Prolonged Survival Following Lung Transplantation in the Absence of Conventional Immunosuppression

Purpose The modern era of lung transplantation (LTx) coincided with the discovery of the calceneurin inhibitors and the ability to limit the the immune response to LTx. Most LTx recipients are maintained on triple immunosuppression. Prolonged survival without conventional immunosuppression has not been reported. Methods We report extended survival following LTx in a recipient who has not received oral immunosuppression for over 2 years and is only maintained on 4-weekly extracorporeal photophoresis (ECP) and intravenous immunoglobulin (IVIg). Results The index case is a 13 year old male with cystic fibrosis who underwent LTx in 2015. He was colonized with mycobacterium abscessus pre-LTx and despite combinational antibiotics peri-operatively and antimicrobial washes of the chest cavities, soft tissue infections developed along the operative site. Wound swabs confirmed infection with mycobacterium abscessus and treatment included escalating doses of combinational intravenous (iv) antibiotics. Despite reduced immunosuppression and toxicity from the combinational antibiotics, the wound infection progressed (Figure 1. PET scan) He underwent surgical rib resection on two occasions separated by 6 months. At the time of initial surgery in 2017, a decision was made to cease his oral triple immunosuppression. To create a potential immunomodulatory environment he has been maintained on 4-weekly IVIg and ECP, and two years on he maintains stable allograft function (FEV1 47% predicted). There have been no episodes of acute cellular rejection. HLA class II donor specific antibodies (mfi 19025) have been chronically present but are not associated with allograft dysfunction. Conclusion We report the first case of prolonged survival following lung transplantation in the absence of conventional immunosuppression. We speculate that the combination of IVIg and ECP is maintaining a tolerogenic environment for the transplanted lung allograft.