Autoimmunity versus autoinflammation--friend or foe?

Abstract

"Autoimmunity" is a designation dependent on the conventional immunological issue of self/non-self discrimination. Identification of novel target autoantigens is still an important issue ongoing in classical tissue-specific autoimmune bullous diseases and autoimmune connective tissue diseases. In contrast, synchronized with the paradigm shift of the fundamental aspect of immunity to danger sensing/signaling, distinct collagen-like diseases have been defined by the genetic mutations causing dysregulated innate immunity/inflammation and have been designated as “autoinflammatory”diseases. Due to the clinical and etiological similarities,the concept of autoinflammatory diseases has expanded to include non-hereditary collagen-like diseases, tissue-specific chronic idiopathic inflammatory diseases and metabolic diseases. On the other hand, various genetic causes of autoimmune diseases have been identified and the border of these two pathophysiologies is becoming obscure. Instead, a variable mixture of both autoimmunity and autoinflammation can cause each inflammatory phenotype with a variable level of antigen specificity