Autoinflammatory diseases (AIDs) are diseases of the innate immune system, with clinical and laboratory evidence of attacks of inflammation. The more common AIDs are those associated with periodic fevers: Familial Mediterranean fever (FMF); Mevalonate Kinase Deficiency (MKD)/Hyperimmunoglobulin D Syndrome (HIDS); Cryopyrin-associated Autoinflammatory Syndrome (CAPS): and Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS). Classification criteria have been developed for all these types. FMF is the most common AID worldwide with a particularly high prevalence in the Eastern Mediterranean region. Environmental factors are thought to affect the course of the disease. Outcome measures are being developed for AIDs. (C) 2018 Elsevier Ltd. All rights reserved.