Sjogren syndrome is a chronic inflammatory autoimmune disease that occurs mainly in middle-aged women. The cause of primary Sjogren syndrome is unknown, whereas secondary Sjogren syndrome is associated with underlying autoimmune diseases (e.g., rheumatoid arthritis). As the immune system mainly attacks lacrimal and salivary glands, patients typically present with xerophthalmia (dry eyes) and xerostomia (dry mouth), the combination of which is also known as sicca syndrome. The disease may also involve the skin, joints, internal organs, and nervous system. It is diagnosed via the detection of autoantibodies (anti-SSA/Ro, ANA) and salivary gland biopsy. Management focuses on supportive measures and, in more severe cases, immunosuppressants.
Epidemiological data refers to the US, unless otherwise specified.
- Primary Sjogren syndrome: idiopathic (association with HLA-DR52) 
- Secondary Sjogren syndrome
Glandular symptoms 
- Inflammation of the salivary glands: decreased production of saliva → xerostomia (dry mouth)
Inflammation of the lacrimal glands (): decreased secretion of tears → xerophthalmia (dry eyes) →
- Redness, itching, burning of eyes
- Sensation of sand or foreign body in the eyes
- Blurred vision
- Sicca syndrome: combination of dry mouth and dry eyes
- Nasal dryness: leads to chronic rhinitis, nosebleeds
- Pharyngeal, tracheal, and bronchial dryness: persistent, dry cough
- Vaginal dryness: dyspareunia and increased risk of infections
Extraglandular symptoms 
- General symptoms: fatigue; and arthralgias (∼ 70% of cases)
- Skin manifestations: xerosis ; Raynaud phenomenon (∼ 15–30% of cases)
- Vasculitis (∼ 10% of cases)
- Neurological and psychiatric manifestations
- Pulmonary manifestation: interstitial lung disease
- Gastrointestinal manifestations: e.g., dyspepsia, reflux esophagitis
Sjogren syndrome is diagnosed based on the typical clinical features and is confirmed via detection of specific antibodies and a pathologically low tear production. Biopsy of the salivary gland is the most accurate test, but is not needed to establish the diagnosis.
Laboratory tests 
- Nonspecific findings: ↑ ESR, normocytic anemia, leukopenia, eosinophilia, hypergammaglobulinemia
- Immunological findings (for additional information, see )
- Urinalysis: possible signs of glomerulonephritis or interstitial nephritis (e.g., proteinuria, red cell casts)
- Schirmer test: : shows decreased tear production
- Slit lamp examination
- Method: usually taken from the labial salivary glands
Ultrasound of the parotid gland
- Secondary Sjogren syndrome: treat the underlying disease
- For dry mouth
- For dry eyes
- Treatment of extraglandular disease: depends on the specific manifestation
- Risk of developing associated conditions (e.g., systemic lupus erythematosus, rheumatoid arthritis)
- Corneal scarring, ulcer, rupture, and infection
- B-cell lymphomas, such as MALT lymphoma (may manifest with parotid swelling)
- Pregnancy: fetal loss, infant with neonatal lupus syndrome and associated complete heart block
Individuals with Sjogren syndrome have an increased risk of developing MALT lymphoma.
We list the most important complications. The selection is not exhaustive.