PORTRESS - the PORTuguese Reuma.pt registry for Sjögren'S disease

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.

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.

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 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.

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.