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- PORTRESS – the PORTuguese Reuma.pt registry for Sjögren’S diseasePublication . Bandeira, Matilde; Silvério-António, Manuel; Costa, Roberto Pereira da; Lopes, Ana Rita; Santos, Filipe Cunha; Pereira, Paulo J.; Raimundo, Diana Belchior; Cunha, Anita; Oliveira, Cláudia Pinto; Duarte, Ana Catarina; Dias, João; Santos, Mariana Emília; Gonçalves, Maria João; Moniz, Ana Catarina; Maduro, Ana Isabel; Luís, Mariana; Valido, Ana; Oliveira, Margarida; Brites, Luísa; Tenazinha, Catarina; Vieira, Ana; Khmelinskii, Nikita; Barcelos, Filipe; Fonseca, João Eurico; Romão, Vasco C.Aims: Sjögren's disease (SjD) is a complex disease with a wide variety of manifestations and outcomes. We recently created PORTRESS, the Portuguese SjD registry within Reuma.pt. We aim to describe this registry and characterize our national cohort. 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: A total of 1375 patients were included. Patients fulfilled AECG 2002 or ACR/EULAR 2016 classification criteria in 62% and 57% of cases, respectively, although more than half didn't have a complete assessment of all items. Of note, the vast majority (93%) had both SjD manifestations and a positive anti-Ro and/or minor salivary gland biopsy. Most patients (88%) exhibited at least one active ESSDAI domain during the course of their disease. Hydroxychloroquine and corticosteroids were used in 52% and 30% of patients, while other immunosuppressants and pilocarpine were used in 12% and 18% of cases, respectively. The mean ESSDAI at inclusion was 3.0±4.4 (range 0-42), and, at the last follow-up, 2.1±3.7 (0-31), corresponding to a significant decrease. Dryness, pain, and fatigue PROs were scored high, with a significant increase from baseline to follow-up. Conclusion: PORTRESS is a web-based SjD registry facilitating efficient nationwide data storage. It enables research, trial recruitment, and a comprehensive longitudinal view of patients' evolution. Although systemic activity improved over follow-up, symptom burden worsened when compared to baseline, underlining a major unmet need in SjD.
- Synovial biopsy in a case of nivolumab-induced arthritisPublication . Costa, Roberto Pereira da; Pinho, Mafalda; Patrocínio, João; Torres, Sofia; Ferreira, Cristina; Fonseca, João Eurico; Khmelinskii, NikitaImmune checkpoint inhibitors (ICIs) are valuable therapeutic weapons against cancer that potentiate a T cell-mediated immune response. Nivolumab is an ICI that targets the PD-1 receptor. Rheumatic immune-related adverse events (irAEs), which are induced by ICIs, occur in 10% of cases. However, their pathogenesis remains unclear. Synovial biopsy has allowed for a better understanding of the underlying mechanisms of different arthropathies. Key message: Synovial biopsy has a role in understanding the aetiopathogenesis of immune checkpoint inhibitor-induced arthritis.