Sjögren Big Data Project, the first example of data sharing in autoimmune diseases: analysis of 10475 worldwide patients with primary Sjögren syndrome

NIHAN ACAR-DENIZLI; ASTRID RASMUSSEN; XIAOMEI LI; DEBASHISH DANDA; GABRIELA HERNANDEZ-MOLINA; SEUNG-KI KWOK; DAMIEN SENE; MAUREEN RISCHMUELLER; VALERIA VALIM; GUADALUPE FRAILE; ROBERTO GIACOMELLI; MICHELE BOMBARDIERI; TAMER A GHEITA; CRISTINA VOLLENVEIDER; RETAMOZO SOLEDAD; MARGIT ZEHER; RAPHAELE SEROR; JACQUES-ERIC GOTTENBERG; ANTONINA MINNITI; AIKE A. KRUIZE; SONJA PRAPROTNIK; ROSER SOLANS; DAVID ISENBERG; GUNNEL NORDMARK; TAKASHI NAKAMURA; BENEDIKT HOFAUER; W. FAI NG; SANDRA G. PASOTO; THOMAS MANDL; JACQUES MOREL; CHIARA BALDINI; RAMOS CASALS MANUEL; LUCA QUARTUCCIO; UMUT KALYONCU; MARIE WAHREN-HERLENIUS; ELENA BARTOLONI; YASUNORI SUZUKI; PIOTR WILAND; HENDRIKA BOOTSMA; VALERIE DEVAUCHELLE-PENSEC; DANIEL HAMMENFORS; FABIOLA ATZENI; BRITO ZERON PILAR

Amsterdam

Congreso; EUROPEAN LEAGUE AGAINST RHEUMATISM (EULAR); 2018

EULAR

Objective. To take a ?high-definition? picture of the main features of primary Sjögren syndrome (SjS) following a worldwide data-sharing cooperative merging of international clinical SjS databases. Methods. The Big Data Sjögren Project Consortium is an international, multicentre registry created in 2014. As a first step, baseline clinical information from leading centers on clinical research in SjS of the 5 continents was collected. The centers shared a harmonized data architecture and conducted cooperative online efforts in order to refine collected data under the coordination of a big data statistical team. Inclusion criteria were the fulfilment of the 2002 classification criteria.Patients. By January 2018, the participant centres had included 10475 valid patients from 22 countries, including 7637 (72.9%) patients from Europe, 1420 (13.6%) from America, 1186 (11.3%) from Asia, 167 (1.6%) from Australia and 65 (0.6%) from Africa. The cohort included 9781 (93.4%) women and 694 (6.6%) men (female: male ratio, 14:1), with a mean age at diagnosis of primary SjS of 53 (SD 14.1) years. The frequencies of fulfilment of the 2002 classification criteria items were 92.2% for dry eye (item I), 93.6% for dry mouth (item II), 83.2% for abnormal ocular tests (item III), 81.8% for positive minor salivary gland biopsy (item IV), 78.5% for abnormal oral diagnostic tests (item V) and 75.6% for positive anti-Ro/La antibodies (item VI). The frequency of positive immunological markers at diagnosis was 79.3% for ANA, 73.2% for anti-Ro, 48.6% for RF, 45.1% for anti-La, 13.4% for low C3 levels, 14.5% for low C4 levels and 7.3% for cryoglobulins.Conclusion. International data sharing-based projects merging disperse clinical registries may be essential tools to increase current knowledge and to improve patient care in specific systemic autoimmune diseases.