Abstract
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.