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dc.contributor.authorGattorno, Marco
dc.contributor.authorHofer, Michael
dc.contributor.authorFederici, Silvia
dc.contributor.authorVanoni, Federica
dc.contributor.authorBovis, Francesca
dc.contributor.authorAksentijevich, Ivona
dc.contributor.authorAnton, Jordi
dc.contributor.authorIgnacio Arostegui, Juan
dc.contributor.authorBarron, Karyl
dc.contributor.authorBen-Cherit, Eldad
dc.contributor.authorBrogan, Paul A.
dc.contributor.authorCantarini, Luca
dc.contributor.authorCeccherini, Isabella
dc.contributor.authorDe Benedetti, Fabrizio
dc.contributor.authorDedeoglu, Fatma
dc.contributor.authorDemirkaya, Erkan
dc.contributor.authorFrenkel, Joost
dc.contributor.authorGoldbach-Mansky, Raphaela
dc.contributor.authorGul, Ahmet
dc.contributor.authorHentgen, Veronique
dc.contributor.authorHoffman, Hal
dc.contributor.authorKallinich, Tilmann
dc.contributor.authorKone-Paut, Isabelle
dc.contributor.authorKuemmerle-Deschner, Jasmin
dc.contributor.authorLachmann, Helen J.
dc.contributor.authorLaxer, Ronald M.
dc.contributor.authorLivneh, Avi
dc.contributor.authorObici, Laura
dc.contributor.authorOzen, Seza
dc.contributor.authorRowczenio, Dorota
dc.contributor.authorRusso, Ricardo
dc.contributor.authorShinar, Yael
dc.contributor.authorSimon, Anna
dc.contributor.authorToplak, Natasa
dc.contributor.authorTouitou, Isabelle
dc.contributor.authorUziel, Yosef
dc.contributor.authorvan Gijn, Marielle
dc.contributor.authorFoell, Dirk
dc.contributor.authorGarassino, Claudia
dc.contributor.authorKastner, Dan
dc.contributor.authorMartini, Alberto
dc.contributor.authorSormani, Maria Pia
dc.contributor.authorRuperto, Nicolino
dc.contributor.authorPaediat, Eurofever Registry
dc.date.accessioned2021-06-02T10:39:32Z
dc.date.available2021-06-02T10:39:32Z
dc.date.issued2019
dc.identifier.issn0003-4967
dc.identifier.urihttp://dx.doi.org/10.1136/annrheumdis-2019-215048
dc.identifier.urihttp://hdl.handle.net/11655/23779
dc.description.abstractBackground Different diagnostic and classification criteria are available for hereditary recurrent fevers (HRF)-familial Mediterranean fever (FMF), tumour necrosis factor receptor-associated periodic fever syndrome (TRAPS), mevalonate kinase deficiency (MKD) and cryopyrin-associated periodic syndromes (CAPS)-and for the non-hereditary, periodic fever, aphthosis, pharyngitis and adenitis (PFAPA). We aimed to develop and validate new evidence-based classification criteria for HRF/PFAPA. Methods Step 1: selection of clinical, laboratory and genetic candidate variables; step 2: classification of 360 random patients from the Eurofever Registry by a panel of 25 clinicians and 8 geneticists blinded to patients' diagnosis (consensus >= 80%); step 3: statistical analysis for the selection of the best candidate classification criteria; step 4: nominal group technique consensus conference with 33 panellists for the discussion and selection of the final classification criteria; step 5: cross-sectional validation of the novel criteria. Results The panellists achieved consensus to classify 281 of 360 (78%) patients (32 CAPS, 36 FMF, 56 MKD, 37 PFAPA, 39 TRAPS, 81 undefined recurrent fever). Consensus was reached for two sets of criteria for each HRF, one including genetic and clinical variables, the other with clinical variables only, plus new criteria for PFAPA. The four HRF criteria demonstrated sensitivity of 0.94-1 and specificity of 0.95-1; for PFAPA, criteria sensitivity and specificity were 0.97 and 0.93, respectively. Validation of these criteria in an independent data set of 1018 patients shows a high accuracy (from 0.81 to 0.98). Conclusion Eurofever proposes a novel set of validated classification criteria for HRF and PFAPA with high sensitivity and specificity.
dc.language.isoen
dc.relation.isversionof10.1136/annrheumdis-2019-215048
dc.rightsAttribution 4.0 United States
dc.rightsinfo:eu-repo/semantics/openAccess
dc.rights.urihttps://creativecommons.org/licenses/by/4.0/
dc.titleClassification Criteria For Autoinflammatory Recurrent Fevers
dc.typeinfo:eu-repo/semantics/article
dc.typeinfo:eu-repo/semantics/publishedVersion
dc.relation.journalAnnals Of The Rheumatic Diseases
dc.contributor.departmentÇocuk Sağlığı ve Hastalıkları
dc.identifier.volume78
dc.identifier.issue8
dc.description.indexWoS
dc.description.indexScopus


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