Ten Years of ECP for CLAD: A Real Life Experience

Purpose We hereby report our real-life experience with extracorporeal photopheresis (ECP) for CLAD. Methods This was an observational retrospective study on LuTx adult patients who received a diagnosis of chronic lung allograft dysfunction (CLAD) from January 2008 to December 2018. They were referred to an ECP Service on a regional basis. Both automated closed and open loop devices were used. Results Over the period considered, 42 patients were diagnosed with CLAD, 10 (24%) restrictive allograft syndrome (RAS) and 32 BOS. Of those 42, 6 individuals were considered ineligible to initiate ECP due to their excessively compromised conditions; they all died soon after (less than 6 months). The rest (36 patients) were scheduled to receive ECP treatments. Median time from CLAD diagnosis to ECP initiation was 2 (1; 4) months. Median rate of FEV1 decline in the first 6 months was -13 mL/month; however, 11 (31%) did not respond (FEV1 decreased > 10% from ECP initiation). 14 (38%) patients were hospitalized at least once for respiratory infection. The majority of treated patients (21, 58%) reported a good quality of life and worked or were fit to work. 6 patients died, 5 of CLAD-related death. 3 individuals received a re-transplant, and 2 continued with ECP even afterwards. Given also that the majority of patients at our centre are affected by cystic fibrosis (24, 58% of this cohort), one of the major problems with performing ECP was represented by the lack of good vascular access; 4 patients needed a long term catheter insertion. In one of these patients early termination of treatment was decided after a sepsis due to S. epidermidis starting from her Tesio access. No other significant adverse event occurred. No difference was observed between closed and open loop devices. Conclusion Our data seem to further support the efficacy and safety of ECP to treat CLAD, not only in terms of pulmonary function but also of survival and quality of life. Vascular access related problems were the major issue. Several limits to be acknowledged: retrospective nature, single centre, no control group.