Sjögren's Syndrome: Causes, Symptoms, Diagnosis and Treatment

Sjögren's syndrome is a lifelong autoimmune disorder that reduces the amount of moisture produced by glands in the eyes and mouth. It is named for Henrik Sjögren, a Swedish eye doctor who first described the condition. While dry mouth and dry eyes are the primary symptoms, most people who have these problems don't have Sjögren's syndrome. Dry mouth is also called xerostomia.

Sjögren's Syndrome

There are two forms of Sjögren's syndrome:

Primary Sjögren's syndrome develops on its own, not because of any other health condition.
Secondary Sjögren's syndrome develops in addition to other autoimmune diseases like rheumatoid arthritis, lupus and psoriatic arthritis.

Who might get Sjögren's syndrome?

An estimated one to four million Americans have Sjögren's syndrome. The disease affects people of all races, ethnicities and ages. However, women are nine times more likely to develop this condition than men.

SYMPTOMS AND CAUSES

What causes Sjögren’s syndrome?

Sjögren’s syndrome is an autoimmune disease, which means something triggers your immune system to attack healthy cells. This attack damages the tear system in your eyes and the salivary glands in your mouth.

Exactly what causes this abnormal immune system response is not clear. These factors may play a role:

Environmental factors.
Genetics.
Sex hormones (the condition affects more women than men).
Viral infections.

What are the symptoms of Sjögren’s syndrome?

In addition to extremely dry eyes and mouth, some people experience muscle pain and joint pain all over the body, similar to fibromyalgia. Other symptoms include:

Abnormal sense of taste.
Burning or redness in eyes, or grittiness (like sand).
Blurry vision.
Difficulty chewing, swallowing or talking.
Dry cough or hoarseness.
Dry, itchy skin.
Enlarged salivary glands.
Fatigue.
Tooth decay or early tooth loss.
Vaginal dryness.

How is Sjögren’s syndrome diagnosed?

If you have dry mouth, dry eyes or other signs of Sjögren’s syndrome, your doctor may use these methods to confirm a diagnosis:

Blood tests: These tests detect specific antibodies in the blood. They look for anti-nuclear antibodies (ANA), anti-Sjögren’s syndrome antibodies (anti-SSA, also called anti-Ro) and anti-Sjögren’s syndrome type B (anti-SSB, also called anti-La). A blood test can also detect rheumatoid factor, an antibody found in many people who have rheumatoid arthritis.
Biopsy: Your doctor may remove tissue or cells from a salivary gland or the inside of your lip. This biopsy sample goes to a lab to check for signs of inflammation.
Eye exam: An eye specialist, such as an ophthalmologist, can measure tear production. During an eye exam, your doctor will examine the cornea, the clear part of the eye, for dryness.
Imaging tests: These include sialometry, which measures how much saliva you produce by using X-rays that can see dye injected into salivary glands. There is also salivary scintigraphy, a way to track how long it takes for a radioactive isotope to travel from an injection point in your vein to your salivary glands.
Health history: If you have a pre-existing autoimmune disease, plus dry eyes and dry mouth, your doctor may conclude that you have developed secondary Sjögren’s syndrome.

MANAGEMENT AND TREATMENT

What kind of a doctor treats Sjögren’s syndrome?

Many types of doctors might be involved in your care if you have SS. These include your own primary care provider, your dentist, and specialists such as rheumatologists, ophthalmologists and otolaryngologists, also called ear, nose and throat (ENT) doctors.

How is Sjögren’s syndrome managed or treated?

There is no cure for Sjögren’s syndrome, but treatments can relieve symptoms. Depending on your specific issues, your doctor may recommend one or more of these therapies.

Treatments for dry eyes:

Artificial tears: Over-the-counter artificial tear eye solutions and artificial tear eye ointments moisturize dry eyes. These products relieve irritation and discomfort.
Prescription eye drops: Cyclosporine (Restasis®) and lifitegrast (Xiidra®) prescription eye drops soothe inflamed tear glands and stimulate tear production.
Punctal plugs: An ophthalmologist inserts tiny silicone plugs into the tear ducts. The plugs block the ducts so tears stay on the eyes, keeping them wet.
Surgery: If punctal plugs work for you, your doctor may recommend surgery to close the tear ducts permanently.
Autologous serum drops: Your doctor can make customized artificial tears. The process involves mixing your blood serum (a clear liquid separated from your blood) with a sterile liquid solution. You receive a one-of-a-kind tear substitute unique to your body. While effective, the pricey treatment isn’t always covered by insurance.

Treatments for dry mouth:

Saliva producers: Products such as gum and hard candies that contain sweeteners like sorbitol or xylitol can stimulate saliva production. You can also use an over-the-counter or prescription saliva substitute. Prescription products include sorbitol oral lozenges and sorbitol oromucosal solutions (solutions that are directed toward the cheeks).
Prescription medications: Pilocarpine (Salagen®) and cevimeline (Evoxac®) pills increase the natural production of saliva.
Dental care: A dry mouth increases the risk of dental cavities, infections and tooth decay. Your doctor may recommend a prescription toothpaste and mouthwash, as well as regular fluoride treatments.

Treatments for joint or organ problems:

Over-the-counter pain relievers: Acetaminophen (Tylenol®) and nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen (Motrin®) and naproxen (Aleve®), can relieve joint pain and muscle aches.
Anti-rheumatics: Hydroxychloroquine prescription pills can diminish pain from rheumatoid arthritis or lupus. This medication may also reduce salivary gland swelling.
Immunosuppressants: These prescription medications slow the immune system’s response. They lessen inflammation and prevent organ damage.
Steroids: Prednisone prescription pills soothe inflammation of the joints, skin and organs.
Antifungals: These medications treat yeast overgrowth in your mouth (oral thrush) or in your vagina (vaginal yeast infection).

Treatments for vaginal dryness:

If your vagina is always dry, itchy and sore, you should check with your healthcare provider to make sure that the issue is not something more than hormonal changes. You might have some type of infection or skin issue. These conditions would require specific treatments. In general, though, women with Sjögren’s syndrome are two to three times more likely to have issues with vaginal dryness and atrophy than women of similar ages around and after menopause. Tips for helping with everyday vaginal dryness include:

Trying vaginal moisturizers or lubricants to add moisture to the vagina daily and to ease sexual intercourse.
Using unscented soaps for cleansing. Perfumes and other additives can cause irritation.
Asking your healthcare provider about vaginal estrogen therapy.

What are the complications of Sjögren’s syndrome?

Most people who have Sjögren’s syndrome live their lives without any significant problems. However, a dry mouth means that you are more likely to have dental problems, such as tooth decay and infection. Dry eyes can also place you at risk for eye infections.

If you have secondary Sjögren’s syndrome, you may also have problems like joint paint caused by rheumatoid arthritis or lupus.

Rarely, people with Sjögren’s syndrome develop these complications:

Abnormal liver or kidney function.
Lymphomas (cancerous tumors in the lymph nodes).
Lung problems that may be mistaken for pneumonia.
Neurological problems that cause weakness or numbness.
Skin rashes (red skin).

How can I prevent Sjögren’s syndrome?

Because no one knows exactly what causes Sjögren’s syndrome or other autoimmune diseases, there is no known way to prevent it.

What is the prognosis (outlook) for people with Sjögren’s syndrome?

Constantly having a dry mouth or dry eyes is certainly uncomfortable. Fortunately, symptoms tend to lessen over time. With the right therapies, you can manage symptoms so they do not interfere with your ability to enjoy life.

Does Sjögren’s syndrome cause weight gain?

Sjögren’s syndrome doesn’t cause weight gain. However, medications (like steroids) used to treat symptoms may cause weight gain. Also, there are conditions like hypothyroidism that may be linked to Sjögren’s syndrome that can result in unintended weight gain.

Does Sjögren’s syndrome affect ears?

Sjögren’s syndrome, like other autoimmune conditions, can affect ears, too. You might have trouble with hearing loss or with balance.

What should I eat if I have Sjögren’s syndrome? What foods should I avoid?

An estimated 90% of people with Sjögren’s syndrome have problems related to eating. In some cases, these problems can be enough to cause malnutrition. Troubles can be related to dryness and swelling of the throat, as well as intestinal tract or nerve damage. There also seems to be a significant number of people who have both Sjögren’s syndrome and celiac disease and/or symptoms of irritable bowel syndrome (bloating, abdominal pain, diarrhea and/or constipation).

If you have gastrointestinal symptoms, you can work with a registered dietitian (RD) to find out what food sensitivities you have. An RD can then also help you to create a food plan that works well for you and provides the nutrients you need.

Problems with gastroesophageal reflux (GERD) are also common among people in general and among people with Sjögren’s syndrome in particular. Your care team can provide you with tips for dealing with GERD. These might include not eating two to three hours before you lie down to sleep and avoiding greasy foods.

There are some recommendations that people who have autoimmune disorders like Sjögren’s syndrome should follow an anti-inflammatory diet to help with joint pain and other symptoms. Some people have found relief from a completely plant-based diet.

Here are some tips that might help you manage other types of eating issues:

Eat smaller amounts of food at one time, but eat more frequently throughout the day.
Include soft foods in your diet.
Be sure to chew your food completely.
Avoid cavities by reducing the amount of sugar you eat and drinking only water before you go to bed.

Is Sjögren’s syndrome considered a disability?

The U.S. government has awarded disability to people with severe cases of Sjögren’s syndrome. However, this is a question that you should discuss with your healthcare provider to see if you are eligible.

Does Sjögren’s syndrome cause hair loss?

If you have Sjögren’s syndrome, you might see some hair loss, and it might be as a result of the condition. There is a condition known as frontal fibrosing alopecia that is being found in higher numbers in people (mostly women) with autoimmune diseases. This condition causes slow hair loss at the front hairline and sometimes the eyebrows.

However, hair loss can be triggered by many things, including stressful life events and medications. You should ask your dermatologist about problems with hair loss.

When should I call the doctor if I have Sjögren’s syndrome?

Severe mouth or gum pain could indicate infection or tooth decay. Itchy eyes, eye pain, or blurred or double vision can signal infections or other vision problems. You should also call your doctor if you notice swollen lymph nodes in your neck, armpits or groin. Enlarged lymph nodes could indicate lymphoma or another health condition.