POS1267 COMMON SYSTEMIC INVOLVEMENT AND WORSENING DISEASE BURDEN IN PATIENTS WITH SJÖGREN’S DISEASE: DATA FROM PORTRESS, THE PORTUGUESE REGISTRY OF SJÖGREN’S DISEASE
Authors: M. Bandeira, M. Silvério-António, R. Pereira Da Costa, A. R. Lopes, M. Silva, 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, L. Brites, C. Tenazinha, N. Khmelinskii, F. Barcelos, J. E. Fonseca, V. C Romão
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> We recently created PORTRESS, the Portuguese registry of Sjögren's Disease (SjD), as a specific module embedded within the Portuguese Registry of Rheumatic Diseases (Reuma.pt). <h3>Objectives:</h3> To characterise the largest nationwide cohort of SjD patients. <h3>Methods:</h3> We included patients with a clinical diagnosis of SjD, registered in PORTRESS up to November 2023. Demographic, clinical, treatment and patient-reported outcomes (PROs) data were collected. Variables were compared according to parametric or non-parametric tests, as applicable. <h3>Results:</h3> 1375 patients were included. Patients fulfilled AECG 2002 or ACR/EULAR 2016 classification criteria in 62.1% and 57.4% of cases, respectively. However, a large percentage of patients (n=769/1375, 55.9%) did not have a complete assessment of all criteria, which translates common clinical practice reality. Importantly, the vast majority of patients had both sicca symptoms (or extraglandular involvement) and a positive anti-Ro and/or minor salivary gland biopsy (n=1130/1210, 93.4%). Most patients were anti-Ro positive and almost half had circulating rheumatoid factor. Hypergammaglobulinemia (49%) and raised immunoglobulin G (40%) were common, unlike cryoglobulinemia (6%). Although Schirmer's test, SG ultrasound and minor SG biopsy were positive in 52-59% of patients, only 22% had reduced unstimulated whole salivary flow. Most patients (88%) had at least one active ESSDAI domain over the course of the disease. Around half had laboratorial features of B cell hyperactivity (52%), whereas articular, hematologic and glandular involvements were seen in 31-43% of patients (Table 1). Excluding biological and hematological involvement, both involvements that poorly correlate with disease impact, systemic involvement was still observed in almost two thirds of patients (64%). Hydroxychloroquine and corticosteroids were used in 65% and 29% of patients, whereas 1 in 5 was treated with pilocarpine. Of note, up to 12% of patients were treated with other immunosuppressants. The mean ESSDAI was 3.0±4.4 (range 0-42), corresponding to 77.0% (n=800/1039) of patients with low systemic disease activity (ESSDAI&lt;5). At the last follow-up, the mean ESSDAI was 2.1±3.7 (range 0-31), corresponding to a significant decrease from baseline (Figure 1A). Around 24% of patients experienced disease activity worsening, whereas 46% improved (Figure 1B). Finally, dryness, pain and fatigue PROs were high, and more importantly, had a significant increase from baseline to follow-up (Figure 1C). <h3>Conclusion:</h3> In the PORTRESS registry, a considerable number of patients do not have a full assessment of classification criteria, reflecting clinical practice. Systemic involvement was seen in most patients, although reduced salivary flow was present in only 1/5 of cases. Although systemic activity improved over follow-up, symptom burden worsened when compared to baseline, underlining a major unmet need in treating SjD. <h3>REFERENCES:</h3> <b>NIL.</b> <h3>Acknowledgements:</h3> This project was funded by SPCare; PORTRESS Reuma.pt Task Force: 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 work: SPCare, Manuel Silvério-António Menarini; Theramex, Roberto Pereira da Costa: None declared, Ana Rita Lopes: None declared, Margarida Silva: 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, Luísa Brites: 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.