Sjögren's syndrome: an autoimmune disorder with otolaryngological involvement.
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.
Tucci
M
M
DIMO, Department of Internal Medicine and Clinical Oncology, University of Bari, Italy.
Quatraro
C
C
Silvestris
F
F
D000888:Antibodies, Anti-Idiotypic / Q000276:immunology*; D017153:Antibodies, Anticardiolipin / Q000276:immunology; D001706:Biopsy; D015834:Cochlear Diseases / Q000453:epidemiology* / Q000276:immunology*; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000453:epidemiology; D011976:Receptors, Muscarinic / Q000276:immunology; D012470:Salivary Glands, Minor / Q000473:pathology; D012859:Sjogren's Syndrome / Q000453:epidemiology / Q000473:pathology*; D014985:Xerophthalmia / Q000453:epidemiology; D014987:Xerostomia / Q000453:epidemiology*
D000888:Antibodies, Anti-Idiotypic; D017153:Antibodies, Anticardiolipin; D011976:Receptors, Muscarinic