An International Delphi Survey for the Definition of New Classification Criteria for Familial Mediterranean Fever, Mevalonate Kinase Deficiency, TNF Receptortextendashassociated Periodic Fever Syndromes, and Cryopyrin-associated Periodic Syndrome

Silvia Federici, Federica Vanoni, Eldad Ben-Chetrit, Luca Cantarini, Joost Frenkel, Raphaela Goldbach-Mansky, Ahmet Gul, Hal Hoffman, Isabelle Koné-Paut, Jasmin Kuemmerle-Deschner, Helen J. Lachmann, Alberto Martini, Laura Obici, Seza Ozen, Anna Simon, Michael Hofer, Nicolino Ruperto, Marco Gattorno

Research output: Contribution to journalArticlepeer-review


Objective. Provisional evidence-based classification criteria for hereditary periodic fever (HPF) have been recently developed. However, no consensus on how to combine clinical criteria, laboratory tests, and results of molecular analysis has been reached. The objective of this study is to understand which variables physicians consider important for the classification of patients with HPF.Methods. Two Delphi surveys were sent to health professionals in the field of autoinflammation. In the first open survey, 124 researchers could list all the variables they consider useful for the diagnosis of each monogenic periodic fever. The variables could be of any type and each researcher could complete the survey for 1 or more diseases. In the second survey, 162 researchers were asked to select, from a list of items coming from the first survey, the 10 top variables and to rank them by assigning a score from 10 to 1.Results. The response rates to the Delphi surveys were 85% for the first session and 87% for the second. The variables selected for each disease (corresponding to the third quartile, considering the total score obtained by the variables after the second Delphi survey) were 21 for mevalonate kinase deficiency, 22 for cryopyrinopathies, 18 for familial Mediterranean fever, and 20 for tumor necrosis factor receptortextendashassociated periodic fever syndrome. A positive genetic test reached the top rank in all the HPF.Conclusion. Our process led to the identification of those features considered the most important as candidate variables to be included in a new set of evidence-based classification criteria for HPF.
Original languageUndefined/Unknown
Pages (from-to)429-436
Number of pages8
JournalThe Journal of rheumatology
Issue number4
Publication statusPublished - 2019

Cite this