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Nat Rev Rheumatol. 2013 Oct;9(10):624-9. doi: 10.1038/nrrheum.2013.101. Epub 2013 Jul 9.

How should we approach classification of autoinflammatory diseases?

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French Reference Centre for Amyloidosis of Inflammatory Origin and Familial Mediterranean Fever, Hôpital Tenon, Assistance Publique-Hôpitaux de Paris (AP-HP), 4 rue de la Chine, Paris, F-75020 France.


The notion of 'autoinflammatory' disease was introduced at the end of the 1990s, and, since then, this concept has rapidly evolved. As a result, multiple definitions of autoinflammatory disease, and classifications of conditions encompassed by these definitions, have been proposed; this succession highlights advances that have been made in understanding of the innate immune system, and especially the roles of IL-1β and the inflammasome in autoinflammtory conditions. However, the definitions and classifications that have been suggested to date face a number of structure and content issues. We therefore propose another, more clinically-oriented, definition: autoinflammatory diseases are diseases with clinical signs of inflammation, associated with elevated levels of acute-phase reactants, which are attributable to dysfunction of the innate immune system, genetically-determined or triggered by an endogenous factor. From this foundation, we propose a clinically-based classification of autoinflammatory diseases, and go on to discuss how immunological diseases as a whole, including autoimmune diseases, can be appropriately located within a continuum only if the classification process is multidimensional. For this purpose, we appeal to the philosophical concepts of family resemblance and signature.

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