Sjögren’s syndrome, or sicca syndrome, is a chronic, autoimmune, rheumatic disorder primarily affecting the exocrine glands – lacrimal and salivary glands – leading to dry eyes and dry mouth.
There are two types of Sjögren’s syndrome:
- Primary Sjögren’s syndrome (PSS)
- Secondary Sjögren’s syndrome. This disorder may develop 10 years after the onset of an associate autoimmune, rheumatic disorder, such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), or scleroderma.
Classification criteria for Sjögren’s syndrome have been established.
There is a wide spectrum of organ specific and systemic complications associated with Sjögren’s syndrome, such as fatigue, myalgia, arthralgia (mild, non-erosive, non-deforming), irritable bowel syndrome, dysphagia, sensory polyneuropathy, nephritis, interstitial lung involvement, liver involvement, hypothyroidism, or B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT) type.
Hematologic abnormalities may include anemia, neutropenia and thrombocytopenia. These abnormalities are seldom clinically significant.
Treatment for Sjögren’s syndrome is mostly palliative, but more specific treatment is indicated when underlying systemic manifestations are present.
Stimulants for residual lacrimal secretion, such as muscarinic agonists (pilocarpine, civimeline) and novel secretagogues, as well as immunomodulatory drugs (cyclosporine A, tacromilus, pimecrolimus), and topical glucocorticosteroids may be used. Anti-inflammatory and disease-modifying antirheumatic drugs are commonly used when more severe non-exocrine manifestations are present.
- Oral Health Care Considerations
- Laboratory Values
- Classifications and Definitions
- Shiboski CH, et al. 2016 American College of Rheumatology/European League against rheumatism classification criteria for primary Sjögren's syndrome. Arthritis Rheumatol. 2017 Jan;69(1):35-45
- Vitali C, et al. Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis. 2002 Jun;61(6):554-8