In the early 20th century, Swedish physician Henrik Sjögren’s (SHOW-gren) first described a group of women whose chronic arthritis was accompanied by dry eyes and dry mouth. Sjögren’s syndrome (SS) can develop on its own (called "primary SS") or as a complication of another autoimmune disorder (called "secondary SS"), most often lupus or rheumatoid arthritis. Symptoms vary in type and intensity, and serious complications are rare.
Sjögren’s syndrome is an inflammatory disease with unknown cause that can affect many different parts of the body, but most often affects the tear and saliva glands. Patients with this condition may notice irritation, a gritty feeling, or painful burning in the eyes. Dry mouth or difficulty eating dry foods, and swelling of the glands around the face and neck. Some patients experience dryness of other mucous membranes (such as the nasal passages, throat, and vagina) and skin.
Most of the complications of Sjögren’s occur because of decreased tears and saliva. Patients with dry eyes are at increased risk for infections around the eye and may have damage to the cornea. Dry mouth may cause an increase in dental decay, gingivitis (gum inflammation), and oral yeast infections (thrush) that may cause pain and burning. Some patients have episodes of painful swelling in the saliva glands around the face.
Complications in other parts of the body occur rarely in patients with Sjögren’s syndrome. Pain and stiffness in the joints with mild swelling may occur in some patients, even in those without RA or lupus. Rashes on the arms and legs related to inflammation in small blood vessels (vasculitis) and inflammation in the lungs, liver, and kidney may occur rarely and be difficult to diagnose. Neurological complications that cause symptoms such as numbness, tingling, and weakness have also been described in some patients.
Who develops Sjögren’sSyndrome?
According to the American College of Rheumatology, between 400,000 and 3.1 million adults have Sjögren’s Syndrome. This condition can affect people of any age, but symptoms usually appear between the ages of 45 and 55. It affects 10 times as many women as men. About half of affected patients also have rheumatoid arthritis or other connective tissue diseases, such as lupus, scleroderma, or polymyositis.
Approximately 1.3 million adults (or 0.6 percent of the U.S. adult population) have rheumatoid arthritis, according to the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS). Doctors at Johns Hopkins Arthritis Center share that approximately 10-15% of patients with rheumatoid arthritis, mostly women, develop Sjögren’s syndrome. In contrast Dr. Steven Mandel, Clinical Professor of Neurology at Jefferson Medical College, says that 25% of patients with RA also have Sjögren’s. With 10-25% of the RA population developing Sjögren’s Syndrome, we as patients should be aware of this risk.
How can Sjögren’s Syndrome affect the body?
- Dry eyes, corneal ulcerations, and infections
- Dry nose, recurrent sinusitis, nose bleeds
- Dry mouth, mouth sores, dental decay
- Difficulty with chewing, speech, taste and dentures
- Dry skin, vasculitis, Raynaud’s phenomenon
- Vaginal dryness, painful intercourse
- Arthritis, muscle pain
- Recurrent bronchitis, pneumonia, interstitial lung disease
- Difficulty swallowing, heartburn, reflux esophagitis
- Stomach upset, gastroparesis, autoimmune pancreatitis
- Abnormal liver function tests, chronic active autoimmune hepatitis, primarily biliary cirrhosis
- Neurological problems, concentration/memory loss (brain fog)
- Peripheral neuropathy (numbness and tingling in the extremities)
How is SjÃ¶gren’s Syndrome treated?
Treatment is designed to lessen the most bothersome symptoms. Dry eyes usually respond to the use of artificial tears applied regularly during the day or to gels applied at night. Other measures, such as plugging or blocking tear ducts, can be used in more severe cases. Eyedrops that reduce inflammation in the glands around the eyes (cyclosporine- Restasis) may be used to increase tear production. Dry mouth can be relieved by drinking water, chewing gum, or using saliva substitutes. Some patients benefit from using prescription medications that stimulate saliva flow, such as pilocarpine (Salagen) or cevimuline (Evoxac). If patients develop yeast infections, these can be relieved by anti-fungal therapies. The currently available treatments may help relieve some of the dryness but usually some dryness persists.
All patients should receive regular dental care in order to prevent cavities and tooth loss that may occur as a complication of the disorder. Patients with dry eyes should see an ophthalmologist (eye doctor) regularly for signs of damage to the cornea. Patients with excessive redness and pain in the eyes should be evaluated for infections.
Hydroxychloroquine (Plaquenil), an antimalarial drug used in lupus and rheumatoid arthritis, may be helpful in some patients with SjÃ¶gren’s syndrome by reducing joint pain and rash. Patients with rare but serious systemic symptoms (such as fever, rashes, abdominal pain, or lung or kidney problems) may require treatment with corticosteroids such as prednisone and/or immunosuppressive agents methotrexate (Rheumatrex), azathioprine (Imuran), mycophenolate (Cellcept), cyclophosphamide (Cytoxan). In addition, rituximab (Rituxan) and other biological therapies (as used in rheumatoid arthritis) are undergoing evaluation for treating patients with severe systemic manifestations of disease.
Living with SjÃ¶gren’s Syndrome and Rheumatoid Arthritis
People with SjÃ¶gren’s syndrome are usually able to live normal lives with very few adjustments. When a diagnosis is made, many patients must focus a great deal of attention dealing with dry eyes and dry mouth, but these symptoms tend to subside with time. Patients should see their physician regularly for general health screening, and should pay close attention to any abnormal swelling in the glands around the face or neck, under the arms, or in the groin areas as this may be a sign of lymphoma.
SjÃ¶gren’s syndrome cannot be cured, but in many cases proper treatment helps to alleviate symptoms. Rheumatologists are specialists in musculoskeletal disorders and therefore are more likely to make a proper diagnosis. They can also advise patients about the best treatment options available.
As Lene points out in the comments below, dryness is not always caused by a new disease process. It can be a side-effect of the medications we commonly take to battle rheumatoid arthritis. A trade-off which is certainly worth it for many. “Better living through chemistry” - right Lene?
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Lisa Emrich is a patient advocate, accomplished speaker, author of the award-winning blog Brass and Ivory: Life with MS and RA, and founder of the Carnival of MS Bloggers. Lisa uses her experience to educate patients, raise disease awareness, encourage self-advocacy, and support patient-centered research. Lisa frequently works with non-profit organizations and has brought the patient voice to health care conferences and meetings worldwide. Follow Lisa on Facebook, Twitter, and Pinterest.