Monogenic lupus is associated with the alteration of three main pathways: (1) defects in the clearance of apoptotic bodies and self-derived nucleic acids such as complement deficiencies and mutations in extracellular DNase genes; (2) IFN signaling pathway defects involving either nucleic acid-sensing or the production and response to IFNs; and (3) B-cell and T-cell tolerance defects (98) (Table 2). The gene discussed is IFNA1; the disease is systemic lupus erythematosus.