One year in review
Sjögren’s syndrome: one year in review 2023
S. Longhino1, L.G. Chatzis2, R. Dal Pozzolo3, S. Peretti4, G. Fulvio5, G. La Rocca6, I.C. Navarro Garcia7, M. Orlandi8, L. Quartuccio9, C. Baldini10, E. Bartoloni11
- Division of Rheumatology, Academic Hospital Santa Maria della Misericordia, Azienda Sanitaria Universitaria Friuli Centrale (ASUFC), Udine, Italy.
- Pathophysiology Department, School of Medicine, National and Kapodistrian University of Athens, Greece.
- Rheumatology Unit, Department of Medicine and Surgery, University of Perugia, Italy.
- Department of Clinical and Experimental Medicine, University of Florence, Italy.
- Department of Clinical and Experimental Medicine, Rheumatology Unit, University of Pisa, Italy.
- Department of Clinical and Experimental Medicine, Rheumatology Unit, University of Pisa, Italy.
- Department of Clinical and Experimental Medicine, Rheumatology Unit, University of Pisa, Italy.
- Department of Clinical and Experimental Medicine, University of Florence, Italy.
- Division of Rheumatology, Academic Hospital Santa Maria della Misericordia, Azienda Sanitaria Universitaria Friuli Centrale (ASUFC), Udine, Italy.
- Department of Clinical and Experimental Medicine, Rheumatology Unit, University of Pisa, Italy.
- Rheumatology Unit, Department of Medicine and Surgery, University of Perugia, Italy. elena.bartolonibocci@unipg.it
CER17288
2023 Vol.41, N°12
PI 2343, PF 2356
One year in review
Free to view
(click on article PDF icon to read the article)
PMID: 38149515 [PubMed]
Received: 10/11/2023
Accepted : 05/12/2023
In Press: 23/12/2023
Published: 23/12/2023
Abstract
Primary Sjögren’s syndrome (pSS) is a systemic autoimmune disorder characterised by the T-cell-mediated hyperactivation of B-cells and cytokine production. The condition may evolve from an asymptomatic, indolent course, with glandular involvement, to extra-glandular systemic manifestations up to lymphoma development. On tissue level, the typical feature is the lymphocytic infiltration of the salivary gland by B-, T- and antigen presenting cells, as mirrored by the diagnostic cornerstone role of minor salivary gland (MSG) biopsy. Recently, increasing research focused on the investigation of mechanisms underlying the complex pathogenesis of the disease and highlighted the multi-factorial nature of SS consisting of concomitant involvement of environmental, genetic, neuroendocrine and immune factors. In particular, many aspects have been investigated regarding genetic and epigenetics, the role of specific B- and T-cell phenotypes and the investigation of disease-specific biomarkers as predictors of disease development, activity, and lymphomagenesis. Surely, a deeper understanding of these multiple mechanisms may facilitate earlier diagnosis, enable subphenotyping of patients and open novel therapeutic possibilities to address the unmet needs of the disease in the upcoming years. In this review, following the others of this series, we will summarise the most recent literature on pSS pathogenesis and clinical features focusing in particular on new insights into pSS molecular stratification and therapeutic advances in the era of precision medicine.
