Sjogren's Syndrome Symptoms and Treatments

Sjogren's syndrome is an autoimmune disease that affects moisture-producing glands and tissues, causing dry eyes and mouth, joint pain, mouth infections, and other chronic (long-lasting) symptoms. Over time, the lack of moisture can start to damage the lungs, kidneys, and nervous system.

The diagnosis of Sjogren's syndrome involves multiple tests, including blood tests that can detect autoimmune antibodies. The treatment may involve immunosuppressant drugs like steroids as well as eye drops and oral painkillers for symptomatic relief.

This article describes the symptoms and causes of Sjogren's syndrome, including how this relatively common autoimmune disease is diagnosed and treated.

What Are the Symptoms of Sjogren's Syndrome?

Sjogren's syndrome—described by Swedish physician Henrik Sjögren in the early 1900s—is a chronic autoimmune disorder with symptoms that come and go in episodic flares.

The symptoms develop when moisture-producing glands and tissues sustain damage due to the autoimmune assault. This includes salivary glands that produce saliva, lacrimal glands that produce tears, and mucosal membranes that produce mucus.

The severity of symptoms can vary, with some people only experiencing eye irritation while others develop serious complications.

Common signs and symptoms of Sjogren's syndrome include:

• Dry eyes, causing redness, grittiness, or burning
• Dry mouth
• Dry skin
• Chronic cough
• Fatigue
• Mental fogginess ("brain fog")
• Cracked or sore tongue
  
• Changes in taste or smell
• Vaginal dryness
• Blurred vision
• Oral yeast infections
• Muscle pain
• Joint pain
• Swollen lymph nodes

Complications

In some people, the decreased output of saliva and mucus can start to damage tissues and organs over time, leading to complications like:

• Tooth decay and periodontal disease
• Interstitial pneumonitis (inflammation of lung tissues)
• Bronchiectasis (irreversible widening of airways of the lungs)
• Interstitial nephritis (inflammation of kidney tissues)
• Hypothyroidism (low thyroid function)
• Irritable bowel syndrome (IBS)
• Peripheral neuropathy (burning, numbness, or tingling sensation caused by nerve damage)
  
• Vasculitis (inflammation of blood vessels)
• Pregnancy complications

Sjogren's syndrome is closely associated and often co-occurs with other autoimmune diseases like lupus, celiac disease, multiple sclerosis, Hashimoto's disease, rheumatoid arthritis, scleroderma, and polymyositis.

Sjogren's syndrome also appears to increase your risk of non-Hodgkin's lymphoma, a type of cancer affecting the lymphatic system.

What Causes Sjogren's Syndrome?

Sjogren's syndrome is one of the most common autoimmune disorders affecting as many as four million people in the United States. Around 90% of cases are in females. People of any age can get it, although the average age of onset is around 40.

The cause of Sjogren's syndrome is unknown. For reasons not entirely clear, the immune system will suddenly attack moisture-producing glands and tissues with chronic inflammation.

Genetics are thought to predispose certain people to Sjogren's syndrome, with six gene mutations currently identified: RF5, STAT4, BLK, IL12A, TNIP1, and CXCR5.

Having these gene mutations does not mean you will automatically get Sjogren's syndrome; other factors are thought to "trigger" the disease, including infection with the Epstein-Barr virus and hepatitis C virus and hormonal abnormalities like estrogen deficiency (which starts to decline steeply in females after the age of 40).

Having another autoimmune disease also increases your risk of Sjogren's syndrome.

How Is Sjogren's Syndrome Diagnosed?

No single test by itself can diagnose Sjogren's syndrome. A combination of factors is considered together in your diagnosis.

Because the symptoms are somewhat vague, it can take years for Sjogren's syndrome to be diagnosed. Your symptoms, physical examination, specialized tests, and blood tests can help identify the condition.

Your healthcare provider or dentist may observe that your mouth is dry. Your nose and skin may appear dry and your eyes may also appear red or dry. Your joints may appear swollen, and you may have a rash on your skin.

Lab Tests and Procedures

Along with your symptoms and physical examination, several diagnostic tests may help confirm the diagnosis of Sjogren's syndrome, including:

• Schirmer's test for dry eyes: This involves the placement of a paper strip under your lower eyelids to measure how much moisture is produced over five minutes.
• Slit lamp exam: An ophthalmologist can check for eye problems with a special magnifying device called a slit lamp. Certain harmless dyes applied by an eye dropper can highlight abnormalities.
• Mouth exam: Your healthcare provider will visually check for abrasions and swelling and obtain a saliva sample to evaluate in the lab.
• Lip biopsy: Your healthcare provider can surgically remove tiny salivary glands from your lower lip for a microscopic examination.
• Imaging studies: Ultrasound, computed tomography (CT), or magnetic resonance imaging (MRI) can check for abnormalities in your salivary glands or damage caused to the kidneys or lungs.
  

Blood Antibody Tests

Several blood tests can be used to identify certain antibodies commonly seen in people with Sjogren's syndrome. These include:

• Antinuclear antibody (ANA test): ANA is an antibody that attacks the body's cells. It is positive in approximately 70% of people who have Sjogren's syndrome. This antibody may also be present in people without any autoimmune disease.
• SSA (anti-Ro) and SSB (anti-La): These antibodies are common with primary Sjogren's syndrome, but you may not test positive for SSA and SSB even if you have the condition.
• Rheumatoid factor: Rheumatoid factor is a protein found in rheumatoid arthritis, and it is positive among approximately 65% of people who have Sjogren's syndrome.

How Is Sjogren's Syndrome Treated?

There is no cure for Sjogren's syndrome. However, the disease can be managed with medications that decrease the inappropriate immune response and over-the-counter or prescription remedies that help ease symptoms.

Treatment is tailored for each individual, depending on which parts of the body are affected and how severe the symptoms are. For example, joint pain or muscle pain may be managed with non-steroidal anti-inflammatory drugs (NSAIDs), while serious complications such as lymphoma need to be treated with chemotherapy.

Immunosuppressive therapy is often used to reduce the inflammatory process that damages the glands, joints, and organs in Sjogren's syndrome—this approach is often referred to as disease-modifying therapy.

Relief of Oral Symptoms

Mouthwashes, lip balm, saliva substitutes, sprays, gels, and gum can relieve dry mouth and tenderness. Medication options may include saliva and mucus-stimulating drugs, such as:

• Salagen (pilocarpine hydrochloride)
• Evoxac (cevimeline HCI)

Relief of Eye Symptoms

There are some practical ways to manage dry eyes. Sunglasses can protect your eyes from wind and drafts outdoors, and a humidifier can relieve dry air indoors to help alleviate the symptoms. Smoke and eye makeup can be irritating and should be avoided.

Artificial tears and eye ointments can help relieve chronic dry eyes. Medication options for dry eye associated with Sjogren's syndrome may include:

• Restasis (cyclosporine ophthalmic emulsions)
• Hydroxypropyl cellulose eye drops or pellets

Disease-Modifying Drugs

The risk of lung, kidney, blood vessel, or nervous system complications can be reduced with medications that suppress the immune system. They also treat severe flare-ups (exacerbation) in people with Sjogren's syndrome.

Disease-modifying treatments include:

• Corticosteroids (steroids) like prednisone
• DMARDs (disease-modifying antirheumatic drugs)
• Immunosuppressants like cyclosporine

These medications can cause side effects, including an increased risk of infection.

Summary

Sjogren's syndrome is an autoimmune disease that affects moisture-producing glands and tissues, causing dry eyes, skin, mouth, and nose as well as fatigue, joint pain, and blurred vision. Over the long term, the disease can cause damage to the kidneys, lungs, and nerves in some people.

Sjogren's syndrome is diagnosed with multiple tests, including blood antibody tests. Certain disease-modifying drugs can temper the overactive immune response. Eye drops, oral pain relievers, and mucus-stimulating medications may help ease symptoms.