Skip to main content

IL-1 inhibition in autoinflammatory diseases

Inherited autoinflammatory syndromes (IAS) are a group of recently identified monogenic diseases characterized by recurrent episodes of systemic inflammation.

Muckle-Wells Syndrome, Familial Cold Autoinflammatory Syndrome and Chronic Infantile Neurological Cutaneous and Articular Syndrome are IAS due to mutations in a single gene, CIAS1 (cold-induced autoinflammatory syndrome 1, or NALP-3), encoding a protein called cryopyrin which is an essential component of an intracellular multiprotein complex named inflammasome, that play a crucial role in the production and secretion of interleukin (IL)-1 These diseases are characterized by excessive production of IL-1 and have a dramatic response to IL-1 inhibition.

More recently, IL-1 inhibition has also been shown to be effective in another AID (TNF-receptor associated periodic syndrome or TRAPS) as well as in other conditions such as systemic juvenile idiopathic arthritis and recurrent idiopathic pericarditis. This has suggested that also these two last diseases may represent autoinflammatory conditions.

Author information

Affiliations

Authors

Corresponding author

Correspondence to Alberto Martini.

Rights and permissions

This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

Reprints and Permissions

About this article

Cite this article

Martini, A. IL-1 inhibition in autoinflammatory diseases. Arthritis Res Ther 14, A4 (2012). https://doi.org/10.1186/ar3711

Download citation

Keywords

  • Juvenile Idiopathic Arthritis
  • Systemic Inflammation
  • Pericarditis
  • Excessive Production
  • Recurrent Episode