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POS1267 COMMON SYSTEMIC INVOLVEMENT AND WORSENING DISEASE BURDEN IN PATIENTS WITH SJÖGREN’S DISEASE: DATA FROM PORTRESS, THE PORTUGUESE REGISTRY OF SJÖGREN’S DISEASE

2024; BMJ; Linguagem: Inglês

10.1136/annrheumdis-2024-eular.214

1468-2060

Matilde Bandeira, M. Silvério-António, R. Pereira Da Costa, Amanda R. Lopes, Matheus de Freitas Silva, F. Cunha Santos, P Ricardo Pereira, D. B Raimundo, Álvaro Cunha, Cristina Oliveira, Ana Duarte, João Madruga Dias, Maria Santos, Marcel Jefferson Gonçalves, A. C. Moniz, A. Maduro, M L Luís, Ana Valido, Mário Oliveira, L. Brites, Catarina Tenazinha, Nikita Khmelinskii, Filipe Barcelos, João Eurico Fonseca, V. C Romão,

Systemic Sclerosis and Related Diseases

Background: 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). Objectives: To characterise the largest nationwide cohort of SjD patients. Methods: 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. Results: 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<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). Conclusion: 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. REFERENCES: NIL. Acknowledgements: 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. Disclosure of Interests: 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.