Sjögren’s syndrome is a condition associated most typically with reduced production of tears in the eyes and saliva in the mouth. However, Sjögren’s is a systemic disease that can affect the entire body, including the blood vessels, kidneys and the nervous system.
Sjögren’s syndrome is an autoimmune disease, meaning it affects how the immune system responds. Usually, the immune system helps your body fight off bacteria, infections and viruses. With an autoimmune disease, your immune system mistakenly attacks your body.
It’s not uncommon for a person to know that they have an autoimmune disease such as rheumatoid arthritis and only later discover they also have Sjögren’s, says Dr. Esen K. Akpek, director of the Johns Hopkins Jerome L. Greene Sjögren’s Syndrome Center at Johns Hopkins Bayview Medical Center. Alpek is also director at the Ocular Surface Disease and Dry Eye Clinic at the Wilmer Eye Institute at Johns Hopkins University in Baltimore.
There are about 4 million people in the U.S. living with Sjögren’s syndrome, according to the Sjögren’s Foundation. Most of those who have it are middle-aged women. Tennis star Venus Williams is one well-known person with Sjögren’s.
The causes of Sjögren’s aren’t entirely clear. There may be a role for genetics or hormones such as estrogen, which would help explain why it affects more women, says Dr. Alireza Meysami, division head of the Division of Rheumatology, Department of Internal Medicine with the Henry Ford Health System and a clinical associate professor of medicine at Wayne State University School of Medicine, both in Detroit. There also may be other triggers, such as infections or chemical exposures, adds Dr. Sheeja Francis, a rheumatologist with Premier Medical Group in New Windsor and Fishkill, New York.
[SEE: Lupus Diet and Nutrition.]
Symptoms of Sjögren’s Syndrome
The two most common symptoms of Sjögren’s syndrome involve dryness in the eyes and mouth. Doctors may call this dryness sicca.
— Blurry vision.
— Changes to vision.
— Infections in the front part of the eye (the cornea).
— Corneal ulcers, or an open sore on the cornea.
— Light sensitivity.
Symptoms related to a dry mouth in Sjögren’s include:
— Difficulty chewing and swallowing.
— Increased dental cavities.
— Thrush, a type of yeast infection in the mouth.
— Having difficulty eating dry food, such as crackers.
Because Sjögren’s syndrome can potentially affect your whole body, it may cause many different health complications. Not every person with Sjögren’s will have these. Arthritis is the most common symptom associated with Sjögren’s, aside from dry eyes and mouth, Meysami says.
Also, because Sjögren’s can take a long time to diagnose, some of these complications could be part of the symptoms you or your doctor identify.
Here are some of Sjögren’s most common symptoms and complications:
— Dry skin and nose.
— Skin rashes.
— Enlarged salivary glands in the face and neck.
— Profound fatigue.
— Arthritis/joint pain.
— Abnormal liver function.
— Changes in taste or smell.
— Having a dry cough or shortness of breath.
— Interstitial cystitis, also known as painful bladder syndrome.
— Kidney function problems.
— Memory loss.
— Neuropathy — nerve pain and/or numbness.
— Pneumonia, bronchitis or other lung problems.
— Problems with sexual intimacy due to vaginal dryness.
Diagnosis of Sjögren’s Syndrome
It takes an average of almost three years to diagnose Sjögren’s syndrome, the Sjögren’s Foundation reports. That’s because the symptoms can be slow to develop and they can mimic other disorders. Or, a patient may be using a medication that causes dryness, so the dryness is only thought to be a drug-induced side effect, Francis says. Also, the symptoms may require care from several health providers — including a rheumatologist, ophthalmologist, ENT specialist and even a dentist. That can make it harder to coordinate care if there’s not a firm diagnosis.
A rheumatologist is the primary doctor who will diagnose and treat Sjögren’s syndrome, although other health providers may suspect it based on a patient’s symptoms. Dry eye symptoms often occur before other Sjögren’s symptoms, Akpek says.
In addition to physical exam findings, the rheumatologist will order lab work to measure markers that can indicate Sjögren’s syndrome. These include Sjögren’s-specific antibodies found in the blood, such as anti-Ro (SS-A) and anti-La (SS-B). Antibodies are a type of protein your immune system makes in response to specific invaders. Seventy percent of people with Sjögren’s syndrome will have a positive blood test for the antibody SS-A, says Dr. Lindsay Lally, an assistant attending rheumatologist at the Hospital for Special Surgery in New York. And 40% will have a positive test for SS-B, according to the Sjögren’s Foundation.
Doctors also may measure dry eye severity and salivary gland function. Some eye tests include what’s called a tear break-up time test and a staining test that can assess for specific dry areas on the eye. In the mouth, a test called sialometry can measure salivary flow, and doctors also frequently perform an inner lip salivary gland biopsy, Francis says.
Because Sjögren’s is traditionally associated with women, men with the disease may get overlooked, Akpek says. Some of her research has focused on diagnosing Sjögren’s in men to make sure the disease is also detected in them.
Treatment for Sjögren’s Syndrome
There is no cure for Sjögren’s syndrome, so doctors will offer treatments for the symptoms. Many of the treatments focus on helping dry eye or dry mouth, which both can be uncomfortable when you have the disease.
For dry eye, treatments include:
— Over-the-counter artificial tears. It’s always best to get tears that are preservative-free, as the preservatives can irritate the eyes, Akpek says. Eye ointments and gels also may be used at night.
— Prescription eye drops such as cyclosporine A and lifitegrast, which help the eyes to produce more tears and improve tear quality.
— Punctal plugs, which are inserted via a quick procedure and can help block the drainage of tears.
— Autologous serum tears, which are eye drops created in a lab using your own blood.
For dry mouth, treatments include:
— Alcohol-free mouthwashes.
— Sugar-free gum and candy to increase salivary flow. Products with the sugar-free sweetener xylitol often are recommended, Francis says.
— Medications that increase saliva production such as pilocarpine and cevimeline.
If Sjögren’s affects other parts of the body, doctors may prescribe nonsteroidal anti-inflammatory drugs as a first-line therapy. Over-the-counter NSAIDs include aspirin, ibuprofen and naproxen. They can help to lower inflammation and pain.
Another type of medication called corticosteroids can help some patients with Sjögren’s. They can help to stop severe symptoms or a flare-up.
Immunosuppressives are another drug used for Sjögren’s. They are often part of the treatments for autoimmune diseases.
Rituximab, a type of medication that targets a type of white blood cell called B-cells, has recently been studied and has had good results for some Sjögren’s symptoms, Lally says.
A group of medications called disease-modifying anti-rheumatic drugs, or DMARDs, are another commonly used drug for Sjögren’s joint pain and other autoimmune diseases. These drugs change the way the immune system works. Hydroxychloroquine, a type of drug also used to treat malaria, is the most common DMARD used in Sjögren’s.
Tips for Better Living With Sjögren’s Syndrome
1. Always schedule regular follow-ups with your doctors. Ask your rheumatologist how often you should be seen. Over a 15- to 20-year period, 8% to 15% of patients with Sjögren’s develop a cancer of the lymph nodes called lymphoma, according to the Sjögren’s Foundation. That’s why regular screenings with lab tests are so important, Francis says. Also ask how often to see an eye doctor and dentist. For his patients, Meysami recommends every six months for eye doctor visits and every three months for dentist visits.
2. Know when to see a doctor beyond your routine checks, Meysami says. Here are some symptoms that should prompt you to seek an appointment sooner rather than later when you have Sjögren’s:
— A change in your vision with no apparent cause.
— Fatigue that’s worse than usual.
— A new rash.
— Pain in the eye.
— Sensitivity to light.
— Swollen glands.
— Unexplained weight loss.
3. Provide a medication list to your doctor, Lally advises. This can help both of you to see if you’re using any meds that make dryness worse, such as antihistamines or certain sleep aids.
4. Make environmental changes to lessen the effects of dryness, Lally recommends. This can include:
— Avoiding dry or windy environments that can make dry eyes worse.
— Wearing sunglasses to protect the eyes, especially on windy days.
— Traveling with a wet washcloth to help provide humidity to the face or eye area, Meysami advises
— Using a humidifier at night.
5. Drink enough water and stay well-hydrated.
6. Avoid smoking or getting exposed to secondhand smoke. Smoking can make dry eyes and mouth worse, Lally says.
More from U.S. News