TY - JOUR T1 - Air-leak syndrome caused by pleuroparenchymal fibroelastosis after bone marrow transplantation JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.congress-2015.PA810 VL - 46 IS - suppl 59 SP - PA810 AU - Tomoya Ishii AU - Takuya Inoue AU - Nobuyuki Kita AU - Akira Tadokoro AU - Naoki Watanabe AU - Nobuhiro Kanaji AU - Shuji Bandoh Y1 - 2015/09/01 UR - http://erj.ersjournals.com/content/46/suppl_59/PA810.abstract N2 - Objective: Air-leak syndrome (ALS) is a life-threatening pulmonary complication after allogeneic bone marrow transplantation (allo-BMT) and is thought to be associated with graft-versus-host disease (GVHD). Recently, it has been reported that pleuroparenchymal fibroelastosis (PPFE) also occurs after allo-BMT and often causes ALS. We sought to extract common features of ALS caused by PPFE after allo-BMT.Methods: Clinical data of the patients who developed ALS caused by PPFE after allo-BMT (ALS-PPFE) between April 1996 and December 2007 in our institution were collected and reviewed retrospectively. The clinical aspects, radiological and pathological features, and treatment outcomes of ALS-PPFE were assessed.Results: Five patients who developed ALS had histologically proven PPFE (four men, one woman: median age, 37 years). The onset of ALS-PPFE was 13 to 109 months (median, 68.8 months) after BMT. Alkylating agents had been used as conditioning chemotherapy for BMT in all patients. Only one patient developed chronic GVHD (limited type). The common radiological findings were subpleural thickening and traction bronchiectasis predominant in the bilateral upper lung fields. Histological pulmonary specimens contain no bronchiolitis obliterans (BO) and GVHD findings. Immunosuppressive therapy was not effective. All patients died of respiratory failure with or without receiving lung transplantation.Conclusions: ALS-PPFE is an extremely late-onset noninfectious pulmonary complication after allo-BMT. It is progressive, resistant to immunosuppressive treatment, and has a poor prognosis. The association was not found between PPFE and GVHD. ER -