¿Qué es lo que hoy debo saber sobre los síndromes autoinflamatorios?

During the last five years almost ten different syndromes resembling systemic onset Juvenile Idiopathic Arthritis (and also having many clinical features in common) have been identified. Although chronic inflammation is present, neither specific autoantibodies nor antigen-driven lymphocytes have been found. Therefore, the term autoinflammatory syndromes or systemic autoinflammatory diseases was chosen to designate them. The inflammasome plays a key role in their pathogenesis. It is composed of a series of proteins that modulate the inflammatory process. The clinical presentation of the familial mediterranean fever (FMF), hypergammaglobulinemia D associated to periodic fever syndrome (HIDS), TNFα-receptor associated periodic syndrome (TRAPS), CINCA/NOMID syndrome, Muckle-Wells syndrome, familial cold associated syndrome (FCAS), systemic granulomatosis diseases of childhood, and PAPA syndrome (pyogenic arthritis/pyoderma gangrenosum/acne) is reviewed with especial attention to the clinical features more related to children. Finally, their treatment is reviewed including the present and future of biological agents.