Sjögren’s syndrome (SS) is a chronic systemic autoimmune disease that initially presents in the head and neck region, affecting the lacrimal and salivary glands. Clinically, the patient will have objective findings of lacrimal and salivary dysfunction with subjective complaints of dry eyes and dry mouth.
Diagnosing SS is a challenge, as there are many conditions associated with dry eyes and dry mouth that can mimic the ocular and oral components. Documentation of objective tests of ocular and salivary dysfunction (Schirmer I test; ocular surface staining with a vital dye; labial salivary gland biopsy) is the most reliable for diagnosis of this syndrome.
SS may be the second most common autoimmune disease after rheumatoid arthritis, yet recent data suggests that there is an average delay in diagnosis of 6.5 years and many individuals remain undiagnosed. SS represents a spectrum of disease with great diversity in its initial presentation and clinical course, but with common underlying themes of lacrimal and salivary gland dysfunction associated with an autoimmune process. The diagnostic process is complicated by the lack of any single diagnostic test for SS. The three components, ocular, salivary, and systemic, are often assessed independently; and conditions that can mimic each component must be ruled out.
Reported ear, nose, and throat manifestations of SS include swallowing disorders, mucosal dryness, dysgeusia, anosmia, epistaxis, gastric reflux, major salivary gland swelling, and lymphoma.