Sjogren's Syndrome

What is Sjögren’s Syndrome?

Sjögren’s syndrome is an autoimmune disease that happens when your immune system damages the glands that produce and control moisture in your body.  Both sebaceous glands and sweat glands are organs in your body that protect your skin from becoming too dry. Sebaceous glands produce an oily substance called sebum and sweat glands produce a watery substance called sweat. Sweat glands have ducts, which are pathways to excrete substances to the surface of your skin. The main symptoms are dry eyes and mouth, but other parts of the body may be affected as well, with many people reporting fatigue and joint and muscle pain. In addition, the disease can damage the lungs, kidneys, and nervous system. Sjögren’s syndrome predominantly affects women.

Sjögren’s syndrome can occur by itself or alongside other autoimmune conditions such as rheumatoid arthritis or systemic lupus erythematosus.

There is no cure for Sjögren’s syndrome, but there are several ways to treat and manage the symptoms.

What causes Sjögren’s syndrome?

Sjögren’s syndrome is an autoimmune disorder that happens when the immune system attacks healthy tissues. Normally, the immune system protects the body from infection and disease. Researchers do not know what causes the immune system to turn on the body, but they believe that both genetic and environmental factors are involved. Studies have linked Sjögren’s syndrome to variants (changes) in several genes, many of which are involved in immunity. In Sjögren’s syndrome, the immune system attacks the glands that make tears and saliva. The resulting inflammation damages the glands, limiting their production of the fluids that normally keep the eyes and mouth moist. In some cases, the immune system attacks additional parts of the body, damaging other organs and tissues and causing a range of other symptoms.

Primary Sjögren’s syndrome happens with no known trigger or cause.

Other health conditions trigger secondary Sjögren’s syndrome, especially other autoimmune diseases and some viral infections.

Viral infections that can trigger secondary Sjögren’s syndrome include:

→ Hepatitis C

→ Cytomegalovirus (CMV)

→ Epstein-Barr virus

→ Human T-lymphotropic virus 1 (HTLV)

→ COVID-19

Any autoimmune disease can trigger secondary Sjögren’s syndrome. 

Some autoimmune diseases that are related to Sjögren’s syndrome include:

→ Rheumatoid arthritis

→ Psoriatic arthritis.

→ Lupus

Even though studies have linked Sjögren’s syndrome to other conditions, there’s no guarantee you’ll develop it if you have these conditions.

Similarly, Sjögren’s syndrome might make you more likely to develop other autoimmune conditions, but that doesn’t mean you definitely will.

People with Sjögren’s syndrome have a higher chance of developing a type of cancer called lymphoma, but the risk of developing it is low.

What are the risk factors for Sjögren’s syndrome?

Anyone can develop Sjögren’s syndrome, but certain groups of people are more likely to have it:

→ Women and people assigned female at birth (AFAB). More than 90% of people with Sjögren’s syndrome are AFAB. People assigned male at birth (AMAB) can develop it, but it’s much less common.

→ People with other autoimmune diseases. Around half of people with Sjögren’s syndrome have at least one other autoimmune condition.

→ People between the ages of 45 and 55. Children, younger adults and adults older than 55 can have Sjögren’s syndrome, but it usually develops in adults in that age range.

→ People who have a biological relative with Sjögren’s syndrome. Around 10% of people with Sjögren’s syndrome have a direct relative (a biological parent or sibling) with it, too.

Who Gets Sjögren’s Syndrome?

Most people with Sjögren’s syndrome are women. You can get it at any age, but it is most common in people in their 40s and 50s. It occurs across all racial and ethnic backgrounds.

What are the different types of Sjögren’s Syndrome?

Doctors divide Sjögren’s syndrome into two categories:

Primary form: You have this form if you do not have another rheumatic disease.

Secondary form: You have this form if you also have another rheumatic disease, such as rheumatoid arthritis, systemic lupus erythematosus, scleroderma, or polymyositis.

What are the symptoms of Sjögren’s syndrome?

Sjögren’s syndrome may have different effects on the body, and the symptoms vary from person to person. In some people, symptoms cycle between mild and severe.

The classic symptoms are:

→ Dry eyes: Your eyes may burn or itch or feel like they have sand in them. Sometimes, the dryness causes blurry vision or sensitivity to bright light. You may get irritated, itchy eyelids due to inflammation.

→ Dry mouth: Your mouth may feel chalky, and you may have trouble swallowing, speaking, and tasting. Because you lack the protective effects of saliva, you may develop more dental decay (cavities) and mouth infections, such as candidiasis (also called thrush).

In some people, the main problem is dry mouth, while for others it is dry eyes, and some people experience both problems equally. In some cases, Sjögren’s syndrome affects other tissues and organs and has more widespread effects on the body. 

These other effects may cause:

- Fatigue

- Joint pain

- Dry skin

- Dry nasal passages and throat, and a dry cough

- Skin rashes

- Muscle aches

- Acid reflux

- Vaginal dryness

- Swelling of the glands around the face and neck

- Trouble sleeping

- Poor concentration and memory problems

- Numbness, tingling, and weakness, especially in the extremities

- Shortness of breath or trouble breathing

- Muscle weakness

The symptoms can be severe, with some people reporting debilitating pain and fatigue.

How is Sjögren’s syndrome diagnosed?

There is no single test for Sjögren’s syndrome, so doctors will typically ask about your symptoms and conduct a series of tests to diagnose the disorder. A rheumatologist (a specialist in autoimmune diseases) may diagnose the disease. However, an ophthalmologist (eye doctor) or a dentist may also perform certain tests to help make the diagnosis. The diagnosis is based on how well the tear and salivary glands are working and whether there is evidence of autoimmunity.

A healthcare provider will diagnose Sjögren’s syndrome with a physical exam and some tests. They’ll examine your body and ask about your symptoms. Tell your provider when you first noticed changes in your body and if certain times of day or activities seem to make the symptoms worse. Diagnosing Sjögren’s syndrome is usually part of a differential diagnosis. This means your provider will probably use a few tests to rule out other conditions and causes of your symptoms before diagnosing you with Sjögren’s syndrome. 

Some tests you might need include:

→ Blood tests

Blood tests can identify antibodies that are typically present in people with Sjögren’s syndrome and other autoimmune disorders. While the presence of these antibodies can help doctors diagnose Sjögren’s syndrome, this alone cannot diagnose the disorder because these antibodies may be present in healthy individuals and people with other disorders as well.

→ An eye exam

Eye tests to see if you produce a normal amount of tears, and to find out if there has been any damage to your eyes due to dryness.

→ Salivary gland tests

Salivary gland tests can measure how much saliva your mouth produces. Ultrasound imaging and biopsy can help determine if the salivary gland tissues are altered by inflammation.

→ A dental cleaning and checkup

→ Urinalysis

→ Biopsies

→ X-rays

You might need to see a few specialists, including:

→ A rheumatologist.

→ Eye care specialists

→ A dentist

→ An otolaryngologist (ear, nose and throat specialist)

How is Sjögren’s syndrome treated?

There is no cure for Sjögren’s syndrome, so treatment focuses on relieving symptoms and preventing complications. Treatments are different for each person and will depend on which parts of your body are affected. Your treatment plan will likely include a combination of self-management approaches and over-the-counter and prescription medications. Be sure to tell your doctor which medications you are currently taking because some make eye and mouth dryness worse.

Which treatments you’ll need depends on where Sjögren’s syndrome affects you.

Some common treatments for dryness in your eyes, mouth or vagina include:

→ Artificial tears: Your eye care specialist might suggest over-the-counter (OTC) eye drops, prescription drops or lubricants to keep your eyes moist.

→ Dry eye surgery (punctal plugs): An ophthalmologist (eye surgeon) can surgically close some or all of your tear ducts to keep your natural tears in your eyes for longer.

→ Saliva producers: Your dentist may suggest over-the-counter saliva (spit) supplements or prescriptions to help your body make more saliva. They might suggest you chew gum, sip water throughout the day or suck on ice chips to help moisturize your mouth.

→ Special mouthwash or dental care products: People with Sjögren’s syndrome have a higher risk of dental health issues, so you may need more frequent cleanings, special fluoride treatments or prescription toothpaste and mouthwash.

→ Vaginal moisturizers or lubricants: Your provider will suggest over-the-counter or prescription lubricants to add moisture to your vagina every day or during sexual intercourse.

→ Hormone therapy: Some people need hormone therapy (like supplemental estrogen) to treat vaginal dryness caused by a hormonal imbalance.

Treatments for pain and other symptoms can include:

→ Over-the-counter pain relievers: Over-the-counter NSAIDs (nonsteroidal anti-inflammatory drugs) or acetaminophen can relieve pain and reduce swelling. Don’t take these medications for more than 10 days in a row without talking to your provider.

→ Corticosteroids: Prescription anti-inflammatory medications your provider will give you as pills or injections.

→ Immunosuppressants: Prescription medications that calm your immune system and reduce how active it is. Your provider might recommend disease-modifying antirheumatic drugs (DMARDs).

Who treats Sjögren’s Syndrome?

Sjögren’s syndrome is primarily treated by:

Rheumatologists: doctors who treat diseases of the joints, muscles, and bones. Rheumatologists are also specialists in autoimmune diseases.

Other specialists who may be involved in your care include:

→ Dentists, who care for your gums and teeth.

→ Mental health professionals, who can help people cope with difficulties in the home and workplace that may result from their medical conditions.

→ Nephrologists, who treat kidney disease problems. 

→ Neurologists, who specialize in treating diseases of the nervous system, which includes the brain and spinal cord.

→ Ophthalmologists, who specialize in the care of the eyes.

→ Otolaryngologists, who specialize in caring for the ears, nose, and throat.

→ Primary care doctors, such as family physicians or internal medicine specialists, who coordinate care between the different health care providers and treat other problems as they arise.

→ Pulmonologists, who specialize in treating diseases of the lungs.

How to live with Sjögren’s syndrome?

The symptoms of Sjögren’s syndrome can largely be managed, and most people can expect to live a normal life. The following tips can make living with Sjögren’s syndrome easier.

→ Caring for your eyes

Protect your eyes from drafts, breezes, and smoky rooms.  Have your glasses fitted with shields on the sides, or use wraparound glasses. Do not use eye drops that irritate your eyes. If one brand or prescription bothers you, try another. Eye drops that do not contain preservatives are usually essential if you use them four or more times per day on a regular basis. Put humidifiers in the rooms where you spend the most time, including the bedroom, or install a humidifier in your heating and air conditioning unit. If you get blepharitis (eyelid inflammation), use warm compresses on your eyes to alleviate the discomfort. You may also gently wash the eyelids with a dilute solution of a mild detergent such as baby shampoo.

→ Caring for your mouth

Brush and floss your teeth regularly. There are toothpastes designed for people with dry mouths. Most people should use toothpaste with fluoride to help prevent cavities. Carry a water bottle and sip on it throughout the day to keep your mouth moist. Keeping hydrated will also help combat dry eyes. Chewing gum or sucking on hard candy helps your glands make more saliva. Try to use sugar-free gum and candy. Visit a dentist at least twice a year to have your teeth examined and cleaned. Ask your dentist about fluoride treatments. See your doctor or dentist if you have symptoms of candidiasis (also called thrush), such as burning, soreness, and white patches inside your mouth. 

→ Managing other Sjögren’s syndrome-related symptoms

Moisturize other dry areas. For dry skin, moisturize your skin regularly, especially with products made for extra dry skin. Use lip balms, such as those containing petroleum jelly, for dry lips. Use products such as vaginal moisturizers or estrogen creams for vaginal dryness. Use saline sprays to help with dry nose. Educate yourself and get support. Learn as much as you can about the syndrome and talk with others who are dealing with it by joining a support group. Having a support network can help you manage difficult times. Having a long-term condition can be challenging, so visit a mental health professional if emotional problems arise. Maintain a healthy weight and watch what you eat to help control acid reflux. Eat slowly and avoid common triggers such as fried and fatty foods, tomato sauce, and onions. Eat a healthy and balanced diet, and exercise regularly to help combat fatigue and to help you sleep better. Check with your doctor before beginning an exercise routine. Remember to visit your health care providers regularly and to follow their recommendations.

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Links:

https://www.niams.nih.gov/health-topics/sjogrens-syndrome

https://www.nhs.uk/conditions/sjogrens-syndrome/symptoms/