Survival and outcomes after lung transplantation for connective tissue disease-associated interstitial lung diseases

Background Lung transplantation (LTx) is the most important treatment for end-stage lung diseases. However, the treatment of connective tissue disease-associated interstitial lung diseases (CTD-ILD) using LTx is still controversial especially for polymyositis/dermatomyositis-associated interstitial lung disease (PM/DM-ILD). Methods Patients diagnosed with idiopathic pulmonary fibrosis (IPF) ( n =180) and CTD-ILD ( n = 36) from 1 st January 2015 to 31 st December 2019 were recruited into the study. We set polymyositis/dermatomyositis (PM/DM) as a single subgroup, and all the patients underwent LTx at the Wuxi People’s Hospital. Results We found that patients with non-myositis connective tissue-related ILD (NM-CTLD) were younger ( p =0.007) and had a higher percentage of females ( p =0.000) than patients with IPF. PM/DM-ILD was associated with a higher incidence of primary graft dysfunction (PGD) ( p =0.006) and a longer time in the intensive care unit (ICU) ( p =0.000). The cumulative survival rates of patients with PM/DM-ILD were significantly lower than those with IPF (log rank, p =0.003). However, there were no significant differences when compared with the cumulative survival rates of patients with NM-CTLD and IPF (log rank, p =0.528). Age- and gender-adjusted Cox proportional hazard analyses indicated that post-LTx PGD (HR 1.498, 95% CI 1.227–1.828, p =0.000) and duration of ICU (HR 1.027, 95% CI 1.007–1.047, p =0.000) were the independent contributors of disease status to survival. Lung infection was the leading cause of post-LTx death in the groups, where the incidence was 65.3% (47/72) in IPF, 66.7% (8/12) in NM-CTLD, and 66.7% (4/6) in PM/DM-ILD. Conclusions This study found that patients with NM-CTLD had a similar survival outcome with IPF. However, patients with PM/DM-ILD-performed LTx had a lower survival rate than those with IPF. Key Points • Previous studies have shown that the myopathies associated ILD patients had similar post-LTx outcomes with IPF patients. However, our retrospective analysis indicated that patients with PM/DM-ILD-performed LTx had a lower survival rate than those with IPF. • Patients with NM-CTLD had a similar survival outcome with IPF. • We also found that PM/DM-ILD was associated with a higher incidence of PGD and a longer time in the ICU.