[What's new in autoinflammatory diseases?].
OBJECTIVE
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.


BACKGROUND
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.


CONCLUSIONS
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.
Stankovic
K
K
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.
Grateau
G
G
D000328:Adult; D001327:Autoimmune Diseases / Q000145:classification / Q000235:genetics / Q000276:immunology / Q000503:physiopathology; D002352:Carrier Proteins / Q000235:genetics / Q000276:immunology; D002648:Child; D010505:Familial Mediterranean Fever / Q000235:genetics / Q000276:immunology; D006801:Humans; D007249:Inflammation / Q000235:genetics / Q000276:immunology; D007375:Interleukin-1 / Q000502:physiology; D009154:Mutation; D014409:Tumor Necrosis Factor-alpha / Q000502:physiology
D002352:Carrier Proteins; D007375:Interleukin-1; D014409:Tumor Necrosis Factor-alpha