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.
Index: Connective tissue disease, Idiopathic pulmonary fibrosis, Lung transplantation, Outcomes, Polymyositis/dermatomyositis