What's new in autoinflammatory diseases?].

Service de médecine interne, centre de référence des amyloses d'origine inflammatoire et de la fièvre méditerranéenne familiale, hôpital Tenon, Assistance publique-Hôpitaux de Paris (AP-HP), faculté de médecine, université de Paris-Paris cedex 20, France.

La Revue de médecine interne / fondée ... par la Société nationale francaise de médecine interne 2008 Dec

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The concept of auto-inflammation was initially coined to define the group of mendelian disorders characterized by recurrent inflammatory symptoms. The core of this group mainly consists of hereditary recurrent fevers, which has been lately enlarged to other inflammatory mendelian disorders as well as to some sporadic diseases with a genetic component relevant to innate immunity. Cryopyrin, the product of the CIAS1/PYPPAF1/NALP3/NLRP gene, whose mutations underline some mendelian syndromes (Mückle-Wells and chronic infantile neurological cutaneous and articular (CINCA), familial cold urticaria) can now be considered as a major factor of the regulation of interleukin-1 production within the multiprotein complex called inflammasome. This discovery has lit up our view of innate immunity. The contribution of the innate immunity mechanisms in inflammatory disorders have led to a new look to the current nosology of this vast group of diseases and to suggest a classification with two poles. The first would be defined by the predominance of auto-inflammation, whereas in the second one auto-immunity predominates.

Citation

K Stankovic, G Grateau. What's new in autoinflammatory diseases?]. La Revue de médecine interne / fondée ... par la Société nationale francaise de médecine interne. 2008 Dec;29(12):994-9