Swollen Necks, Dry Mouths: Diagnosing Salivary Gland Diseases

Gordon H. Sun, MD, MS


June 06, 2019

Sjögren Syndrome

Primary Sjögren syndrome is a systemic autoimmune disease classically defined as the presence of dry eyes and mouth, fatigue, and limb pain in a previously healthy patient.[13,14] Associated Sjögren syndrome occurs with a comorbid systemic autoimmune disease such as rheumatoid arthritis or systemic lupus erythematosus.[15] B-cell lymphoma is not a prerequisite for the diagnosis of primary or associated Sjögren syndrome; however, the risk for B-cell lymphoma is about 15-20 times higher in primary Sjögren syndrome patients compared with the general population and is believed to be due to chronic B-cell activation.[13]

Although sicca syndrome (also referred to as sicca complex), with dry mouth and eyes, is a common finding in Sjögren syndrome, the two are not synonymous. Many patients with sicca syndrome do not meet the full criteria for Sjögren syndrome; moreover, the cause of a patient's sicca symptoms may be other conditions or a side effect of some medications (eg, monoclonal antibodies, phenobarbital).[16,17,18]

The presence of anti-SSB/La autoantibodies was a historical biomarker for primary Sjögren syndrome but is no longer considered a diagnostic criterion.[13,19,20]

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