Madisons Foundation - Moms And Dads In Search Of Needed Support

What

Sjögren’s syndrome is an autoimmune disease in which the body’s immune system mistakenly attacks its own moisture-producing glands. Normally, our immune system targets foreign bodies such as bacteria and viruses in order to protect us from disease. However, in Sjögren’s syndrome, the immune system malfunctions and attacks the body’s own cells. The glands that produce tears and saliva become destroyed, and patients develop dryness of the mouth, eyes, and other mucous membranes. Sjögren’s syndrome can affect the entire body, and is usually associated with other autoimmune disorders, such as rheumatoid arthritis, systemic lupus erythematosus, scleroderma, and polymyositis. When the condition occurs alone, it is called “Primary Sjögren’s syndrome.” When it occurs with another disease, the condition is referred to as “Secondary Sjögren’s syndrome.”

Who

Sjögren’s syndrome usually occurs in women 40-50 years old although it can occur in all age groups in both women and men. It rarely occurs in children, but it is possible. Approximately 3% of the population (or 4 million Americans) is affected by Sjögren’s syndrome.

Signs and Symptoms

The main symptoms of Sjögren’s syndrome are dry or itchy eyes and dryness of the mouth. Sjögren’s can also affect other parts of the body (ie. kidneys, gastrointestinal tract, lung, liver, pancreas, and blood vessels), and as a result, people may exhibit some or all of the following additional symptoms:

1. Dry skin, or skin rashes.
   
2. Difficulty swallowing.
   
3. Loss of taste, or impaired taste.
   
4. Severe dental cavities.
   
5. Hoarseness.
   
6. Swollen glands.
   
7. Cloudy cornea, which can affect vision.

Possible Causes

The exact case of Sjögren’s syndrome is still unknown. Researchers believe that Sjögren’s syndrome may be caused by a combination of genetic and environmental factors. Most likely, individuals with Sjögren’s may have carried a gene that predisposed them to developing the condition. Then, a trigger such as a viral or bacterial infection may have brought on the syndrome

Diagnosis

Diagnosis of Sjögren’s syndrome usually involves blood tests, eye and dental exams, and tissue biopsy. In Sjögren’s, the body’s immune system produces specific antibodies towards cells of the moisture-producing glands. As a result, a doctor will test for the presence of antibodies in the patient’s blood to help diagnose Sjögren’s. These antibodies include SS-A, SS-B, anti-nuclear antibody and rheumatoid factor. While these antibodies are almost always present in Sjögren’s syndrome, they can be present in other diseases, or even normal people, as well. They need to be considered in the context of the patient’s symptoms and physical findings before a diagnosis is made. Eye exams are also done to test for tear production, while dental exams are done to measure saliva production. Lastly, a biopsy of the salivary glands can be performed to see if cells called lymphocytes are present, which would indicate that the immune system has indeed mounted an attack on these cells.

Treatment

Treatment for patients with Sjögren’s syndrome consists of alleviating symptoms. For example, dry eyes can be treated with artificial tears or a lubricating ointment. Dry mouth can be helped by sipping water, using a mouth lubricant, or taking saliva-stimulating drugs such as Pilocarpine. Joint or muscle pain can be treated with nonsteroidal anti-inflammatory drugs. Lung, kidney, blood vessel, or nervous system problems can be treated by suppressing the immune system with drugs (ie. methotrexate and hydroxychloroquine). Lastly, certain medications such as antihistamines and decongestants should be avoided because they can exacerbate symptoms of dryness.

Prognosis

The prognosis for individuals with Sjögren’s syndrome is variable and can depend on the additional autoimmune diseases the patient has. Generally, Sjögren’s syndrome itself is not life threatening, and children properly treated for the condition can lead normal lives.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

The Sjögren’s Syndrome Foundation
An outstanding website with information about Sjogren's as well as about ongoing research, advocasy and patient support.

Lupus Foundation of America
Well written information on Sjogren's Syndrome with links to other useful sites. This site also contains information on ongoing lupus related research.

National Institute of Arthritis and Musculoskeletal and Skin Diseases
A great website organized in questions and answer form. It has detailed information about Sjogren's Syndrome as well as other usefull resources.

Google Search for Sjögren's Syndrome

References and Sources

Sjögren’s Syndrome and Lupus: www.lupus.org/education/brochures/sjogren.html MedlinePlus: Sjögren’s Syndrome: www.nlm.nih.gov/medlineplus/ency/article/000456.htm National Institute of Arthritis and Musculoskeletal and Skin Diseases: “Questions and Answers of Sjögren’s Disease” www.niams.nih.gov/hi/topics/sjogrens/index.htm The Sjögren’s Syndrome Foundation: www.sjogrens.org/syndrome/diagnosis.html