How immunological profile drives clinical phenotype of primary Sjögren's syndrome at diagnosis: analysis of 10,500 patients (Sjögren Big Data Project).

WAHREN-HERLENIUS MARIE; DE VITA SALVATORE; HORVATH IF; ATZENI FABIOLA; PASOTO SG; BOMBARDIERI MICHELLE; GIACOMELLI ROBERTO; FRAILE, GUADALUPE; MARIETTE XAVIER; SANCHEZ-GUERRERO, JORGE; VALIM VALERIA; SIVILS KATHY; RISCHMUELLER, MAUREEN; SENE, DAMIEN; MOREL, JACQUES; KWOK, SEUNG-KI; RETAMOZO SOLEDAD; MOÇA TREVISANI, VIRGINIA; HERNANDEZ-MOLINA GABRIELA; DEVAUCHELLE-PENSEC, VALERIE; DANDA, DEBASHISH; BOOSTMA HENDRIKA; LI X; RAMOS CASALS MANUEL; PIOTR WILAND; KILIC L; ASTRID RASMUSSEN; SUZUKI YASUNORI; OLSSON P; ACAR-DENIZLI, NIHAN; BARTOLONI ELENA; VOLLENVEIDER, CRISTINA; KVARNSTRÖM MARIKA; GHEITA, TAMER A.; ARMAGAN BERKAN; HAMMENFORS DANIEL; QUARTUCCIO LUCA; KNOPF A; BALDINI CHIARA; TAKASHI NAKAMURA; MANDL THOMAS; NORDMARK GUNNEL; WAN-FAI NG; ISENBERG DAVID; SOLANS LAQUÉ R; PRAPROTNIK SONJA; KRUIZE AIKE; PRIORI ROBERTA; GOTTENBERG JAQUES; RAPHAELE SEROR; ZEHER MARGIT; BRITO ZERON PILAR

CLINICAL AND EXPERIMENTAL RHEUMATOLOGY

CLINICAL & EXPER RHEUMATOLOGY

Lugar: Pisa; Año: 2018

0392-856X

OBJECTIVES: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 harmonised 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 fulfillment 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 22 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 hypocomplementaemia and especially cryoglo-bulinaemia 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