AB0854 OLDER AGE AT SJÖGREN’S DISEASE DIAGNOSIS IS AN INDEPENDENT PREDICTOR OF LYMPHOMA IN A COHORT WITH A LOWER PREVALENCE OF HEMATOLOGICAL MALIGNANCY — DATA FROM PORTRESS, THE PORTUGUESE SJÖGREN’S DISEASE REGISTRY
Authors: M. Bandeira, M. Silvério-António, R. Pereira Da Costa, A. R. Lopes, F. Cunha Santos, P. Pereira, D. B Raimundo, A. Cunha, C. Pinto Oliveira, A. C. Duarte, J. Madruga Dias, M. E. Santos, M. J. Gonçalves, A. C. Moniz, A. Maduro, M. Luis, A. Valido, M. Oliveira, C. Tenazinha, N. Khmelinskii, F. Barcelos, J. E. Fonseca, V. C Romão
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> One of the most impacting complications of Sjögren's disease (SjD) is the development of lymphoma, reported to occur in around 5-10% of patients with SjD. There have been multiple studies proposing possible risk factors for lymphoma in SjD. <h3>Objectives:</h3> Our goal is to assess the prevalence of lymphoma in a nationwide cohort of SjD and identify potential risk factors for its occurrence in these patients. <h3>Methods:</h3> Multicentre retrospective cohort study, including patients with a clinical diagnosis of SjD, registered in PORTRESS — the Portuguese Registry of SjD — up to November 2023. Patients with information on the presence or absence of lymphoma were included. Demographic, clinical, treatment and patient-reported outcomes (PROs) data were collected. Univariate analysis was performed using chi-square, Fisher's exact, Mann-Whitney or t-test, and correlation between continuous variables using Pearson or Spearman, according to their distribution. Predictors of the development of lymphoma were identified through binomial logistic regression modelling. <h3>Results:</h3> A total of 846 patients were included. Hematological neoplasia was found in 24/846 patients (2.8%), most commonly lymphoma (20/846, 2.4%). The most frequent type of lymphoma was MALT lymphoma (n=7). Patients with SjD-associated lymphoma were more frequently male (p=0.030) and had a higher frequency of pulmonary (p=0.039) and renal involvement (p=0.040; Table 1). They were also more frequently treated with Rituximab (p=0.016), as expected. Both age at diagnosis and at symptom onset were non-significantly higher in the subgroup of patients with lymphoma. Known risk factors for lymphoma were present in a significant part of the cohort. Lymphopenia and low C3 were seen in 22.8 and 17.9%, respectively. Lymphadenopathies were found in 12.3% of patients. Furthermore, decreased C4 was present in 9.1%, persistent SG swelling in 8.4%, cutaneous vasculitis in 6.5% and, less frequently, monoclonal gammopathy (5.5%) and cryoglobulins (3.1%). When submitted to a salivary gland biopsy, ectopic lymphoid structures (ELS) were seen in 44/480 patients (9.2%). From the previously studied risk factors for the development of lymphoma in SjD patients, decreased C3, persistent salivary gland swelling, lymphadenopathies and ELS on the minor salivary gland biopsy were significantly more frequent in the population with lymphoma (Table 1). On multivariate analysis, persistent salivary gland swelling (OR 9.6, 95%CI: 1.5-61.9, p=0.018) and age at diagnosis (OR 1.2, 95%CI: 1.0-1.2, p=0.035) were found to be independent predictors of lymphoma, irrespective of sex, disease duration, decreased C3, lymphadenopathies and ectopic lymphoid structures on the minor salivary gland biopsy. <h3>Conclusion:</h3> The PORTRESS cohort had a lower prevalence of SjD-associated lymphoma than previously reported. Persistent salivary gland swelling was the predominant risk factor for lymphoma in our cohort. Furthermore, older age at SjD diagnosis was independently associated with the development of lymphoma. <h3>REFERENCES:</h3> <b>NIL</b> <h3>Acknowledgements:</h3> This project was funded by SPCare; PORTRESS Reuma.pt Task Force: Margarida Silva, Sara P Dinis, Filipe Vinagre, Maria H Lourenço, Ana B Silva, Alexandra Daniel, Paula Valente, Inês Almeida, Joana Dinis, Lígia Silva, Lídia Teixeira, Filipe Araújo, Carlos M Gomes, Patrícia Pinto, Filipa Farinha, Teresa Nóvoa, Ana Rodrigues, Sara Cortes. <h3>Disclosure of Interests:</h3> Matilde Bandeira Research grant for this registry: SP Care, Manuel Silvério-António menarini, theramex, Roberto Pereira da Costa: None declared, Ana Rita Lopes: None declared, Filipe Cunha Santos: None declared, Paulo Pereira: None declared, Diana B Raimundo: None declared, Anita Cunha: None declared, Cláudia Pinto Oliveira: None declared, Ana Catarina Duarte Boehringher Ingelheim, João Madruga Dias: None declared, Mariana Emília Santos: None declared, Maria João Gonçalves: None declared, Ana Catarina Moniz: None declared, Ana Maduro: None declared, Mariana Luis: None declared, Ana Valido: None declared, Margarida Oliveira: None declared, Catarina Tenazinha: None declared, Nikita Khmelinskii Paid instructor: GSK. Paid speaker: Astrazeneca, GSK, Vifor Pharma. Paid consultant: Abbvie, Astrazeneca, GSK, Novartis., Filipe Barcelos: None declared, Joao Eurico Fonseca: None declared, Vasco C Romão Speaking fees: Astrazeneca, Abbvie, GSK, Janssen, Lilly, Pfizer, Sobi, Research Grants: MSD, SPCare Travel grants and scientific support: Abbvie, Lilly, Medac, MSD, Novartis, Pfizer, Roche.