TY - JOUR AU - Maggio, Maria Cristina AU - Corsello, Giovanni PY - 2020 DA - 2020/01/15 TI - FMF is not always “fever”: from clinical presentation to “treat to target” JO - Italian Journal of Pediatrics SP - 7 VL - 46 IS - 1 AB - Familial Mediterranean Fever, a monogenic autoinflammatory disease secondary to MEFV gene mutations in the chromosome 16p13, is characterized by recurrent self-limiting attacks of fever, arthritis, aphthous changes in lips and/or oral mucosa, erythema, serositis. It is caused by dysregulation of the inflammasome, a complex intracellular multiprotein structure, commanding the overproduction of interleukin 1. Familial Mediterranean Fever can be associated with other multifactorial autoinflammatory diseases, as vasculitis and Behçet disease. SN - 1824-7288 UR - https://doi.org/10.1186/s13052-019-0766-z DO - 10.1186/s13052-019-0766-z ID - Maggio2020 ER -