TY - JOUR AU - Alperin, Jessie M. AU - Ortiz-Fernández, Lourdes AU - Sawalha, Amr H. PY - 2018 M3 - Review TI - Monogenic Lupus: A Developing Paradigm of Disease JO - Frontiers in Immunology UR - https://www.frontiersin.org/articles/10.3389/fimmu.2018.02496 VL - 9 SN - 1664-3224 N2 - Monogenic lupus is a form of systemic lupus erythematosus (SLE) that occurs in patients with a single gene defect. This rare variant of lupus generally presents with early onset severe disease, especially affecting the kidneys and central nervous system. To date, a significant number of genes have been implicated in monogenic lupus, providing valuable insights into a very complex disease process. Throughout this review, we will summarize the genes reported to be associated with monogenic lupus or lupus-like diseases, and the pathogenic mechanisms affected by the mutations involved upon inducing autoimmunity. ER -