Session Type: ACR Poster Session C
Session Time: 9:00AM-11:00AM
Background/Purpose:To analyse the main characteristics of patients with primary Sjögren syndrome (SjS) with focal lymphocytic sialadenitis (FLS) unrelated to the presence of circulating anti-Ro/La autoantibodies in a large international cohort of patients.
Methods:The Big Data Sjögren Project Consortium is an international, multicentre registry designed, in 2014, to take a “high-definition” picture of the main features of primary SjS at diagnosis by merging international databases including patients fulfilling the 2002 AE classification criteria. Patients came principally from Europe (n=6045), America (n=1134) and Asia (n=940).
Results:The cohort included 7748 (93%) women and 562 (7%) men, with a mean age at diagnosis of primary SS of 53 years. We selected 5245 patients who showed a Chisholm-Mason grade III or IV in minor salivary gland biopsy and that were also tested for Ro/La autoantibodies, and compared the main features at diagnosis between immunonegative patients (n=2073) and those carrying anti-Ro/La antibodies (n=3172). Immunonegative biopsy-proven patients were predominantly White (p<0.001), were 7 years older at diagnosis (57.44 vs 50.74 years, p<0.0001) and had a higher frequency of oral and ocular dryness (p<0.001), a lower frequency of abnormal salivary flows (p=0.046) and parotid scintigraphy (p=0.012), and a lower frequency of ANA (p<0.001), low C3 (p<0.001), low C4 (p<0.001), rheumatoid factor (p<0.001) and cryoglobulinemia (p=0.001) in comparison with Ro/La+ biopsy-proven patients (Figure 1). Abnormal salivary flows, parotid scintigraphy and ANA remained independent variables statistically associated with negative Ro/La autoantibodies after adjusting by age and gender.
Conclusion:Biopsy-proven patients with primary SS without circulating anti-Ro/SSA and anti-La/SSB antibodies have a specific phenotypic profile at diagnosis of the disease, characterized by an older age, a higher frequency of sicca symptoms, a lower frequency of abnormal diagnostic tests and a milder immunological profile. Patients carrying Ro/La autoantibodies are diagnosed earlier in spite of having sicca symptoms less frequently, probably due to the development of extraglandular features.
To cite this abstract in AMA style:Brito-Zerón P, Acar-Denizli N, Zeher M, Rasmussen A, Seror R, Theander E, Li X, Baldini C, Gottenberg JE, Danda D, Quartuccio L, Priori R, Hernandez-Molina G, Kruize AA, Valim V, Kvarnström M, Sene D, Gerli R, Praprotnik S, Isenberg DA, Solans R, Rischmueller M, Kwok SK, Nordmark G, Suzuki Y, Giacomelli R, Devauchelle-Pensec V, Bombardieri M, Hofauer B, Bootsma H, Brun JG, Fraile G, Carsons SE, Gheita T, Morel J, Vollenweider CF, Atzeni F, Retamozo S, Horvath IF, Sivils K, Mandl T, Sandhya P, De Vita S, Sánchez-Guerrero J, van der Heijden E, Moça Trevisano V, Wahren-Herlenius M, Mariette X, Ramos-Casals M. How Does Primary Sjogren Syndrome Present in Biopsy-Proven Patients without Circulating Ro/La Autoantibodies? Characteristics at Diagnosis of 2073 Patients from the SjöGren Big Data Project [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/how-does-primary-sjogren-syndrome-present-in-biopsy-proven-patients-without-circulating-rola-autoantibodies-characteristics-at-diagnosis-of-2073-patients-from-the-sjogren-big-data-project/. Accessed July 3, 2020.
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