Objective. Immunoglobulin-G4-(IgG4-) related disease (IgG4 RD) is a fibrosing process characterized by a significant infiltration of IgG4-secreting plasma cells. IgG4 RD can affect almost all organs including salivary glands. Whether IgG4 RD plays a role in the development of sicca syndrome and particularly dry mouth syndrome remains to be investigated. Methods. We conducted a monocentric cohort study for two years to search for IgG4 RD features in patients with dry mouth syndrome using immunostainings of labial salivary gland specimens with anti-IgG4 antibody. Results. Among 60 patients presenting with dry mouth syndrome who underwent labial salivary gland biopsy, 18 showed positive immunostaining with the anti-IgG4 antibody including 4 patients with typical systemic IgG4 RD. Five also fulfilled criteria for Sj？gren's syndrome. Conclusion. These findings suggest that clinical forms of IgG4 RD salivary involvement without salivary swelling may occur. This salivary involvement is probably overlooked in everyday practice and could represent a mild form of IgG4 RD. 1. Introduction The symptoms of dry mouth syndrome frequently cause patients to consult with physicians. More than 10% of the population reports symptoms linked to oral and/or ocular dryness. The causes of xerostomia are numerous including Kuttner’s tumor (KT) and Mikulicz’s disease (MD). Both are mainly characterized by enlargement increase in the volume of salivary glands, with submandibular glands involvement in KT and parotid glands in MD associated with lacrimal induration. Until 2005, MD and KT were classified as subtypes of Sj？gren’s syndrome (SS) [1] but the discovery of lymphoplasmacytic infiltrates rich in IgG4+ plasma cells in the salivary and lacrimal glands led to the reclassification of MD and KT as separate entities from SS. Nowadays, MD and KT are considered to be constituents of salivary involvement caused by immunoglobulin-G4-(IgG4-) related disease (IgG4 RD) [2]. KT is the third most frequent involvement of IgG4 RD (after bile ducts and lymphadenopathy involvement) in patients suffering from autoimmune pancreatitis (AIP) [3]. The concept of IgG4 RD was suggested in 2003 by Kamisawa et al. [4]. AIP, which was described much earlier, is part of this pathology and many publications are dedicated to this subject. IgG4 RD is a fibrosing process that can affect most organs and is characterized by a high number of plasmacytes that secrete IgG4. Essentially for epidemiological reasons, IgG4 RD has been the subject of Japanese-Korean studies although the first description was in French
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