TY - JOUR T1 - Fludarabine in paediatric steroid-refractory inflammatory lung injury after stem cell transplantation JF - European Respiratory Journal JO - Eur Respir J SP - 479 LP - 483 DO - 10.1183/09031936.00029312 VL - 41 IS - 2 AU - Martine F. Raphael AU - Bart L. Rottier AU - Jaap Jan Boelens Y1 - 2013/02/01 UR - http://erj.ersjournals.com/content/41/2/479.abstract N2 - To the Editor:In children, the incidence of pulmonary complications after haematopoietic stem cell transplantation (HSCT) varies from 10% to 20%. Noninfectious causes, such as idiopathic pneumonia syndrome (IPS), bronchiolitis obliterans syndrome (BOS) and bronchiolitis obliterans organising pneumonia (BOOP), are considered nonspecific inflammatory injuries to the lung [1, 2]. These may represent a form of alloimmune injury to the lung after HSCT.The current view is that there is a three-step process in the development of alloreactivity: 1) tissue damage results in 2) a release of inflammatory cytokines, which results in 3) activation and influx of T-cells [3].First-line treatments for nonspecific inflammatory lung injuries are intravenous corticosteroids and supportive care. In four steroid-refractory, nonspecific inflammatory lung injury patients, we studied the efficacy and toxicity of fludarabine given on clinical grounds as a second-line treatment, despite the presence of respiratory viral infections.In our study, nonspecific inflammatory lung injury was defined as IPS, BOS and BOOP (also referred to as cryptogenic organising pneumonia) diagnosed by clinical symptoms, typical high-resolution computed tomography changes and/or results of pulmonary function tests (PFTs), if feasible. Clinical symptoms were defined as cough, dyspnoea, fever and oxygen requirement. Three preschool study patients were not able to perform PFTs.In patients with upper respiratory tract infection symptoms, nasopharyngeal aspirate (NPA) samples were obtained, testing for respiratory virus infection by PCR. Bronchoalveolar lavage (BAL) for bacterial/fungal cultures, viral PCR and galactomannan testing were performed as clinically indicated. Quantitative real-time PCR (TaqMan®; Life Technologies Europe BV, Bleiswijk, the Netherlands) for Epstein–Barr virus, cytomegalovirus, human herpesvirus 6 and adenovirus, and galactomannan monitoring in plasma were performed twice a week.All patients diagnosed … ER -