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Annals of the rheumatic diseases · 2019

Classification criteria for autoinflammatory recurrent fevers.

СтатьяПоддержка исследования: вне правительства СШАОбзорНазвание и аннотация: Перевод пока не подготовлен
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Gattorno Marco, Hofer Michael, Federici Silvia, Vanoni Federica, Bovis Francesca, Aksentijevich Ivona, Anton Jordi, Arostegui Juan Ignacio, Barron Karyl, Ben-Cherit Eldad, Brogan Paul A, Cantarini Luca, Ceccherini Isabella, De Benedetti Fabrizio, Dedeoglu Fatma, Demirkaya Erkan, Frenkel Joost, Goldbach-Mansky Raphaela, Gul Ahmet, Hentgen Veronique, Hoffman Hal, Kallinich Tilmann, Kone-Paut Isabelle, Kuemmerle-Deschner Jasmin, Lachmann Helen J, Laxer Ronald M, Livneh Avi, Obici Laura, Ozen Seza, Rowczenio Dorota, Russo Ricardo, Shinar Yael, Simon Anna, Toplak Nataša, Touitou Isabelle, Uziel Yosef, van Gijn Marielle, Foell Dirk, Garassino Claudia, Kastner Dan, Martini Alberto, Sormani Maria Pia, Ruperto Nicolino, Eurofever Registry and the Paediatric Rheumatology International Trials Organisation (PRINTO)

Аннотация

BACKGROUND: 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.

Предметные рубрики MeSH

ИП Урсолов Владлен Сергеевич

ИНН 500118482352 · ОГРНИП 314500117700049

Реквизиты и контакты