1. Nat. Rev. Rheumatol. doi:10.1038/nrrheum.2017.198
Figure 6 Defects in the clearance of apoptotic debris and immune complexes
Figure 6 | Defects in the clearance of apoptotic debris and immune complexes. a | Cellular apoptotic debris is normally cleared through binding to complement protein C3b or to naturally occurring IgM antibodies. Immune complexes consisting of C3b or IgM and debris bind to complement receptor 1 (CR1) on erythrocytes and are transported to the liver and spleen where they are phagocytosed and removed from the circulation. b | In patients with complement deficiencies or selective IgM deficiency, cellular debris is not removed from the circulation, but instead stimulates the production of IgG autoantibodies. Immune complexes of IgG and debris bind to Fc receptors on macrophages and dendritic cells, inducing the production of type I interferon (IFN) and other cytokines. In particular, increased type I IFN production can lead to the development of systemic lupus erythematosus (SLE). APC, antigen-presenting cell; FcγR, Fcγ receptor.
Schmidt, R. E. et al. (2017) Autoimmunity and primary immunodeficiency: two sides of the same coin?
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