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AB0550 PHENOTYPE OF BIOPSY-PROVEN PATIENTS WITH PRIMARY SJÖGREN SYNDROME LACKING RO AUTOANTIBODIES: HIGH FREQUENCY OF DRYNESS SYMPTOMS WITH LOW SYSTEMIC ACTIVITY (BIG DATA SJÖGREN PROJECT)

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Retamozo, Soledad | Acar-Denizli, Nihan | Ng, Wan Fai | Horváth, Ildiko Fanny | Rasmussen, Astrid | Seror, Raphaèle | Xiaomei, Li | Baldini, Chiara | Gottenberg, Jacques-Eric | Sandhya, Pulukool | Quartuccio, Luca | Priori, Roberta | Hernandez-Molina, Gabriela | Armagan, Berkan | Kruize, Aike | Kwok, Seung-Ki | Kvarnstrom, Marika | Praprotnik, Sonja | Sene, Damien | Gerli, Roberto | Solans-Laqué, Roser | Rischmueller, Maureen | Mandl, Thomas | Suzuki, Yasunori | Isenberg, David | Valim, Valeria | Sebastian, Agata | Nordmark, Gunnel | Bootsma, Hendrika | Nakamura, Hideki | Giacomelli, Roberto | Devauchelle-Pensec, Valerie | Hofauer, Benedikt | Bombardieri, Michele | Moça Trevisani, Virginia Fernandes | Hammenfors, Daniel | Pasoto, Sandra | Gheita, Tamer | Atzeni, Fabiola | Morel, Jacques | Vollenveider, Cristina | Consani-Fernández, Sandra | Mariette, Xavier | Ramos-Casals, Manuel | Brito-Zerón, Pilar

Edité par CCSD ; BMJ Publishing Group -

International audience. Objectives To analyze the clinical phenotype of primary Sjögren syndrome (SjS) with focal lymphocytic sialadenitis (FLS) without Ro autoantibodies (FLS+/Ro-).Methods The International Big Data Sjögren Project was designed in 2014 to take a “high-definition” picture of the primary SjS at diagnosis (2002 criteria) by merging international databases. 2716 FLS+/Ro- patients were compared with 8315 Ro+ patientsResults FLS+/Ro- patients were predominantly White (82% vs 77%), had a higher frequency of oral (97% vs 92%) and ocular (95% vs 91%) dryness, a lower frequency of ANA (60% vs 87%), hypocomplementemia (13% vs 23%), rheumatoid factor (26% vs 56%), cryoglobulins (5% vs 9%), a lower mean ESSDAI score (4.6 vs 6.6), and a lower systemic activity in the constitutional, lymphadenopatic, glandular, cutaneous, renal, hematological and biological domains (p<0.001 for all comparisons). Abnormal salivary flows and ANA remained significant independent variables after adjustment by age and gender.Conclusion Biopsy-proven primary SjS with negative anti-Ro antibodies is characterized by high frequency of sicca symptoms, mild immunological profile and low systemic activity.

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