PT  - JOURNAL ARTICLE
AU  - Robin Vos
AU  - Stijn Verleden
AU  - David Ruttens
AU  - Elly Vandermeulen
AU  - Hannelore Bellon
AU  - Jonas Yserbyt
AU  - Lieven Dupont
AU  - Olivier Gheysens
AU  - Nathalie De Raedt
AU  - Dirk Van Raemdonck
AU  - Bart Vanaudenaerde
AU  - Geert Verleden
TI  - Pirfenidone stabilizes pulmonary function and reduces <sup>18</sup>F-FDG-uptake in restrictive allograft syndrome following lung transplantation
DP  - 2014 Sep 01
TA  - European Respiratory Journal
PG  - P3322
VI  - 44
IP  - Suppl 58
4099  - http://erj.ersjournals.com/content/44/Suppl_58/P3322.short
4100  - http://erj.ersjournals.com/content/44/Suppl_58/P3322.full
SO  - Eur Respir J2014 Sep 01; 44
AB  - Introduction: We recently reported stabilization of FVC and FEV1 with regression of subpleural consolidations and ground-glass opacities following pirfenidone (PFD) treatment for Restrictive Allograft Syndrome (RAS) after lung transplantation (LTx) (Vos et al. Am J Transplant, 2013). Increased 18F-FDG uptake was present at diagnosis of RAS in this case. In a second case of RAS treated with PFD presented herein, we sought to confirm our prior findings and to describe the PET/CT-evolution over time with PFD.Methods: a 67-year old patient was diagnosed with RAS more than 10 years after SSLTx for emphysema. PFD in compassionate use was started add-on to his treatment with FK-MPA-steroids after informed consent and ethical approval. PET/CT was performed at start and after 3 months of PFD.Results: After initiation of PFD stabilization of FVC, FEV1 and TLC as well as decreased 18F-FDG-uptake was seen (SUVmax right 5.10 before vs. 2.6 after PFD; SUVmax left 4.44 before vs. 2.4 after PFD). Because of severe subjective intolerance (headache, nausea and anorexia) PFD was stopped by the patient after 3.4 months, following which he opted for palliative sedation and finally succumbed 5 months after PFD had been started.Conclusion: PFD may stabilize the decline in pulmonary function and decrease 18F-FDG-uptake in RAS following LTx, yet side-effects may make it difficult to tolerate treatment.