An expanding spectrum of acute and chronic inflammatory diseases is considered 'autoinflammatory' diseases. This review considers autoinflammatory diseases as being distinct from 'autoimmune' diseases. Autoimmune diseases are associated with dysfunctional T cells and treated with 'biologicals', including antitumour necrosis factorα, CTLA-Ig, anti-IL-12/23, anti-CD20, anti-IL-17 and anti-IL-6 receptor. In contrast, autoinflammatory diseases are uniquely attributed to a dysfunctional monocyte caspase 1 activity and secretion of IL-1β; indeed, blocking IL-1β results in a rapid and sustained reduction in the severity of most autoinflammatory diseases. Flares of gout, type 2 diabetes, heart failure and smouldering multiple myeloma are examples of seemingly unrelated diseases, which are uniquely responsive to IL 1β neutralization.
© 2010 The Association for the Publication of the Journal of Internal Medicine.