title: Long-Term Off-Line Extracorporeal Photochemotherapy in Patients with Chronic Lung Allograft Rejection Not Responsive to Conventional Treatment: A 10-Year Single-Centre Analysis.
abstract: BACKGROUND
  Extracorporeal photochemotherapy (ECP) for chronic lung allograft dysfunction (CLAD) has been reported as beneficial in a few short-term studies.
  
  
  OBJECTIVE
  In this retrospective cohort study on 48 CLAD patients treated by ECP (off-line technique) for a period of >8 years (compared to 58 controls), we explored potential predictors of survival and response.
  
  
  METHODS
  Failures were defined as a decrease in forced expiratory volume in 1 s (FEV1) of >10% from ECP initiation.
  
  
  RESULTS
  ECP patients were enrolled between February 2003 and December 2013; 14 (29.2%) with restrictive allograft syndrome (RAS) and 34 with bronchiolitis obliterans syndrome. Grade 1 severity was indicated in 58.3%, grade 2 in 20.8%, and grade 3 in 20.8% of patients. The median follow-up was 65 months (cumulative 2,284.4 person-months). Twenty (41.7%) patients died, including 17 (85%) CLAD-related deaths. Among the controls, there were 42 deaths (72.4%), of which 32 (76.2%) were CLAD related, over a median of 51 months (cumulative 3,066.5 person-months; p = 0.09). Among ECP patients, the FEV1 slope flattened out after a decline in the initial months (slope -19 ml/month in months 0-6, +4 in months 36-48 and later; p = 0.001). RAS was associated with poorer survival, whereas a 'rapid decline in the previous 6 months' was not. No ECP side effects or complications were observed.
  
  
  CONCLUSIONS
  Long-term ECP for CLAD is safe and reduces FEV1 decline over time; the RAS phenotype might show a poorer response. ECP deserves to be evaluated in a randomized controlled trial.
authors.affiliation: Immunohaematology and Transfusion Service, Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy.
authors.forename: Claudia
authors.initials: C
authors.lastname: Del Fante
authors.forename: Luigia
authors.initials: L
authors.lastname: Scudeller
authors.forename: Tiberio
authors.initials: T
authors.lastname: Oggionni
authors.forename: Gianluca
authors.initials: G
authors.lastname: Viarengo
authors.forename: Francesca
authors.initials: F
authors.lastname: Cemmi
authors.forename: Monica
authors.initials: M
authors.lastname: Morosini
authors.forename: Alessandro
authors.initials: A
authors.lastname: Cascina
authors.forename: Federica
authors.initials: F
authors.lastname: Meloni
authors.forename: Cesare
authors.initials: C
authors.lastname: Perotti
mesh_terms: D000328:Adult; D064591:Allografts / Q000473:pathology / Q000503:physiopathology; D001989:Bronchiolitis Obliterans / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D004351:Drug Resistance; D005260:Female; D005500:Follow-Up Studies; D006084:Graft Rejection / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D007558:Italy / Q000453:epidemiology; D016040:Lung Transplantation / Q000009:adverse effects*; D008297:Male; D008875:Middle Aged; D017893:Photopheresis / Q000379:methods*; D011379:Prognosis; D012129:Respiratory Function Tests / Q000379:methods; D012720:Severity of Illness Index; D016019:Survival Analysis; D013995:Time; D016896:Treatment Outcome