Percorrer por autor "Costa, Roberto Pereira da"
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- NeuroPORTRESS: prevalence, clinical spectrum, and factors associated with neurological involvement in Sjögren’s diseasePublication . Costa, Roberto Pereira da; Bandeira, Matilde; Silvério-António, Manuel; Lopes, Ana R.; Cunha-Santos, Filipe; Pereira, Paulo J.; Raimundo, Diana Belchior; Cunha, Anita; Oliveira, Cláudia Pinto; Duarte, Ana C.; Dias, João Madruga; Santos, Mariana Emília; Gonçalves, Maria JoãoObjectives: Neurological involvement is a heterogeneous complication of Sjögren's disease (SjD). We aimed to describe its prevalence and spectrum and identify factors associated with peripheral (PNS) and central nervous system (CNS) involvement. Methods: We conducted a cross-sectional, multicentre study based on PORTRESS, the Portuguese SjD registry. Demographic and clinical data were compared between groups. Independent associations of overall neurological, PNS, and CNS involvement were identified through logistic regression models using complete-case analysis. Results: Among 1234 patients, 62 (5.0%) had neurological involvement: 46 (3.7%) PNS, 17 (1.4%) CNS, with one patient having both. Patients with neurological manifestations had higher ESSDAI, more cryoglobulinaemia, purpura/cutaneous vasculitis and monoclonal gammopathy, and higher mortality. Cryoglobulinaemia and higher baseline ESSDAI (excluding neurological domains) were independently associated with neurological involvement. PNS involvement was associated with older age at symptom onset, cryoglobulinaemia, lymphopenia and higher ESSDAI. A sensitivity analysis revealed that low C4, persistent salivary gland swelling, and purpura/cutaneous vasculitis were also independently associated with PNS involvement. CNS involvement was associated with male sex and younger age at diagnosis. Conclusion: Neurological involvement in SjD was infrequent. PNS involvement clustered with vasculitic and B-cell-driven features and worse outcomes, whereas CNS manifestations appeared to show a distinct demographic profile, characterised by younger age and male sex. These findings support separate evaluation of PNS and CNS involvement and may help identify patients requiring neurological surveillance. Given the limited number of cases, particularly of CNS involvement, these findings remain exploratory and require confirmation in larger cohorts.
- 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.