PORTRESS – the PORTuguese Reuma.pt registry for Sjögren’S disease

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.

Description

This work has been supported by SPCare.

Keywords

bDMARDs Disease activity Healthcare quality Sjogren’s syndrome DMARDs Epidemiology PORTRESS Portugal

URI

http://hdl.handle.net/10400.21/22404

Citation

Bandeira M, Silvério-António M, Costa RP, Lopes AR, Santos FC, Gonçalves MJ, et al. PORTRESS: the PORTuguese Reuma.pt registry for Sjögren'S disease. ARP Rheumatol. 2025;4(3):183-93.