Sjogren’s Syndrome

Sjogren’s syndrome is a long-term autoimmune disease where the body’s immune system attacks the glands that produce moisture, leading to widespread dryness throughout the body, especially in the eyes and mouth.

What is Sjogren’s syndrome?
What causes this condition?
Signs and symptoms
Who is most affected?
How is it diagnosed?
Treatment options
Living with Sjogren’s syndrome
Outlook and life expectancy

What is Sjogren’s syndrome?

Sjogren’s syndrome is a disease where your immune system (the body’s defense against germs and illness) makes a mistake and attacks your own healthy tissues instead of protecting them. This type of disease is called an autoimmune disease^[1].

In Sjogren’s syndrome, the immune system specifically targets the glands that produce moisture in your body. These include the glands that make tears for your eyes and saliva (spit) for your mouth. When these glands are damaged, they cannot produce enough moisture, which leads to dryness^[3].

The condition can affect the entire body beyond just the eyes and mouth. It may also cause problems in other organs such as the lungs, kidneys, joints, skin, and nervous system^[2].

Doctors classify Sjogren’s syndrome into two types. Primary Sjogren’s syndrome develops on its own without any other health condition causing it. Secondary Sjogren’s syndrome happens when another disease, especially another autoimmune condition, triggers it^[3].

Sjogren’s syndrome is more common than many people realize. An estimated four million Americans live with this disease, making it one of the most common autoimmune diseases^[2].

What causes this condition?

Scientists do not fully understand what causes Sjogren’s syndrome. They believe it happens due to a combination of genetic factors (traits passed down in families) and environmental triggers^[7].

Several genes appear to be involved in the development of Sjogren’s syndrome. Some researchers think that a previous infection with a virus or bacteria might trigger the condition in people who are genetically prone to developing it^[7].

For secondary Sjogren’s syndrome, other health conditions can trigger the disease. These triggers include other autoimmune diseases and some viral infections^[3].

Viral infections that have been linked to secondary Sjogren’s syndrome include hepatitis C, cytomegalovirus (a common virus in the herpes family), Epstein-Barr virus, human T-lymphotropic virus 1, and COVID-19^[3].

Autoimmune diseases that are commonly associated with Sjogren’s syndrome include rheumatoid arthritis, psoriatic arthritis, and lupus. About half of people with Sjogren’s syndrome also have another autoimmune disease^[3]^[4].

Signs and symptoms

The two main symptoms of Sjogren’s syndrome are dry eyes and dry mouth. However, the condition can cause many other problems throughout the body^[1].

People with dry eyes may feel like their eyes burn, itch, or feel gritty, as if there is sand in them. The eyes might turn red or be sensitive to light^[1]^[4].

Dry mouth can make it feel like your mouth is full of cotton. This makes it difficult to swallow or speak, especially when eating dry foods. Without enough saliva, you may have trouble tasting food properly. Dry mouth also increases the risk of tooth decay, gum disease, and infections in the mouth^[1]^[4].

The salivary glands (the glands that produce saliva) may become swollen, particularly the ones located behind your jaw and in front of your ears^[1]^[4].

Many people with Sjogren’s syndrome experience additional symptoms. These include joint pain and swelling, muscle pain or weakness, and swollen lymph nodes (small organs that are part of the immune system)^[3].

Severe tiredness, called fatigue, is very common. This is not ordinary tiredness but an overwhelming feeling of exhaustion that does not improve with rest^[2]^[4].

Other areas of the body can also be affected. Dryness may occur in the skin, nose, throat, and vagina. Some people develop skin rashes. You may experience a persistent dry cough, a hoarse or quiet voice, or frequent nosebleeds. Heartburn and other digestive problems can occur. Some people have difficulty thinking clearly, which is sometimes called brain fog^[3]^[6].

The condition affects people differently. Some individuals have mild symptoms that barely affect their daily life. Others have more severe symptoms that make everyday activities challenging^[6].

Symptoms may come and go in cycles. There may be times when symptoms get worse (called flare-ups) and times when they improve (called remission). It is not always clear why symptoms change, but things like not getting enough rest or doing too much may trigger flare-ups^[6].

Who is most affected?

Sjogren’s syndrome can affect anyone, regardless of age, race, or background. However, certain groups of people are more likely to develop this condition^[7].

Women are much more likely to have Sjogren’s syndrome than men. More than 90% of people with this condition are women. The disease is about ten times more common in women than in men^[3]^[4].

Most people are diagnosed between the ages of 45 and 55, though the condition can develop at any age. While children can be affected, this is less common^[3]^[4].

People who already have another autoimmune disease are at higher risk. Around half of people with Sjogren’s syndrome have at least one other autoimmune condition^[3].

How is it diagnosed?

Diagnosing Sjogren’s syndrome can be challenging. The symptoms are similar to those caused by other diseases, and side effects from certain medications can mimic the signs of Sjogren’s syndrome. Because of this, it may take several years to reach a proper diagnosis^[8].

A doctor will start by taking your medical history and performing a physical examination. During the exam, the doctor checks for physical signs of Sjogren’s syndrome, such as a dry mouth, and looks for signs of other related diseases^[7]^[8].

Several tests can help confirm the diagnosis. Blood tests check for different things, including levels of various types of blood cells, the presence of antibodies (proteins made by the immune system) that are common in Sjogren’s syndrome, evidence of inflammation, and signs of problems with your liver and kidneys^[8].

The most common antibodies found in people with Sjogren’s syndrome are called anti-SSA (also known as Ro) and anti-SSB (also known as La). Tests may also look for a positive antinuclear antibody (ANA) and rheumatoid factor^[4].

Eye tests can measure how dry your eyes are. One common test is called a Schirmer tear test, where a small piece of filter paper is placed under your lower eyelid to measure how much tears you produce. An eye doctor may also examine the surface of your eyes with a special magnifying device called a slit lamp^[8].

Tests to check your salivary glands may include measuring how much saliva you produce. For this test, you might be asked to spit or drip saliva into a container for five minutes^[6].

Special imaging tests can check how well your salivary glands are working. These might include X-rays or nuclear medicine tests that track how quickly a special substance reaches your salivary glands^[8].

Sometimes a lip biopsy is needed to confirm the diagnosis. During this procedure, a small piece of tissue containing salivary glands is removed from your lip and examined under a microscope to look for clusters of inflammatory cells^[8].

Treatment options

There is currently no cure for Sjogren’s syndrome. However, many treatments are available to help relieve symptoms and prevent complications. Treatment focuses on managing the dryness and other symptoms to improve quality of life^[1]^[6].

Your treatment plan should be personalized based on your specific symptoms and how they affect you. Most people will need a combination of different treatments^[9].

For dry eyes, you can use artificial tears (eye drops) throughout the day. These are available without a prescription. Some people need prescription eye drops that help increase tear production, such as cyclosporine or lifitegrast^[4]^[6].

For severe dry eyes, a doctor may recommend a procedure to seal the tear ducts in your eyes. This helps keep more moisture on the surface of the eye^[3].

For dry mouth, drinking water frequently throughout the day helps keep your mouth moist. Chewing sugar-free gum can help stimulate saliva production. Some people need prescription medications such as pilocarpine or cevimeline to increase saliva production^[4]^[6].

Good dental care is very important. You should brush your teeth two or three times a day with fluoride toothpaste, floss daily, and see your dentist every six months for check-ups^[6].

For joint and muscle pain, your doctor may recommend pain relievers such as paracetamol or nonsteroidal anti-inflammatory drugs (NSAIDs). NSAIDs are medications that reduce inflammation and pain^[6]^[9].

For more severe symptoms or when the disease affects internal organs, stronger medications may be needed. These might include hydroxychloroquine, corticosteroids (medications that reduce inflammation), or other drugs that suppress the immune system such as methotrexate, azathioprine, or mycophenolate^[4]^[6].

For life-threatening cases or severe complications, doctors may use a medication called rituximab or intravenous immunoglobulin therapy (IVIG), which involves giving antibodies through a vein^[4].

New treatments are being studied in clinical trials. These include drugs called dazodalibep, ianalumab, and nipocalimab^[4].

Living with Sjogren’s syndrome

While Sjogren’s syndrome is a chronic condition, there are many things you can do to manage your symptoms and improve your quality of life.

Staying hydrated is very important. Drink plenty of water throughout the day by taking small sips. Carry a water bottle with you so you always have water available^[6].

Using a humidifier in your home can add moisture to the air, which helps with dryness in your eyes, mouth, nose, and skin^[6].

Wearing sunglasses when you go outside can protect your eyes from wind, dust, and sun, which can make dry eyes worse. Choose sunglasses with sides that cover the area around your eyes^[6].

Eating a healthy, balanced diet is important. Include foods rich in omega-3 fatty acids, such as oily fish and walnuts. Avoid spicy, acidic, and salty foods that can irritate your dry mouth. Also avoid sugary foods and drinks, which can increase the risk of tooth decay^[6].

If you smoke, quitting is strongly recommended. Smoking can irritate your eyes and mouth and makes saliva dry up faster^[6].

Try to avoid spending long periods in smoky, dry, dusty, or windy places, or in air-conditioned or heated rooms. These environments can make dryness worse^[6].

Getting adequate rest is crucial. Listen to your body and take breaks when you need them. Try to get enough sleep each night. Dividing your day into parts and resting during one of those parts can help manage fatigue^[16].

Regular gentle exercise can help with mobility, joint problems, and overall wellbeing. However, avoid overdoing it, as too much activity can trigger symptoms^[16].

Managing stress is important. Consider techniques such as meditation or mindfulness. Talking with a mental health professional can also help^[16].

It can be helpful to connect with other people who have Sjogren’s syndrome. Consider joining a support group where you can share experiences and learn from others^[16].

Regular check-ups with your healthcare providers are essential. This includes visits to your doctor who manages your Sjogren’s syndrome (often a specialist called a rheumatologist), regular dental check-ups, and regular eye examinations^[4].

Outlook and life expectancy

Sjogren’s syndrome is a lifelong condition, but with proper treatment and self-care, most people can manage their symptoms and continue with their normal activities^[4].

The condition affects everyone differently. Some people are able to work and do their usual activities with minimal disruption. Others may find that symptoms affect their daily life more significantly. For example, severe dry mouth can make eating difficult, and dry eyes may cause vision problems. Fatigue and pain can also impact what you are able to do^[6].

Early diagnosis and proper treatment are important. They can prevent serious complications and greatly improve a person’s quality of life^[2].

Most people with Sjogren’s syndrome live into old age and have the same life expectancy as the general population. Rarely, the condition can cause serious complications, including an increased risk of a type of cancer called lymphoma^[4]^[6].

Regular monitoring by your healthcare team helps ensure that any complications are caught early and treated appropriately.