Sjögren's syndrome: an autoimmune disorder with otolaryngological involvement

Abstract

Sjorgen's syndrome (SS) is an autoimmune exocrinopathy characterized by lymphocyte infiltration of salivary and lacrimal glands that leads to progressive xerostomia and xerophtalmia. One-third of patients suffer of systemic manifestations including arthritis, fever, fatigue and mucosal dryness whereas those with major salivary involvement show an increased risk to develop low-grade non-Hpdgkin lymphomas. In addition, a minority of patients show symptoms related to progressive hearing loss whose pathogenesis remains undefined. Both deposition of autoantibodies to antigens of the inner-ear structures and infiltration by autoreactive T-cells have been implicated in its pathogenesis. In this context, high levels of autoantibodies to both cardiolipin and M3 muscarinic receprtors as well as to ciliar epitopes of the cochlear cells have been recently described. Here we review recent advances on the pathodgenesis of SS with a particular focus to otolaryngological manifestations.

Fig. 1

A) Biopsy of minor salivary gland from SS patient. Left panel: haematoxylin/eosin staining reveals typical infiltration in form of large foci of both lymphocytes and plasma cells (arrow) adjacent to acinar and ductal structures of gland (x20). Right panel: lymphoid foci (x100). B) Apoptosis of acinar and ductal cells by immunofluorescence. Left panel: Fas expression (green fluorescence) on acinar cells of minor salivary gland from patient with active SS. Right panel: epithelial cell apoptosis is confirmed by presence of apoptotic TUNEL-positive bodies (arrow).