OP0207 NATIONWIDE EPIDEMIOLOGY OF LYMPHOPROLIFERATIVE DISORDERS COMPLICATING SJÖGREN DISEASE (SD): DATA ON 46,012 PATIENTS WITH SD AND 1749 LYMPHOMAS FROM THE FRENCH HEALTHCARE INFORMATION SYSTEM
Authors: J. E. Gottenberg, P. M. Duret, C. Yamdjieu Ngadeu, E. Sauleau, N. Meyer, D. Cornec, A. Saraux, V. Devauchelle-Pensec, J. Sibilia, G. Nocturne, X. Mariette, R. Seror
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> The epidemiological and immunopathogenic association between Sjögren disease (SD) and non-Hodgkin's lymphoma (NHL) is well-established. However, the epidemiological data on this complication mostly relies on data from tertiary centers, introducing selection bias and limiting comprehensive case capture. <h3>Objectives:</h3> This study aimed to evaluate accurately the characteristics of lymphoma in primary or associated SD using data from the French nationwide healthcare information system ("système national des données de santé" [SNDS]). <h3>Methods:</h3> SD patients were identified in the SNDS database based on a validated algorithm [1] (ICD10 code for SD and reimbursement for at least 2 SD-prescribed drugs). Primary SD was defined in the absence of an ICD10 code for other autoimmune diseases, and associated SD was defined otherwise. The study analyzed, primary, associated and all SD patients and lymphoma occurrences between 2009 and 2022. Analysis of risk factors of lymphoma included demographic variables such as age, sex, disease duration, and proxy reflecting disease activity including notably cryoglobulinemic vasculitis and monoclonal gammopathy of unknown significance (MGUS). <h3>Results:</h3> Patients with primary SD In the 30,221 patients with primary SD (65.7% of the total cohort of patients with SD; 88.8% women, median age 60 [48;70], median follow-up 11.6 years [9.4;13.2]; hydroxychloroquine use in 35.1%, methotrexate [11.5%], leflunomide [1.2%], azathioprine [4.4%], mycophenolate [3.1%], cyclophosphamide [0.4%], and rituximab [5.5%]), lymphoma occurred in 1228 patients with a prevalence of 4.1%. Marginal zone lymphomas (MZL) were the predominant lymphoma subtype (53.8%) (especially MALT-MZL [30.9%]) followed by diffuse large B-cell lymphoma (DLBCL) (25.4%), follicular lymphoma (FL) (12.1%), Hodgkin's lymphoma (8.2%), and T/NK lymphoma (7.2%). Mortality was higher in patients with lymphoma (379/1228 [30.9%] versus 4335/28993 [15%], p&lt;0.001). Preliminary risk factor analysis revealed associations with MGUS and cryoglobulinemic vasculitis: MGUS was observed in 3.2% of SD patients without lymphoma, compared to 10.8% in those with lymphoma (p &lt; 0.001), and cryoglobulinemic vasculitis occurred in 2.2% of SD patients without lymphoma, as compared with 10% of patients with lymphoma (p &lt; 0.001). Patients with associated SD In the 15,791 patients with associated SD (34.3% of the total cohort, including rheumatoid arthritis [20.4%], systemic lupus [10%], systemic sclerosis [6.2%], other connective tissue disorders [3.2%], and juvenile arthritis [0.3%]), 483 patients developed lymphoma (3.1%). Total SD cohort In the total SD cohort, including 46,012 patients with either primary or associated SD (89.4% women, median age 59 [47;69], median follow-up 11.8 years [9.5;13.2]), lymphoma occurred in 1749 patients, with a 3.8% prevalence. Lymphoma subtypes were distributed similarly to the primary SD cohort, and associations between lymphoma and MGUS and cryoglobulinemic vasculitis were observed. Mortality was higher in patients with lymphoma compared to those without lymphoma (30.9% versus 14.8% [p &lt; 0.001]). <h3>Conclusion:</h3> This extensive epidemiological nationwide study demonstrates a 4% prevalence of lymphoma in primary SD, and 3% prevalence in associated SD. Preliminary risk factor analysis confirms the association between SD-related lymphoma and MGUS and cryoglobulinemic vasculitis. <h3>REFERENCES:</h3> [1] Seror et al. (2024 in press) Development of an Algorithm to Identify Sjögren's Syndrome Patients in the French National Healthcare Claims Database. RMD Open. <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>