COVID-19 in patients with pulmonary alveolar proteinosis: a European multicentre study. Issue 1 (3rd January 2023)

• Record Type: Journal Article
• Authors: Papiris, Spyros A.; Campo, Ilaria; Mariani, Francesca; Kallieri, Maria; Kolilekas, Lykourgos; Papaioannou, Andriana I.; Gonca Chousein, Efsun; Cetinkaya, Erdogan; Bonella, Francesco; Borie, Raphael; Kokosi, Maria; Pickworth, Thomas; Molina-Molina, Maria; Gasa, Mercè; Radzikowska, Elżbieta; Fijolek, Justyna; Jouneau, Stéphane; Gomez, Emmanuel; McCarthy, Cormac; Bendstrup, Elisabeth; Piotrowski, Wojciech J.; Pabary, Rishi; Hadchouel, Alice; Coolen-Allou, Nathalie; Alfaro, Tiago; Robalo Cordeiro, Carlos; Antonogiannaki, Elvira-Markela; Tomos, Ioannis P.; Papakosta, Despoina; Kontakiotis, Theodoros; Panagiotou, Panagiota; Douros, Konstantinos; Schams, Andrea; Lettieri, Sara; Papaevangelou, Vassiliki; Kanaka-Gantenbein, Christina; Karakatsani, Anna; Loukides, Stelios; Costabel, Ulrich; Crestani, Bruno; Morgan, Cliff; Tazawa, Ryushi; Bush, Andrew; Griese, Matthias; Manali, Effrosyni D.;
• Is Part Of: ERJ open research. Volume 9:Issue 1(2023)
• Publication Date: 2023-01-03
• Subjects: Respiratory organs -- Diseases -- Periodicals; Respiration -- Periodicals; Respiration; Respiratory organs -- Diseases; Respiratory organs -- Diseases -- Treatment; Respiratory Tract Diseases; Electronic journals; Fulltext; Internet Resources; Periodicals; Periodical; 616.2005
• DOI: 10.1183/23120541.00199-2022
• Languages: English
• ISSNs: 2312-0541

Abstract

Granulocyte–macrophage colony-stimulating factor (GM-CSF) signalling is essential in both alveolar macrophage (AM) 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 AMs 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 [1–3]. 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 (iGM-CSF), which is off-label but increasingly used worldwide [5–9]. Adult PAP patients experience similar #COVID19 rates to the general population, and high rates of hospitalisation and deaths, underscoring their vulnerability and the need for measures to prevent infection. The impact of iGM-CSF must be considered. https://bit.ly/3M0wKnZ