Sjögren's Syndrome: Diagnosis and Management
Sjögren's syndrome is a systemic autoimmune exocrinopathy primarily affecting the salivary and lacrimal glands, causing characteristic dry eyes and dry mouth, with potential for multiple systemic manifestations that should be managed with both symptomatic treatments and immunomodulatory therapy for systemic disease. 1
Definition and Classification
Sjögren's syndrome is characterized by:
- Primary form: Dry eyes and dry mouth occur alone
- Secondary form: Dry eyes and dry mouth occur alongside another rheumatic disease (commonly rheumatoid arthritis) 2
Clinical Presentation
Common Manifestations
- Sicca symptoms:
- Xerostomia (dry mouth)
- Xerophthalmia (dry eyes)
- Systemic manifestations (affecting approximately 50% of patients):
Diagnosis
Disease activity should be regularly assessed using the EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) 1.
Key diagnostic elements include:
- Detection of autoantibodies (anti-Ro/SSA and anti-La/SSB)
- Histological analysis of biopsied salivary gland tissue showing lymphocytic infiltration
- Objective measures of decreased tear and saliva production 3
Management Approach
1. Dry Mouth Management
Non-pharmacological approaches:
- Increase water intake throughout the day
- Sugar-free gum or lozenges to stimulate saliva production
- Saliva substitutes containing xylitol, betaine, or olive oil
- Meticulous oral hygiene to prevent dental complications
- Regular dental check-ups every 3-6 months 1
Pharmacological treatment:
- Pilocarpine (Salagen): The recommended dose is 5 mg four times daily for Sjögren's syndrome patients, with efficacy established by 6 weeks of use 4
- Starting dose should be reduced to 5 mg twice daily in patients with moderate hepatic impairment
- Not recommended for patients with severe hepatic insufficiency
- Common side effects include sweating (40%), urinary frequency (10%), nausea (9%), and flushing (9%) 4
2. Dry Eye Management
- Regular ophthalmological examination annually 1
- Artificial tears and lubricating eye drops
- Punctal occlusion in severe cases 5
3. Systemic Disease Management
Based on disease severity:
Mild systemic features:
- Hydroxychloroquine 200-400mg daily for fatigue, arthralgia, and mild systemic features 1
Moderate to severe systemic disease (ESSDAI score >5):
- Glucocorticoids for acute flares
- Immunosuppressive agents as glucocorticoid-sparing therapy:
- Methotrexate
- Azathioprine
- Mycophenolate
- Leflunomide
- Cyclophosphamide 1
Refractory cases:
- Rituximab (anti-CD20) for glandular and extraglandular manifestations
- Abatacept and belimumab are under investigation 1
Monitoring and Follow-up
- Regular assessment of disease activity using ESSDAI
- Annual ophthalmological examination
- Regular dental evaluation every 3-6 months
- Daily fluoride application to prevent dental caries
- Monitoring for development of lymphoma, which represents a serious complication 1, 3
Special Considerations
Adverse Effects of Pilocarpine
The most common adverse events with pilocarpine include:
- Sweating (40%)
- Urinary frequency (10%)
- Nausea (9%)
- Flushing (9%)
- Headache (13%)
These effects are dose-dependent, with elderly patients experiencing higher rates of urinary frequency, diarrhea, and dizziness 4.
Disease Complications
Sjögren's syndrome is associated with:
- Increased risk of lymphoma
- Excess mortality related to systemic involvement
- Potential for other autoimmune diseases 3, 6
Treatment Algorithm
| Disease Manifestation | Treatment Approach |
|---|---|
| Dry mouth (mild) | Increased hydration, sugar-free gum/lozenges, saliva substitutes |
| Dry mouth (moderate-severe) | Above measures plus pilocarpine 5 mg four times daily |
| Dry eyes | Artificial tears, annual ophthalmological examination |
| Mild systemic disease | Hydroxychloroquine 200-400mg daily |
| Moderate-severe systemic disease | Glucocorticoids for flares + immunosuppressive agents |
| Refractory systemic disease | Consider biologics (rituximab) |
Remember that Sjögren's syndrome requires a multidisciplinary approach involving rheumatologists, ophthalmologists, and dentists for optimal management of this complex systemic disease.