Is Lung Transplantation a Valuable Therapeutic Option for Patients With Pulmonary Polymyositis? Experiences From the Leuven Transplant Cohort

Interstitial lung disease (ILD) is one of the most critical complications associated with idiopathic inflammatory myopathies (IIM). If medical treatment fails, the only option is lung transplantation (LTx), however, this is still controversial mainly because the outcome is unknown. This case series compared patients with IIM who underwent transplantation in the University Hospitals of Leuven among a total of 90 LTxs for ILD between January 2004 and August 2013. From the 5 IIM patients with associated ILD there were 4 males and 1 female. The mean age at transplantation was 54.4 ± 4.3 years. Three patients underwent sequential single lung (SSLTx) and 2 underwent single lung transplantation (SLTx). Their mean pre-LTx % predicted forced expiratory volume in the first second (FEV1) was 42.8 ± 7.5%, forced vital capacity (FVC) was 49.8 ± 9.6%, total lung capacity (TLC) was 60.8 ± 8.1%, and transfer coefficient for carbon monoxide (DLCO) was 35.1 ± 9.3%. Mean 6-minute walking test (SMWT) before LTx was 316.0 ± 146 meters. In one patient there was an acute rejection (AR) after 20 days. No lymphocytic bronchiolitis (LB) nor chronic rejection was observed. The 1-year survival rate was 100%, and the 2- and 5-year survival rates were 75% (follow-up period of 32.6 ± 4.4 months) compared with 86%, 67%, and 58%, respectively, for patients undergoing LTx for idiopathic pulmonary fibrosis (IPF) (follow-up period of 35.2 ± 3.9) and 86%, 63%, and 57%, respectively, for patients undergoing LTx for non-IPF non-IIM ILD (follow-up period of 40.6 ± 20.5 months). LTx could be a valid option in well-selected patients with ILD related to IIM, yielding a good postoperative course and acceptable 1-, 2-, and 5-year survival rates, compared with patients undergoing LTx for IPF and non-IPF non-IIM–related ILD.