Sjögren's Syndrome and Associated Conditions
Systemic Manifestations
- Sjögren's syndrome is a systemic autoimmune disorder characterized by lymphocytic infiltration of the lacrimal and salivary glands, leading to dry eyes and dry mouth, with a female predominance and a female-to-male ratio of 20:1, affecting approximately 0.4% of the general population, and causing systemic manifestations including arthralgia, myalgia, and fatigue, according to the American Academy of Ophthalmology 1, 2
- The condition has the potential for multiple organ involvement beyond exocrine glands, as noted by the American College of Rheumatology 1, 2, 3
Known Comorbidities
- Primary biliary cholangitis occurs in 8% of cases of limited cutaneous systemic sclerosis (lcSSc), usually in those positive for anti-centromere antibodies, as reported by the European League Against Rheumatism 3
- Thyroid dysfunction or autoimmune thyroiditis is associated with Sjögren's syndrome, according to the American Thyroid Association 1, 2
- Patients with Sjögren's syndrome have an increased risk for lymphoproliferative disorders, with approximately 5% of patients developing lymphoma, as stated by the American College of Rheumatology 4
- Decreased C4 levels at diagnosis are associated with a higher risk of developing lymphoma in patients with Sjögren's syndrome, as noted by the American College of Rheumatology 1, 2, 4
Management Considerations
- Co-management with a rheumatologist is essential for patients with Sjögren's syndrome due to potential systemic complications, as recommended by the American College of Rheumatology 1, 2, 4
- Regular monitoring for signs of lymphoma development is recommended for patients with Sjögren's syndrome, according to the American College of Rheumatology 4
- Screening for other autoimmune conditions may be warranted for patients with Sjögren's syndrome, as suggested by the European League Against Rheumatism 3