TY - JOUR T1 - A preliminary score for the assessment of disease activity in hereditary recurrent fevers: results from the AIDAI (Auto-Inflammatory Diseases Activity Index) Consensus Conference JF - Annals of the Rheumatic Diseases JO - Ann Rheum Dis SP - 309 LP - 314 DO - 10.1136/ard.2010.132613 VL - 70 IS - 2 AU - Maryam Piram AU - Joost Frenkel AU - Marco Gattorno AU - Seza Ozen AU - Helen J Lachmann AU - Raphaela Goldbach-Mansky AU - Véronique Hentgen AU - Bénédicte Neven AU - Katia Stankovic Stojanovic AU - Anna Simon AU - Jasmin Kuemmerle-Deschner AU - Hal Hoffman AU - Silvia Stojanov AU - Agnès Duquesne AU - Pascal Pillet AU - Alberto Martini AU - Jacques Pouchot AU - Isabelle Koné-Paut Y1 - 2011/02/01 UR - http://ard.bmj.com/content/70/2/309.abstract N2 - Background 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). Methods The study was conducted using two well-recognised consensus formation methods: the Delphi technique and the nominal group technique. The results from a two-step survey and data from parent/patient interviews were used as preliminary data to develop the agenda for a consensus conference to build a provisional scoring system. Results 24 of 65 experts in SAID from 20 countries answered the web questionnaire and 16 attended the consensus conference. There was consensus agreement to develop separate activity scores for each disease but with a common format based on patient diaries. Fever and disease-specific clinical variables were scored according to their severity. A final score was generated by summing the score of all the variables divided by the number of days over which the diary was completed. Scores varied from 0 to 16 (0–13 in CAPS). These scores were developed for the purpose of clinical studies but could be used in clinical practice. Conclusion Using widely recognised consensus formation techniques, preliminary scores were obtained to measure disease activity in four main SAID. Further prospective validation study of this instrument will follow. ER -