Development Of Lymphoma In Patients With Sjogren's Syndrome

LLOVES SCHENONE N; MELO F; DERMARCHI JULIA; SALVATIERRA, GABRIELA; AMITRANO, CRISTINA; RATTI L; CAEIRO FRANCISCO; BAENAS DIEGO FEDERICO; ALBIERO EDUARDO; MAMANI MARTA; BARREIRA, JUAN CARLOS; SANTIAGO L; ASNAL, CECILIA; CROW C; PAPASIDERO, SILVIA BEATRIZ; SAURIT VERONICA; GOBBI CARLA; SECCO ANASTACIA; ADROVER, MARIANELA; SEGURA ESCOBAR, CAROLINA; NITSCHE, ALEJANDRO; PUCCI P; CRUZAT V; RETAMOZO SOLEDAD; RISCANEVO NADIA; CATALAN PELLET A

Buenos Aires

Congreso; XX CONGRESO DE LA LIGA PANAMERICANA DE ASOCIACIONES DE REUMATOLOGÍA (PANLAR); 2018

PANLAR

Introduction: Non-Hodgkin lymphoma (NHL) is one of the most feared complications of primary Sjogren´s syndrome (pSS). The most frequent is MALT type lymphoma, with localization in the parotid glands being common. There are no multicentric data in the Argentinean population regarding the frequency of appearance of this type of cancer in patients with pSS and the possible predictors of this outcome.Objectives: To describe the prevalence and incidence rate of lymphoma in patients with pSS in nine centers in Argentina. To determine the frequency of involvement of the domains of the baseline clinical ESSDAI in those patients who developed lymphoma during their follow-up and compare them with the rest of the sample.Materials and Methods: To respond to the primary objective, the design was observational, descriptive and historical. We included patients older than 18 years of age with a diagnosis of pSS according to ACR/EULAR 2002 criteria, included in a multi-center Argentinean database. Patients diagnosed with another associated autoimmune rheumatic disease were excluded.Results:We included 708 patients, 95% female, with a mean age of 54.4 years (SD +/− 13.67), mean age at diagnosis of 49.7 years (SD +/− 13.32) and mean age at symptom onset of 47.19 (SD +/− 13.03). Fifteen patients presented lymphoma (prevalence: 2.12%). Six hundred thirty-six patients provided information for the survival analysis. The average follow-up time was 5 years (SD +/− 6.5). The incidence rate of lymphoma was 0.47 per 100 patient-years. The median time from the diagnosis of pSS to the development of lymphoma was 4 years (IQR: 1-6). The most frequently lymphoma type was MALT. The main predictor of lymphoma development was recurrent parotid gland enlargement (H.R: 4.17, 95% CI: 1.42-12.22). Table 1 reports the results regarding theclinical ESSDAI/.Conclusion: The prevalence of lymphoma was 2.12%and the incidence rate of 0.47 lymphomas per 100 patients/year. Patients who developed lymphoma had a higher frequency of involvement of most of the domains of the baseline clinical ESSDAI compared to patients who did not present this complication. We found recurrent parotid glands enlargement as the main predictor of the development of this cancer.