AB0819 SJOGRENSER REGISTRY: PROSPECTIVE EVALUATION OF A COHORT OF PATIENTS WITH PRIMARY SJÖGREN’S SYNDROME AFTER 8 YEARS OF FOLLOW-UP
Authors: M. Fernandez Castro, Z. Plaza, J. Rosas Gómez de Salazar, V. Martinez Taboada, A. Olive, R. Menor-Almagro, B. Serrano-Benavente, J. Font, Á. García-Aparicio, S. Manrique-Arija, J. A. García Vadillo, R. López-González, J. Narváez, R. L. Beatriz, C. Galisteo, J. J. González Martin, P. Vela Casasempere, C. Bohórquez, C. Erausquin, B. Paredes Romero, E. Aurrecoechea, S. Melchor Díaz, J. M. Pego-Reigosa, S. Heredia Martin, C. Moriano, M. Á. Blázquez, P. Estrada, E. Judez, J. M. Belzunegui Otano, C. Ramos Giráldez, F. Sánchez-Alonso, J. L. Andréu Sánchez
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Sjögren's syndrome is a chronic autoimmune systemic disease that accumulates extragladular manifestations and complications such as lymphoma over time. Poor prognostic factors are described in the scientific literature [1]. <h3>Objectives:</h3> To describe the clinical evolution of patients with Sjögren's syndrome (SS) in relation to the appearance of new systemic manifestations and disease activity, as well as factors associated with an unfavorable outcome. <h3>Methods:</h3> SJÖGRENSER PROS (SS-PROS) is an observational, longitudinal, and multicentric study of patients with SS who met the 2002 classification criteria under active follow-up in rheumatology clinics of 28 Spanish hospitals that participated in the cross-sectional phase of the study (SJÖGRENSER TRANS; SS-TRANS) [2]. For the prospective phase, an 8 year follow-up visit was performed (during 2021-2022) and results were compared to the baseline visit (SS-TRANS performed in 2013-2014, 437 patients included). Medical record was reviewed and a medical interview was performed. Epidemiological, clinical and serological variables, as well as causes of death were recorded. Continuous and categorical variables were analyzed using means, medians, and frequencies, with their respective deviations and interquartile ranges (p25-p75). Student's T test was used to establish statistical associations, considering p&lt;0.05 as significant. <h3>Results:</h3> Three hundred and fourteen patients have been included, 95% were women, with a mean age of 66 years old (SD 11.5) and mean evolution time from diagnosis to inclusion of 17 years (SD 6.5). Mean ESSDAI scores were similar in SS-PROS and SS-TRANS: 3.67 (SD 5.5) vs 3.5 (SD 5.2), respectively. In the analysis by domains, the joint, hematological and biological domains were the most frequently involved and those that accumulate more changes (decrease or increase) over time; for the rest of the domains, stability was evidenced in ≥95% of the cases (Table 1). By organs, the most affected organ in the follow-up was the lung, with 35 new patients who scored in to some degree of at this domain, being 53 the total number of accumulated patients who scored to some degree at this domain during the 8 year period of follow up. After 8 years, 15 lymphomas (4.8%) have accumulated in this cohort, 6 in the SS-TRANS and 9 new lymphomas in the SS-PROS. The mean ESSPRI improved slightly from SS-TRANS to SS-PROS: 5.24 (SD 2.5) vs 4.66 (SD 2), respectively; The improvement was greater in the pain VAS, 5 (SD 3.2) vs 4 (SD 2.9), respectively; The mean VAS for dryness and fatigue in SS-TRANS and SS-PROS respectively was: 6 (SD 2.6) vs 5.6 (SD 2.5) and 4.6 (SD 3.2) vs 4 (SD 3). Forty-two patients died since their inclusion in SS-TRANS (13.4%), 88% women, with a mean age of 75 years old (SD 11.5), and a 20 years long of disease evolution (SD 7.4). Comparing baseline visit data (SS-TRANS) from the group of deceased (during SS-PROS) and non-deceased (remaining under follow-up in SS-PROS), we observed that age (70 years; SD 10), lasting of the disease (11 years; SD 7.33) and ESSDAI score (6.12; SD 7.7), were higher in deceased vs non-deceased (age 57 years, SD 11; time of evolution 8 years, SD 6; ESSDAI 3, SD 4.6), this difference being significant in age (p&lt;0.001) and with a marked trend in the lasting of the disease and the ESSDAI score, although statistical significance was not reached (p=0.078 and p=0.087 respectively). <h3>Conclusion:</h3> Patients with SS develop new systemic manifestations over the years, despite maintaining or improving ESSDAI score, suggesting the need for close follow-up. ESSPRI experiences few variations over time, which represents a great challenge for the scientific community. The incidence of lymphoma in this cohort was 4.8%. Mortality in this cohort is 13.4%. Older age, longer duration of the disease, and higher baseline ESSDAI score were associated with a worse outcome. <h3>REFERENCES:</h3> [1] Mariette X. N Engl J Med 2018;378:931-9. [2] Fernandez -Castro M. <i>Rheumatol Clin</i> 2016;12(4):184-9. <h3>Acknowledgements:</h3> "Proyecto SJOGRENSER del grupo de enfermedades autoinmunes sistémicas de la Sociedad Española de Reumatologia." <h3>Disclosure of Interests:</h3> <b>None declared.</b>