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Treatment of systemic and extraglandular manifestations of Sjögren’s syndrome

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


Sjögren’s syndrome (SS) is a chronic multisystem inflammatory disorder characterized by diminished lacrimal and salivary gland function. This results in the “sicca complex,” a combination of dry eyes (xerophthalmia) and dry mouth (xerostomia). A variety of other disease manifestations may also be present, including systemic signs and extraglandular features.

SS occurs in a primary form not associated with other diseases and in a secondary form associated with other autoimmune rheumatic conditions, including rheumatoid arthritis and systemic lupus erythematosus (SLE).

The treatment and prognosis of systemic and extraglandular manifestations of SS will be reviewed here. Other issues in SS are discussed separately, including the clinical manifestations and diagnosis; the treatment of dry eyes, dry mouth (including the use of muscarinic agonists such as pilocarpine and cevimeline), and other nonocular sicca symptoms is presented separately. (See "Clinical manifestations of Sjögren's syndrome: Exocrine gland disease" and "Clinical manifestations of Sjögren's syndrome: Extraglandular disease" and "Diagnosis and classification of Sjögren's syndrome" and "Treatment of dry mouth and other non-ocular sicca symptoms in Sjögren's syndrome" and "Treatment of dry eye in Sjögren's syndrome: General principles and initial therapy".)


Both local and systemic medical therapies are used in the treatment of Sjögren’s syndrome (SS) [1]. These include:

Preventive therapies and attention to other conditions, medications, and environments that may exacerbate dryness complaints


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Literature review current through: Jun 2017. | This topic last updated: Dec 04, 2015.
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