Sjögren’s disease represents a complex systemic autoimmune disorder mainly driven by T and B lymphocytic infiltration of exocrine gland, activation of different signalling pathways and systemic cytokine production. These interacting pathogenic mechanisms may differently contribute to characterise highly variable phenotypic expression of the disease, ranging from an asymptomatic, indolent course with only glandular involvement to several extra-glandular systemic manifestations. Moreover, approximately 5–10% of patients develop lymphoproliferative disease, with an overall risk reported to be up to 48 times higher in comparison to healthy population. Due to the substantial clinical heterogeneity of the disease, in recent years, research focused to investigate biomarkers able to identify distinct subtypes of Sjögren’s disease, facilitate earlier patient recognition and homogenise patient subgroups in clinical trials aiming to develop tailored therapies. Surely, a more detailed understanding of pathogenetic mechanisms and recognition of different disease phenotypes may facilitate earlier diagnosis, enable recognition of patient clusters and suggest novel therapeutic modalities to address the unmet needs of the disease in the upcoming years. In this review, following the others of this series, we will update the most recent literature on Sjögren’s disease focusing in particular on new insights into clinical stratification, imaging techniques and targeted therapeutic advances.

Clinical manifestations, imaging and treatment of Sjögren’s disease: one year in review 2025

Quartuccio L.;
2025-01-01

Abstract

Sjögren’s disease represents a complex systemic autoimmune disorder mainly driven by T and B lymphocytic infiltration of exocrine gland, activation of different signalling pathways and systemic cytokine production. These interacting pathogenic mechanisms may differently contribute to characterise highly variable phenotypic expression of the disease, ranging from an asymptomatic, indolent course with only glandular involvement to several extra-glandular systemic manifestations. Moreover, approximately 5–10% of patients develop lymphoproliferative disease, with an overall risk reported to be up to 48 times higher in comparison to healthy population. Due to the substantial clinical heterogeneity of the disease, in recent years, research focused to investigate biomarkers able to identify distinct subtypes of Sjögren’s disease, facilitate earlier patient recognition and homogenise patient subgroups in clinical trials aiming to develop tailored therapies. Surely, a more detailed understanding of pathogenetic mechanisms and recognition of different disease phenotypes may facilitate earlier diagnosis, enable recognition of patient clusters and suggest novel therapeutic modalities to address the unmet needs of the disease in the upcoming years. In this review, following the others of this series, we will update the most recent literature on Sjögren’s disease focusing in particular on new insights into clinical stratification, imaging techniques and targeted therapeutic advances.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11390/1322028
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