OP0258 LESSON FROM EUROFEVER REGISTRY AFTER THE FIRST TEN YEARS OF ENROLLMENT. (June 2019)

• Record Type: Journal Article
• Authors: Finetti, Martina; Gueli, Ilaria; Frenkel, Joost; Özen, Seza; Lachmann, Helen J.; Benedetti, Fabrizio De; Koné-Paut, Isabelle; Wouters, Carine; Brogan, Paul; Girschick, Hermann; Neven, Benedicte; Martini, Alberto; Ruperto, Nicolino; Gattorno, Marco
• Is Part Of: Annals of the rheumatic diseases. Volume 78(2019)Supplement 2
• Page Start: 209
• Page End: 210
• Publication Date: 2019-06
• Subjects: Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2019-eular.3743
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : 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 assess the impact of the Eurofever Registry on scientific community with particular interest in the geographical coverage, diagnostic delay, access to treatment and pubblications. Methods: The data analyzed in the study were extracted from the Eurofever registry, which is hosted in the PRINTO website. Results: Up to date 4175 patients have been enrolled from 62 countries (3843 of them with complete demographic data). Most of patients (72%) are resident in Western Europe, 8% in Central-Eastern Europe, 11% in Southern-Eastern Mediterranean, 2% in South America and 7% in other countries. Compared to the first Eurofever report (Toplak et al, 2012) we have observed an increase of enrolled patients from 1388 to 2651 in Western Europe, from 106 to 313 in Central-Eastern Europe, from 294 to 406 in Southern-Eastern Mediterranean. The median onset age is 4 years (range 1 month – 75 years), the median diagnosis age is 8 years (range 1 month – 78 years). The median diagnostic delay observed in 2012 was 7.3 years (range 0.3–76), from patients enrolled after 2012 it was 1.9 years (range 0-57). Comparing the mean diagnostic delay from 1980 to 2018, we have observed an encouraging constant reduction of period between AIDs onset e diagnosis (from a mean diagnostic delay value of 20 years for patients born before 1980, to a mean value of 1 year for patients born after 2011, Figure 1). Complete information on access to treatment were available in 2430 patients. DMARDs were used in 1031 (42%), biologics in 396 (16%) patients. According to the number of enrolled patients, biologics were used in 361/1782 (20%) of Western europeans, 17/342 (5%) of Central-Eastern europeans, 6/259 (2%) of Southern-Eastern Mediterranean patients. Regarding Eurofever impact on Scientific Community, during this first 10 years the Registry provided 12 papers with more than 800 citations. Detailed analysis of clinical features collected in Eurofever database allowed to perform studies with large cohort of patients, to purpose new classification criteria (Federici et al, 2015), to validate damage and activity score (Piram et al, 2014 and N Ter Haar et al, 2017) and to evaluated genotype/phenotype correlation (Papa et al, 2017). Conclusion: In the last years we have observed an encouraging increase of involved Countries, with a greater number of patients coming from geographic area poorly represented in the first epidemiologic study of Toplak et al. Eurofever data analysis has confirmed an improvement of diagnostic ability during the last years, with a significant reduction of mean diagnostic delay. Longterm studies will help understand the efficacy and safety of different treatments used in these rare conditions. Disclosure of Interests: Martina Finetti: None declared, Ilaria Gueli: None declared, Joost Frenkel: None declared, Seza Özen Consultant for: Seza Ozen is receiving consultancy fees from Novartis, Speakers bureau: Roche, Helen J. Lachmann Grant/research support from: SOBI, Novartis, Consultant for: Novartis, Takeda, Speakers bureau: SOBI. Novartis, Fabrizio De Benedetti Grant/research support from: Abbvie, SOBI, Novimmune, Roche, Novartis, Sanofi, Pfizer, Isabelle Koné-Paut: None declared, Carine Wouters Grant/research support from: Grant/research support to Istituto Gaslini from GlaxoSmithKline immune-inflammation: unrestricted grant to study Blau syndrome; Roche: unrestricted research grant; Pfizer: grant for psychological care of patients with JIA, Grant/research support from: GSK, Roche, Pfizer, Paul Brogan Grant/research support from: SOBI, Novartis, Roche, Novimmune, Chemocentryx, Consultant for: Roche, SOBI, Speakers bureau: SOBI, Roche, Novartis, UCB, Hermann Girschick: None declared, Benedicte Neven: 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 (< 10.000 USD each) from the following pharmaceutical companies in the past 3 years: Ablynx, AbbVie, Astrazeneca-Medimmune, Biogen, Boehringer, Bristol-Myers Squibb, Eli-Lilly, EMD Serono, GlaxoSmithKline, Hoffmann-La Roche, Janssen, Merck, Novartis, Pfizer, R-Pharma, SanofiServier, Sinergie, Sobi and Takeda., Speakers bureau: Received honoraria for consultancies or speaker bureaus (< 10.000 USD each) from the following pharmaceutical companies in the past 3 years: Ablynx, AbbVie, Astrazeneca-Medimmune, Biogen, Boehringer, Bristol-Myers Squibb, Eli-Lilly, EMD Serono, GlaxoSmithKline, Hoffmann-La Roche, Janssen, Merck, Novartis, Pfizer, R-Pharma, SanofiServier, Sinergie, Sobi and Takeda., Marco Gattorno Grant/research support from: MG has received unrestricted grants from Sobi and Novartis