Autoinflammatory diseases

Abstract

The term autoinflammatory syndrome was first proposed in 1999 to describe diseases with a lack of apparent provocation for inflammation and absence of high titer autoantibodies or antigen-specific T lymphocytes. The term autoinflammation was proposed in order to draw a distinction between diseases caused by dysregulation of the adaptive immune system (autoimmune) versus innate immune system (autoinflammation). Since that time, the definition of autoinflammatory diseases has evolved to include dysregulatory disorders characterized by significant excessive inflammation mediated predominantly by components of the innate immune system. As our understanding of immune dysregulatory diseases evolves, we are now beginning to understand that diseases characterized by excessive inflammatory response lie on an immunological disease continuum, in which both innate and adaptive immune system components may play pivotal roles in disease propagation.