What are the treatment options for Sjögren's (Sjögren's syndrome)?

From the Guidelines

Treatment of Sjögren's syndrome should focus on managing symptoms and preventing complications, with a multidisciplinary approach involving various health professionals, and the first therapeutic approach to dryness should be symptomatic relief using topical therapies, as recommended by the European League Against Rheumatism (EULAR) 1.

Treatment Options

• For dry eyes, artificial tears used frequently throughout the day provide relief, with preservative-free options preferred for frequent use.
• Prescription medications like cyclosporine eye drops (Restasis) or lifitegrast (Xiidra) can reduce inflammation and increase tear production.
• For dry mouth, patients should sip water frequently, use sugar-free gum or lozenges to stimulate saliva, and consider prescription medications like pilocarpine (5-10mg three times daily) or cevimeline (30mg three times daily) to increase saliva production.
• Systemic symptoms may require hydroxychloroquine (200-400mg daily), which can improve fatigue and joint pain with effects typically seen after 2-3 months of treatment.
• For more severe systemic disease, immunosuppressants like methotrexate (starting at 7.5mg weekly, potentially increasing to 20mg) or rituximab may be necessary.

Additional Recommendations

• Patients should practice good oral hygiene, including regular dental check-ups, fluoride treatments, and avoiding sugary foods to prevent dental caries.
• Vaginal dryness can be treated with lubricants or moisturizers.
• Treatment should be tailored to each patient's specific symptoms and disease severity, with regular monitoring for disease progression and medication side effects.

Key Considerations

• The use of systemic therapies (glucocorticoids, antimalarials, immunosuppressive agents, intravenous immunoglobulins, biologics) should be restricted to patients with active systemic disease, as defined by the ESSDAI score 1.
• The management of systemic features must be tailored to the specific organ involved and the severity evaluated by the ESSDAI.
• The addition of immunosuppressive agents as GC-sparing agents is justified in patients requiring long-term therapy with glucocorticoids, especially those with severe organ impairments 1.

From the FDA Drug Label

Pilocarpine hydrochloride tablets are indicated for ... the treatment of symptoms of dry mouth in patients with Sjogren’s Syndrome. The recommended dose of pilocarpine hydrochloride tablets is one tablet (5 mg) taken four times a day. Efficacy was established by 6 weeks of use.

The treatment option for Sjögren's syndrome is pilocarpine (PO), with a recommended dose of 5 mg taken four times a day 2 2.

From the Research

Treatment Options for Sjögren's Syndrome

The treatment options for Sjögren's syndrome are varied and depend on the severity of the symptoms. Some of the treatment options include:

• Topical therapies such as saliva substitutes and preservative-free artificial tears to manage sicca manifestations 3, 4
• Stimulation of salivary flow with a sialogogue (pilocarpine or cevimeline) for patients with residual salivary gland function 5, 3, 4, 6, 7
• Topical cyclosporine A for severe cases of keratoconjunctivitis sicca 3
• Hydroxychloroquine for patients with fatigue, arthralgia, and myalgia 3, 7
• Glucocorticoids and immunosuppressive agents for severe systemic involvement 3
• B-cell depleting agents, which have shown promising results in clinical trials 3
• LASER therapy, which has been reported to have therapeutic effects 7
• Oral pilocarpine, which has been found to be highly beneficial in reducing hyposalivation in SS patients 5, 6, 7
• Rituximab and interferon-alpha (IFN-α), which have been found to be moderately effective in reducing hyposalivation in SS patients 7

Systemic Medications

Systemic medications may be used to treat extraglandular features of Sjögren's syndrome, including:

• Antimalarials such as hydroxychloroquine 3, 7
• Glucocorticoids 3
• Immunosuppressive drugs 3
• Biologic agents such as rituximab and B-cell depleting agents 3, 7

Safety and Efficacy

The safety and efficacy of these treatment options have been evaluated in several studies. For example, a study found that oral pilocarpine was well tolerated and effective in relieving dry mouth and dry eye symptoms in patients with Sjögren's syndrome 5. Another study found that topical cyclosporine A was effective in treating severe cases of keratoconjunctivitis sicca 3. However, the use of these medications may be associated with adverse events, and the potential risks and benefits must be carefully considered 3, 7.