Abstract
Interleukin 1 (IL-1) is one of the major cytokines involved in the regulation of systemic inflammation. The availability of drugs that specifically inhibit IL-1 has dramatically improved the prognosis of a number of diseases with a systemic inflammation as a dominant feature.
This group of diseases, called autoinflammatory, is characterized by a dysregulation of the inflammatory reaction with excessive and uncontrolled overproduction of IL-1β in particular. There is an increasing evidence that one form of juvenile idiopathic arthritis (JIA), systemic JIA (sJIA) could be included in autoinflammatory diseases.
There are currently two IL-1 blockers with proven efficacy in the treatment of autoinflammatory diseases (AID), including sJIA, for which the European Medicines Agency (EMA) has approved an indication for administration in AID. They are anakinra and canakinumab.
Recently published studies with IL-1 inhibitors confirm a sustained reduction in disease activity in most autoinflammatory disorders with an acceptable safety profile and satisfactory tolerability. In addition, the results of studies in patients with sJIA suggest that early initiation of IL-1 blockade as a first-line therapy fulfill the concept of therapeutic "window of opportunity" in which the disease pathophysiology can be altered to prevent the development of chronic arthritis.
This review provides up-to-date information on the use of IL-1 inhibitors, their efficacy, safety and tolerability in the treatment of autoinflammatory diseases and discusses the reasons for use of these drugs.