Sjögren disease

Sjögren disease (SjD), previously known as Sjögren’s syndrome, is a chronic systemic autoimmune disease in which the immune system mistakenly attacks the body’s moisture-producing glands. The disease most commonly affects the salivary and lacrimal glands, leading to dry mouth and dry eyes, but it can also involve the joints, lungs, kidneys, nervous system, skin, blood vessels, and other organs. While some people develop Sjögren disease on its own (primary SjD), others develop it alongside another autoimmune disease such as rheumatoid arthritis or systemic lupus erythematosus (secondary SjD), though recent terminology increasingly emphasizes Sjögren disease as a single disease entity regardless of associated autoimmune conditions.

Learn More: Why Experts Now Call It Sjögren Disease Instead of Sjögren Syndrome

Dry eyes, dry mouth, difficulty swallowing dry foods, dental cavities, swollen salivary glands, fatigue, joint pain, muscle pain, dry skin, dry nose, dry cough, vaginal dryness, numbness or tingling, skin rashes, shortness of breath, and symptoms related to involvement of the lungs, kidneys, nervous system, or other organs.

Rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis (scleroderma), autoimmune thyroid diseases (including Hashimoto’s thyroiditis and Graves disease), primary biliary cholangitis, autoimmune hepatitis, autoimmune gastritis, celiac disease, Raynaud phenomenon, interstitial lung disease, peripheral neuropathy, and an increased risk of B-cell non-Hodgkin lymphoma.

Sjögren disease affects women far more often than men, with approximately 90% of diagnosed cases occurring in females. The disease most commonly develops during middle adulthood but can occur at any age, including in children. Having a family history of autoimmune disease or certain genetic risk factors may increase susceptibility. Environmental triggers, including viral infections, are also thought to contribute in genetically susceptible individuals.

Research continues to identify immune pathways involved in disease development. Recent studies have highlighted abnormal T-cell regulation, interferon signaling, calcium signaling pathways, and proteins involved in immune cell activation and gland function, although many of these findings remain under investigation.

Optimizing Outcomes for Patients Experiencing Systemic Manifestations of Sjögren’s Disease

Patients with Sjögren disease (SjD) face a variety of complex systemic symptoms that are difficult to recognize and manage. In this engaging clinical case challenge, learn tips to identify the non-sicca features of SjD, as well as the latest recommendations for the use of systemic therapies. Test your skills and see how you stack up against other health care professionals.

This activity is delivered by PRIME Education in partnership with United Rheumatology and the Sjögren’s Foundation.