What is the prognosis and management for a patient with Sjögren's (Sjögren's syndrome) syndrome and an overlapping syndrome, who is otherwise healthy?

From the Guidelines

The prognosis for a patient with Sjögren's syndrome and an overlapping syndrome is generally favorable with appropriate management, though it requires lifelong monitoring and treatment. Management should focus on symptom control and preventing complications through a combination of medications and supportive care. For dry eyes, artificial tears (preservative-free) should be used frequently during the day, with more viscous lubricants at night. Cyclosporine eye drops (Restasis) 0.05% twice daily or lifitegrast (Xiidra) 5% twice daily can reduce inflammation, as recommended by the latest guidelines 1. For dry mouth, patients should use sugar-free lozenges, frequent water sips, and saliva substitutes, while maintaining meticulous oral hygiene. Pilocarpine (5-10mg three times daily) or cevimeline (30mg three times daily) can stimulate saliva production.

For systemic manifestations, hydroxychloroquine (200-400mg daily) often helps with fatigue and joint pain, as suggested by the EULAR recommendations 1. More severe organ involvement may require immunosuppressants like methotrexate (starting at 7.5mg weekly, increasing as needed) or mycophenolate mofetil (1-2g daily in divided doses). For overlapping conditions like rheumatoid arthritis or lupus, additional targeted therapies may be necessary. Regular monitoring should include eye examinations every 6-12 months, dental check-ups twice yearly, and laboratory tests to monitor organ function and medication effects. Patients should be educated about increased lymphoma risk (5-10% lifetime risk) and the importance of reporting new, persistent symptoms promptly, as highlighted in the recent study 1.

Key considerations in management include:

• Symptomatic relief using topical therapies for dryness, as the first therapeutic approach 1
• Systemic therapies for active systemic disease, with a focus on minimizing glucocorticoid use and employing immunosuppressive agents as GC-sparing agents 1
• B-cell targeted therapies for severe, refractory systemic disease, with rituximab being the most widely studied option 1
• A multidisciplinary approach involving various health professionals, with a central role for specialists in autoimmune diseases 1

This comprehensive approach addresses both the sicca symptoms and potential systemic complications while tailoring treatment to the specific overlapping syndrome present, ultimately aiming to improve morbidity, mortality, and quality of life outcomes for patients with Sjögren's syndrome and overlapping syndromes.

From the Research

Prognosis for Overlapping Syndrome and Sjögren's Syndrome

• The prognosis for a patient with Sjögren's syndrome and an overlapping syndrome can vary depending on the specific overlap and the severity of the disease 2.
• In general, patients with Sjögren's syndrome and an overlapping syndrome may experience more severe symptoms and a poorer prognosis than those with Sjögren's syndrome alone 2.
• However, early identification and treatment of the overlapping syndrome can help improve the prognosis and prevent complications 2.

Management of Overlapping Syndrome and Sjögren's Syndrome

• The management of Sjögren's syndrome and an overlapping syndrome typically involves a multidisciplinary approach, including symptomatic treatment of sicca symptoms and management of extraglandular features 3, 4, 5.
• Topical therapies, such as saliva substitutes and artificial tears, may be used to manage sicca symptoms, while sialogogues like pilocarpine and cevimeline may be used to stimulate salivary flow 3, 4, 5, 6.
• For patients with severe systemic involvement, glucocorticoids and immunosuppressive agents may be necessary, although the use of these agents should be carefully considered due to potential risks and benefits 3, 4, 5.
• Biologic agents, such as rituximab, may also be considered for patients with severe disease, although larger controlled trials are needed to establish their efficacy 6.

Treatment Options for Specific Symptoms

• For dry eyes, topical cyclosporine A may be effective in improving symptoms 3, 6.
• For dry mouth, pilocarpine and cevimeline may be effective in improving symptoms 3, 4, 5, 6.
• For extraglandular features, such as fatigue, arthralgia, and myalgia, hydroxychloroquine may be considered, while glucocorticoids and immunosuppressive agents may be necessary for severe systemic involvement 3, 4, 5.