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Article Dans Une Revue La Revue de Médecine Interne Année : 2018

Clinical overview of auto-inflammatory diseases

F. Rodrigues
  • Fonction : Auteur
A. Fayand
  • Fonction : Auteur
C. Bachmeyer
  • Fonction : Auteur
L. Savey
  • Fonction : Auteur

Résumé

Monogenic auto-inflammatory diseases are characterized by genetic abnormalities coding for proteins involved in innate immunity. They were initially described in mirror with auto-immune diseases because of the absence of circulating autoantibodies. Their main feature is the presence of peripheral blood inflammation in crisis without infection. The best-known auto-inflammatory diseases are mediated by interleukines that consisted in the 4~following diseases familial Mediterranean fever, cryopyrinopathies, TNFRSF1A-related intermittent fever, and mevalonate kinase deficiency. Since 10~years, many other diseases have been discovered, especially thanks to the progress in genetics. In this review, we propose the actual panorama of the main known auto-inflammatory diseases. Some of them are recurrent fevers with crisis and remission; some others evaluate more chronically; some are associated with immunodeficiency. From a physiopathological point of view, we can separate diseases mediated by interleukine-1 and diseases mediated by interferon. Then some polygenic inflammatory diseases will be shortly described: Still disease, Schnitzler syndrome, aseptic abscesses syndrome. The diagnosis of auto-inflammatory disease is largely based on anamnesis, the presence of peripheral inflammation during attacks and genetic analysis, which are more and more performant.
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Dates et versions

hal-01908992 , version 1 (30-10-2018)

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Citer

S. Georgin-Lavialle, F. Rodrigues, V. Hentgen, A. Fayand, P. Quartier, et al.. Clinical overview of auto-inflammatory diseases. La Revue de Médecine Interne, 2018, 39 (4), pp.214-232. ⟨10.1016/j.revmed.2018.01.004⟩. ⟨hal-01908992⟩
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