MBL2 and systemic sclerosis: While not investigated in SSc experimental models, evidence from comprehensive animal and human studies suggests that MBL-mediated damage in I/R injury is caused by binding of MBL to IgM on stressed endothelial and parenchymal cells causing apoptosis [38], activation of the complement and the coagulation cascade (via MASP-1) and recruitment of inflammatory cells [39].