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Treatment of Sjögren's syndrome: Constitutional and non-sicca organ-based manifestations

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

INTRODUCTION

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.

The treatment of salivary gland enlargement, extraglandular manifestations affecting other organs and tissues, and constitutional symptoms will be reviewed here. An overview of the systemic treatment and prognosis of SS, 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), and other nonocular sicca symptoms are described in detail separately. (See "Overview of the management and prognosis of Sjögren's syndrome" and "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".)

OVERVIEW OF MANAGEMENT

An overview of the management and prognosis of Sjögren's syndrome (SS) is presented separately (see "Overview of the management and prognosis of Sjögren's syndrome"). Key elements are reviewed briefly here together with the evidence supporting our approach to drug therapy. (See 'Approach to drug therapy' below and 'Therapy for organ-based disease and constitutional symptoms' below and 'Therapeutic rationale and evidence overview' below and 'Efficacy of specific therapeutic agents' below.)

Treatment goals and principles — The goals of therapy in patients with 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.

                                 

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Literature review current through: Jul 2017. | This topic last updated: Jul 31, 2017.
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