Abstract: Acute chest syndrome (ACS) is a common and potentially lethal form of acute lung injury in sickle cell disease (SCD). Because pathophysiology remains unclear, therapeutic options are limited to supportive care with empiric antibiotics and red cell transfusion in case of aggravation. A role of inflammation mediated by endothelial and immune cells has been suspected but the levels of pro-inflammatory cytokines and chemokines in the lungs during ACS have not yet been investigated. Here the inventors report dramatically high levels of IL-6, unlike IL-1? and TNF-?, in the sputum from SCD children during ACS (n=12) compared with non-ACS sputum (n=6). By contrast, plasma IL-6 levels were not significantly increased during ACS (n=12), compared with vaso-occlusive crisis (n=12), steady state (n=12) and healthy controls (n=9). IL-6 levels were more than 150-fold higher in sputum than in plasma, suggesting increased local production by inflammatory cells during ACS.

Abstract: A 2-aminoarylthiazole derivative or a pharmaceutically acceptable salt or solvate thereof, in particular masitinib or a pharmaceutically acceptable salt or solvate thereof, for use in the treatment of sickle cell disease in a patient in need thereof. Also, a 2-aminoarylthiazole derivative or a pharmaceutically acceptable salt or solvate thereof, in particular masitinib or a pharmaceutically acceptable salt or solvate thereof, for use in the prevention and/or treatment of acute chest syndrome (ACS) in a sickle cell disease patient in need thereof.