COVID-19 in patients with pulmonary alveolar proteinosis: a European multicentre study
Spyros A. Papiris, Ilaria Campo, Francesca Mariani, Maria Kallieri, Lykourgos Kolilekas, Andriana Ι. Papaioannou, Efsun Gonca Uğur Chouseın, Erdoğan Çetınkaya, Francesco Bonella, Raphaël Borie, Maria Kokosi, Thomas Pickworth, María Molina‐Molina, Mercè Gasa, Elżbieta Radzikowska, Justyna Fijołek, S. Jouneau, E. Gomez, Cormac McCarthy, Elisabeth Bendstrup, Wojciech Piotrowski, Rishi Pabary, Alice Hadchouel, Nathalie Coolen-Allou, Tiago M. Alfaro, Carlos Robalo Cordeiro, Elvira-Markela Antonogiannaki, Ioannis Tomos, Despoina Papakosta, Theodoros Kontakiotis, Panagiota Panagiotou, Konstantinos Douros, Andrea Schams, Sara Lettieri, Vassiliki Papaevangelou, Christina Kanaka‐Gantenbein, Anna Karakatsani, Stelios Loukides, Ulrich Costabel, Bruno Crestani, Cliff Morgan, Ryushi Tazawa, Andrew Bush, Matthias Griese, Effrosyni D. Manali
Abstract
Granulocyte-Macrophage Colony Stimulating Factor (GM-CSF) signaling is essential in both alveolar macrophages (AMs) differentiation and activation of lung immune cells [1]. Differentiated AMs are crucial in both the elimination of alveolar microbes and surfactant clearance. The disruption of the GM-CSF axis in alveolar macrophages leads to the development of pulmonary alveolar proteinosis (PAP) [1]. In the majority of patients this relates to the presence of autoantibodies against GM-CSF autoimmune (a)PAP but there are multiple other causes [1, 2, 3]. GM-CSF deficient animals may have impaired lung inflammatory response to commensal microbes and humans with PAP may occasionally develop opportunistic lung infections [4]. The mainstay of pharmacological treatment in aPAP is inhaled GM-CSF which is off-label but increasingly used worldwide [5, 6, 7, 8, 9]. Footnotes This manuscript has recently been accepted for publication in the ERJ Open Research . It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJOR online. Please open or download the PDF to view this article. Conflict of interest: Dr. Papiris reports grants, personal fees and non-financial support from La Roche Hoffman , grants, personal fees and non-financial support from Boehringer Ingelheim , other from SAVARA , outside the submitted work. Conflict of interest: Dr. Bonella reports grants and personal fees from Savara Pharma, outside the submitted work. Conflict of interest: Raphael Borie has received consulting fees from Boerhinger Ingelheim, Roche, and Sanofi, outside the submitted work. Payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events received from Boerhinger Ingelheim and Roche, outside the submitted work. Support for attending meetings and/or travel received from Boerhinger Ingelheim, Roche, and Chiesim outside the submitted work. Participation on a Data Safety Monitoring Board or Advisory Board for SAvara, outside the submitted work. Conflict of interest: Dr. MOLINA MOLINA reports personal fees and grants from Esteve-Teijin, Roche, and Boehringer Ing., outside the submitted work. Conflict of interest: Dr. Bendstrup reports grants and personal fees from Boehringer Ingelheim, grants and personal fees from Hofmann la Roche, personal fees from Galapagos, outside the submitted work. Conflict of interest: Dr. Jouneau reports personal fees and other from Actelion, personal fees and other from AIRB, personal fees and other from AstraZeneca, personal fees and other from Bellerophon Therapeutics, personal fees and other from Biogen, personal fees and other from BMS, personal fees and other from Boehringer Ingelheim, personal fees and other from Chiesi, personal fees and other from FibroGen, personal fees and other from Galecto Biotech, personal fees and other from Genzyme, personal fees and other from Gilead, personal fees and other from GlaxoSmithKline, personal fees and other from LVL, personal fees and other from Mundipharma, personal fees and other from Novartis, personal fees and other from Pharm-Olam, personal fees and other from Pfizer, personal fees and other from Pliant Therapeutics, personal fees and other from F. Hoffmann-La Roche, Ltd., personal fees and other from Sanofi, personal fees and other from Savara-Serendex, outside the submitted work. Conflict of interest: Dr. McCarthy reports and Scientific advisory board member of Savara Inc. Unrelated to this work and have not received any payment from Savara Inc for any work to date. Conflict of interest: Matthias Griese has received grants or contracts from Böhringer Ingelheim, outside the submitted work. Participation on a Data Safety Monitoring Board or Advisory Board for Böhringer Ingelheim, outside the submitted work. Leadership or fiduciary role in other board, society, committee or advocacy group, unpaid for Head chILD-EU, outsde the submitted work. Conflict of interest: Dr. MANALI reports personal fees and non-financial support from La Roche Hoffman , grants, personal fees and non-financial support from Boehringer Ingelheim , other from SAVARA , outside the submitted work. Conflict of interest: The remaining authors have nothing to disclose.