Features of Sjögren's Syndrome
Sjögren's syndrome is characterized by keratoconjunctivitis sicca (dry eyes) and xerostomia (dry mouth) associated with systemic immune dysfunction, resulting from lymphocytic infiltration of the lacrimal and salivary glands with secondary compromise of gland function. 1
Key Clinical Manifestations
Cardinal Features
Ocular symptoms:
- Dry eyes (keratoconjunctivitis sicca)
- Irritation, itching, or pain
- Foreign body sensation
- Light sensitivity
- Frequent use of eye drops
- Blurry vision or unexplained vision changes 1
Oral symptoms:
- Dry mouth (xerostomia)
- Need for liquids to swallow dry foods
- Frequent sipping/drinking water
- Burning sensation in the mouth
- Angular cheilitis
- Frequent dental cavities
- Teeth chipping, cracking, or eroding
- Gum inflammation 1
Systemic Manifestations
- Extreme fatigue (>50% of patients) 1
- Arthralgia and myalgia 2, 1
- Swollen parotid or submandibular glands 1
- Skin dryness or itching 1
- Vaginal dryness 1
- Peripheral neuropathy 1
- Raynaud's phenomenon 1
- Pulmonary complications (in ~10% of patients):
- Interstitial pneumonitis
- Xerotrachea
- Bronchiectasis 1
- Cough (observed in >50% of patients) 1
Epidemiology
- Prevalence: approximately 0.4% of the population 2, 1
- Annual incidence: 3.9-5.3 per 100,000 2, 1
- Female-to-male ratio: 20:1 2, 1
- About 10% of patients with clinically significant dry eye have underlying Sjögren's syndrome 2
Classification
- Primary Sjögren's syndrome: Dry eyes and dry mouth occur alone 3
- Secondary Sjögren's syndrome: Dry eyes and dry mouth occur in the context of another rheumatic disease (most commonly rheumatoid arthritis, scleroderma, or systemic lupus erythematosus) 2, 3
Diagnostic Criteria
The 2016 ACR-EULAR classification criteria for primary Sjögren's syndrome require a total score of ≥4 based on the weighted sum of:
- Anti-SSA/Ro antibody positivity (score 3)
- Focal lymphocytic sialadenitis on lip biopsy with focus score ≥1 foci/4 mm² (score 3)
- Abnormal ocular staining score ≥5 or van Bijsterveld score ≥4 (score 1)
- Schirmer's test result ≤5 mm/5 minutes (score 1)
- Unstimulated salivary flow rate ≤0.1 ml/minute (score 1) 2, 1
Diagnostic Workup
Serological panel:
- Anti-SSA/Ro antibodies
- Anti-SSB/La antibodies
- Antinuclear antibody (ANA)
- Rheumatoid factor (RF) 1
Tear film assessment:
- Tear meniscus height
- Tear break-up time
- Ocular surface staining with fluorescein and lissamine green
- Schirmer's test 1
Salivary gland assessment:
- Unstimulated salivary flow rate
- Minor salivary gland biopsy 1
Pulmonary assessment:
- Baseline pulmonary function tests
- High-resolution CT with expiratory views if respiratory symptoms are present 1
Complications
Ocular manifestations of immune dysfunction:
Increased risk for potentially life-threatening conditions:
Interstitial lung disease (found in 10-20% of patients) 1
Decreased quality of life due to chronic symptoms 1
Congenital heart block risk (2-18%) in offspring of pregnant women with anti-Ro antibodies 1
Management Considerations
Co-management with a rheumatologist is essential due to potential systemic complications 2, 1
Regular monitoring for lymphoma development, particularly in patients with decreased C4 levels 2, 1
Treatment options include:
Monitoring of associated conditions:
Sjögren's syndrome remains a complex autoimmune disorder with diverse manifestations that can significantly impact quality of life. Early recognition of its features is crucial for timely diagnosis and appropriate management.