Sjögren syndrome (SS) is one of the three most common systemic autoimmune illnesses in the United States, affecting up to two million people. It can be a primary disorder (i.e., unaccompanied by another condition), or it can be secondary to other autoimmune diseases like rheumatoid arthritis, lupus, scleroderma, Hashimoto’s thyroiditis, or mixed connective tissue disease. Approximately 30% of patients with autoimmune illnesses eventually develop SS.
Sjögren syndrome is characterized by dryness of the eyes, mouth, and other mucous membranes. Its cause is unknown; viruses have been theorized to trigger the condition, but no clear association with any known infectious agent has been proven. (Hansen A, et al. Immunopathogenesis of primary Sjögren syndrome: implications for disease management and therapy. Curr Opin Rheumatol 2005;17(5):558-65)
It appears that a genetic predisposition and exposure to an unidentified environmental stimulus causes activation of certain components of the immune system. Salivary and tear glands, as well as other exocrine organs, become infiltrated with white blood cells that produce inflammatory cytokines. These cytokines eventually damage the secretory ducts, leading to a reduction in normal lubricating secretions.
Signs and Symptoms of Sjögren Syndrome
- Xerophthalmia (dry eyes): dryness; grittiness; itching; sensation of foreign body in eye; redness; clouding of cornea
- Xerostomia (dry mouth): stickiness; difficulty speaking, eating, or swallowing; redness of mucous membranes; oral ulcers; fissuring of tongue; increased dental caries; tender or swollen parotid glands
- Extraglandular manifestations: tender, swollen joints; gastrointestinal reflux; diarrhea; constipation; chronic cough; recurrent bronchitis; pulmonary fibrosis (scarring); Raynaud’s phenomenon; peripheral neuropathy; cutaneous vasculitis; kidney damage; swollen lymph nodes
(From Kruszka P and O’Brian R. Diagnosis and management of Sjögren syndrome. Am Fam Phys 2009;79(6):465-70)
Diagnosis of Sjögren Syndrome
People who complain of dry mouth and eyes—particularly those who have autoimmune illnesses—may be subjected to a battery of tests:
- Blood tests: antibodies to specific antigens (anti-SSA, anti-SSB, antinuclear antibody, rheumatoid factor) are often found in SS patients, but they may also be present in healthy persons or those with other illnesses
- Salivary gland biopsy: infiltration by lymphocytes, with degeneration of glandular structures, is a hallmark of SS
- Salivary flow tests or radiographic examinations to determine salivary gland involvement
- Tests for tear production: Schirmer's test; rose bengal test
Treatment of Sjögren Syndrome
There is currently no cure for SS; treatment is therefore based on symptom relief and the prevention of complications.
- Eye involvement: topical replacement of tears with preservative-free “artificial tears”; medications to increase tear production (pilocarpine [Salagen]; cevimeline [Evoxac])
- Oral involvement: good oral hygiene to reduce cavity risk; sugar-free chewing gums or lemon lozenges to help stimulate saliva production; saliva substitutes (e.g., glycerine, mucin, or carboxymethylcellulose); medications to increase salivary flow (Salagen; Evoxac)
- Studies on interferon-alpha have shown promise for relieving symptoms of both oral and eye involvement.
- Systemic symptoms: treatment of associated autoimmune disorders, if any; corticosteroids and antimalarial medications (e.g., hydroxychloroquine) may relieve joint pain and fatigue; studies with monoclonal antibodies (e.g., rituximab) show promise for treating severe inflammatory manifestations
Prognosis for People with Sjögren Syndrome
Although primary SS does not appear to increase mortality in comparison to the general population, it does confer a much higher risk for developing lymphomas. Since most deaths occur in elderly individuals, statistical all-cause mortality is unaltered.
People with secondary SS do have a higher mortality rate, probably due to their associated autoimmune diseases.
Other complications (oral ulcerations, corneal clouding, dental caries, etc.) are largely preventable.
Sjögren syndrome is a relatively common disorder whose cause is uncertain. It is often associated with other autoimmune conditions, but it can occur as an isolated illness. Treatment is aimed at symptom control.
