What is Sjögren's syndrome?

From the Guidelines

Sjögren's syndrome should be managed with a focus on symptom relief, using artificial tears and saliva substitutes, and considering immunosuppressants for systemic symptoms, as recommended by the most recent guidelines 1.

Key Considerations

• The condition is characterized by dry eyes and dry mouth due to inflammation that damages moisture-producing glands.
• Treatment focuses on symptom management rather than cure.
• For dry eyes, artificial tears (preservative-free) should be used regularly throughout the day, with more viscous gels at night.
• Prescription options include cyclosporine eye drops (Restasis) or lifitegrast (Xiidra) to reduce inflammation.
• For dry mouth, patients should sip water frequently, use sugar-free lozenges, and consider saliva substitutes like Biotene.
• Pilocarpine (5-10mg three times daily) or cevimeline (30mg three times daily) can stimulate saliva production.
• For systemic symptoms, hydroxychloroquine (200-400mg daily) may help with fatigue and joint pain.
• More severe cases might require immunosuppressants like methotrexate or rituximab.

Management Approach

• Patients should maintain good oral hygiene with regular dental visits, avoid smoking, and limit alcohol and caffeine consumption.
• Regular monitoring is essential as patients have an increased risk of developing lymphoma and other complications.
• The condition occurs when the immune system attacks the body's moisture-producing glands, with 90% of cases affecting women, typically in their 40s and 50s.

Treatment Options

• Topical lubricants, topical anti-inflammatory therapy, and tear-conserving strategies seem effective for dry eye secondary to Sjögren's syndrome 1.
• Oral secretagogues, such as pilocarpine and cevimeline, are effective for sicca features, mostly oral 1.
• Cyclosporine eye drops are effective for moderate or severe dry eye 1.
• Hydroxychloroquine may help with fatigue and joint pain, but its efficacy for dry eye is not strongly supported by evidence 1.

From the Research

Definition and Classification of Sjögren Syndrome

• Sjögren syndrome is a systemic autoimmune disease characterized by dry eyes and dry mouth, with other organ systems affected in many patients 2.
• It is classified as primary or secondary, with primary disease being a solitary process and secondary disease accompanying another autoimmune disease, often rheumatoid arthritis 2, 3.

Symptoms and Diagnosis

• The disease is associated with xerostomia and xerophthalmia, which can be challenging to diagnose and require coordination with a team of specialists, including dentists, otolaryngologists, rheumatologists, and ophthalmologists 2.
• Patients with Sjögren syndrome often have anti-Ro and/or anti-La (ENA) antibodies, ANA positivity, and hypergammaglobulinaemia, with a positive rheumatoid factor (RF) also common 3.

Treatment Options

• Pilocarpine and cevimeline can help relieve dry eyes and dry mouth 2.
• Pilocarpine has been shown to stimulate the secretion of saliva and is more effective than artificial saliva for enhancing salivary and lacrimal secretion in patients with Sjögren syndrome 4.
• Topical and systemic medications, such as saliva substitutes and artificial tears, can be used to treat sicca features, while extraglandular features require tailored treatment based on the specific organ or organs involved 5.
• Pharmacological stimulants, such as Salagen and Evoxac, are FDA-approved salivary stimulants that can increase salivary output and provide an alternative to palliative measures like salivary substitutes 6.