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NeuroPORTRESS: prevalence, clinical spectrum, and factors associated with neurological involvement in Sjögren’s disease

2026-08Artigo científico Acesso aberto
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datacite.subject.sdg03:Saúde de Qualidade
dc.contributor.authorCosta, Roberto Pereira da
dc.contributor.authorBandeira, Matilde
dc.contributor.authorSilvério-António, Manuel
dc.contributor.authorLopes, Ana R.
dc.contributor.authorCunha-Santos, Filipe
dc.contributor.authorPereira, Paulo J.
dc.contributor.authorRaimundo, Diana Belchior
dc.contributor.authorCunha, Anita
dc.contributor.authorOliveira, Cláudia Pinto
dc.contributor.authorDuarte, Ana C.
dc.contributor.authorDias, João Madruga
dc.contributor.authorSantos, Mariana Emília
dc.contributor.authorGonçalves, Maria João
dc.date.accessioned2026-09-02T08:47:32Z
dc.date.available2026-09-02T08:47:32Z
dc.date.issued2026-08
dc.description.abstractObjectives: 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.eng
dc.identifier.citationCosta RP, Bandeira M, Silvério-António M, Lopes AR, Cunha-Santos F, Gonçalves MJ, et al. NeuroPORTRESS: prevalence, clinical spectrum, and factors associated with neurological involvement in Sjögren’s disease. Front Immunol. 2026;17:1791369.
dc.identifier.doi10.3389/fimmu.2026.1791369
dc.identifier.issn1664-3224
dc.identifier.urihttp://hdl.handle.net/10400.21/23034
dc.language.isoeng
dc.peerreviewedyes
dc.publisherFrontiers Media SA
dc.relation.hasversionhttps://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2026.1791369/full
dc.relation.ispartofFrontiers in Immunology
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/
dc.subjectSjogren disease
dc.subjectCentral nervous system
dc.subjectIndependent associations
dc.subjectNeurological involvement
dc.subjectPeripheral nervous system
dc.subjectNeuroPORTRESS
dc.titleNeuroPORTRESS: prevalence, clinical spectrum, and factors associated with neurological involvement in Sjögren’s diseaseeng
dc.typejournal article
dspace.entity.typePublication
oaire.citation.startPage1791369
oaire.citation.titleFrontiers in Immunology
oaire.citation.volume17
oaire.versionhttp://purl.org/coar/version/c_970fb48d4fbd8a85

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