Autoinflammatory disorders

Abstract

Autoinflammatory disorders constitutes a new term that covers a group of diseases characterized by recurrent generalized inflammation in the absence of infectious or autoimmune causes. Dysregulation of the innate immune system causes inflammation by affecting the pathways connected to the NOD-like receptors. The patients are at risk of developing amyloidosis, the main long-term sequelae of many of these disorders. The term was initially used only for the inherited periodic fever syndromes. Over the past few years, the concept of autoinflammatory disorders has expanded to encompass an increasing number of polygenic/multifactorial diseases. © 2008 Springer Berlin Heidelberg.