Fig. 2

a Chest-CT in patient case 1, d1 of CFZ-TMA onset: Bipulmonary extensive ground-glass opacities in all lung fields, mostly omitting the periphery and predominantly occurring in the right lung. b Mechanism of CFZ-TMA: Within the proximal complement activation C3 is cleaved by the C3 convertase to anaphylactic 3a and C3b. C3b then cleaves C5 to C5a and C5b, the latter together with C6-C9 forming the membrane attack complex (MAC) within the terminal pathway. Activity of the C3 convertase is regulated by complement factor H and regulated proteins. Proteasome inhibition blocks CFH [11]. The monoclonal antibody eculizumab counteracts the resulting overactivation of C3b by binding C5