Overview

Overview of Sjögren’s Syndrome

Sjögren’s syndrome is a chronic (long-lasting) autoimmune disorder that happens when the immune system attacks the glands that make moisture in the eyes, mouth, and other parts of the body. 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.

Who Gets

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.

Types

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.

Symptoms

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.

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.

Causes

Causes of 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.

Diagnosis

Diagnosis of Sjögren’s Syndrome

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.

To diagnose Sjögren’s syndrome, your doctor may ask about your medical history, including about dryness in your eyes and mouth, such as when it started and whether you feel it every day. Your doctor may also order the following tests.

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

Your doctor may order other laboratory or imaging tests to determine if another disease or problem is causing your symptoms.

Treatment

Treatment of Sjögren’s Syndrome

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.

Eye Treatments

  • Eye drops (artificial tears). There are many different types of eye drops, and you may have to try a few to find the one that works best for you. Some people need prescription eye drops that contain medications to suppress the immune system and reduce inflammation in the eye.
  • Eye ointments. These are thicker than eye drops and keep the eyes wet for several hours. They can blur your vision, so most people use them while they sleep.
  • Plugs to block the tear ducts. Small plugs placed in the tear duct in the corners of the eyes block drainage and keep tears in the eyes longer. The procedure only takes a few minutes and is done in an ophthalmologist’s (eye doctor’s) office.

Mouth Treatments

  • Artificial saliva. Using a saliva substitute prescribed by a doctor helps to make the mouth feel wet.
  • Saliva production stimulators. These medications cause salivary glands to make more saliva. These medications also stimulate tear production.
  • Anti-fungal medications. These medications treat fungal infections, such as candidiasis (also called thrush), which are more common in people with dry mouths.

Treatments for Other Problems Related to Sjögren’s Syndrome

  • Over-the-counter or prescribed pain relievers. These medicines alleviate joint and muscle pain, and discomfort from swollen glands.
  • Disease-modifying anti-rheumatic drugs (DMARDs) and anti-malarial drugs are often prescribed in people with joint pain, rashes, and other serious effects of the disease. While these medicines have not specifically been approved for Sjögren’s syndrome, they may be helpful in some people with the disorder.
  • Corticosteroids. These medications help control inflammation and pain. Because they are potent drugs, your doctor will prescribe the lowest dose possible to achieve the desired benefit. They are usually reserved for people with rare, serious effects of the disorder.
  • Acid reflux medications. Reduced saliva production may raise the stomach’s acidity in people with Sjögren’s syndrome. Some people may take these medicines to counteract this effect.

Who Treats

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.

Living With

Living 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.

Research Progress

Research Progress Related to Sjögren’s Syndrome

Investigators at the National Institutes of Health (NIH), as well as investigators across the country, many supported by the NIH, are working to understand what causes Sjögren’s syndrome, which may lead to new treatment strategies. A number of efforts are also aimed at testing the efficacy of medications that have been shown to work for other autoimmune diseases.

Following are examples of the types of studies that are ongoing.

  • Scientists are working to uncover the molecules and immune cells involved in the autoimmune attack in Sjögren’s syndrome. This work may help identify new therapeutic targets.
  • Clinical trials are underway to test the efficacy of disease-modifying anti-rheumatic drugs in people with Sjögren’s syndrome. Some of these drugs are novel, while others are used to treat other rheumatic conditions. They work by targeting specific immune messages and interrupting the signal, helping to decrease or stop inflammation.
  • Researchers believe that genetics is a contributing factor in who gets Sjögren’s syndrome, but the role of genes remains unclear. It appears that multiple genes are involved, and research is underway to identify them.
  • The characteristics of Sjögren’s syndrome can vary widely from person to person, and investigators are working to group patients according to patterns of symptoms, which will help researchers develop tailored therapies that better address the problems associated with each subset.
  • It can take years to diagnose Sjögren’s syndrome because the symptoms are so diverse, and they are shared with other diseases and conditions. Scientists are looking for ways to diagnose the disease earlier, such as by identifying biomarkers or using imaging techniques such as ultrasound scanning of salivary glands. Pinpointing the syndrome earlier will enable doctors to treat it sooner, potentially leading to better outcomes for patients.

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