IgG4‐related sialadenitis and Sjögren's syndrome

IgG4‐related disease (IgG4‐RD) has emerged as a new entity in the last decade. It comprises numerous conditions previously thought to be unrelated. Macroscopically, these diseases cause diffuse organ swelling and formation of pseudotumorous masses. Histopathologically, they are characterized by a lymphoplasmacytic infiltrate with increased IgG4+ plasma cells and storiform fibrosis. Despite rapid progress within the last years, our knowledge on these conditions is still fragmented. To date, more than forty organs have been reported to be included in IgG4‐RD, and salivary gland involvement is amongst the most common organs affected [IgG4‐related sialadenitis (IgG4‐RS)]. Interestingly, IgG4‐RS shares commonalities with Sjögren's syndrome (SS), like glandular enlargement, sicca symptoms, arthralgias, hypergammaglobulinemia, hypocomplementemia, and circulating antinuclear antibodies. Nonetheless, they differ in that the incidence of anti‐Ro and anti‐La reactivity is not frequently found in patients with IgG4‐RS, their salivary glands are infiltrated by a large number of IgG4+ plasma cells and IgG4‐RS symptoms respond promptly to steroids. The aim of this review was to describe the clinical, serological, histopathological and pathophysiological aspects of IgG4‐RS in the context of IgG4‐RD and highlight the differences between IgG4‐RS and SS.     

Follicular dendritic cells confirm lymphoid organization in the minor salivary glands of primary Sjögren’s syndrome

Background: Sjögren’s syndrome (SS) is an autoimmune chronic inflammatory disorder affecting the salivary and lacrimal glands. The aim of this study was to explore immunophenotypic features of chronic inflammatory reactions in the minor salivary glands in patients with primary SS (pSS). Methods: Formalin‐fixed, paraffin‐embedded labial minor salivary gland tissue sections from randomly selected patients with pSS (n = 60) were investigated for the expression of CD21, CD23, CD35 and IgD by immunohistochemistry. Results: Based on the distribution and staining pattern of CD21, CD23, CD35 and IgD in lymphoid aggregates, several stages of chronic inflammatory reactions were observed. In 12/60 (20%) patients, lymphoid infiltrates with germinal centre (GC)‐like features such as extensive networks of CD21‐, CD23‐ and CD35‐positive cells were observed in the minor salivary gland tissue. Smaller networks and /or focal infiltrates with scattered CD21⁺, CD23⁺ and CD35⁺ cells were observed in the remaining 48/60 (80 %) cases. When dividing patients according to the presence (GC+) or the absence (GC?) of GC in the minor salivary glands, the mean focus score was significantly higher in the GC+ patients (P < 0.05). Double staining of the minor salivary glands revealed focal infiltrates with follicular dentritic cell networks and B cells resembling normal GCs in tonsillar tissue. Conclusion: A particular cellular profile was demonstrated in a sub‐group of patients with pSS and could be linked to serological aberrations. These findings warrant further prospective studies.     

Effect of omega‐3 and vitamin E supplementation on dry mouth in patients with Sjögren's syndrome

To determine whether omega‐3 (n‐3) increases saliva production in patients with Sjögren's syndrome, 61 patients with Sjögren's received either wheat germ oil (n = 23) or n‐3 supplement (TheraTears Nutrition^®) (n = 38) in a prospective, randomized, double‐masked trial. The outcomes assessed were salivary secretion and markers for oral inflammation. The differences between the n‐3 group and wheat germ oil group were not statistically significant for either unstimulated (US) or stimulated (SS) salivary secretion (p= 0.38 and p= 0.346, respectively) nor for the number of sites with probing depth (PD) a 4 mm (p= 0.834). In this pilot study, supplementation with n‐3 was not found to be significantly better than wheat germ oil in stimulating saliva production in patients with Sjögren's syndrome.     

Oral manifestations and their treatment in Sjögren′s syndrome

Sjögren's syndrome (SS) is a complex, chronic, systemic, autoimmune disease that mainly affects the exocrine glands, especially the salivary and lacrimal glands, leading to dryness of the oral and ocular mucosae. Several factors have been studied that could explain the glandular hypofunction primarily related to water transport. Recent reports have shown alterations in secretory route and trafficking in labial salivary glands, explaining alterations in the saliva quality. The decrease in salivary flow and qualitative alterations in saliva could explain many of the oral manifestations. The exocrine manifestations and systemic involvement significantly impact the patient's perception of health‐related quality of life. For this reason and given its systemic nature, the treatment of these patients should be multidisciplinary. This review addresses some particular oral health aspects of SS patients and focuses on relevant topics concerning the treatment and prevention of common oral disorders associated with this disease.