LYMPHOMA ARISING AT THE TIME OF DIAGNOSIS OF PRIMARY SJÖGREN SYNDROME: A HIGHLY-ACTIVE SYSTEMIC SUBSET OF THE DISEASE

ACAR-DENIZLI, NIHAN; ASTRID RASMUSSEN; BALDINI CHIARA; QUARTUCCIO LUCA; BERKAN ARMAGAN; MARIKA KVARNSTROM; WAN FAI NG; ROSER SOLANS-LAQUÉ; RAPHAELE SEROR; YASUNORI SUZUKI; JACQUES-ERIC GOTTENBERG; AGATA SEBASTIAN; ROBERTA PRIORI; HIDEKI NAKAMURA; AIKE A. KRUIZE; BENEDIKT HOFAUER; SONJA PRAPROTNIK; DANIEL HAMMENFORS; MAUREEN RISCHMUELLER; FABIOLA ATZENI; DAVID ISENBERG; SANDRA CONSANI-FERNÁNDEZ; RETAMOZO SOLEDAD; BRITO ZERON PILAR; GUNNEL NORDMARK; ANTÓNIA SZÁNTÓ; ROBERTO GIACOMELLI; LI XIAOMEI; MICHELE BOMBARDIERI; PULUKOOL SANDHYA; SANDRA PASOTO; GABRIELA HERNANDEZ-MOLINA; JACQUES MOREL; SEUNG-KI KWOK; MARIETTE XAVIER; DAMIEN SENE; THOMAS MANDL; ELENA BARTOLONI BOCCI; VALERIA VALIM; HENDRIKA BOOTSMA; VALERIE DEVAUCHELLE-PENSEC; VIRGINIA FERNANDES MOÇA TREVISANI; TAMER A GHEITA; CRISTINA VOLLENVEIDER; RAMOS CASALS MANUEL

Madrid

Congreso; EULAR: Annual European Congress of Rheumatology; 2019

EUROPEAN LEAGUE AGAINST RHEUMATISM (EULAR)

Objectives: To analyse the phenotype of patients with primary Sjogren syndrome(SjS) in whom a lymphoproliferative disease is diagnosed concomitantly.Methods: By January 2019, The Big Data Sjögren Project included 11,420 consecutive patients with primary SjS recruited from 24 countries of the fivecontinents.Results: 117 (1%) patients were diagnosed with lymphoma and primary SjS synchronously. Age-gender adjusted multivariate analysis identified the following features associated with lymphoma (OR; CI95%): male gender (4.61; 2.88-7.18), White ethnicity (3.51; 1.78-7.91), abnormal oral tests (3.4; 1.38-10.88), positive biopsy (3.2; 1.3-10.17), positive RF (2.27; 1.48-3.53), hypocomplementemia (3.39; 2.06-5.54), and cryoglobulins (4.74; 2.57-8.38). Activity (score > 1) in the constitutional (2.97; 1.86-4.62), glandular (3.11; 2.1-4.57), cutaneous (2.17; 1.28-3.52), peripheral nerve (2.56; 1.4-4.41) and hematological (2.49; 1.64-3.75) ESSDAI domains was associated with lymphoma Conclusion: Patients diagnosed concomitantly with primary SjS and lymphomahave a very specific, highly-active phenotype (men, White, severe oral nvolvement, cryoglobulinemic-related immunological markers, and high systemicactivity).