FRI0222 ANALYSIS OF CLINICAL AND SEROLOGICAL PICTURE OF PATIENTS WITH PRIMARY SJöGREN’S SYNDROMEAND AN EARLY DISEASE ONSET AT AGE BEFORE 35 YEARS
Authors: Ourania Argyropoulou, Andreas Goules, Evangelia Zampeli, Maria Mavromati, Clio Mavragani, Fotini Skopouli, Haralampos M. Moutsopoulos, Athanasios Tzioufas
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> Primary Sjögren's Syndrome (pSS) is a chronic systemic autoimmune disease with diverse clinical picture, extending from exocrinopathy to systemic disease and non-Hodgkin's lymphoma (NHL). Usually, it affects middle aged women, but early disease onset (≤35 years old) has been also observed. So far, there is a limited number of studies to explore whether the age of SS onset affects the clinical SS phenotype<sup>1</sup>. To definitely answer this question a well-organized, large, harmonized cohort of patients with a continuous follow up is needed, through the ongoing European project (HarmonicSS, grant agreement no: 731944). <h3>Objectives:</h3> To investigate whether the clinical and serological picture of pSS patients with early disease onset (≤35 years old) differs to that of middle-age onset. <h3>Methods:</h3> Medical records of 717 pSS patients, included in two Greek cohorts fulfilling the 2002 revised European/American International classification criteria for SS, were evaluated. The study group included 133 (18,5%) patients with disease onset ≤35 years (median disease duration, range: 10, 0-32 years; group A) matched at 1:1 ratio according to gender and disease duration with 133 pSS patients with middle-age onset (mean age at disease onset 52±5 years; median disease duration, range: 10, 0-30; group B). Clinical and laboratory data were collected through an extensive clinical chart review. All parameters were compared by chi-square or Fisher's exact test, when appropriate. <h3>Results:</h3> The two pSS groups had similar frequencies of non-specific clinical findings (chronic fatigue, arthralgias-myalgias, arthritis, Raynaud's phenomenon), periepithelial manifestations (interstitial nephritis, lung and liver disease), splenomegaly, leucocyte and neutrophil counts. However, patients with disease onset ≤35 years old, exhibited increased proportion of salivary gland enlargement (SGE; 43,8% vs 25,4%, p=0,003), lymphadenopathy (18,2% vs 7,6%, p=0.016), palpable purpura (20,3% vs 9,8%, p=0,025), anti-Ro/SSA antibodies (90,2% vs 74%, p=0,001), rheumatoid factor positivity (73,3% vs 53,6% p=0,002), low C4 levels (64,5% vs 42,4%, p=0,001) and NHL (18% VS 8,3%, p=0.028). <h3>Conclusion:</h3> Our findings suggest that young patients with pSS display increased prevalence of systemic features, B cell hyperreactivity, as well as heightened risk for lymphoma development. Further prospective studies with a larger number of patients are needed to address whether early disease onset may also serve as an additional risk factor for NHL development. <h3>References:</h3> [1] Anquetil C et al, Rheumatology, 2019 <h3>Acknowledgement:</h3> The project was supported by the EU grant "HarmonicSS" (grant agreement no: 731944) <h3>Disclosure of Interests:</h3> Ourania Argyropoulou: None declared, Andreas Goules: None declared, Evangelia Zampeli: None declared, Maria Mavromati: None declared, Clio Mavragani: None declared, Fotini Skopouli: None declared, Haralampos M. Moutsopoulos: None declared, Athanasios Tzioufas Grant/research support from: ABBVIE, PFIZER, AMGEN, NOVARTIS, GSK