Sjogren's Syndrome

Sjogren’s syndrome (SS) is an autoimmune condition that causes dry eyes and mouth and sometimes other symptoms like joint pain or lung problems. In SS, an overactive immune system damages the glands that make secretions like tears and saliva, leading to symptoms. Since Sjogren's can occur alongside other autoimmune diseases, it is relatively common for those with Sjogren's to experience other symptoms as well.

Sjogren's syndrome affects 0.5-1% of people, with women affected ten times more often than men. Taking a medical history, conducting a physical exam, and performing blood tests may be all that’s needed for diagnosis. Treatments like eye drops and medications can help decrease symptoms, although some may still be bothersome. It’s also important to monitor for complications, like lymphoma (cancer of the lymphatic system), which occurs in a minority of people.

Types of Sjogren’s Syndrome

Sjogren’s syndrome sometimes occurs along with other autoimmune illnesses, such as rheumatoid arthritis, lupus, or systemic sclerosis. Less commonly, it might occur with autoimmune liver disease, autoimmune thyroid disease, or multiple sclerosis. This is sometimes called “secondary Sjogren’s.” The SS-type symptoms might come before or after you are diagnosed with the other illness. 

In contrast, the term “primary Sjogren’s syndrome” is sometimes used to describe Sjogren’s that occurs without any of these other syndromes. 

Symptoms

Many symptoms of Sjogren’s syndrome are “sicca” type symptoms, meaning they are caused by excess dryness. Such symptoms are the most common manifestation of Sjogren’s disease, occurring in roughly 80% of people who have it.

Sicca Symptoms 

In Sjogren’s syndrome, your eyes are often too dry because your glands do not make enough tears to properly lubricate your eyes. This can lead to symptoms like eye irritation and soreness, poor vision, and feeling like something is stuck in your eye.

Because SS reduces saliva production, it can also cause dryness in the mouth and throat. This can lead to oral irritation and bad breath. It can make eating more difficult, and you might need to drink water to swallow dry food. People with SS are also more prone to dental cavities.

Sjogren’s can also cause vaginal dryness, which can cause an increased risk of pain with sex and increased susceptibility to some infections. 

Other Sjogren’s Symptoms

Fluctuating joint pain affects up to 70% of people with Sjogren’s syndrome, mostly often affecting the hands and feet. A less common cause of pain in Sjogren’s syndrome is neuropathy (nerve damage), which can cause a burning or pins-and-needles-type feeling.

Around 70% of people also experience fatigue, which many find to be their most debilitating symptom.

About one-third of people with Sjogren’s have swelling of one or more of their parotid glands, the largest salivary glands in your body. This can cause you to feel a slightly painful lump near the back of your jaw near your ear. 

Roughly 10% of people with Sjogren’s syndrome have skin symptoms, which can include vasculitis (swelling of the blood vessels). An even smaller minority develop kidney issues due to Sjogren’s.

Lung symptoms occur in roughly 10-20% of people with Sjogren’s syndrome, which might cause a chronic cough. The most serious manifestation is interstitial lung disease, which can cause shortness of breath and lead to a decreased life expectancy.

People with secondary Sjogren’s syndrome may have additional symptoms related to other autoimmune conditions.

What Causes Sjogren’s Syndrome?

In Sjogren’s syndrome, part of your immune system becomes mistakenly activated against proteins in your own body, causing your body to attack its own cells. Part of the immune system targets the glands that make tears and saliva, so they can’t secrete in normal amounts. It may affect other parts of the body, causing additional symptoms.

Researchers are still learning about the genetic and environmental factors that contribute to this. It likely takes a series of steps, including the development of abnormal antibodies—anti-SSA and anti-SSB—which are usually present in SS and rheumatic diseases. More research is needed to determine the exact cause.

Risk Factors

The factors that increase your risk of Sjogren’s syndrome aren’t well known. However, research tells us that some of the following may increase your risk:

• Being assigned female at birth
• Having certain kinds of infections, like hepatitis C
• Having other autoimmune diseases yourself and/or in your family history
• Experiencing stressful life events

People who already have an autoimmune disease such as rheumatoid arthritis are also at higher risk of developing secondary Sjogren’s. 

Diagnosis

Taking a medical history and conducting a physical exam are key starting points for diagnosing Sjogren’s and ruling out other potential causes of your symptoms. Notably, evaluating your eye and mouth dryness is important, as dryness from Sjogren’s is more severe, consistent, and prolonged than other symptoms.

Key elements for diagnosis may include:

• Thorough oral exam and examination of the parotid glands: This can help identify decreased saliva production and/or enlarged secretory glands.
• SS antibody blood tests: Testing for anti-SSA (also called anti-Ro) and anti-SSB (also called anti-La) can indicate if SS is a likely cause of your symptoms. These blood tests are often positive in SS, but not 100% of the time
• Other blood tests: Blood tests like antinuclear antibody (ANA) and rheumatoid factor can help identify other potential causes of symptoms or secondary Sjogren’s.
• Schirmer eye testing: This test assesses the amount of tears your eyes produce over a few minutes and can indicate if you're experiencing decreased tear production.
• Biopsy of the labial gland (on the inner side of the lip): This procedure involves taking a small tissue sample to examine under a microscope. It's only necessary for diagnosis if anti-SSA or anti-SSB antibodies aren’t found to be positive.
• Salivary flow tests: These tests are only needed if eye tests haven’t first identified that you have dry eyes.
• Imaging tests: If your diagnosis is still unclear, imaging may be helpful. Ultrasound is usually preferred over computed tomography (CT) scan or magnetic resonance imaging (MRI) scan.

Treatments for Sjogren’s Syndrome

There is no cure for Sjögren's syndrome. However, treatment can help minimize symptoms and improve your comfort and quality of life. Treatment varies based on the specific symptoms you're experiencing and their severity.

Treatment for Dryness (Sicca) Symptoms

Eye drops and tear substitutes may be needed multiple times a day. You might also use drops that contain a medication like Cequa (cyclosporine), which can reduce your immune system's overactive response. Drops containing corticosteroids can temporarily decrease symptoms, but they're not used long-term.

Other options are punctual plugs, which are tiny devices used to block the flow of tears out of the eye. Punctal plugs can help keep tears in the eye for increased lubrication. Scleral lenses, a special kind of contact lens, can also help keep tears in the eye (although standard contact lenses should be avoided).

For dry mouth, some people benefit from a device that electrically stimulates their saliva glands, like SaliPen. The electrical stimulation it creates helps promote natural saliva flow.

Some medications may also help increase tears and saliva. These include Salagen (pilocarpine) and Evoxac (cevimeline).

Treatment for Additional Symptoms

For additional symptoms, you might need medication to suppress part of your immune system, either temporarily or long-term. For example, you might need medication if symptoms affect your joints, lungs, kidneys, blood vessels, or nerves. 

This might include corticosteroids, such as methylprednisolone. Due to the risk of side effects, healthcare providers usually only prescribe corticosteroids for limited periods of time. 

Other therapies that suppress the immune response associated with Sjogren's syndrome include: 

• Plaquenil (hydroxychloroquine)
• Imuran (azathioprine)
• Trexall (methotrexate)
• CellCept (mycophenolate) 
• Rituxan (Rituximab) 

Many of these same therapies can be used to simultaneously treat issues from secondary Sjogren’s syndrome, if present. 

Prevention

Scientists haven’t identified a way to prevent Sjogren’s syndrome, but you can take steps to reduce symptoms and lower your risk of complications. These include:

• Not smoking
• Increasing the humidity in your home (e.g., using a humidifier)
• Using nasal irrigation and/or inhaled saline
• Taking sips of water during the day and avoiding caffeinated beverages
• Sucking on sugar-free candies or chewing sugar-free gum
• Brushing and flossing after every meal
• Seeing your dentist every six months
• Using vaginal lubricants or moisturizers
• Getting regular aerobic exercise

It’s also important to reduce or eliminate medications that might worsen dry eyes or dry mouth. Anticholinergic drugs like Benadryl (diphenhydramine) are an important category.

Some people with SS may also need periodic screening tests to diagnose potential lung disease before symptoms become severe.

Related Conditions

Due to inflammation and immune changes in Sjogren’s, about 5% of people with Sjogren’s syndrome eventually develop a type of blood cancer, B-cell non-Hodgkin’s lymphoma. This might require chemotherapy, radiotherapy, or other types of cancer treatment.

Sjogren’s syndrome also greatly increases your risk of complications during pregnancy, such as infant heart problems, spontaneous abortion, and premature delivery, so pregnant people need special monitoring.

People with Sjogren’s syndrome are also at increased risk of yeast infections (candidiasis), both orally and vaginally, which may require antifungal treatment.

Additionally, people who initially are diagnosed with primary Sjogren’s syndrome are at risk of developing a related autoimmune condition, such as rheumatoid arthritis or lupus. 

Living With Sjogren’s Syndrome

Sjogren’s syndrome can range from a mild nuisance to a condition that much more severely impacts your quality of life. There are many treatment and management options to help you adapt, but some symptoms may still be bothersome, particularly if you have symptoms in addition to dry eye and mouth. 

Sjogren’s syndrome can be stressful, but stress may also worsen flare-ups of symptoms. If you can, find ways to reduce and process your stress. Try to find time to do what you enjoy and eliminate unnecessary responsibilities. 

Don’t hesitate to lean on others for support as well. Depending on your preferences, this might include your friends and family, a mental health professional, and/or a support group of others with Sjogren’s.