Sjögren's syndrome: one year in review 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 extraglandular 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 multifactorial 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.

Involvement of CCL2 in Salivary Gland Response to Hyperosmolar Stress Related to Sjögren’s Syndrome

“…Several studies have shown that an increase in local salt concentration caused CD4+ T cell differentiation into Th17 [29,30] and the development of autoimmune diseases [30,31]. Although the biological mechanisms leading to SS sicca symptoms are poorly understood, increasing evidence suggests that dryness of the eyes and mouth are not only the result of SG destruction but primarily the consequences of cytokine, autoantibody, and soluble factor production exacerbating the pathological inflammatory process [3,5,32,33].…”

Role of chemokines CXCL9, CXCL10, CXCL11, and CXCR3 in the serum and minor salivary gland tissues of patients with Sjögren’s syndrome

“…Sjogren’s syndrome (SS) is an autoimmune disorder that manifests as dryness of the eyes and mouth accompanied by lymphocytic infiltrates in the lacrimal and salivary glands. This chronic condition, categorized as primary (pSS) or secondary SS, is often linked to other rheumatic diseases and presents with extraglandular manifestations across various systems [ 1 ]. The diagnosis of SS relies on a combination of clinical, serological, and functional tests and histopathological biomarkers based on the 2016 American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR) classification criteria [ 2 ].…”