TY - JOUR T1 - <em>Aspergillus</em>-induced pneumonia in adult without obvious immunodeficiency: test the burst! JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.02711-2017 VL - 51 IS - 4 SP - 1702711 AU - Héloïse Flament AU - Vanessa Granger AU - Corinne Vezinet AU - Viviana Marzaioli AU - Caroline Kannengiesser AU - Luc de Chaisemartin AU - Margarita Hurtado-Nedelec AU - Elena Litvinova AU - Jonathan Messika AU - Nicolas Adam AU - Marie-Anne Gougerot-Pocidalo AU - Pham My-Chan Dang AU - Renato Monteiro AU - Jamel el Benna AU - Olivier Langeron AU - Sylvie Chollet-Martin AU - Antoine Monsel Y1 - 2018/04/01 UR - http://erj.ersjournals.com/content/51/4/1702711.abstract N2 - Chronic granulomatous disease (CGD) is a rare primary immunodeficiency caused by a germline defect in one of the genes (CYBB, NCF1, CYBA, NCF2) encoding the NADPH oxidase 2 (NOX2) components responsible for the phagocyte oxidative burst and the production of reactive oxygen species (ROS). An early diagnosis and a prompt treatment are crucial for improving outcomes in affected patients. CGD is usually diagnosed in early childhood, and more than 95% of cases are diagnosed by the age of 5 years [1, 2]. However, CGD can be diagnosed late in adulthood [1–3]. Aside from residual NOX2 activity, factors underlying late diagnosis of CGD remain poorly investigated. Here, we report a case of Aspergillus-induced pneumonia rapidly extending to fatal acute respiratory distress syndrome (ARDS) revealing a late diagnosis of CGD, which was associated with a residual mitochondria-driven neutrophil extracellular trap release (NETosis) counterbalancing the lack of functional NOX2. Written informed consent was obtained from the deceased patient's next-of-kin for publication of this case report and accompanying images.Unusual pathogen-driven sepsis should prompt to screen for chronic granulomatous disease, even in adult patients http://ow.ly/2FSi30iXUsaWe thank Benoît Noël (Unité mixte de Recherche 996-Inflammation, Chemokines and Immunopathology, Institut National de la Santé et de la Recherche Médicale, Université Paris Sud, Université Paris-Saclay, Châtenay-Malabry, France) and Claire Oudin (AP-HP, Centre Hospitalier Universitaire Xavier Bichat, Service d'Immunologie et d'Hématologie et Service de Biochimie Hormonale et Génétique, CIB Phenogen, Paris F-75018, France) (molecular exploration of CYBB) for excellent technical help. ER -