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Overview of the management and prognosis of Sjögren's syndrome

Alan N Baer, MD, FACP
Frederick B Vivino, MD, MS, FACR
Section Editor
Robert Fox, MD, PhD
Deputy Editor
Paul L Romain, MD


Sjögren's syndrome (SS) is a chronic, multisystem autoimmune disease characterized by lacrimal and salivary gland inflammation, with resultant dryness of the eyes and mouth and occasional glandular enlargement. In addition, a variety of systemic (so-called "extraglandular") manifestations may occur, including fatigue, musculoskeletal symptoms, rashes, and internal organ (eg, pulmonary, renal, hepatic, and neurologic) disease. There is also increased risk of non-Hodgkin B-cell lymphoma.

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

An overview of the systemic treatment and prognosis of SS will be reviewed here. The clinical manifestations and diagnosis of SS and the treatment of dry eyes, dry mouth (including the use of muscarinic agonists such as pilocarpine and cevimeline as secretagogues), other nonocular sicca symptoms, and manifestations affecting other organs and tissues are described in detail 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 eye in Sjögren's syndrome: General principles and initial therapy" and "Treatment of dry mouth and other non-ocular sicca symptoms in Sjögren's syndrome" and "Treatment of Sjögren's syndrome: Constitutional and non-sicca organ-based manifestations".)


The goals of therapy in patients with Sjögren's syndrome (SS) are to ameliorate symptoms of dry eye and mouth, prevent complications of mucosal dryness (such as dental decay, corneal ulceration, or oral candidal infection), and detect and manage systemic manifestations and glandular lymphoproliferative disease. The following general principles apply to the management of patients with SS:

Evaluation and management should be provided by a multidisciplinary team; a rheumatologist, an eye care professional, and a dentist or oral medicine specialist are often required for optimal care.

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Literature review current through: Sep 2017. | This topic last updated: Jul 29, 2017.
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