@article {Hostettler HaackPA3923, author = {Katrin Esther Hostettler Haack and J{\"o}rg Halter and Michael Tamm}, title = {Pirfenidone treatment in patients with bronchiolitis obliterans syndrome following allogeneic hematopoietic stem cell transplantation}, volume = {48}, number = {suppl 60}, elocation-id = {PA3923}, year = {2016}, doi = {10.1183/13993003.congress-2016.PA3923}, publisher = {European Respiratory Society}, abstract = {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 {\textpm} 0.4, corresponding to 36.2\% {\textpm} 11,5 percentage predicted (\%P) FEV1, mean FVC-value was 2.3 l {\textpm} 0.8, 60,4\% {\textpm} 19,9 FVC,\%P, and mean absolute DLCO-value was 6.0 mmol/(min*kPa) {\textpm} 1.7, 68.4\% {\textpm} 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.}, issn = {0903-1936}, URL = {https://erj.ersjournals.com/content/48/suppl_60/PA3923}, eprint = {https://erj.ersjournals.com/content}, journal = {European Respiratory Journal} }