Sjögren’s Syndrome

Understanding Sjögren’s syndrome and the immune system

In a healthy body, the immune system attacks foreign invaders.

However, sometimes the immune system starts attacking the body because it (mistakenly) thinks foreign material is present. If this happens, it causes the destruction of healthy tissue. This condition is called an autoimmune disorder.

Sjögren’s syndrome is an autoimmune disorder that primarily affects salivary and lacrimal glands. These glands help the body create moisture in the eyes and mouth, in the form of saliva and tears.

In a person with Sjögren’s syndrome, the body fails to produce enough moisture.

This is a chronic, systemic disorder that affects 1 to 4 million people in the United States, according to the National Institute of Neurological Disorders and Stroke.

The condition is typically diagnosed as either primary or secondary. In primary Sjögren’s syndrome, there’s no other autoimmune disease present. Secondary Sjögren’s syndrome is diagnosed when an individual has another autoimmune disease. 

Primary Sjögren’s syndrome tends to be more aggressive and can cause more dryness than the secondary type.

What are the symptoms of Sjögren’s syndrome?

Dry mouth is a common symptom, which can increase your risk of cavities. It can also make it more difficult to speak or swallow. Chewing gum or sucking on candies may help with this symptom.

Dryness of the eyes often occurs, too. This may feel like a burning sensation or like something is in your eye.

Sjögren’s syndrome can affect the whole body. Some individuals have vaginal dryness, dry skin, fatigue, rashes, or joint pain. Sjögren’s syndrome can cause inflammation of organs like the kidneys or lungs.

If you have constant inflammation, your doctor might prescribe medications to help prevent organ damage. These medications are called disease-modifying antirheumatic drugs. They help tamp down the immune system even more than immune-suppressing drugs.

Risk factors for Sjögren’s syndrome

There’s no one specific cause or risk factor for Sjögren’s syndrome. Nine out of 10 people who have the condition are women, and postmenopausal women are particularly likely to develop the problem.

Research is currently being done to see if estrogen is associated with the condition.

Other autoimmune disorders are often present, and a family history of the condition appears to increase your risk of developing the syndrome.

How is Sjögren’s syndrome diagnosed?

No one diagnostic test exists for this condition. Because the symptoms of Sjögren’s syndrome are generalized symptoms, your doctor will run a variety of tests to diagnose the problem.

In addition to a physical exam and a medical history, your doctor may perform blood tests to check for certain antibodies that are linked to Sjögren’s syndrome.

Eye tests and a lip biopsy can help check eye moisture and salivary gland production. A special X-ray of the salivary glands, called a sialogram, may also be ordered.

Tell your doctor about any medications or supplements you’re taking. Side effects of certain drugs are similar to the symptoms of Sjögren’s syndrome.

How is Sjögren’s syndrome treated?

There’s no cure for Sjögren’s syndrome, but it can be treated. Treatment is aimed at relieving symptoms. Treatments that replace moisture are typically prescribed, such as eye drops or lotions.

If an individual has joint problems, nonsteroidal anti-inflammatory drugs are recommended. Severe symptoms may require immunosuppressants or corticosteroids. Getting plenty of rest and eating a healthy diet can help combat fatigue.

Are there any complications of Sjögren’s syndrome?

A possible complication of Sjögren’s syndrome is an increased risk of developing lymphoma, a cancer of the lymphatic system, which is related to the immune system.

Tell your doctor if your main salivary gland changes size or seems swollen. The following can all be symptoms of lymphoma:

  • night sweats
  • fever
  • fatigue
  • unexplained weight loss

Call your doctor if you have any of these symptoms.

What is secondary Sjogren’s syndrome?

Sjogren’s syndrome is an autoimmune disorder that damages moisture-producing glands, making it difficult to produce saliva and tears. A hallmark of the disease is infiltration of target organs by lymphocytes. When Sjogren’s syndrome occurs by itself, it’s called primary Sjogren’s syndrome. 

If you already have another autoimmune disease, the condition is called secondary Sjogren’s syndrome. With secondary Sjogren’s, you might have a milder form of the condition. But you’ll still experience symptoms of the coexisting disease. The most common cause of secondary Sjogren’s is rheumatoid arthritis (RA), another type of autoimmune disease.

Learn more: What do you want to know about rheumatoid arthritis? »

Symptoms

The symptoms of Sjogren’s can include dry eyes, mouth, throat, and upper airways. You may have difficulty tasting or swallowing your food. You may also develop a cough, hoarseness, dental issues, or have difficulty speaking. For women, vaginal dryness may occur. 

Primary and secondary forms of Sjogren’s can have similar symptoms, which include:

Less often, Sjogren’s causes:

Secondary Sjogren’s can accompany the following conditions: 

  • RA
  • primary biliary cholangitis
  • lupus
  • scleroderma 

While symptoms of RA usually include inflammation, pain, and stiffness of the joints, it can also cause other symptoms similar to Sjogren’s. These include:

  • slight fever
  • fatigue
  • loss of appetite 
Risk factors

According to the Cleveland Clinic, more than a million people in the United States have primary Sjogren’s. More than 90 percent are women. You can develop Sjogren’s at any age, but it’s most often diagnosed after age 40, according to the Mayo Clinic. The exact cause of Sjogren’s is unknown. But like RA, it’s a disorder of the immune system.

The precise cause of RA is also unknown, but there’s a genetic component involved. If you have a family member with any autoimmune disease, like RA, you’re at risk for developing one, too.

Diagnosis

There’s no single test for Sjogren’s. Diagnosis can occur after you’ve been diagnosed with another autoimmune disease and develop dryness of the mouth and eyes. Or you may experience severe gastrointestinal problems or nerve pain (neuropathy). 

To diagnose secondary Sjogren’s with RA, you’ll need to undergo a series of tests. Most often these include SSA/SSB antibodies and a lower lip biopsy to look for focal areas of lymphocytes. You may be referred to an eye doctor to test for dry eye. Your doctor will also rule out other potential causes of your symptoms.

Tests for Sjogren’s

Your doctor will first look at your complete medical history and conduct a physical exam. They will also likely order the following tests: 

  • blood tests: These are used to see if you have certain antibodies characteristic of Sjogren’s. Your doctor will look for anti-Ro/SSA and anti-La/SSB antibodies, ANA, and rheumatoid factor (RF).
  • biopsy: During this procedure, your doctor will focus on your saliva glands.
  • Schirmer’s test: During this five-minute eye test, your doctor places filter paper over the corner of your eye to see how wet it gets.
  • Rose-Bengal or lissamine green staining test: This is another eye test that measures dryness of the cornea.
Conditions that mimic Sjogren’s

Be sure to tell your doctor about over-the-counter (OTC) and prescription medications you are taking. Some medications can cause symptoms associated with Sjogren’s. These medications include:

  • tricyclic antidepressants such as amitriptyline (Elavil) and nortriptyline (Pamelor) 
  • antihistamines such as diphenhydramine (Benadryl) and cetirizine (Zyrtec)
  • oral contraceptives
  • blood pressure medications

Radiation treatments can also cause similar symptoms, especially if you receive these treatments around the head and neck area.

Other autoimmune disorders may also mimic Sjogren’s. It’s important that you take all the recommended tests and follow up with your doctor to determine the exact cause of your symptoms.

Treatment options

There’s no cure for Sjogren’s or arthritis, so treatment is essential to alleviating symptoms and improving your overall quality of life. Your treatment plan depends on the severity of your symptoms. You’ll likely need to try a combination of treatments. Some options include: 

Medications

If you have aches and pains in your joints and muscles, try OTC pain relievers or anti-inflammatory medications. Nonsteroidal anti-inflammatory drugs (NSAIDS) such as ibuprofen (Advil, Motrin) may help. 

If they don’t do the trick, ask your doctor about corticosteroids and antirheumatic or immunosuppressive medications. These work by decreasing inflammation and preventing your body from attacking its own healthy tissues.

With secondary Sjogren’s, you may also need medications to help increase secretions such as tears and saliva. Common prescription drugs include cevimeline (Evoxac) and pilocarpine (Salagen). You might need prescription eye drops to help dry eye. Cyclosporine (Restasis) and lifitegrast ophthalmic solution (Xiidra) are two options.

Lifestyle

Certain lifestyle choices can also help you combat secondary Sjogren’s and RA. First, you can fight fatigue by getting a good night’s sleep and taking breaks during the day. Also, ask your doctor about exercises that can help you increase flexibility and ease muscle and joint pain. Regular exercise can improve flexibility and lessen discomfort. It’ll also help maintain proper body weight and put less stress on joints and muscles.

Maintaining a diet rich in nutrients can improve your overall health. Stick with plant-based foods and anti-inflammatory fats found in fish and plant oils. Avoid sugar and processed foods. These can increase inflammation. 

Read more: Foods that reduce inflammation »

If you have arthritis, you may also find relief with complementary treatments such as yoga, tai chi, or acupuncture. You can even temporarily soothe aches and pains by applying heat or cold compresses. 

Oral hygiene

Chronic dry mouth causes oral and dental problems, so proper oral hygiene is important. Limit your sugar intake and get regular dental checkups. Choose dental products made for dry mouth and be sure to floss every day. Hard candy and chewing gum can help you produce saliva, but make sure they’re sugar-free. 

For dry lips, use lip balm that has an oil or petroleum base. Your doctor can recommend mouth rinses or ointments to ease pain and inflammation. These may be used along with your prescription medications.

Eye treatments

There are several things you can do to help eye-related symptoms of Sjogren’s syndrome. Use a humidifier to keep the air moist at home. Avoid smoke, and protect your eyes from wind. You should also avoid eye makeup or skin creams that could irritate your eyes. 

Your doctor might suggest you try OTC artificial tears. It they don’t help, ask your doctor to prescribe something stronger. 

Thicker eye ointments can be used while you sleep. But talk with your doctor before applying ointments or gels to your eyes. Also, a surgical procedure called punctual occlusion can temporarily or permanently close off tear ducts that drain tears from your eyes. Placing warm compresses on the eyes before bedtime can open up eyelid oil glands. This helps protect the cornea and lessen dry eye symptoms.

What kind of doctor do I need?

Doctors who specialize in diseases like arthritis are called rheumatologists. If you’ve been diagnosed with arthritis, your rheumatologist will most likely also be able to treat Sjogren’s.

Depending on the severity of your symptoms, your rheumatologist or general physician may refer you to other specialists. They will include an ophthalmologist, dentist, or an otolaryngologist, also known as an ear, nose, and throat specialist.

Read more: 5 types of health professionals you should know about »

Long-term outlook

There’s no cure for Sjogren’s or RA. But there are many treatments and lifestyle choices that can improve your quality of life. 

Symptoms of arthritis vary from very mild to debilitating, but arthritis in primary Sjogren’s is rarely damaging. The key is to work with your doctor to find the best treatments. In rare cases, people with Sjogren’s may develop lymphoma. Report signs of unusual swelling or neurologic problems to your doctor.

Rheumatoid Arthritis Complications
The effects of rheumatoid arthritis

Rheumatoid arthritis (RA), unlike osteoarthritis, affects more than just your joints. RA is an autoimmune disease that can also affect your organs and cause symptoms that range from mild to severe. Treatment can prevent or delay many of the complications of RA.

Bone and joint health

RA not only causes joint pain and stiffness. It can also cause long-term problems with bone and joint health.

Joint destruction

Progressive inflammation from RA can destroy the cartilage and bone around affected joints. Severe loss of cartilage can lead to bones becoming deformed and fusing. This can cause the joint to become immobilized.

Joint damage is often irreversible. Total joint replacement surgery may be an option for some joints, like the knees.

Early and aggressive treatment with disease-modifying antirheumatic drugs (DMARDs) may prevent or delay joint damage.

Osteoporosis

Osteoporosis is a loss of bone density. It makes fractures more likely. According to the Mayo Clinic, RA sufferers are at increased risk of osteoporosis. Some reasons for this include:

  • RA and osteoporosis being more common in older women and smokers
  • the use of corticosteroids in the treatment of RA
  • the potential for RA to directly cause bone loss in affected joints

Talk to your doctor about steps you can take to prevent bone loss. Your doctor may recommend calcium and vitamin D supplements, or even medications named bisphosphonates.

Lifestyle disruption

Both RA and its treatment can affect quality of life in a number of ways.

Sleep

Pain from RA may wake patients several times during the night, preventing restorative sleep. RA patients may also have fibromyalgia, which can disturb sleep.

Disability

Joint damage and pain can keep you from performing normal everyday tasks. It may become difficult to perform such simple tasks as getting dressed or using a computer mouse.

RA symptoms can also affect your ability to work. The Centers for Disease Control and Prevention (CDC) report that people with RA are substantially more likely to:

  • change jobs
  • reduce work hours
  • retire early
  • lose their job

RA especially affects the work life of service workers and people whose jobs are physically demanding.

Psychological problems

The stress of RA and the lifestyle changes it causes can lead to:

  • loss of self-esteem
  • feelings of helplessness
  • clinical depression
  • anxiety disorders
Other conditions

RA increases the risk of other conditions as well. People with RA are at higher risk of cardiovascular disease and infections.

Anemia

Anemia is a low level of red blood cells in the body. It causes:

  • fatigue
  • weakness
  • dizziness

Widespread inflammation caused by RA can lower production of red blood cells. This makes anemia more common among RA patients, according to an article published in the Journal of the American Dietetic Association.

Rheumatoid lung

Rheumatoid lung is a group of lung conditions that may be found in RA patients. These include:

  • fluid in the lungs or chest cavity (pleural effusions)
  • scarring of the lungs (pulmonary fibrosis)
  • lumps of tissue (nodules)
  • high blood pressure in the lungs (pulmonary hypertension)

Symptoms of rheumatoid lung include:

  • shortness of breath
  • chest pain
  • persistent cough
Heart disease

RA sometimes causes inflammation in or around the heart. It can cause both myocarditis and pericarditis. Pericarditis is inflammation of the membrane covering the heart. Myocarditis is inflammation of the heart muscle itself.

Both conditions can lead to congestive heart failure (CHF). CHF is a serious condition in which the heart cannot adequately pump blood to the rest of the body, and fluid collects in the lungs.

People with RA also have an increased risk of:

  • heart attack
  • hardening of the arteries
  • blood vessel inflammation
Sjogren’s syndrome

Sjogren’s syndrome is an autoimmune condition commonly linked to RA. The disease attacks moisture-producing cells, such as the salivary and tear glands.

Sjogren’s syndrome is most prevalent in women. It’s characterized by:

  • dry eyes
  • dry mouth and increased dental cavities
  • vaginal dryness
  • problems with swallowing and talking

Sjogren’s can also cause lung, kidney, digestive, and neurological problems.

The importance of RA treatment

It’s important to get the proper treatment for RA as early as possible. Proper RA care can increase the likelihood of remission and reduce the amount of joint damage and inflammation you experience. There are many treatments available, ranging from physical and occupational therapy to medications and surgery. Work closely with your doctor to develop a treatment plan that works for you.

References:
Dunkin MA. (n.d.). Sjögren’s syndrome and your body.
arthritis.org/diseases/more-about/sjogrens-syndrome-and-your-body
Sjögren’s syndrome. (n.d.).
arthritis.org/diseases/sjogrens-syndrome
https://www.healthline.com/health/secondary-sjogrens-syndrome-and-arthritis
Sjögren’s syndrome information page. (2019).
ninds.nih.gov/Disorders/All-Disorders/Sj%C3%B6grens-Syndrome-Information-Page
https://www.healthline.com/reviewers/nancy-carteron-md-facr
https://www.healthline.com/reviewers/kristen-m-moyer-md
Written by Jaime Herndon, MS, MPH, MFA — Updated on December 7, 2020
https://www.healthline.com/health/sjogren-syndrome#symptoms
https://www.healthline.com/authors/ann-pietrangelo
Carsons SE. (2017). Treatment guidelines for rheumatologic manifestations of Sjögren's syndrome: Use of biologic agents, management of fatigue, and inflammatory musculoskeletal pain.
ncbi.nlm.nih.gov/pubmed/27390247
Kassan S. (2013). What you need to know about Sjögren’s syndrome.
resources.lupus.org/entry/what-you-need-to-know-about-sj%25C3%25B6grens-syndrome
Mayo Clinic Staff. (2014). Sjögren’s syndrome: Definition.
mayoclinic.org/diseases-conditions/sjogrens-syndrome/basics/definition/con-20020275
Questions and answers about Sjögren’s syndrome. (2016).
niams.nih.gov/Health_Info/Sjogrens_Syndrome/
Rheumatoid arthritis. (2017).
rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Rheumatoid-Arthritis
Sjögren's syndrome. (2014).
my.clevelandclinic.org/health/articles/sjogrens-syndrome
Sjögren’s syndrome. (n.d.).
arthritis.org/about-arthritis/types/Sjogrens-syndrome/
Sjogren’s Syndrome Foundation.
sjogrens.org/home/about-sjogrens/symptoms
https://www.healthline.com/reviewers/nancy-carteron-md-facr
https://www.healthline.com/authors/-560
https://www.healthline.com/health/rheumatoid-arthritis-complications
Mayo Clinic Staff. (2016, March 18). Rheumatoid arthritis: Symptoms and causes
mayoclinic.org/diseases-conditions/rheumatoid-arthritis/symptoms-causes/dxc-20197390
Mayo Clinic Staff. (2014, July 8). Sjogren’s syndrome: Risk factors
mayoclinic.org/diseases-conditions/sjogrens-syndrome/basics/risk-factors/con-20020275
Rheumatoid arthritis (RA). (2016, July 22)
cdc.gov/arthritis/basics/rheumatoid.htm
Rheumatoid arthritis treatment. (2016, March 21)
hopkinsarthritis.org/arthritis-info/rheumatoid-arthritis/ra-treatment/
Thomson, C. A., Stanaway, J., Neuhouser, M. L., Snetselaar, L. G., Stefanick, M. L., Arendell, L., & Chen, Z. (2011, April). Nutrient intake and anemia risk in the WHI Observational Study. (4), 532-541
ncbi.nlm.nih.gov/pmc/articles/PMC3066454/
Wilson, A., Yu, H. T., Goodnough, L. T., & Nissenson, A. R. (2004, April 5). Prevalence and outcomes of anemia in rheumatoid arthritis: a systematic review of the literature. (Suppl 7A), 50S-57S
ncbi.nlm.nih.gov/pmc/articles/PMC3066454/

コメントを残す