title: How should we approach classification of autoinflammatory diseases?
abstract: 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.
authors.affiliation: 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.
authors.forename: Gilles
authors.initials: G
authors.lastname: Grateau
authors.forename: Véronique
authors.initials: V
authors.lastname: Hentgen
authors.forename: Katia Stankovic
authors.initials: KS
authors.lastname: Stojanovic
authors.forename: Isabelle
authors.initials: I
authors.lastname: Jéru
authors.forename: Serge
authors.initials: S
authors.lastname: Amselem
authors.forename: Olivier
authors.initials: O
authors.lastname: Steichen
mesh_terms: D001327:Autoimmune Diseases / Q000145:classification* / Q000503:physiopathology; D056660:Hereditary Autoinflammatory Diseases / Q000145:classification* / Q000503:physiopathology; D006801:Humans; D007113:Immunity, Innate / Q000502:physiology; D058847:Inflammasomes / Q000502:physiology; D053583:Interleukin-1beta / Q000502:physiology; D012216:Rheumatic Diseases / Q000145:classification* / Q000503:physiopathology; D015398:Signal Transduction / Q000502:physiology
chemical_list: D058847:Inflammasomes; D053583:Interleukin-1beta