Background: The development of non-Hodgkin’s Lymphoma (NHL) in Sjögren’s syndrome (SS) contributes to an inferior survival compared to SS patients without lymphoma.

Objectives: To record the long-term outcomes of SS patients with and without NHLs with 10-year survival curves and standardized mortality ratio (SMR) for first time in the literature.

Methods: In order to estimate the effect of NHL development in SS outcome, we expanded the follow-up time of the previously published cohort of SS-associated NHL cases and we retrospectively compared them with age- and sex-matched SS patients without NHL from our center. As outcome end-points, overall and event-free survivals (OS and EFS, respectively) were used. An event was defined as disease relapse or progression, histological transformation or death. Ten-year survival plots and SMR compared to General Greek population were calculated. The impact of rituximab in the outcome of SS associated NHL treated patients was also studied.

Results: From a total of 712 consecutive SS patients who fulfill the 2002 AECG classification criteria for Sjögren’s, 77 were diagnosed with NHL. The prevalence of MALT and DLBC lymphoma in the total SS cohort was 7.1% and 1,7% respectively. The median follow-up time from SS diagnosis was 16.67 years (range: 4.00-38.91 years) corresponding to a total of 1283.71 person-years for SS-associated NHL patients and 17.92 years (range: 2.00-34.00 years) with a total of 1380 person-years for SS patients without NHL. The median time from SS to lymphoma diagnosis was 5.5 years (range: 0-38 years) and the median follow-up time after lymphoma diagnosis was 8.2 years (range: 0.1-26.2 years) months. During follow-up, 15 patients from the SS-NHL group died, 11 of whom due to lymphoma-related causes, while only 2 patients died in the control group (one due to advanced ovarian cancer and one due to cardiac arrest) (p=0.0013). Patients with SS associated NHL displayed a significantly higher risk of death (HR=5.95, 95%CI: 1.66 - 21.38, log-rank test, p=0.0006) compared to their counterparts without lymphoma. The corresponding age-adjusted SMR of SS with and without NHLs versus the general Greek population was 7.94 (95%CI 6.82-9.06) and 1.06 (95%CI -0.06-2.18) respectively. In addition, 18 patients with SS-associated NHL (23.38%) experienced lymphoma relapse or progression and 2 (2.6%) lymphoma transformation; The vast majority of recorded events (27 of 29 events) were observed within the first ten years from NHL diagnosis and DLBCL was associated with the worst outcome; The 10-year EFS% and OS% for DLBC was 22.22% and 48.61% respectively while for MALTL were 68.15% and 82.03%, respectively. Interestingly, the introduction of rituximab in the anti-lymphoma chemotherapeutic regimens didn’t offer any long-term survival benefit.

Conclusion: Herein, we present the longest, to our knowledge, follow-up time of patients with SS-associated NHL; The majority of events that determine the morbidity and mortality of SS-NHL patients have already occurred within the first ten years from NHL diagnosis. In individuals with SS, the evolution of NHL was found to have a detrimental effect in patients’ survival. Among them, DLBCL patients experienced the worst outcome.

Disclosure of Interests: Marina Sikara: None declared, Dimitris Ziogas: None declared, Ourania Argyropoulou: None declared, Aristea Papageorgiou: None declared, Athanasios Tzioufas Grant/research support from: ABBVIE, PFIZER, AMGEN, NOVARTIS, GSK, Michael Voulgarelis: None declared