PReS-FINAL-2194: Evidence-based clinical classification criteria for periodic fevers
نویسندگان
چکیده
Methods All FMF, TRAPS, MKD and CAPS patients enrolled in the Eurofever registry until March 2013 were evaluated. For each disease gold standards were considered according to the following criteria: i) clinical validation by centers and disease-principal investigator, ii) confirmative molecular analysis (2 mutations for MEFV with at least one mutation in exon 10, 2 mutations of MVK gene, 1 mutation of TNFRFS1A with exclusion of low-penetrance variants, 1 mutation of NLRP3 with exclusion of low-penetrance variants), iii) PFAPA patients validated by disease-principal investigator and confirmed by the centers on the basis of the follow-up. Clinical criteria were formulated on the basis of a univariate and multivariate analysis in a first group of patients (training set) and then validated in an independent set of patients (validation set).
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How experts on autoinflammatory diseases classify inherited periodic fevers: preliminary results of the Eurofever Delphi Survey
Background Provisional evidence-based classification criteria for Familial Mediterranean Fever (FMF), Cryopyrin Associated Periodic Syndrome (CAPS), Tumor Necrosis factor Receptor Associated Periodic Syndrome (TRAPS) and Mevalonate Kinase Deficiency (MKD) have been recently developed based on data coming from the Eurofever registry. However, no consensus on how to combine clinical criteria with...
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