Macitentan in the Treatment of Organ Rejection After Lung Transplantation

Potential therapy with MACITENTAN in the treatment of Chronic Lung Allograft Dysfunction (CLAD) after Lung Transplantation. Pilot Study, Double-blind, \"ADD-ON Therapy\" with MACITENTAN to \"usual standard of care immunosuppressive therapies\" after lung transplantation for established BOS Stages I or II versus a \"matched control group\" who receive \"usual standard of care immunosuppressive therapies\" alone, results in a decrease in the Primary Endpoint: \"rate of decline\" in \"Forced Expiratory Volume-1 sec (FEV1) versus time\" while Secondary Endpoints including: differences in Six minute walk distance (6MWD), BORG Score, corrected single-breath diffusing capacity (DCO corrected) at time intervals of 1, 3, 6 months on therapy. Specific biomarkers for BOS, including inflammatory chemokines, which are routinely collected in the context of post-transplant \"surveillance\" will be analyzed. Chemokines which our group has previously described in the pathogenesis of the continuum of \"acute-to-chronic lung allograft rejection\", have included both C-C (CCL2, CCL5) and CXC (CXCL9, CXCL10, CXCL11) chemokines as determined in bronchial-alveolar lavage (BAL).