Autoinflammatory diseases: how to put the fire inside the body out?

Abstract

Autoinflammatory diseases (AIDs) are a group of distinct hereditable disorders characterized by unexplained, recurrent episodes of fever and severe inflammation, most commonly involving skin, joints, gut, and eyes. Mutations in inflammasome-related proteins, particularly in NOD-like receptor (NLR) genes, have been strongly associated with the occurrence of AIDs. However, new genes and dysfunctional proteins have recently been identified and the spectrum of AIDs is ever-expanding. In fact, it has been suggested to encompass other disorders which share some clinical features with AIDs, but are not clearly familial, or are not characterized by fever as a prominent symptom, or are polygenic. In this issue of Autoimmunity Reviews some novel and burning aspects of AIDs were covered and the relationship between AIDs and autoimmune diseases was discussed.