Familial Mediterranean Fever and Other Autoinflammatory Disorders

Abstract

Autoinflammatory diseases are a group of disorders characterized by seemingly unprovoked inflammation in the absence of high-titer autoantibodies or antigen-specific T-cells (Stojanov and Kastner 2005). The autoinflammatory diseases include the hereditary periodic fever syndromes and are thought to be due to disturbances in the regulation of the innate immunity (Kastner 2005). Familial Mediterranean Fever (FMF) is the archetypal hereditary periodic fever syndrome and autoinflammatory disease. Other disorders include tumor necrosis factor receptor-associated periodic syndrome (TRAPS); hyperimmunoglobulinemia D with periodic fever syndrome (Hyper-IgD); pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome; the cryopyrinopathies: familial cold autoinflammatory syndrome (FCAS), Muckle–Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease (NOMID, also called chronic infantile neurologic cutaneous and articular syndrome, or CINCA syndrome); and chronic recurrent multifocal osteomyelitis (McGonagle and McDermott 2006; Milhavet et al. 2008).