TY - JOUR T1 - Pirfenidone treatment in patients with bronchiolitis obliterans syndrome following allogeneic hematopoietic stem cell transplantation JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.congress-2016.PA3923 VL - 48 IS - suppl 60 SP - PA3923 AU - Katrin Esther Hostettler Haack AU - Jörg Halter AU - Michael Tamm Y1 - 2016/09/01 UR - http://erj.ersjournals.com/content/48/suppl_60/PA3923.abstract N2 - Introduction: Long-term survival after allogeneic hematopoietic stem cell transplantation (HSCT) is limited by chronic graft-versus-host disease, manifesting in the lungs as bronchiolitis obliterans. Treatment for bronchiolitis obliterans syndrome (BOS) is based on augmentation of immunosuppressive therapy, but prognosis remains poor. Pirfenidone exhibits anti-fibrotic effects and in patients with idiopathic pulmonary fibrosis, pirfenidone reduced disease progression. We report the treatment of patients with BOS after HSCT with pirfenidone.Methods: Five patients with BOS and whose lung function has not improved after 3 months immunosuppressive treatment and without active infection were treated with pirfenidone (2403 mg/day) in addition to their basic therapy. Every three months clinical assessments and pulmonary function tests [forced vital capacity (FVC), forced expiratory volume at 1 second (FEV1), diffusion capacity (DLCO)] were performed.Results: Five patients (mean age 55.2 years) received pirfenidone during a mean time of 9.2 months (range 6-13 months). At start of therapy mean absolute FEV1-value was 1.1 l ± 0.4, corresponding to 36.2% ± 11,5 percentage predicted (%P) FEV1, mean FVC-value was 2.3 l ± 0.8, 60,4% ± 19,9 FVC,%P, and mean absolute DLCO-value was 6.0 mmol/(min*kPa) ± 1.7, 68.4% ± 27,5 DLCO,%P. One patient discontinued pirfenidone due to gastro-intestinal symptoms. No serious drug-related adverse events occurred.Conclusions: Pirfenidone was well tolerated and no serious drug-related adverse events were observed. Further studies are needed as pirfenidone might represent a new therapeutic option for patients with BOS after HSCT. ER -