FRI0161 PHENOTYPIC DIFFERENCES BETWEEN SJÖGREN’S SYNDROME PATIENTS WITH LOW AND HIGH-GRADE INFLAMMATION BASED ON SALIVARY GLAND FOCUS SCORE
Authors: L. Chatzis, V. Pezoulas, F. Ferro, V. Donati, A. Venetsanopoulou, E. Zampeli, M. Mavromati, P. Voulgari, C. Mavragani, D. Fotiadis, F. Skopouli, S. De Vita, G. Vassilis, C. Baldini, H. M. Moutsopoulos, A. Goules, A. Tzioufas
Journal: Annals of the Rheumatic Diseases (2020)

Background: Sjögren’s syndrome (SS) is characterized by the presence of lymphocytic infiltration around the ductal epithelium of the salivary and lachrymal glands. The periepithelial inflammatory lesions and the enclosed B cell component are responsible for the glandular and extraglandular manifestations of the disease. Previous studies have shown that the severity of inflammation observed within the salivary glands is correlated with the occurrence of extraglandular manifestations. However, in these studies either the number of patients is small or the SS criteria are not well defined. To explore the association between the degree of inflammation within the salivary glands and the phenotype of the disease, large and well characterized cohorts of SS patients is required. Objectives: To compare the phenotypic features of SS patients with low and high degree of inflammation within the minor salivary glands as reflected by the focus score (FS). Methods: From a total cohort of 1723 consecutive SS patients who fulfill the 2016 EULAR/ACR criteria and are followed up in 4 clinical centers ([Universities of P isa, A thens, H arokopio and I oannina, ( PAHI )], those who had performed a lip biopsy and the focused score was available, were classified into low grade (FS&lt;3) or high grade (FS≥3). Glandular (dry mouth, dry eyes, parotid gland enlargement) and extra-glandular manifestations (Raynaud’s phenomenon, arthralgias/myalgias, arthritis, palpable purpura, liver involvement, kidney involvement, lung involvement, neurologic involvement, long standing lymphadenopathy and lymphoma) as well as serologic features (ANA, RF, anti-Ro/SSA, anti-La/SSB) were compared between the 2 groups. Statistical analysis for categorical variables was performed by Fisher exact or chi-square tests and for continuous variables with t test or Mann-Whitney accordingly. Results: Eight hundred and eight minor salivary gland biopsies were available and evaluated based on focus score at the initial evaluation of SS patients, of whom 753 had low grade (FS&lt;3) and 153 high grade (≥3) inflammation. The median disease duration after SS diagnosis was not statistically significant different for the 2 groups (median: 4 years, range: 0-36 years). SS patients with high grade inflammation displayed higher prevalence of salivary gland enlargement (SGE) (40% vs 25%, p=0,0002), long standing lymphadenopathy (22% vs 14%, p=0,02), ANA (97% vs 88%, p=0,0001), anti-La/SSB (52% vs 32%, p&lt;0,0001), RF (61,5% vs 48%, p=0,003), peripheral neuropathy (PN) (5,3% vs 1,5, p=0,01) and of lymphoma (26% vs 8%, p&lt;0,0001, OR=4,142, 95%CI=2,65 to 6,47) compared to those with low grade inflammation. Conclusion: SS patients with FS ≥3 at the initial evaluation, display higher prevalence of lymphoma as well as higher B cell hyperactivity and certain clinical manifestations (SGE, PNS, lymphadenopathy) that constitute risk factors for lymphoma development. Disclosure of Interests: Loukas Chatzis: None declared, Vasileios Pezoulas: None declared, Francesco Ferro: None declared, Valentina Donati: None declared, Aliki Venetsanopoulou: None declared, Evangelia Zampeli: None declared, Maria Mavromati: None declared, Paraskevi Voulgari: None declared, Clio Mavragani: None declared, Dimitris Fotiadis: None declared, Fotini Skopouli: None declared, Salvatore De Vita Consultant of: Roche, Human Genome Science, Glaxo Smith Kline and Novartis, Gorgoulis Vassilis: None declared, Chiara Baldini: None declared, Haralampos M. Moutsopoulos: None declared, Andreas Goules: None declared, Athanasios Tzioufas: None declared