Background Lung transplantation (LT) has been proposed as a treatment for advanced Interstitial Lung Disease (ILD) and/or Pulmonary Arterial Hypertension (PAH) associated to Systemic Sclerosis (SSc). Few studies are available and interest in this therapeutic tool is growing.
Objectives To describe the clinical features, evolution, complications and survival of patients with SSc who received LT.
Methods Nine patients with SSc that underwent LT between May 2005 and January 2013 were included. Data were obtained from review of medical records and databases, that includes more than 600 LTs. Standard international criteria were used to determine eligibility for LT. Severity of gastro-oesophageal involvement was not considered as a major counter indication. Follow-up was continued until January 2014.
Results All patients were caucasian. Seven (77%) were women with a mean age of 46±7 years at the LT moment. Distribution by SSc subset according to the Le Roy and Medsger's criteria was: 6 limited and 3 diffuse. All patients had Raynaud's phenomenon, mild to severe esophageal involvement and ILD; 8 heart involvement; 7 musculoskeletal manifestations; 5 digital ulcers and PAH. Lung transplantation was carried out due to: ILD in 6 patients, ILD associated to PAH in 2, and PAH in 1. Six lung transplants were bilateral and 3 unilateral. Initial complications were: gastroparesia in 5 patients, diaphragmatic palsy in 2, and acute cellular rejection in 5. Late complications were: bronchial stenosis in 3 patients, and chronic allograft dysfunction (bronchiolitis obliterans syndrome in 2 patients and restrictive allograft syndrome in 1). All patients were immunosupressed with tacrolimus, mycophenolate mofetil and corticosteroids. Mean forced expiratory volume in the first second (FEV1) in the moment of evaluation for LT (range 2-32months before LT) was 45.3%, while the best FEV1 after LT was 61.35% (range 210-2015 days after LT). No patients developed PAH or ILD in the graft during follow-up. Regarding SSc activity, only one patient presented digital ulcerations after LT. Mean survival was 26 months (range 7-52 months). Cumulative survival at 6 months, 1 year and 2 years were 100%, 88% and 80%, respectively. Deaths were due to respiratory infections in 2 patients (39 and 54 months after LT) and lung adenocarcinoma, that went unnoticed in the original lungs, in 1 (7 months after LT).
Conclusions LT is an effective treatment for advanced ILD and/or PAH associated to SSc patients. Bilateral LT was the most common procedure. In our series, infectious complications were the leading cause of death. Early mortality within the first 6 months has improved in comparison with the oldest series. Mortality at one and two years was similar to that described in our own group1 or in others2 for idiopathic PAH or for non-SSc ILD. Gastro-oesophageal involvement seems not to affect results. More studies are needed to assess long term effectiveness of this treatment.
Berastegui C, Monforte V, Bravo C, et al. [Lung transplantation in pulmonary fibrosis and other interstitial lung diseases.]. Med Clin (Barc). doi: 10.1016/j.medcli.2013.06.026
Saggar R, Khanna D, Furst DE, et al. Systemic sclerosis and bilateral lung transplantation: a single centre experience. Eur Respir J. 2010;36(4):893-900.
Disclosure of Interest None declared