Asked by Susan Boyce, Myrtle Creek, Oregon
Can you explain a little about Sjogren's (SHOW-grins) syndrome? I have it at 53. My sister died two years ago at 53; her heart and lungs were involved. Our aunt had the same autoimmune disease and passed away many years ago. Is this becoming more common? What kind of hope to we have? Is science working on a cure?
Dr. Otis Brawley
Chief Medical Officer,
American Cancer Society
Sjögren's syndrome (SS) is a chronic inflammatory or rheumatologic disorder. Patients have decreased gland function, especially of the lacrimal, or tearing, glands, leading to dry eye. They may also have problems with the salivary glands, leading to dry mouth. This combination of dry eyes and dry mouth is called the sicca complex. It is the only manifestation of the disease in one-third of SS patients.
Patients with the dry eye syndrome may complain of a gritty, sandy feeling in the eye instead of dryness. They can also have corneal ulcerations and other eye diseases caused by the dryness. Dry mouth can cause candidiasis, or fungal infections of the mouth. Dry mouth can also lead to severe inflammation of the mouth and difficulty with gum health and difficulty supporting teeth (gingivitis). Dental caries are a significant problem.
Other common problems associated with SS are enlargement of salivary and lacrimal glands. Dryness of the upper airway tract is a reported problem. These patients often have sinus and throat problems, which can lead to a cough and in severe cases, pneumonia. Some female patients will complain of severe vaginal dryness.
Fatigue is common in Sjögren's syndrome. It may be because of the disease itself or to difficulties staying asleep, which might occur if one drinks a lot of water to treat dry mouth and then needs to urinate frequently at night.
SS can occur by itself or with other rheumatoid diseases. When occurring with other diseases, it is most commonly seen with rheumatoid arthritis. Fibromyalgia, a disease involving nonspecific muscle aches, is also common.
Non-Hodgkin lymphoma, a malignancy of the immune system, occurs in 2.5 percent of SS patients followed for 10 years. Many patients who succumb to the disease actually die of infections cause by the immune dysfunction that characterizes this disease.
The diagnosis of SS is usually confirmed through biopsy of a salivary gland, which is a relatively simple surgery, or through a blood test looking for specific antibodies.
The treatment of this disease is largely keeping affected areas hydrated. There are some medications beyond artificial tears for dry eye. There are also some treatments for dry mouth. Some patients find room humidifiers comforting.
Patients with this syndrome do much better if they understand the disease. For example, some common over-the-counter cold medicines need to be avoided as they can worsen symptoms. I have found the Sjögren's Syndrome Foundation, Inc., and the Arthritis Foundation to be very helpful in explaining the syndrome. The National Institute of Neurological Disorders and Stroke of the National Institutes of Health is the largest sponsor of Sjögren's research. It also has a listing of clinical trials that are still being enrolled.
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