Author/Editor		Kačar, Mark; Savic, Sinisa
Title		Autoinflammatory disorders
Type		članek
Source		In: Cellular primary immunodeficiencies Cham : Springer Nature
Publication year		2021
Volume		str. 279-313
Language		eng
Abstract		Autoinflammatory disorders represent an evolving group of conditions which were first mentioned as a separate disease category in 1999, after the genetic basis for tumor necrosis factor (TNF) receptor-associated periodic fever syndrome was identified. Since then, the boundaries of what defines an autoinflammatory condition continue to expand and include diseases which to a varying degree have features of immunodeficiency and classical autoimmunity. There has been considerable progress in mapping the genetic basis of these disorders and understanding the relevant biological pathways responsible for the inflammatory pathology. A targeted therapeutic approach using modern biologicals has in many cases led to significant improvements in disease management and outcomes. According to the latest classification from the International Union of Immunological Societies, there are almost 40 diseases being classed as autoinflammatory. It is beyond the scope of this chapter to describe each condition in detail. Instead, it will shed light on the more common, prototypic, and well-understood conditions which help elucidate autoinflammatory disease pathways in general.
Keywords		avtovnetne bolezni pirin relopatije interferonopatije inflamasomopatije