Epidemiologic subsets drive a differentiated clinical and immunological presentation of Primary Sjögren Syndrome: analysis of 9302 patients from the Big Data International Sjögren Cohort

BRITO ZERON PILAR; RAPHAELE SEROR; CHIARA BALDINI; ROBERTA PRIORI; AIKE A. KRUIZE; SONJA PRAPROTNIK; ROSER SOLANS; MAUREEN RISCHMUELLER; PIOTR WILAND; VALERIA VALIM; MARGIT ZEHER ; BENEDIKT HOFAUER; THOMAS MANDL; DANIEL HAMMENFORS; JACQUES-ERIC GOTTENBERG; JACQUES MOREL; LUCA QUARTUCCIO; CRISTINA VOLLENVEIDER; SEUNG-KI KWOK; RAMOS CASALS MANUEL; DAMIEN SENE; YASUNORI SUZUKI; RETAMOZO SOLEDAD; GUNNEL NORDMARK; KATHY SIVILS; HENDRIKA BOOTSMA; XIAOMEI LI; ROBERTO GIACOMELLI; DEBASHISH DANDA; MICHELE BOMBARDIERI; GABRIELA HERNANDEZ-MOLINA; STEVEN E. CARSONS; MARIE WAHREN-HERLENIUS; TAMER A GHEITA; ROBERTO GERLI; BELCHIN KOSTOV; DAVID ISENBERG; GUADALUPE FRAILE; TAKASHI NAKAMURA; VALERIE DEVAUCHELLE-PENSEC; VIRGINIA FERNANDES MOÇA TREVISANI; SANDRA G. PASOTO; FABIOLA ATZENI; XAVIER MARIETTE

San Diego

Congreso; ACR / ARHP ANNUAL MEETING; 2017

American College of Rheumatology

OBJECTIVE. To analyse whether epidemiologic factors (such as gender or age at diagnosis of the disease) are associated with particular disease expressions and define some specific subsets in patients with primary Sjögren syndrome (SS). PATIENTS. The Big Data Sjögren project is an international, multicentre registry formed in 2014 to take a ?high-definition? picture of the main features of primary SS at diagnosis by merging international SS databases using a Data-Sharing methodological approach. By January 2017, the database included 9302 consecutive patients recruited from 21 countries of the five continents. The main features at diagnosis (time of criteria fulfilment) or at recruitment were collected and analysed.RESULTS. Of the 9032 patients, 8680 (93%) were women and 622 (7%) were men with a mean age at diagnosis of primary SS of 50 years; 76% were Caucasian. The frequency of fulfilment of the 2002 criteria was: 92% for dry eye, 93% for dry mouth, 88% for positive salivary gland biopsy, 93% for positive ocular tests, 85% for positive oral tests and 71% for positive Ro/La autoantibodies. Other immunological tests included positive ANA (81%), RF (49%), low C4 levels (13%), low C3 levels (14%) and cryoglobulins (7%). Men with primary SS presented a higher frequency of White ethnicity (83% vs 76% in women, p < 0.001) and rheumatoid factor (54% vs 49%, p = 0.017), and a lower frequency of dry eyes (89% vs 92%, p = 0.011) and dry mouth (90% vs 94%, p = 0.026) in the multivariate modelanalysis. Patients with a younger onset (< 35 years) showed a lower frequency of White ethnicity (69% vs 77% in aged > 35 yrs, p < 0.001), dry eyes (86% vs 93%, p < 0.001) and positive ocular tests (81% vs 85%, p = 0.001), and a higher frequency of anti-Ro/La autoantibodies (84% vs 70%, p < 0.001), ANA (89% vs 80%, p < 0.001), RF (62% vs 47%, p < 0.001) and low C3 levels (19% vs 13%, p < 0.001) in the multivariate model analysis. Patients with an elderly onset (> 70 years) showed a higher frequency of White ethnicity (85% vs 75% in aged < 70 yrs, p < 0.001), positive oral tests (82% vs 76%, p = 0.003) and a lower frequency of anti-Ro/La autoantibodies (62% vs 72%, p < 0.001) and low C3 levels (9% vs 14%, p < 0.001) in the multivariate model analysis. CONCLUSION. In the largest reported cohort of primary SS patients diagnosed homogeneously around the world according to the 2002 AE criteria, we found that primary SS is a disease that can be presented heterogeneously at diagnosis, depending on specific epidemiologic features such as gender, age and ethnicity.