BackgroundPrimary Sjögren Syndrome (pSS) patients have an increased risk of Non-Hodgkin lymphoma (NHL). There is no consensus on the therapeutic management of low-grade NHL. Two strategies can be proposed; either a ‘‘wait and see’’ strategy or an active therapeutic strategy.ObjectivesTo describe characteristics of NHL in pSS, therapeutic strategies and impact of these strategies on prognosis of lymphoma and of pSS.MethodsThis multicentric retrospective study included all lymphoma patients of the ASSESS cohort, enriched with patients recruited in Rheumatology and Internal Medicine departments. For each patient, we collected biological and clinical manifestations of pSS, lymphoma’s characteristics, and treatment strategy. Progression free survival (PFS) (lymphoma-PFS and pSS relapse free survival) and overall survival (OS) were analyzed.ResultsA total of 106 pSS patients who presented a B cell lymphoma between 1985 and 2019 were included. Among them 14 (13%) had diffuse large B cell lymphoma (DLBCL) and 82 pSS patients presented a low-grade B cell-NHL, mucosa-associated lymphoid tissue (MALT) lymphomas being the most frequent histologic subtype occurring in 68/82 (83%) patients.Among these 82 patients, a “wait and watch strategy” was chosen in 19 (23%) patients; 63 patients received a specific treatment for lymphoma at lymphoma diagnosis including systemic treatment (chemotherapy and/or immunotherapy) in 49 (60%) patients and local therapy (surgery or radiotherapy) in 14 (17%) patients; 10 patients further received rituximab (RTX) maintenance therapy.Comparison of treated versus not treated patients is presented in the Table 1. Untreated patients were older and had less pulmonary lymphoma location.We then analyzed the prognosis after a mean follow up of 6.5 years. Nine patients (11%) died during the follow-up. In multivariate analysis, age (HR= 1.16 [1.06-1.27], p = 0.001) and pulmonary location (HR= 8.15 [1.57-42.3], p = 0.013) were associated with death.Last, we compared OS, lymphoma PFS and pSS relapse PFS in treated versus not treated patients after propensity score weighting We observed that starting an active treatment for NHL at lymphoma diagnosis did not impact OS and lymphoma PFS (HR= 4.81 [0.48-47.9], p=0.2 and HR=1.12 |0.50- 2.48], p=0.8, respectively). Conversely, treating lymphoma had a protective effect on the risk of pSS relapse (HR 0.4 [0.17-0.95], p = 0.038). Last, among patients treated for lymphoma, we observed that no lymphoma relapse occurred in patients who received maintenance therapy with RTX (0/10 events vs 18/53, p = 0.04).ConclusionThis study based on a large number of pSS patients with lymphoma shows that age and pulmonary location are independently associated with the risk of death. The choice of treating lymphoma at its diagnosis or not does not affect OS and PFS for lymphoma. However treatment of lymphoma reduced the risk of pSS relapse. This should be taken into account when deciding therapeutic strategy in our pSS patients with lymphoma.Table 1.VariableWait and watch N=19Active treatment N=63p-val.F18/ 19 (95%)54/ 63 (86%)Age64.0 (54.0, 75.5)56.0 (47.0, 65.5)0.041Time between pSS and lymphoma diagnosis1.0 (0.0, 8.5)3.2 (0.1, 9.0)0.6ESSDAI6.0 (4.0, 8.0)8.0 (5.0, 14.0)0.2clinESSDAI4.0 (3.0, 7.0)6.5 (4.0, 12.8)0.091BiologySSA antibodies14/ 19 (74%)44/ 61 (72%)0.9SSB antibodies8/ 19 (42%)23/ 58 (40%)0.9Positive rheumatoid factor14/ 18 (78%)39/ 54 (78%)0.8Low C42/ 11 (18%)15/ 35 (43%)0.2Hypergammaglobulinemia9/ 16 (56%)30/ 45 (67%)0.5Lymphoma type0.7MALT lymphoma15/ 19 (79%)53/ 63 (84%)Nodal marginal zone lymphoma4/ 19 (21%)10/ 63 (16%)Lymphoma disseminationSalivary and/or ganglionar limited7/ 19 (37%)35/ 63 (56%)0.2Lymphoma spread ≥ 2 locations9/ 19 (47%)28/ 63 (44%)0.8Lymphoma localizationSalivary gland limited lymphoma4/ 19 (21%)20/ 63 (56%)0.4Pulmonary lymphoma involvement0/ 19 (0%)13/ 63 (21%)0.032Salivary gland lymphoma involvement11/ 19 (58%)36/ 63 (57%)>0.9OutcomeMedian follow-up7.0 (4.5, 8.5)6.0 (4.0, 13.5)0.7Death1/ 19 (5.3%)8/ 63 (13%)-REFERENCES:NIL.Acknowledgements:NIL.Disclosure of InterestsNone Declared.

No abstract

No abstract