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Clinical manifestations of Sjögren's syndrome: Exocrine gland disease

Robert Fox, MD, PhD
Section Editor
Peter H Schur, MD
Deputy Editor
Paul L Romain, MD


Sjögren’s syndrome (SS) is a chronic inflammatory disorder characterized by diminished lacrimal and salivary gland function. SS occurs in a primary form not associated with other diseases and in a secondary form that complicates other rheumatic conditions. The most common disease associated with secondary SS is rheumatoid arthritis.

In primary or secondary SS, decreased exocrine gland function leads to the “sicca complex,” a combination of dry eyes (keratoconjunctivitis sicca [KCS]) and dry mouth (xerostomia) [1,2]. In addition, a wide variety of other disease manifestations can occur in SS. The clinical manifestations of SS are divided into the exocrine gland features and the extraglandular disease features [3].

A retrospective study of 80 patients with primary SS followed for a median of 7.5 years reported the following frequencies of clinical manifestations [3]:

KCS and/or xerostomia occurred in all patients and were the only disease manifestation in 31 percent

Extraglandular involvement occurred in 25 percent


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Literature review current through: Sep 2016. | This topic last updated: Oct 1, 2014.
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