Sjögren's syndrome: one year in review 2022

Abstract

Primary Sjögren's syndrome (pSS) is a complex disabling systemic autoimmune disorder. The hallmark of pSS is the T-cell-mediated hyperactivation of B-cells, evolving from asymptomatic conditions to systemic complications and 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. B-cells show multiple possible roles in disease pathogenesis, from autoantibody production, to antigen presentation, and cytokine production. B-cells hyperactivation is supported by genetic risk factors, T-cell dependent and independent mechanisms, and the presence of different pathogenic B-cell subsets must be reminded. Many aspects have been investigated in the last year regarding genetic and epigenetics, B-and T-cell role in pSS pathogenesis, their interaction with salivary gland epithelial cells (SGECs) and in their direct or indirect use as biomarkers and predictors of disease development, activity, and lymphomagenesis.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.

Mechanisms underlying sex bias in oral immune‐mediated conditions, an insight

“…The T-cell mediated hyperactivity of B-cells and the resultant hypergammaglobulinemia, the presence of serum autoantibodies, the dryness of the eyes (xerophthalmia) and dryness of the mouth (xerostomia), in addition to dryness of the other mucosal surfaces (collectively known as sicca syndrome), represent the hallmarks of the disease. 26 The heterogeneity in SS presentation and phenotype have contributed to a wide variation in the reported prevalence rates, ranging from 0.01% to 0.09%. 27,28 More consistent, however, is the marked female predilection of SS, with a female: male ratios reaching more than 10: 1 in some reports.…”

“…Sjögren's syndrome (SS) is a systemic autoimmune rheumatic disease characterized by chronic inflammation, lymphocytic infiltration, and damage of the exocrine glands, primarily the lacrimal and salivary glands. The T‐cell mediated hyperactivity of B‐cells and the resultant hypergammaglobulinemia, the presence of serum autoantibodies, the dryness of the eyes (xerophthalmia) and dryness of the mouth (xerostomia), in addition to dryness of the other mucosal surfaces (collectively known as sicca syndrome), represent the hallmarks of the disease 26 …”

Diagnosis and management of lung involvement in systemic lupus erythematosus and Sjögren’s syndrome: a literature review

“…124 Pulmonary involvement in pSS pSS is defined as an autoimmune epithelitis specifically characterized by impairment of exocrine glands (mainly lacrimal and salivary) due to inflammatory infiltrate. 125 It also shows extraglandular, multi-organ involvement 126,127 and respiratory manifestations (Table 2) are among the most prevalent extra-glandular complications affecting 9% to 75% of patients 128 according to the detection methods and patient selection criteria. [129][130][131] Upper airway disorders with dryness in nasal and oropharyngeal mucosa are definitely among the most frequent manifestations and are due to mucosal dryness, mucus viscosity disorders, and impaired mucociliary clearance caused by the long-standing inflammation that finally leads to atrophy of the submucosal glands of the airway mucosa.…”

The positive effect of JAK inhibitor tofacitinib in the treatment of primary Sjögren’s syndrome: a clinical case

“…Sjögren's syndrome is a systemic autoimmune disease which characterized by damage to exocrine glands, mainly lacrimal and salivary glands, with gradual formation of their secretory insufficiency and various systemic manifestations. Primary Sjögren's syndrome (pSS) develops in previously healthy individuals, unlike secondary Sjogren's syndrome, which can accompany a some of autoimmune diseases (systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis) [1].…”