Frontiers in immunology · 2026
NeuroPORTRESS: prevalence, clinical spectrum, and factors associated with neurological involvement in Sjögren's disease.
СтатьяМногоцентровое исследованиеНазвание и аннотация: Перевод в очереди
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Pereira da Costa Roberto, 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, Madruga Dias João, Santos Mariana Emília, Gonçalves Maria J, Moniz Ana C, Maduro Ana I, Luís Mariana, Valido Ana, Oliveira Margarida, Brites Luísa, Tenazinha Catarina, Khmelinskii Nikita, Barcelos Filipe, Fonseca João E, Romão Vasco C, PORTRESS Reuma.pt Task Force
Аннотация
OBJECTIVES: 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 low C4, persistent salivary gland swelling and purpura/cutaneous vasculitis also as 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.
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 low C4, persistent salivary gland swelling and purpura/cutaneous vasculitis also as 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.