SAT0190 LYMPHOMA IN PRIMARY SJÖGREN’S SYNDROME: A RETROSPECTIVE CLINICAL STUDY WITH PATIENTS FROM THE UPA (UDINE, PISA, ATHENS) GROUP
Authors: Andreas Goules, Chiara Baldini, Saviana Gandolfo, Aristea Papageorgiou, Dimitris Ziogas, Francesco Ferro, Valentina Donati, Cathryn Scott, Salvatore De Vita, Michael Voulgarelis, Athanasios Tzioufas
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> Lymphoma development in Sjögren's syndrome has significant impact on morbidity and mortality of the disease. <h3>Objectives:</h3> To describe and compare clinical, serological and histological features of patients with primary Sjögren's syndrome (pSS) associated non-Hodgkin's lymphomas (NHLs) in 3 cohorts, from Greece (1) and Italy (2). <h3>Methods:</h3> 140 consecutive pSS patients with NHLs who fulfilled the 2002 AECG criteria for Sjögren's were included in the study. Patients were recruited from 3 centers (Udine, Pisa, Athens, cohort named UPA), two from Italy (Udine and Pisa University Hospitals with a total of 63 cases, named cohort UP) and one from Greece (University of Athens with 77 cases, named cohort A). Age at NHL onset, NHL subtype and distribution, SS disease duration until lymphoma development, serum anti-SSA/SSB antibodies and rheumatoid factor, history of salivary gland enlargement, cryoglobulinemia with or without vasculitis and C4 hypocomplementemia before the onset of NHL were recorded and compared. <h3>Results:</h3> The Greek cohort (A) had a median age at lymphoma diagnosis 58 years (range: 28-90 years) and the (UP) cohort a median age at diagnosis of lymphoma 55.6 years (range: 25-77 years). The median time from SS to lymphoma diagnosis was 65.8 months (range: 0-456 months) vs 48 months (range: 0-276 months) for (A) and (UP) cohort respectively. The commonest histologic subtypes such as, MALT (mucosa associated lymphoid tissue), diffuse large B cell (DLBC) and nodal or splenic marginal zone (MZ) lymphomas were observed in similar frequencies between the 2 cohorts. The proportion of pSS NHLs patients with MALT lymphomas and &gt;1 involved sites was significantly higher in cohort (A) compared to (UP) cohort [25/51, (49%) vs 5/40 (12.5%%), p=0,0003)] while extranodal localization of DLBC lymphomas was less frequent in Greek compared to Italian patients [2/12 (16.7%) and 4/10 (40%) respectively]. No significant differences were observed regarding anti-Ro/SSA, anti-La/SSB, rheumatoid factors (RF) or history of parotid gland enlargement. Interestingly, no significant difference was also observed in the frequencies of serum cryoglobulinemia (without vasculitis) and C4 hypocomplementemia. Importantly, cryoglobulinemic skin vasculitis was significantly more frequent in cohort (A) than in cohort (UP) [33/77 (42,9%) vs 12/63 (19,0%) p=0,0027] as well as bone marrow involvement by lymphoma [21/77 (27.2%) vs 4/63 (6.3%) respectively, p=0,0015]. <h3>Conclusion:</h3> The similar lymphoma histologic subtypes, coupled with the differences in the frequency of cryoglobulinemic skin vasculitis and bone marrow involvement by lymphoma between Greek and Italian patients, suggest potential diversities in genetic background, environmental factors, disease progression and pathologic pathways in different cohorts, offering novel perspectives to study the biology of SS associated lymphomagenesis. <h3>Disclosure of Interests:</h3> Andreas Goules: None declared, Chiara Baldini: None declared, Saviana Gandolfo: None declared, Aristea Papageorgiou: None declared, Dimitris Ziogas: None declared, Francesco Ferro: None declared, Valentina Donati: None declared, Cathryn Scott: None declared, Salvatore De Vita Grant/research support from: Roche, Pfizer, Abbvie, Novartis, BMS, MSD, Celgene, Janssen, Consultant for: Roche, Michael Voulgarelis: None declared, Athanasios Tzioufas Grant/research support from: ABBVIE, PFIZER, AMGEN, NOVARTIS, GSK