Sjogren's Syndrome

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Sjogren’s Syndrome: What you need to know

What is Sjögren’s syndrome?

Free-Eyes-400-webSjögren’s (pronounced SHOW-grin’s) is a chronic autoimmune disease in which a person’s immune system turns against and attacks the body’s own cells. In Sjögren’s, white blood cells attack moisture-producing glands, such as the tear ducts and salivary glands, making it difficult for your body to produce saliva and tears.

Sjögren’s is a systemic disease, meaning that it affects the entire body, and is not localized to one area. Symptoms may remain steady, worsen, or uncommonly, go into remission. While some people experience mild discomfort and lead normal lives, others suffer debilitating symptoms that severely impair their daily functioning. Early diagnosis and proper treatment are critical – they may prevent serious complications and greatly improve a patient’s quality of life.

About half of the time Sjögren’s occurs alone, and the other half it occurs in the presence of another autoimmune connective tissue disease such as Rheumatoid arthritis, lupus, or scleroderma which are commonly linked with Sjögren’s. When one or more autoimmune conditions occur at the same time, they are often called overlap diseases.

When occurring alone, Sjögren’s syndrome is known as primary Sjögren’s syndrome (Primary SS) and when it occurs along with another connective tissue disease it is considered to be secondary Sjögren’s syndrome (Secondary SS). People with secondary Sjögren’s tend to have a milder form of the condition. However, they also must deal with the symptoms of the coexisting or ‘overlap’ disease. The most common condition occurring with secondary Sjögren’s is rheumatoid arthritis.

Who develops Sjögren’s?

While nine out of ten patients are women, Sjögren’s syndrome impacts all racial and ethnic groups. You can develop Sjögren’s at any age, but it is most often diagnosed after the age of 40. With anywhere from 400,000 to 3.1 million Americans suffering from Sjögren’s, it is one of the most prevalent autoimmune disorders in the United States.

How are lupus and Sjögren’s related?

People living with lupus and people living with primary Sjögren’s syndrome can both have strikingly similar symptoms. These symptoms may include skin rashes, arthritis, and kidney disease.  An increased level of ANA, or antinuclear antibodies, is also often seen in both diseases. 80% of those with Sjögren’s will have an increase of ANAs. There also may be a genetic link between the two, as people with Sjogren’s and people with lupus have been found to share certain genetic similarities. Unfortunately, Sjogren’s patients have a higher risk of developing lymphoma as well.

What causes Sjögren’s?

The exact cause is unknown, but like rheumatoid arthritis and lupus, it is a disorder of the autoimmune system.

What are the Signs and Symptoms?

Sjögren’s signs and symptoms vary from person to person; from extreme fatigue and joint pain to dysfunction within key organs such as the kidneys, gastrointestinal system, circulatory system, lungs, liver, pancreas and the central nervous system. The most notable symptoms are: dry eyes, dry mouth and dry throat. 

Mouth

The most common oral symptom is xerostomia, or dryness of the mouth, and others listed below:

Sjogren's Syndrome

Throat and Nose

Symptoms that may develop within the throat and nose are:

Eyes

The most prominent symptom surrounding the eyes is a gritty or sandy sensation along with:Sjogren's Syndrome

Glands

Extra Glandular (outside of the glands)

Less often, Sjögren’s can cause symptoms outside of the glands: skin rash, gastrointestinal problems, or inflammation of the liver, kidneys, pancreas, or lungs. These symptoms are seen in one-third of people with primary Sjogren’s, but rarely in those with secondary Sjogren’s.Sjogren's Syndrome

Extra glandular symptoms may include:

It is possible that some of these signs and symptoms may be associated with lung involvement, kidney involvement, and/or vasculitis (inflammation of blood vessels). It is also possible to experience lymphomas (tumors of the lymph glands), which develop in about 6% of people with primary Sjogren’s.

How is it diagnosed?

As with lupus, there is no single, definitive test for Sjögren’s. Diagnosis usually occurs after someone with a diagnosed autoimmune disease develops extreme dryness of the mouth and eyes. Diagnosis involves a complete medical history and physical examination.

On average, it takes 4.7 years to receive a diagnosis of Sjögren’s. Because the disease is often mild in many people, the first signs of dryness in the mucous membranes may be present for years before the disease becomes clearly evident.

Though the disease was first identified in 1933 by Dr. Henrik Sjögren, general awareness about Sjögren’s is still lacking, making getting an early diagnosis more difficult. Like many other chronic autoimmune diseases, including lupus or (SLE), increased awareness among the public and medical community is needed to help spur researchers to find new ways to diagnose and treat Sjögren’s in addition to helping patients receive earlier diagnosis.

Five diagnostic criteria for Sjogren’s are:

  1. Dry mouth
  2. Poor salivary (saliva-producing) gland production- determined by testing
  3. Dry eyes
  4. The presence of antinuclear antibodies (ANA) in a diagnostic blood test
  5. The presence of a positive rheumatoid factor, meaning some sort of rheumatic condition, such as rheumatoid arthritis

Here are several other tests that are often used to confirm a Sjogren’s diagnosis:

Be sure to tell your doctor about any over-the-counter and prescription medications you are taking. Some medications can cause similar symptoms associated with Sjögren’s.

How is it treated?

There is no cure for Sjögren’s or arthritis, so treatment revolves around controlling symptoms. Your treatment will depend on the severity of your individual symptoms and may require a combination of different methods.

Sjogren's SyndromeYou can combat fatigue by getting a good night’s sleep and taking appropriate breaks during the day. Ask your doctor about exercises that can help you increase flexibility and ease muscle and joint pain. Maintaining a diet rich in nutrients can improve your overall health. Many people with arthritis find relief with the use of complementary or alternative treatments/therapies like yoga, tai chi, or acupuncture.

For dry mouth: Dry mouth causes oral and dental problems, so proper oral hygiene is very important. Limit your sugar intake and get regular dental checkups. Choose dental products made specifically for dry mouth and be sure to floss every day. Hard candy and chewing gum can help you produce saliva, but make sure they are sugar-free. For dry lips, use lip balm or lipstick that has an oil or petroleum base. Your doctor can recommend mouth rinses or ointments to ease pain and inflammation. In severe cases, your doctor can prescribe a gel-based saliva substitute or other stronger prescription medications. Patients need to remember to be pro-active in talking with their physicians and dentists about their symptoms and potential treatment options. Increased intake of fluids is often necessary both with, and between, meals because of the decrease in salivary gland secretion.

For dry eyes: There are several things you can do to help with the eye-related symptoms of Sjögren’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 irritate your eyes. Try over-the-counter artificial tears. If they don’t do the trick, ask your doctor to prescribe something stronger. Some thicker eye ointments can be used while you sleep. Consult with your doctor before applying ointments or gels to your eyes. For stubborn cases, a surgical procedure called punctual occlusion can temporarily or permanently close off the tear ducts that drain tears from your eyes. Plugging the lacrimal (tear) ducts with collagen plugs can be very effective in increasing moisture in the eye.

For joint and muscle aches and pains: Over-the-counter pain relievers or [anti-inflammatory medications] can help relieve joint and muscle pain. If they don’t help with these symptoms, ask your doctor about [corticosteroids] , other prescription pain medication options, as well as anti-rheumatic or [immunosuppressive medications]. You can temporarily soothe aches and pains by applying heat or cold compresses. Regular exercise can improve flexibility and lessen discomfort. Additionally, maintaining a proper body weight will put less stress on joints and muscles.

What kind of doctor do I need?

Female doctor examining her patient.Doctors who specialize in diseases like lupus and arthritis are called rheumatologists. If you’ve already been diagnosed with arthritis or even lupus, your rheumatologist will most likely be able to help treat your case of Sjögren’s. Depending on the severity of your symptoms, your rheumatologist or general physician may refer you an internist or to one or more specialists. An eye specialist is called an ophthalmologist, and an ear, nose, and throat specialist is called an otolaryngologist.

How do I cope with a diagnosis?

Like lupus, there is no cure for Sjögren’s, but there are many treatment options and lifestyle choices that can improve quality of life. Most people with Sjögren’s can make the necessary adjustments to live a normal life. The key is to work with your doctor to find the best treatments for your symptoms. It is important to note that in rare cases, people with Sjögren’s may develop lymphoma. Report signs of unusual swelling or neurologic problems to your doctor.

There are many feelings that go along with learning to cope with a chronic illness and they are all normal. You may not know where to turn to for advice, support, or information. Your family, friends, and caregivers may feel the same way.

The first thing to understand is that a person with Sjögren’s, scleroderma, lupus, fibromyalgia, or any other chronic illness is just that, a person. The disease should not become your identity. There is so much more to each individual than their diagnosis, so maintaining a positive but realistic attitude can really help when learning to live with any chronic disease. Try to lead as full of a life as possible and understand that this is just a part of your life, meaning that the diseases is what you have, not who you are. If you are feeling depressed or hopeless, please seek counseling from a professional to help provide the tools to help you cope with these complicated feelings.

Maintain good communication with your treating physician(s) and be your own best advocate. Keep organized medical records, bring a support person along to visits when you can to help you digest and remember information and advice, and be open and honest with your doctor, following the recommended protocol.  A network, or team of friends, family, medical providers, support groups, and resources can help you successfully navigate through your disease.

Sources: www.healthline.comwww.Sjögren’s.orgwww.rheumatology.org

*All resources provided by Molly’s Fund are for informational purposes only and should be used as a guide or for supplemental information, not to replace the advice of a medical professional. The personal views do not necessarily encompass the views of the organization, but the information has been vetted as a relevant resource. We encourage you to be your strongest advocate and always contact your medical provider with any specific questions or concerns.  

You have just read this blog on Sjogren’s Syndrome. You may also be interested in the following blogs as well:

Overlap DiseasesInvisible Illness: “But you look so good?”Scleroderma

 and What is Lupus?

Article by : Frances Ku

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