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Hence, in this article, we try to advance the conceptual understanding of autoinflammatory disease by, proposing a modification of the definition by Daniel Kastner et al. Introduction Autoinflammatory disease was introduced as a concept indemarcating an entirely new group of diseases in clinical, immunological, and conceptual terms 1.

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Here, we map the recent developments in the field and discuss the conceptual challenges that these developments entail, with the goal to propose an updated definition of autoinflammatory diseases that is inclusive broad 6 rather than exclusive narrow 7. This formulation made a clear division between autoinflammation and autoimmunity, at that time an important distinction to make. This continuum Badoo voisin moved the understanding of immunological diseases forward by entwining the concept of autoinflammation with that of autoimmunity and applying the concept of autoinflammation not only to monogenic diseases but also to polygenic diseases. It also recognized that polygenic and multifactorial diseases might have both an autoinflammatory and an autoimmune component 9.

InDaniel Kastner et al. This definition recognizes that cells and molecules of innate immunity, including, monocytes, neutrophils, and NOD-like receptors are fundamental in autoinflammatory disease This is the definition that is generally accepted and used at present. The Expanding Disease Spectrum of Autoinflammatory Diseases Today, several studies show that classical ILdriven autoinflammatory diseases are associated with activation of adaptive immunity 11 — Characterization of monogenic autoinflammatory diseases with complex phenotypes.

For example, in patients with cryopyrin-associated periodic syndromes CAPSthere is an increased number of Th17 cells and IL in the blood as well as an increased percentage of Th17 cells in skin biopsies 12 Therapeutic ILblockade not only leads to inhibition of the ILmediated inflammatory symptoms but also to decreased numbers of Th17 cells, indicating that auto inflammatory dysregulation activates the adaptive immune system and that this activation is part of the autoinflammatory disease phenotype. Faites la connaissance de la personne de votre vie. Viens dialoguer sur la plus conviviale des messageries. Dialogue en direct, gratuit. Enfin une vraie messagerie!

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