AB0826 PREVALENCE OF MALIGNANCIES IN PATIENTS WITH SJOGREN’S SYNDROME OVER A 10-YEAR PERIOD, DATA FROM THE GIRRCS ITALIAN COHORT
Authors: I. Genovali, O. Berardicurti, A. Marino, L. Navarini, M. Vomero, D. Currado, L. La Barbera, R. Irace, P. Conigliaro, V. Pavlych, V. Guardo, G. Carlino, R. D. Grembiale, A. Iagnocco, P. Cipriani, M. S. Chimenti, F. Ciccia, G. Guggino, R. Giacomelli
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Primary Sjogren's syndrome (pSS) is a systemic autoimmune disorder primarily impacting salivary and lacrimal glands, with systemic manifestations occurring in nearly half of the patients. While the prognosis for this disease is generally favorable, approximately 5% of patients develop hematologic malignancies, particularly non-Hodgkin's lymphoma (NHL) during its course. In contrast to well-established data on lymphoma development, information on solid malignancies is both scarce and contentious. A systematic review and meta-analysis, encompassing 25 articles and involving over 47,607 pSS patients, revealed an increased risk of overall cancer, encompassing solid malignancies, among individuals with pSS. <h3>Objectives:</h3> -To assess the prevalence of different types of cancers, subdividing them into lymphomas, MGUS, and solid malignancies, in a cohort of 495 patients with pSS. -To analyze the characteristics of patients at baseline in patients without malignancies, with MGUS, with lymphoma, and with solid malignancies. <h3>Methods:</h3> The GIRRCS (Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale) Sjogren's disease (SD) cohort is an inception cohort enrolling 495 consecutive patients with pSS followed in different rheumatology centers in Italy with experience in SS diagnosis and treatment. All patients were ≥18 years old and fulfilled the 2016 American–European Consensus Criteria for pSS. We provided descriptive statistic analysis, and normally distributed continuous variables were expressed as mean ± SD. Data distribution was tested by using the Shapiro-Wilk test. To compare the clinical characteristics, parametric or nonparametric t-tests were used for all the continuous variables, and the chi-square test for the categorical variables, as appropriate. <h3>Results:</h3> We examined data from 495 patients with primary Sjogren's syndrome (pSS), with a median age of 52.15 (SD±12.61), of whom 94.7% were female, and with an average ESSDAI of 4.16 (SD±5.71). At the baseline assessment, individuals with monoclonal gammopathy of undetermined significance (MGUS) exhibited a younger age at diagnosis compared to other groups, with no differences based on diagnostic delay. A higher baseline ESSDAI value was associated with the presence of MGUS, lymphoma, and solid tumors. Notably, a correlation between a higher ESSPRI value and malignancies was evident solely in patients with MGUS. Elevated focus score values were linked to the presence of MGUS. Gamma globulin values were significantly elevated in patients with MGUS and solid neoplasia. Rheumatoid factor positivity was more frequent in patients developing MGUS, whereas anti-La positivity was observed in individuals with solid neoplasia. An intriguing association emerged between an increase in CRP value and the presence of solid neoplasms, likely attributed to the systemic inflammation induced by this type of malignancy. Comprehensive data are presented in Table 1. <h3>Conclusion:</h3> This study revealed certain connections between the baseline characteristics of SS patients and the occurrence of malignancies, not only for lymphomas. Notably, a high ESSDAI value and increased levels of gamma globulins were identified as associated with lymphomas and also with solid malignancies diagnosis. RF presence was significantly associated with MGUS presence but not with lymphoma diagnosis. CRP elevation could also be an interesting finding, after the exclusion of other underlying causes, to better screen these patients for a possible malignant cause of this condition. Intriguingly, the percentage of patients with anti-La antibodies was significantly higher in patients with solid malignancies. These data could contribute to the SS patients profiling, with the scope of an early identification of patients with malignancies for early recognition and better management. <h3>REFERENCES:</h3> <b>NIL</b> <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>