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Ann Rheum Dis. 2018 Nov;77(11):1558-1565. doi: 10.1136/annrheumdis-2017-212515. Epub 2018 Aug 12.

Consensus proposal for taxonomy and definition of the autoinflammatory diseases (AIDs): a Delphi study.

Author information

Rheumatology Unit, Hadassah Hebrew University Medical Center, Jerusalem, Israel.
UOSD Centro Malattie Autoinfiammatorie e Immunodeficienze, Istituto Giannina Gaslini, Genoa, Italy.
Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
Inflammatory Disease Section, Metabolic, Cardiovascular and Inflammatory Disease Genomics Branch, National Human Genome Research Institute, Bethesda, Maryland, USA.
Division of Medicine, Centre for Amyloidosis & Acute Phase Proteins, University College London Medical School, London, UK.
CEREMAIA, CHU Montpellier, INSERM U1183, Université de Montpellier, Montpellier, France.
Clinica Pediatrica e Reumatologia-PRINTO, Istituto Giannina Gaslini, Genoa, Italy.


Autoinflammatory diseases (AIDs) are a relatively new family of disorders, defined about 19 years ago. Some of them are hereditary and some are not. The names given to these diseases do not follow any systematic guidelines, and sometimes the same disorder carries several names. The aim of this study is to refine the definition of AIDs and to provide some conventions for their naming. We focused mainly on monogenetic AIDs. Delphi technique, which enables consensus among a group of experts through internet and mail communication and questionnaires, was employed. After achieving 100% consensus among six members of a steering committee, the questionnaire containing AID definitions and the agreed-upon conventions were sent to 26 physicians and researchers working in the field of AIDs in order to gain broader support for the committee's proposals. The committee proposed the following definition for AIDs: "Autoinflammatory diseases are clinical disorders caused by defect(s) or dysregulation of the innate immune system, characterized by recurrent or continuous inflammation (elevated acute phase reactants-APR) and the lack of a primary pathogenic role for the adaptive immune system (autoreactive T-cells or autoantibody production)." Several rules were defined for guiding the naming of these diseases among which are: abandoning eponyms and preferring the name of the gene over its encoded protein. The new definition for AIDs allows inclusion of clinical disorders mainly associated with defects in the innate immune system. The new conventions propose names with clinical meaning and in some cases even clues for treatment.


autoimmune diseases; familial mediterranean fever; fever syndromes; inflammation

[Indexed for MEDLINE]

Conflict of interest statement

Competing interests: For EB-C, MG, AG, DLK, HJL, IT: Nothing to disclose for this manuscript. NR received honoraria for consultancy of speaker’s bureau from the following pharmaceutical companies since last 5 years: Abbott, AbbVie, Amgen, Biogenidec, Astellas, Alter, AstraZeneca, Baxalta Biosimilars, Boehringer, BMS, CD Pharma, Celgene, CrescendoBio, EMD Serono, Hoffman La Roche, Italfarmaco, Janssen, MedImmune, Medac, Novartis, Novo Nordisk, Pfizer, Rewind Arms, R Pharma, Sanofi Aventis, Servier, Sinergie, Takeda, Vertex, UCB Biosciences GmbH. The Gaslini Hospital, which is the public Hospital where NR works as full time public employee, has received contributions from the following industries: Abbott, BMS, ‘Francesco Angelini’, GlaxoSmithKline (GSK), Hoffman La Roche, Italfarmaco, Janssen, Novartis, Pfizer, Sanofi Aventis, Schwarz Biosciences, Sobi, Xoma, Wyeth. This money has been reinvested for the research activities of the hospital in a fully independent manner without any commitment with third parties.

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