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Piram, Maryam; Frenkel, Joost; Gattorno, Marco; Ozen, Seza; Lachmann, Helen J.; Goldbach-Mansky, Raphaela; Hentgen, Véronique; Neven, Bénédicte; Stojanovic, Katia Stankovic; Simon, Anna; Kuemmerle-Deschner, Jasmin; Hoffman, Hal; Stojanov, Silvia; Duquesne, Agnès; Pillet, Pascal; Martini, Alberto; Pouchot, Jacques; Koné-Paut, Isabelle (February 2011): A preliminary score for the assessment of disease activity in hereditary recurrent fevers: results from the AIDAI (Auto-Inflammatory Diseases Activity Index) Consensus Conference. In: Annals of the rheumatic diseases, Vol. 70, No. 2: pp. 309-314




The systemic autoinflammatory disorders (SAID) share many clinical manifestations, albeit with variable patterns, intensity and frequency. A common definition of disease activity would be rational and useful in the management of these lifelong diseases. Moreover, standardised disease activity scores are required for the assessment of new therapies in constant development. The aim of this study was to develop preliminary activity scores for familial Mediterranean fever, mevalonate kinase deficiency, tumour necrosis factor receptor-1-associated periodic syndrome and cryopyrin-associated periodic syndromes (CAPS).