How Immunological Profile Drives Clinical Phenotype of Primary Sjögren's Syndrome at Diagnosis: Analysis of 10.500 Patients (Sjögren Big Data Project)

ACAR-DENIZLI, NIHAN; ASTRID RASMUSSEN; XIAOMEI LI; DANDA, DEBASHISH; GABRIELA HERNANDEZ-MOLINA; SEUNG-KI KWOK; DAMIEN SÈNE; MAUREEN RISCHMUELLER; VALERIA VALIM; GUADALUPE FRAILE; ROBERTO GIACOMELLI; MICHELE BOMBARDIERI; SANDRA G PASOTO; JACQUES MOREL; KATHY L. SIVILS; JORGE SÁNCHEZ-GUERRERO; XAVIER MARIETTE; RETAMOZO SOLEDAD; MARGIT ZEHER; SEROR, RAPHAELE; GOTTENBERG JAQUES; PRIORI ROBERTA; AIKE A. KRUIZE; SONJA PRAPROTNIK; ROSER SOLANS; DAVID A. ISENBERG; GUNNEL NORDMARK; TAKASHI NAKAMURA; ANDREAS KNOPF; DANIEL S. HAMMENFORS; FABIOLA ATZENI; ILDIKO-FANNY HORVATH; SALVATORE DE VITA; MARIE WAHREN- HERLENIUS; WAN-FAI NG; BRITO ZERON PILAR; THOMAS MANDL; BALDINI CHIARA; LUCA QUARTUCCIO; BERKAN ARMAGAN; MARIKA KVARNSTRÖM; ELENA BARTOLONI; YASUNORI SUZUKI; PIOTR WILAND; HENDRIKA BOOTSMA; VALÉRIE DEVAUCHELLE-PENSEC; VIRGINIA FERNANDES TREVISANI; TAMER A. GHEITA; CRISTINA VOLLENVEIDER; PETER OLSSON; LEVENT KILIC; RAMOS CASALS MANUEL

Chicago

Congreso; 2018 ACR/ARHP ANNUAL MEETING; 2018

American College of Rheumatology

Background/Purpose: To evaluate the influence of the main immunological markers on the disease phenotype at diagnosis in a large international cohort of patients with primary Sjögren?s syndrome (SjS).Methods: The Big Data Sjögren Project Consortium is an international, multicentre registry created in 2014. As a first step, baseline clinical information from leading centres on clinical research in SjS of the 5 continents was collected. The centres 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. Immunological tests were carried out using standard commercial assays.Results: By January 2018, the participant centres had included 10,500 valid patients from 23 countries. The cohort included 9,806 (93%) women and 694 (7%) men, with a mean age at diagnosis of primary SjS of 53 years, mainly White (78%) and included from European countries (71%). 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. Positive autoantibodies (ANA, Ro, La) correlated with a positive result in salivary gland biopsy, while hypocomplementemia and especially cryoglobulinaemia correlated with systemic activity (mean ESSDAI score of 17.7 for cryoglobulins, 11.3 for low C3 and 9.2 for low C4, in comparison with 3.8 for negative markers). The immunological markers with a great number of statistically-significant associations (p