What Triggers Sjogren's Syndrome?

Reviewed on 1/8/2021

What is Sjogren's syndrome?

Possible triggers of Sjogren's syndrome include infections, rheumatic diseases, and hormonal changes.
Possible triggers of Sjogren's syndrome include infections, rheumatic diseases, and hormonal changes.

Named after the Swedish doctor who first noted its symptoms in the early 1900s, Sjogren’s syndrome causes dryness, fatigue, and joint pain throughout the body. It is an autoimmune disease, which means that the immune system, which normally protects you from infection, turns and attacks the cells of the body.

Symptoms of Sjogren's syndrome

Sjogren’s is a systemic disease, meaning it affects the entire body. Some people have only mild symptoms, while others experience more difficult complications. Symptoms can include:

Dry eyes

In Sjogren’s, the white blood cells (part of the immune system) mistakenly attack the glands that produce moisture. This includes the lacrimal glands that produce tears. Without enough tears, your eyes feel dry and gritty. They may itch or burn or be red, watery, or sensitive to bright light. You may have blurred vision. People with Sjogren’s are at an increased risk of eye infections and damage to the cornea (the front of the eye).

Dry nose

Dryness in the nasal passages can cause frequent nosebleeds and sinus infections. You may experience a change in your senses of smell and taste.

Dry mouth

The white blood cells also attack the glands that produce saliva to keep the mouth and throat moist. This sometimes makes these glands swollen and painful. Without enough saliva, your mouth may be uncomfortably dry and you may have frequent mouth sores, difficulty chewing, or dry, peeling lips.

Dry mouth increases your risk of tooth decay, gingivitis (gum disease), and thrush (a painful infection in the mouth).

Dry throat

The lack of saliva can lead to a burning sensation in the throat, difficulty swallowing, or esophagitis, a painful inflammation of the tube that leads from the throat to the stomach. Some people with Sjogren’s have frequent reflux and heartburn.

Respiratory issues

People with Sjogren’s may have a chronic dry cough or experience shortness of breath (feeling like you can’t breathe). They may frequently get sick with respiratory (breathing-related) illnesses like pneumonia and bronchitis.

Abdominal pain

Sjogren’s can lead to gastroparesis or irritable bowel syndrome, which both cause abdominal pain. Gastroparesis is a condition in which the stomach cannot empty normally. Signs include nausea, vomiting, and weight loss.

The symptoms of irritable bowel syndrome include bloating, constipation, and diarrhea.

Skin problems

Chronic dry skin is a common symptom of Sjogren’s syndrome. Vasculitis, inflammation in small blood vessels, may cause rashes on the arms and legs.

Chronic pain and fatigue

People with Sjogren’s may experience pain, stiffness, weakness, and swelling in their joints and muscles, even if they don’t have lupus or rheumatoid arthritis. Extreme fatigue can make it difficult for some people with Sjogren’s to complete daily tasks.

Causes of Sjogren's syndrome

Someone who has Sjogren’s syndrome most likely inherited the risk from a parent and also experienced some sort of trigger that activated the autoimmune response. Scientists are looking into the following risk factors as possible triggers:

Infection

Researchers are studying the impact of viral and bacterial illnesses on people who have inherited a high risk of developing Sjogren’s. It appears that certain infections may activate Sjogren’s syndrome.

Rheumatic disease

In about half of cases, Sjogren’s is a complication of another rheumatic disease (a condition marked by inflammation and joint or muscle pain), such as lupus or rheumatoid arthritis. This is known as secondary Sjogren’s syndrome. In primary Sjogren’s, the person does not have another rheumatic disorder.

Hormones

Sjogren’s syndrome can occur in people of any age but is typically diagnosed in people who are 40 or older. It is ten times more common in women than in men. Researchers are looking into the role of hormones in Sjogren’s syndrome, as it often starts after menopause.

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Tests for Sjogren's syndrome

The average time from first symptom to diagnosis is three years. Many of the symptoms of Sjogren’s are also symptoms of other disorders and side effects of common medications. It can take time to rule out other possible causes.

There is no single test that can diagnose Sjogren’s syndrome. Information must be gathered from a number of tests, which may include these tests:

  • Bloodwork and urine testing can check for certain proteins that are found in people with Sjogren’s.
  • An antinuclear antibody (ANA) test is a blood test that tells the doctor whether you have an autoimmune disorder.
  • Schirmer’s test shows whether your lacrimal glands are producing enough tears.
  • Ocular surface staining allows your eye doctor to see areas of damage or dryness.
  • A salivary gland scan provides images that show how well the salivary glands are working and can rule out other causes of dry mouth.
  • A salivary gland biopsy, usually taken from the lower lip, can provide information about the type and severity of inflammation.
  • Sialometry measures how much saliva your glands are producing.
  • An ultrasound can show changes in the salivary glands.

Treatments for Sjogren's syndrome

There is no cure for Sjogren’s, but there are treatments that can help ease symptoms and restore quality of life:

Artificial tears, used regularly during the day, and a gel applied to the eyes at night can keep the eyes moist. Medications such as cyclosporine (Restasis) may increase tear production by reducing inflammation in the tear glands.

People with Sjogren’s need to see their eye doctor regularly to check for signs of damage to the eyes. If they notice more eye discomfort and redness than usual, they must go to the eye doctor quickly to rule out infection.

Nasal dryness can be eased with nasal irrigation (flushing the nasal passages with saline) and the use of a humidifier to add moisture to the air.

Dry mouth may be relieved by drinking water frequently, chewing gum, or using a saliva substitute. Prescription medicines like pilocarpine (Salagen) may increase saliva production.

Antifungal medicines can treat fungal infections like thrush. If you have Sjogren’s, it is especially important to stay on top of dental checkups and hygiene (brushing and flossing) to prevent tooth decay and gum disease.

Reflux and heartburn may be reduced by proton-pump inhibitors, H2 blockers, or other medications that reduce the amount of acid in the stomach.

Hydroxychloroquine (Plaquenil) was originally a treatment for malaria but has recently been used to treat lupus and rheumatoid arthritis. It may reduce joint pain and rash in some people with Sjogren’s. Rarely, people experiencing serious complications must take steroids such as prednisone or medicines like methotrexate (Rheumatrex) that suppress the immune system.

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References
American College of Rheumatology: "Sjogren's Syndrome."

Johns Hopkins: "Sjogren's Syndrome."

National Institutes of Health: "Gastroparesis.", "Irritable Bowel Syndrome."

Sjogren's Foundation: "Symptoms."

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