Diseases

Sjögren’s Syndrome

Sjögren’s syndrome (SS) is a chronic (long-term) disease that affects the body’s moisture-producing glands and causes a reduction in a variety of body fluids.

The most common glands that are affected by Sjögren’s are the tear glands and salivary glands. People with Sjögren’s often have dry eyes that can feel gritty and suffer from dry mouth. The dryness usually gets worse as the day goes on.

Other glands and organs can also be affected, potentially causing a wide variety of additional symptoms including joint pain, skin rashes, and numbness and tingling in the feet.

Sjögren’s syndrome can be tricky to diagnose because of its range of symptoms and their similarity to other diseases. It is best diagnosed by a rheumatologist or another specialist who knows a lot about Sjögren’s.

Autoimmune Disease

Sjögren’s Syndrome is an autoimmune disease, meaning that it occurs when the body’s immune system attacks its own healthy cells and tissues. The reason it does this is not well understood. When the body’s immune system is “activated” in this way, it can make a person feel very tired, similar to when they have the flu.

Who Gets Sjögren’s Syndrome

Sjögren’s is relatively rare (0.5%, or 1 in 200 people, will get it) and it affects women about ten times more frequently than men. Most people who get it notice their first symptoms between the ages of 30 and 50.

Understanding Sjögren’s Syndrome

Symptoms

In Sjögren’s syndrome the body’s immune system primarily attacks its glands, small organs that are responsible for producing a variety of body fluids. The disease can also sometimes attack other tissues and organs.

The good news is that most cases of Sjögren’s are mild and symptoms can be limited to dry eyes, dry mouth, achy joints, and fatigue. That being said, there are cases where the condition can be more severe.

Symptoms include:

Dry Eyes

People with Sjögren’s often have dry eyes that feel gritty because the most common glands that are affected by the disease are the tear glands and salivary glands. The dryness usually gets worse as the day goes on and can require the frequent use of eye drops.

Dry Mouth

Another common symptom of Sjö’grens syndrome is a dry mouth. This can make swallowing difficult and can also cause dental problems like cavities.

Dry and Swollen Glands

Sjögren’s syndrome can also affect other glands including those in the skin, airways, esophagus (the tube that connects your mouth to your stomach), and the vagina. These tissues can become dry and uncomfortable.

Sjögren’s syndrome can also cause the glands to become swollen. This is most common in glands around the head and neck area.

Fatigue

Fatigue is a very common problem for people with Sjögren’s syndrome because the immune system is activated (“turned on”) all of the time.

Arthritis

Some people with Sjögren’s syndrome also have arthritis. This means the joints are attacked by the immune system, causing pain, swelling, and stiffness. Painful symptoms or fibromyalgia can also affect people with Sjögren’s syndrome.

Less Common Symptoms

Other less common symptoms that people with Sjögren’s syndrome sometimes have include:

  • Raynaud’s Phenomenon, which causes the ends of the fingers and toes to turn white or blue in the cold
  • Vasculitis, or inflammation of the small blood vessel – this can look like a rash with lots of little red dots on the skin, usually on the lower legs
  • Neuropathy or painful nerves – this happens when blood vessels supplying the nerves are affected. It can cause a tingling or burning feeling in the feet or sometimes the hands
  • Pulmonary fibrosis – a condition affecting the lungs, making it harder to breathe; this is very rare
  • Kidney problems are also very rare
  • Hypothyroidism – low levels of thyroid hormone

Although it is very rare, people with Sjögren’s syndrome are at higher risk for a type of cancer called lymphoma.

Diagnosis

Sjögren’s syndrome can be properly diagnosed by a rheumatologist, a type of doctor that specializes in arthritis and autoimmune disease, or another specialist who knows a lot about the disease. Sjögren’s syndrome can be tricky to diagnose and having someone experienced is very useful.

To diagnose Sjögren’s syndrome, physicians will take a careful and thorough history. They will ask many questions because Sjögren’s can have so many symptoms.

Next, they will perform a complete physical examination including the head and neck, looking for dry eyes and mouth; as well as the lungs, heart, abdomen, joints, and skin. The exam may include the Schirmer’s test that measures the dryness of eyes. The physician may also recommend a biopsy of a salivary gland in the mouth that produces saliva (spit). A biopsy involves taking a very small piece of tissue so that it can be examined.

Finally, special blood tests are ordered to see the patient’s body is making antibodies to itself, to confirm the diagnosis.

Although it is very rare, people with Sjögren’s are at a higher risk for a type of cancer called lymphoma. People diagnosed with Sjögren’s should be screened and monitored for this type of cancer.

Common Tests for SS

Schirmer’s Test (Test for Dry Eyes)

Looking for dry eyes – The Schirmer’s test measures the ability of the eye to make tears. People with SS are expected to have very dry eyes because their tear-producing glands are being attacked by their immune system.

Biopsy (Tissue Sample)

Biopsy (tissue sample) looking for signs of SS in the salivary gland: A very small tissue sample is taken from the patient so it can be examined to look for white blood cells (lymphocytes) that are infiltrating the glandular tissue.

Blood Tests

Looking for antibodies that attack the body – the anti-nuclear antibody (ANA) test is the most important blood test to do first if Sjögren’s is suspected is the test. This test helps determine if the body is making too many antibodies to itself (having a small amount is normal) and checks if they might be attacking unusual places.

If this test comes back negative in a patient, it is unlikely that they have SS. If it comes back positive, then more tests should be run to confirm the disease because a positive Anti-Nuclear Antibody test is possible in healthy people and in people with other diseases.

Looking for antibodies that attack certain proteins: the Extractable Nuclear Antigen (ENA) Panel is a test that measures antibodies to 6 or 7 other proteins in the body. The antibodies in this panel may include: anti-Ro (also called anti-SSA), anti-La (also called anti-SSB), anti-Sm (anti-Smith), anti-RNP, anti-Jo-1, anti-Scl70, and anti-centromere. Things get a bit more tricky here and it’s best for patients to discuss this test and its results with a rheumatologist. In general, anti-Ro and anti-La antibodies can be found in people with Sjogren’s Syndrome.

Rheumatoid Factor: The rheumatoid factor can be found to be positive in some patients with Sjogren’s Syndrome.

Looking for excessive B-lymphocytes: an immunoglobulin (antibody) test measures the total amount of all immunoglobulins (antibodies) a person has. Since people with Sjögren’s have too many of a type of white blood cell called the B-lymphocyte (B-cell), and these cells are responsible for making antibodies, this test is expected to show a high level of them.

Looking for inflammation: Sjoogren’s Syndrome causes inflammation in the body so tests for inflammation are expected to have abnormal results. Common tests include a Complete Blood Count (CBC), Erythrocyte Sedimentation Rate (ESR), and C-Reactive Protein (CRP).

Looking at kidney function: Creatinine is a common blood test that looks at how well the kidneys are working. Abnormally high levels of creatinine can indicate a problem and can mean that the kidneys are involved.

Urine Tests

Looking for kidney involvement: urinalysis testing to detect protein or blood in the urine can give a sign that the kidneys might be involved.

Scans

Looking for lung involvement: Chest x-ray and/or CT scans can help confirm if the lungs are involved.

Nerve Tests

Looking for nerve involvement: Electromyography or Nerve Conduction Studies can be performed to look for nerve involvement.

Causes

Sjögren’s syndrome occurs when the body’s immune system begins to attack its own moisture-producing glands.

It’s not known why the immune system gets turned on in people with Sjögren’s syndrome.

It is known that the immune system makes too much of a white blood cell called the B lymphocyte (or “B-cell”). These B lymphocytes make lots antibodies that can play a role in causing the symptoms of Sjögren’s syndrome.

Treatments

Treatment of Sjögren’s syndrome (SS) often involves a combination of good hygiene and medications. As SS can be a variable disease, each patient’s treatment plan is personalized to suit their needs.

Fatigue is one of the most common symptoms in people with SS, and one most difficult to treat. Patients are encouraged to learn how to balance the demands of their life with their need to rest. Medications such as prednisone can be helpful with fatigue but also have side effects.

Care for Dry Eyes

People with Sjögren’s should keep their eyes clean by washing them every day with warm water. This is especially important after waking up because debris can build up in and around the eyes during sleep.

A good pair of glasses or sunglasses that stops air from blowing across the eyes helps prevent them from becoming too dry.

Artificial tears during the day and/or a jelly lubricant at night can help keep the eyes moist.

Tear Duct Surgery

In some cases, an eye surgeon (ophthalmologist) may perform a simple procedure that blocks the tear ducts (punctual occlusion). This prevents the eyes from draining tears that are formed by the tear glands, and helps keep the eyes moist.

Care for Dry Mouth

People with Sjögren’s often don’t make enough saliva. They are prone to cavities and other dental problems because saliva helps rinse food and bacteria away from the teeth.

It is important to brush teeth regularly with a fluorinated toothpaste, and use an antimicrobial mouthwash.

When brushing, it is important that toothpaste is in contact with the tooth for at least 2 minutes. A good electric toothbrush with a timer (such as the Sonicare brand) can be helpful.

Going to the dentist on a regular basis is an important part of good oral health.

Dry mouth can be relieved by chewing sugar-free gum (containing Xylitol), sucking on sugarless mints, using a lubricant spray, and/or drinking plenty of fluids like plain water.

Medications for Sjögren’s Syndrome

The choice of medications depend on a patient’s specific symptoms. Though they cannot cure the disease, they can make living with the condition much more comfortable.

Pilocarpine for Dry Eyes and Dry Mouth

A medication called pilocarpine (Salagen) encourages the glands to produce more fluids. This may be helpful if you have dry eyes and/or a dry mouth. Pilocarpine comes as a liquid solution (eye drops) or a gel that can be applied to the eyes, and as an oral tablet to increase saliva production. Since pilocarpine increases the body’s production of fluids, it can cause excessive sweat production. This can be a bothersome side effect for some people.

Pilocarpine has been around a long time. It has been used for the treatment of an eye condition called glaucoma for over 100 years.

Pain Medications

Chronic pain can be treated with a variety of medications.

Analgesic medications only control pain. They do nothing to control the disease or to prevent further damage to the glands. Analgesics can range from simple things like Acetaminophen or Paracetamol to more potent narcotics like morphine.

Corticosteroids (prednisone)

Medications like prednisone can help control inflammation. Prednisone is a very effective medication to control many of the symptoms of Sjögren’s syndrome including fatigue, arthritis, and vasculitis. High doses are typically used when any organs are involved.

In the short-term, prednisone works very well to control symptoms caused by inflammation. When used for long periods of time, prednisone can have side effects. Patients should to discuss the risks and benefits of using prednisone with their rheumatologist. Some patients with joint involvement can benefit from cortisone injections directly into the affected joints.

Medications for Arthritis or Vasculitis Symptoms

Swollen and inflamed joints may be treated with medications typically used for people with rheumatoid arthritis. These include prednisone, steroid injections, and disease-modifying anti-rheumatic drugs such as Methotrexate, Sulfasalazine, Leflunomide (Arava), and Hydroxychloroquine (Plaquenil).

like methotrexatesulfasalazinePlaquenil (hydroxychloroquine), Gold (myochrisine), Imuran (azathioprine), Arava (leflunomide), and Cellcept (mycophenolate).

Vasculitis or inflammation of small blood vessels can also be relieved by prednisone and disease-modifying anti-rheumatic drugs.