FRI0149 THE CLINICAL FEATURES OF SJÖGREN’S SYNDROME PATIENTS WITH EARLY AND LATE DISEASE ONSET

Abstract

Background:Sjögren’s syndrome (SS) affects mainly individuals of the 4thor 5thdecade of life, although patients with early (≤35 years old) or late (≥65 years old) disease onset have been described in the literature. The clinical spectrum of the disease extends from mild dryness to severe systemic vasculitis and lymphoproliferative disorders. The phenotypic diversity of SS is defined by many factors, including age, since many parameters related to age may affect the clinical expression of the disease. Few studies have been conducted to study the effect of age on the clinical phenotype of SS, though with limited number of patients. Large and well-defined groups of SS are required to address such questions.Objectives:To study the clinical phenotype of SS patients with early and late disease onset and to explore the association of age with lymphoma development in a unified multicenter cohort.Methods:From a total cohort of 1997 consecutive SS patients who fulfill the 2016 EULAR/ACR criteria and are followed up in 5 clinical centers ([Universities ofUdine,Pisa andAthens,Harokopio andIoannina, (UPAHI)], those with either early (≤35 years) or late (≥65 years) disease onset were identified and matched according to gender and disease duration with middle aged controls whose disease onset was at the 4thor 5thdecade of life. Glandular manifestations, extra-glandular manifestations, serologic characteristics and histologic features were compared between the 2 age groups and the middle-aged control 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:Three hundred seventy-nine (19%) SS patients with early and 293 (15%) with late disease onset were identified and compared with 353 and 285 middle aged SS controls respectively. The median disease duration of patients with early onset was 12 years (range:0-68) and for those with late disease onset was 5 years (range: 0-27). SS patients with early disease onset had statistically significant higher frequency of Raynaud’s phenomenon, lymphadenopathy, hypergammaglobulinemia, anti-Ro/SSA, anti-La/SSB, rheumatoid factor, salivary gland enlargement, low C4 complement levels, leukopenia and lymphoma (10,3% vs 5,7%, p= 0.03, OR= 1,91, 95% CI: 1,11-3,27) while SS patients with late disease onset had more frequently dry mouth, interstitial lung disease and lymphoma (6,8% vs 2,1%, p=0,01, OR= 3,4. 95%CI: 1,35-1,81).Conclusion:In a multicenter cohort of 1997 consecutive SS patients, those with early and late disease onset comprise more than one third of the total SS population. Patients with early disease onset, exhibit robust B cell responses with traditional risk factors for lymphoma as opposed to patients with late disease onset. Both age groups have increased lymphoma prevalence but presumably for different reasons, since late onset patients lack classical predictors of lymphoma. Therefore, these predictors deserve further study in different disease subsets.Disclosure of Interests:Andreas Goules: None declared, Ourania Argyropoulou: None declared, Vasileios Pezoulas: None declared, Francesco Ferro: None declared, Saviana Gandolfo: None declared, Valentina Donati: None declared, Marco Binutti: None declared, Sara Zandonella Callegher: None declared, Loukas Chatzis: None declared, Aliki Venetsanopoulou: None declared, Evangelia Zampeli: None declared, Maria Mavromati: None declared, Paraskevi Voulgari: None declared, Clio Mavragani: None declared, Chiara Baldini: None declared, Fotini Skopouli: None declared, Dimitris Fotiadis: None declared, Salvatore De Vita Consultant of: Roche, Human Genome Science, Glaxo Smith Kline and Novartis, Haralampos M. Moutsopoulos: None declared, Athanasios Tzioufas: None declared