OP0131 COMPARISON OF CLINICAL PHENOTYPE, SEROLOGICAL CHARACTERISTICS AND HISTOLOGIC FEATURES OF MALES VS FEMALES PATIENTS WITH PRIMARY SJÖGREN’S SYNDROME (PSS)
Authors: Loukas Chatzis, Aliki Venetsanopoulou, Mary Pappa, Athanasios Tzioufas, Andreas Goules
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Primary Sjögren's syndrome (pSS) is a female predominant autoimmune disease and very few studies have been conducted to address the phenotypic and laboratory differences of the disease between the two genders. <h3>Objectives</h3> To investigate whether gender in pSS interferes with clinical manifestations, serology, disease course and lymphoma development, in the largest cohort of pSS males in Greece. <h3>Methods</h3> From a cohort of 588 consecutive pSS patients who fulfill the 2016 ACR/EULAR criteria for Sjögren's, 33 males were included in the study. Every male was matched with a female in a 1:2 ratio, according to age of disease onset and disease duration. A 3 year age deviation was permitted. Glandular (dry mouth, dry eyes, parotid gland enlargement) and extra-glandular manifestations (Raynaud's phenomenon, lymphadenopathy, arthralgias/arthritis, palpable purpura, liver involvement, kidney involvement, lymphoma) as well as serology (anti Ro/SSA, anti La/SSB, rheumatoid factor, cryoglobulinemia, low C4 complement levels) and histologic features (focus score, presence of germinal centers) were recorded and compared. Statistical analysis for categorical data was performed by Fisher exact test in SPSS software version 22.0. <h3>Results</h3> The median age of disease onset was 52 years (range: 15-71 years) for the male group and 50 years (range: 15-73 years) for the females. The median disease duration was 8 years (range: 0-26 years) and 7 years (range: 0-26 years) for males and females respectively. Anti-La/SSB antibodies were found in statistically significant higher frequency in males compared to female patients [21/33 (63.3%) vs 23/66 (34.8%), respectively, p=0.009]. A similar trend was observed regarding anti-Ro/SSA antibodies [26/32 (81%) for males vs 44/64 (68%) for females] and rheumatoid factor [18/26 (69%) for males vs 28/57 (49%) for females], however without reaching statistical significance. Furthermore, males with pSS had less frequently Raynaud's phenomenon [3/33 (9%) vs 17/64 (26.5%) respectively] and a tendency to develop lymphomas [6/33 (18%) for males vs 6/65 (9%) for females] compared to females. <h3>Conclusion</h3> This is the first study comparing males and females with pSS after applying the 2016 ACR/EULAR classification criteria. The difference in the prevalence of anti-La/SSB antibodies and to a lesser extend of anti-Ro/SSA and rheumatoid factors implies a potential role of gender and hormones in the production of autoantibodies. Furthermore, higher frequency of lymphoma among males without classical risk factors may suggest distinct lymphomagenesis mechanisms between the 2 genders. <h3>Disclosure of Interests</h3> Loukas Chatzis: None declared, Aliki Venetsanopoulou: None declared, MARY PAPPA Employee of: Bayer, Athanasios Tzioufas Grant/research support from: ABBVIE, PFIZER, AMGEN, NOVARTIS, GSK, Andreas Goules: None declared&nbsp;