Table 2

Results from the Delphi questionnaires for consensus on nomenclature

Definition or diseaseGroup of AIDs experts consensus
 Autoinflammatory diseases are clinical disorders caused by defect(s) or dysregulation of the innate immune system, characterised by recurrent or continuous inflammation (elevated APR) and by the lack of a primary pathogenic role of the adaptive immune system (autoreactive T-cells or autoantibody production).87%
Final names proposed for the AIDs
CARD14-associated psoriasis91%
 CNO: Chronic non-bacterial osteomyelitis87%
 DIRA: Deficiency of the IL-1 receptor antagonist96%
 DITRA: Deficiency of the IL-36 receptor antagonist96%
 IL-10 deficiency-associated inflammatory bowel diseases83%
 PAAD: Pyrin-associated autoinflammatory disease:
 MKD: Mevalonate kinase deficiency87%
NLRP3-AID—NLRP3-associated autoinflammatory disease88%
NLRP12-AID—NLRP12-associated autoinflammatory disease88%
NOD2-associated granulomatous diseases83%
 PAPA: PSTPIP1-associated arthritis, pyoderma gangrenosum and acne87%
 PFAPA: Periodic fever, aphthous stomatitis, pharyngitis and adenitis83%
 PRAAS: Proteasome-associated autoinflammatory syndrome84%
 Schnitzler syndrome87%
 SDCM—SH3BP2 deficiency with multilocular cystic disease of the mandibles94%
 TRAPS—TNF receptor-associated periodic fever syndrome83%
  • AIDs, autoinflammatory disease; APR, acute phase reactants; PAAND, pyrin-associated autoinflammation with neutrophilic dermatosis.