06). The Kaplan-Meier 1-year survival rate was 65.0%, not significantly different from those of patients with scleroderma (76.0%, p = 0.36), IPF (76.0%, p = 0.41), or PPH (67.3%, p = 0.90).
Survival following lung transplantation for CTD-related lung disease is similar to that following transplant for scleroderma, IPF, and PPH.
In carefully selected patients with CTD, lung transplantation represents a viable therapeutic option.
Amy Hajari, No Financial Disclosure Information; No Product/Research Disclosure Information