Overview of the management and prognosis of Sjögren's syndrome
- Alan N Baer, MD, FACP
Alan N Baer, MD, FACP
- Associate Professor of Medicine
- Director, Jerome L. Greene Sjogren’s Syndrome Center
- Johns Hopkins University School of Medicine
- Frederick B Vivino, MD, MS, FACR
Frederick B Vivino, MD, MS, FACR
- Director, Penn Sjögren's Syndrome Center
- Professor of Clinical Medicine
- Perelman School of Medicine, University of Pennsylvania
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".)
TREATMENT GOALS AND PRINCIPLES
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.To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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- TREATMENT GOALS AND PRINCIPLES
- PRETREATMENT EVALUATION
- Confirmation of the diagnosis
- Assessment of disease activity and severity
- - Laboratory evaluation
- Testing for all patients at initial evaluation
- Testing in confirmed SS to define subset, activity, and prognosis
- Additional studies
- Determining disease subset
- NONPHARMACOLOGIC AND PREVENTIVE INTERVENTIONS
- GENERAL APPROACH TO DRUG THERAPY
- THERAPY FOR ORGAN-BASED DISEASE AND CONSTITUTIONAL SYMPTOMS
- SPECIAL CONSIDERATIONS
- Management during pregnancy
- General anesthesia
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS