THU0517 THE LONGITUDINAL EUROFEVER PROJECT: AN UPDATE ON ENROLLMENT

Background In 2008 the Paediatric Rheumatology European Society (PReS) promoted an International Project for the study of Autoinflammatory Diseases (AIDs) named Eurofever, whose main purpose is to create a web-based registry for the collection of information in AIDs patients. Objectives To implement the Registry with the new recently described AIDs and to increase the collection of longitudinal data. Methods The data were extracted from the Eurofever registry, which is hosted in the PRINTO website (http://www. printo.it). From February 2015 we started the longitudinal collection of follow-up data with particular focus on treatment, modification of the clinical picture, onset of complication/adverse events. We have enrolled patients included in the registry up to 28 September 2018. Results Up to date 4175 patients have been enrolled (3843 of them with complete demographic information, 1903 M e 1940 F) from 62 countries. For 3356 (87%) patients also clinical data from onset to diagnosis, collected during the first visit performed at referred pediatric or adult center, are available. For each disease the number of enrolled patients is: FMF 1086 pts (951 with complete clinical data); TRAPS 273 pts (256 complete); CAPS 298 pts (279 complete); MKD 205 pts (190 complete); Blau’s disease 49 pts (26 complete); PAPA 42 pts (41 complete); NLRP12 mediated periodic fever 13 pts (11 complete); DADA2 14 pts (9 complete); DIRA 3 pts (all complete); SAVI 3 pts (all complete); CANDLE 1 pt (complete) and Majeed 4 pts (all complete). Among multifactorial autoinflammatory diseases: PFAPA 676 pts (551 complete); CNO 581 pts (540 complete); Behcet 214 pts (186 complete), undefined periodic fever 368 pts (292 complete) and Schnitzler 13 pts (all complete). The median onset age is 4 years (range 1 month – 75 years), the median diagnosis age is 8 years (range 1 month – 78 years). Most of patients (3509, 91%) presented disease onset during pediatric age ( Conclusion The enrollment in Eurofever Registry is still ongoing. The analysis of data will improve our knowledge both on the natural history of the single disease and on the efficacy/safety of treatment commonly used in the clinical practice. Disclosure of Interests: Ilaria Gueli: None declared, Martina Finetti: None declared, Fabrizio De Benedetti Grant/research support from: Abbvie, SOBI, Novimmune, Roche, Novartis, Sanofi, Pfizer, Jordi Anton Grant/research support from: JA has received grant/research support, consulting fees and/or honoraria from AbbVie, Alexion, BMS, ChemoCentryx, Gebro, GSK, Novartis, Novimmune, Pfizer, Roche, Sanofi and Sobi, Consultant for: JA has received grant/research support, consulting fees and/or honoraria from AbbVie, Alexion, BMS, ChemoCentryx, Gebro, GSK, Novartis, Novimmune, Pfizer, Roche, Sanofi and Sobi, Maria Alessio: None declared, Joost Frenkel: None declared, Luca Cantarini: None declared, Romina Gallizzi: None declared, Judith Sanchez Manubens: None declared, Marco Cattalini: None declared, Efimia Papadopoulou-Alataki: None declared, Rolando Cimaz: None declared, Donato Rigante: None declared, Alma Nunzia Olivieri: None declared, Pavla Dolezalova: None declared, Alberto Martini Consultant for: I do not have any conflict of interest to declare since starting from 1 March 2016 I became the Scientific Director of the G. Gaslini Hospital; therefore, my role does not allow me to render private consultancies resulting in personal income. I perform consultancy activities on behalf of the Gaslini Institute for the companies listed below: AbbVie, Biogen, Boehringer Ingelheim, Bristol-Myers Squibb, EMD Serono, Janssen, Novartis, Pfizer, R-Pharm. The money received for these activities are directly transferred to the Gaslini Institute’s bank account. Before March 2016, I was the head of the Pediatric Rheumatology Department at the G. Gaslini Hospital, where the PRINTO Coordinating Centre is located. For the coordination activity of the PRINTO network, the Gaslini Hospital received contributions from the industries listed in this section. This money has been reinvested for the research activities of the hospital in fully independent manners besides any commitment with third parties., Nicolino Ruperto Grant/research support from: The Gaslini Hospital, where NR works as full-time public employee, has received contributions (> 10.000 USD each) from the following industries in the last 3 years: BMS, Eli-Lilly, GlaxoSmithKline, F Hoffmann-La Roche, Janssen, Novartis, Pfizer, Sobi. This funding has been reinvested for the research activities of the hospital in a fully independent manner, without any commitment with third parties., Consultant for: Received honoraria for consultancies or speaker bureaus (