Sjögren’s Disease

Sjögren's disease can affect people of any age, sex or ethnicity, but it most commonly occurs in middle-aged adults and is diagnosed more frequently in women than men (approximately 9:1).

People at increased risk include:

• Women, who account for approximately 90% of cases 
• Adults, particularly during middle age, although the disease can develop at any age 
• Individuals with another autoimmune disease, such as rheumatoid arthritis, systemic lupus erythematosus or systemic sclerosis 
• People with a family history of autoimmune diseases, reflecting a shared genetic predisposition to autoimmunity 

Sjögren's disease affects millions of people worldwide, but many individuals remain undiagnosed because symptoms often develop gradually, vary considerably between patients, and may initially be mistaken for other conditions.

The exact cause of Sjögren's disease is not yet fully understood. Like many autoimmune diseases, it is believed to develop through a combination of genetic susceptibility, environmental exposures and changes in the immune system.

In people with Sjögren's disease, the immune system, which normally protects the body from infections, becomes misdirected and mistakenly attacks the body's own tissues, particularly the glands that produce tears and saliva. Over time, this inflammation can also affect joints, nerves, lungs, kidneys, skin and other organs.

Researchers believe several factors contribute to the development of the disease, including:

• Genetic susceptibility: Certain inherited genes increase the likelihood of developing autoimmune diseases, although most people with these genes never develop Sjögren's disease.
• Environmental triggers: Viral infections and other environmental exposures may trigger the disease in genetically susceptible individuals, although no single infection has been proven to cause Sjögren's disease.
• Immune system dysregulation: Abnormal activation of immune cells leads to persistent inflammation and the production of autoantibodies that contribute to tissue damage.
• Hormonal influences: Sjögren's disease occurs much more frequently in women than men, suggesting that sex hormones may influence disease development, although the exact role of hormones remains unclear.

Current research suggests that Sjögren's disease develops when these factors interact over time, rather than from a single identifiable cause. Understanding these underlying mechanisms is helping researchers develop more targeted therapies aimed at controlling the immune response and preventing long-term organ damage.

The symptoms of Sjögren's disease vary widely from person to person. While dryness of the eyes and mouth is the hallmark of the disease, Sjögren's is a systemic autoimmune disease that can affect many organs throughout the body. Symptoms often develop gradually over months or years and may range from mild to severe.

Because Sjögren's disease is systemic, it can affect many other parts of the body. Some patients may develop:

• Dry skin, rashes or inflammation of small blood vessels (vasculitis)
• Numbness, tingling, burning pain or weakness due to peripheral neuropathy
• Chronic cough or shortness of breath caused by inflammation of the airways or lung tissue
• Inflammation that can affect kidney function or the body's ability to regulate minerals and acid-base balance
• Recurrent swelling of the salivary glands, particularly near the jaw or in front of the ears

Not everyone with Sjögren's disease develops systemic complications. However, because the disease can affect multiple organs, regular follow-up with your healthcare team is important to monitor for new symptoms, detect organ involvement early and guide treatment when needed.
 

Diagnosing Sjögren's disease can be challenging because symptoms such as dryness, fatigue and joint pain can occur with many other conditions. There is no single test that can diagnose Sjögren's disease. Instead, a rheumatologist combines your symptoms, physical examination, blood tests and objective tests of tear and saliva production to determine whether Sjögren's is the likely diagnosis.

Sjögren's disease can affect many parts of the body, so diagnosis often requires collaboration between rheumatology, ophthalmology, oral medicine or dentistry, pathology and other specialists when needed.

Your rheumatologist brings these findings together to determine whether Sjögren's disease is present and, importantly, whether the disease is limited primarily to dryness or is affecting other organs.

Many people with Sjögren's disease primarily experience dryness, fatigue and joint or muscle symptoms. However, because Sjögren's is a systemic autoimmune disease, some people may develop complications involving other parts of the body.

Potential complications include:

• Dental and oral problems: Reduced saliva can increase the risk of cavities, tooth decay, gum disease and oral infections.
• Eye complications: Severe or untreated dry eye can damage the surface of the eye (cornea) and, in some cases, affect vision.
• Nervous system involvement: Inflammation affecting the nerves may cause numbness, tingling, burning pain, weakness or other neurologic symptoms.
• Lung involvement: Sjögren's can affect the airways or lung tissue, sometimes causing persistent cough, shortness of breath, or interstitial lung disease.
• Kidney involvement: Inflammation can affect the kidneys and, in some patients, interfere with the body's ability to maintain normal electrolyte and acid-base balance.
• Vasculitis: Inflammation of blood vessels can cause skin rashes and, less commonly, affect nerves or internal organs.

Sjögren's disease and lymphoma

People with Sjögren's disease have an increased risk of developing certain types of lymphoma, particularly B-cell non-Hodgkin lymphoma, compared with the general population. However, lymphoma still develops in only a minority of people with Sjögren's disease.

Regular follow-up is important. Your healthcare team will monitor your overall disease activity and evaluate concerning changes such as persistent or unexplained salivary gland swelling, enlarged lymph nodes, unexplained weight loss, fevers or night sweats.

Routine monitoring allows complications to be recognized early and treated appropriately.

There is currently no cure for Sjögren's disease, but treatment can relieve symptoms, protect the eyes and mouth from complications and control inflammation when the disease affects other parts of the body.

Treatment is tailored to each individual based on which organs are affected and how active or severe the disease is.

Sjögren's disease can look very different from one person to another. Some people primarily need treatment for dryness, while others require treatment for systemic inflammation. Regular follow-up and individualized treatment are important to control symptoms, identify organ involvement early and protect long-term health.

Many people with Sjögren's disease lead active and fulfilling lives. In addition to medical treatment, simple daily strategies can help manage dryness, protect the eyes and teeth, reduce complications and improve overall well-being.

Helpful strategies include:

• Drinking water regularly and maintaining good hydration
• Using a humidifier, particularly in dry environments
• Using recommended eye lubricants consistently
• Practicing careful oral hygiene and receiving regular preventive dental care
• Using sugar-free gum or lozenges to help stimulate saliva when appropriate
• Avoiding smoking and other exposures that can worsen dryness
• Reviewing medications with your healthcare provider, as some medications can worsen dry eyes or dry mouth
• Staying physically active with regular exercise appropriate for your health and abilities
• Maintaining healthy sleep habits and balancing activity with adequate rest

Managing fatigue

Fatigue is common in Sjögren's disease and can significantly affect quality of life. Regular physical activity, healthy sleep habits, balanced nutrition and pacing activities may help. Persistent or worsening fatigue should also be discussed with your healthcare provider, as other treatable conditions can contribute to fatigue.

Staying connected with your care team

Regular follow-up with your rheumatologist, eye-care provider, dentist and primary care provider is an important part of Sjögren's care. These visits help manage symptoms, protect eye and dental health, monitor for systemic organ involvement and identify complications early.

With appropriate treatment, preventive care and regular monitoring, many people with Sjögren's disease can successfully manage their symptoms and maintain a good quality of life.

Consider seeking medical evaluation if you have persistent symptoms that could suggest Sjögren's disease, particularly when several symptoms occur at the same time.

These may include:

• Persistent dry eyes or dry mouth
• Recurrent dental cavities or other problems related to dry mouth
• Unexplained or persistent fatigue
• Joint pain, stiffness, or swelling
• Recurrent or persistent swelling of the salivary glands, particularly around the jaw or in front of the ears
• Numbness, tingling, burning pain, or weakness in the hands or feet

A rheumatologist can evaluate whether Sjögren's disease, another autoimmune condition, medications or other medical conditions may be contributing to your symptoms.

Because Sjögren's can affect different parts of the body, care often involves a multidisciplinary team. Your rheumatologist may coordinate care with ophthalmology, dentistry or oral medicine, neurology, pulmonology, nephrology and other specialists depending on your symptoms and organ involvement.

This coordinated approach helps address both the dryness-related symptoms and the systemic manifestations of Sjögren's disease.

Sjögren’s disease is a chronic autoimmune disease that primarily causes dry eyes and dry mouth, but it can also affect other organs in the body. Early diagnosis and appropriate management can help control symptoms, prevent complications and improve quality of life.

If you have symptoms of Sjögren’s disease, speak with your healthcare provider or schedule an evaluation with a UK HealthCare rheumatologist.

• American College of Rheumatology
• Sjögren’s Foundation
• Sjögren’s Society of Canada