Systemic Sjögren presenting without sicca syndrome: characterization of 240 patients according to the new 2017 ACR/ EULAR Classification Criteria

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

Washington DC

Simposio; 14th International Symposium on Sjögren's Syndrome; 2018

Johns Hopkins University School of Medicine

Background. To analyse the epidemiological, clinical and immunological characteristics of patients presenting with systemic disease in the absence of sicca manifestations in a large international cohort of patients diagnosedwith primary Sjögren syndrome (SS). Methods. The new 2017 ACR/EULAR Classification Criteria for SS allows the application of the criteria to any patient with a suspicion of SS due to systemic features with at least 1 positive ESSDAI domain. We have identifiedhow many patients from the Big Data Sjögren Registry showed this early systemic presentation at the time of the disease diagnosis. As a control group, we selected those patients who presented with the typical sicca syndrome(subjective dry mouth and dry eyes).Results. We have identified 240 (2.5%) patients presenting with a nonsicca systemic disease among the 9545 patients included in the Registry: 211 (88%) were women and 29 (12%) were men (female: male ratio, 7:1),with a mean age at diagnosis of 46.6 years. The frequency of fulfilment of the 2017 criteria was: 63% for positive ocular staining, 76% for abnormal Schirmer test, 79% for abnormal unstimulated whole salivary flow, 84%for positive salivary gland biopsy and 88% for Ro autoantibodies. Other immunological tests included positive ANA (88%), RF (54%), low C3 levels (25%), low C4 levels (13%), and cryoglobulins (9%). In comparison with patients presenting with the typical sicca syndrome, those presenting as systemic Sjögren?s with no sicca syndrome were younger (46.6 vs 52.9, p