- Clinical science
Sjögren syndrome is a chronic inflammatory autoimmune disease that occurs mainly in middle-aged women. The cause of primary Sjögren syndrome is unknown, whereas secondary Sjögren 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.
- Sex: ♀ > ♂ (∼ 9:1)
- Age of onset: : typically 40–60 years
Epidemiological data refers to the US, unless otherwise specified.
- Primary Sjögren syndrome: idiopathic (association with HLA-DR52 estimated in 87% of cases)
- Secondary Sjögren syndrome
Inflammation of the salivary glands: decreased production of saliva → xerostomia (dry mouth)
- Increased formation of dental caries; and tendency to oral infections
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 → chronic rhinitis, nosebleeds
- Pharyngeal, tracheal, and bronchial dryness → persistent, dry cough
- Vaginal dryness → dyspareunia (painful intercourse) and increased risk of infections
- General symptoms: : fatigue and arthralgias (∼ 70% of cases)
- Skin manifestations: xerosis ; Raynaud's phenomenon (∼ 15–30% of cases)
- Vasculitis (∼ 10% of cases)
Neurological and psychiatric manifestations
- Variety of focal and/or diffuse findings (e.g., impaired gross motor control, paresis, seizures, peripheral neuropathy)
- Gastrointestinal manifestations: e.g., dyspepsia, reflux esophagitis
Sjögren 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.
- Nonspecific findings: ↑ ESR; , normocytic anemia, leukopenia, eosinophilia, hypergammaglobulinemia
- Immunological findings (also found in )
- Urinalysis: possible signs of glomerulonephritis or interstitial nephritis (e.g., proteinuria, red cell casts)
Schirmer's test: : shows decreased tear production
- < 5 mm of moisture on the filter paper after 5 minutes are indicative of Sjögren syndrome
- Slit lamp examination
- Taken from the salivary gland or the inner mucosa of the lip
- Shows destruction of minor salivary glands (fibrosis, parenchymal atrophy)
- Sialadenitis with dense lymphocytic infiltrations
- Treat the underlying disease in secondary Sjögren syndrome.
- For dry mouth
- For dry eyes
- Treatment of extraglandular disease depends on the specific manifestation: arthralgia may be managed with NSAIDs or hydroxychloroquine, whereas neurological manifestations, for instance, often require aggressive immunosuppression.
- Risk of developing associated conditions (e.g., systemic lupus erythematosus, rheumatoid arthritis)
- Corneal scarring, ulcer, rupture, and infection
- Pregnancy: fetal loss, infant with neonatal lupus syndrome and associated complete heart block
We list the most important complications. The selection is not exhaustive.