What is Sjögren’s Syndrome?
That’s a good question! While many people are unfamiliar with Sjögren’s syndrome, it is estimated that as many as four million people in the United States are affected by Sjögren’s syndrome.
Sjögren’s (pronounced “SHOW-grens”) syndrome is a chronic autoimmune disorder where the immune cells attack and destroy the exocrine glands, which are the glands that prevent dryness and produce moisture needed in your mouth, skin, eyes, vaginal area, gastrointestinal tract (stomach and intestine) and respiratory tract (airway and lungs).
While the exact cause of Sjögren’s remains unknown, scientists believe that genetic factors, as well as exposure to viruses or bacteria, may trigger the development of the syndrome.
Symptoms of Sjögren’s Syndrome
The most common symptoms, or the “hallmark” symptoms, of the disorder include dry mouth and dry eyes.
Dry eyes – Because of the lack of tears being produced, your eyes may burn or itch. Some people say it feels like they have sand in their eyes. It may also cause you to have blurry vision, or to be bothered by bright lights, especially fluorescent ones.
Dry mouth – Since there is a lack of saliva being produced, your mouth may feel chalky or like it is full of cotton. It may be difficult to swallow, speak or taste things. Left untreated, dry-mouth symptoms can lead to medical conditions like tooth decay, gastroesophageal reflux disease (GERD) or oral yeast infections.
Although often overlooked, saliva has several important functions. These include buffering, protection, lubrication, repairing tissue, forming and maintaining the protective dental tissues, initiating digestion of food, and facilitating the access of food to the taste buds.
Saliva is much more than water. In fact, saliva normally contains more than 60 important, protective elements, including electrolytes, buffers, antimicrobial enzymes, digestive enzymes, and many others. All of these components make saliva an essential contributor to the health of the mouth.
If you cannot produce enough saliva (known as dry mouth or xerostomia) you may be at risk for the following:
- Increased risk of infection, including oral yeast infections such as candidiasis
- Decreased ability to heal sores in the mouth
- Gastroesophageal reflux disease (GERD)
- Tooth decay
- Difficulty chewing, swallowing and speaking
- Loss of protective coating to ward off irritating substances
- Intolerance to spicy or acidic foods
- Altered speech
Symptoms of Sjögren’s can vary from person to person. Among these symptoms, people may experience:
- Difficulty talking, chewing or swallowing
- Sore or cracked tongue
- Dry or burning throat
- Dry or peeling lips
- Change in taste or smell
- Burning, gritty or itchy eyes
- Oral yeast infections, such as candidiasis
- Enlarged parotid glands(a type of gland that produces saliva)
- Increased dental cavities
- Joint pain
- Vaginal and skin dryness
- Digestive problems
- Dry nose
Conditions Associated with Sjögren’s Syndrome
Sjögren’s is a serious disease, but it can be managed effectively with help and oversight from your doctor. Discuss your Sjögren’s symptoms with your doctor because in some cases certain comorbidities (additional conditions associated with the disorder) may exist. Sometimes Sjögren’s is present along with other autoimmune diseases, such as rheumatoid arthritis or lupus. Additionally, some patients must be monitored carefully for development of internal organ involvement and other serious complications. While rare, the incidence of lymphomas (cancer of the lymph nodes), is higher in people with Sjögren’s compared to that of the general population.
How is Sjögren’s Syndrome diagnosed ?
Diagnosing Sjögren’s syndrome
Sjögren’s can be difficult to diagnose because the symptoms can sometimes be similar to those caused by other diseases or side effects from medications and can vary from person to person. In fact, the average time from onset of symptoms to diagnosis is approximately seven years.
Although Sjögren’s is typically diagnosed by rheumatologists, most patients talk to a variety of different doctors about their symptoms including ophthalmologists, dentists, ENTs (Ear, Nose & Throat specialists) or general healthcare providers.
Sjögren’s Syndrome Tests
There are a number of different tests to help diagnose Sjögren’s:
Blood tests – Your doctor may order blood tests to check the levels of your different types of blood cells, as well as your blood glucose levels. Blood tests may also be used to detect the presence of antibodies common in Sjögren’s, evidence of inflammatory conditions or indications of problems with your liver or kidneys.
Salivary Flow tests – Your doctor may order salivary flow tests to see how much saliva you produce, including:
Spit test: During this test, your doctor will have you spit into a test tube every minute for 15 minutes. The total amount of saliva collected is then measured to determine the severity of your dry mouth.
Sialogram: Your doctor may order a special X-ray called a sialogram. During this test, dye is injected into your parotid glands (located behind your jaw and in front of your ears). From this, your doctor can see how much saliva flows into your mouth.
Eye tests – There are two different types of eye tests that doctors may use to diagnose Sjögren’s:
The Schirmer test: This test measures the dryness of your eyes by putting a small piece of paper under your lower eyelid to measure your tear production.
The Slit-lamp test: This test examines the dryness of the surface of your eye by staining it with vital dyes and using a magnifying device, called a slit lamp.
Other tests – Since symptoms of Sjögren’s can vary from person to person, a number of other tests may be used depending on what symptoms you may be experiencing. Other diagnostic options include:
Imaging: A number of different imaging tests are used to diagnose Sjögren’s. These common tests check the condition of your salivary glands or see if you are experiencing inflammation of the lungs.
Biopsy: You doctor may also want to do a lip biopsy of your salivary glands to detect the presence of inflammatory cells, which can indicate Sjögren’s.
Urine sample: Your doctor may also want you to provide a urine sample to determine whether Sjögren’s has affected your kidneys.
If you are experiencing symptoms of Sjögren’s, make an appointment to talk to your doctor. Click here for tips on discussing Sjögren’s with your doctor.
Sjögren’s Syndrome Treatment
There is no known cure for Sjögren’s so the goal of treatment is to reduce symptoms of the disorder. The most common symptoms of the disorder are dry mouth and dry eyes, but symptoms vary from person to person, so treatment may vary as well. Your doctor will be able to help you figure out what treatment options may work best for you.
The good news: with early diagnosis and proper treatment, many patients with Sjögren’s are able to manage their symptoms so that they can continue to lead full lives.
Treatment for Dry-Mouth Symptoms
Patients with Sjögren’s syndrome may produce at least a third less saliva than the average person. Saliva is important because it helps neutralize acid from bacteria in the mouth which in turn helps prevent dental decay. If you are not producing enough saliva then your risk for tooth decay can increase.
To help relieve the dry-mouth symptoms associated with Sjögren’s, doctors may recommend the following:
Saliva substitutes – Saliva substitutes mimic some of the properties of saliva, which means they make the mouth feel wet and can be helpful if you produce small amounts of saliva, or none at all. They can come in a gel form or as a solution and because they can reduce oral symptoms and do not cause excessive urine formation, it is sometimes suggested that they be used at night instead of drinking water. However, their effectiveness is limited by the fact that you eventually swallow them.
Prescription medications – Medications such as cholinergic agonists can help to stimulate the salivary glands and increase the production of saliva. Side effects may include sweating, abdominal pain, flushing and increased urination. Be sure to talk to your doctor to find out what is right for you.
Treatment for Dry-Eye Symptoms
Depending on how severe your dry-eye symptoms are your doctor may suggest the following:
Artificial tears or ointments – There are a number of different options that are available over-the-counter or by prescription. These products keep eyes moist by replacing natural tears.
Surgery – In more extreme cases, a surgical procedure called punctal occlusion may be an option. During this procedure, the tear ducts that drain tears from your eyes are sealed, and collagen or silicone plugs are inserted into the ducts to close them temporarily. The tear ducts can also be sealed with a laser for a more permanent effect.
Other Treatments for Sjögren’s Symptoms
If your symptoms go beyond those affecting the moisture-producing glands of your mouth and eyes (extraglandular involvement), your doctor may recommend one or more of the following:
Nonsteroidal anti-inflammatory drugs (NSAIDs) – Some Sjögren’s patients may develop arthritis symptoms, and may benefit from nonsteroidal anti-inflammatory drugs (NSAIDs) or other arthritis remedies to help relieve joint and muscle pain.
Antifungal medications – Oral thrush, a yeast infection in the mouth, should be treated with antifungal medications.
Treatments for systemic symptoms – For some patients, treatments such as hydroxychloroquine, a medication used to treat malaria, may be helpful. In some cases corticosteroids such as prednisone and/or immunosuppressive agents, such as methotrexate, may be appropriate.
Health Implications Due to Sjögren’s Syndrome
If you are experiencing symptoms of Sjögren’s it is important that you talk to your doctor. While most patients with Sjögren’s are able to manage their symptoms and lead full lives, if symptoms are left untreated, patients may experience additional complications such as:
Dental cavities – Saliva helps protect the teeth from bacteria, so you’re more prone to developing cavities if your mouth is dry.
Yeast infections (oral thrush) – About one-third of people with Sjögren’s experience yeast infections of the mouth, called candidiasis, due to a lack of saliva.
Vision problems – Dry eyes can lead to light sensitivity, blurred vision and possibly corneal ulcerations.
Inflammation – Inflammation of a variety of organs, including the lungs, liver and kidneys, can also be found in patients with Sjögren’s. These can lead to more serious conditions such as pneumonia, problems with kidney functioning, and cirrhosis of the liver.
It is also important that patients with Sjögren’s talk to their doctor as they are often more likely to experience other conditions in addition to the disorder, such as:
Additional autoimmune disorders – Some patients may be diagnosed with another autoimmune disorder, such as rheumatoid arthritis or lupus.
To find tips for talking to your doctor, click here