Sjögren's syndrome is an autoimmune disease in which the body's immune system mistakenly attacks its own moisture producing glands. Sjögren's is one of the most prevalent autoimmune disorders, striking as many as 4,000,000 Americans. Nine out of ten patients are women. The average age of onset is late 40s although Sjögren's occurs in all age groups in both women and men.
About 50% of the time Sjögren's syndrome occurs alone, and 50% of the time it occurs in the presence of another connective tissue disease. The four most common diagnoses that co-exist with Sjögren's syndrome are Rheumatoid Arthritis, Systemic Lupus, Systemic Sclerosis (scleroderma) and Polymyositis/Dermatomyositis. Sometimes researchers refer to the first type as "Primary Sjögren's" and the second as "Secondary Sjögren's." All instances of Sjögren's syndrome are systemic, affecting the entire body.
Sjögren Syndrome Symptoms
The defining symptoms of Sjögren syndrome are dry eyes (xerophthalmia) and dry mouth (xerostomia). Other areas can be dry as well, such as the inside of the nose, the skin, the airways of the lungs, and the vagina. These symptoms often are referred to as the sicca (dryness) complex.
Dry eyes can cause:
Almost every system of the body can be affected. Symptoms depend on which areas are affected and may include any of the following:
Symptoms are mild in some people and very severe in others. Symptoms can vary over time and may improve, worsen, or even go away completely for periods of time.
Dry eyes and mouth do not always mean Sjögren syndrome. The symptoms are common and can be caused by other medical disorders, by some medications, and by anxiety. Dryness also may be a result of normal changes in glands and tissues that occur with aging.
Sjogren's syndrome is an autoimmune disorder. This means that your body attacks its own cells and tissues.
It's unknown why this happens, but researchers believe that a combination of factors causes something to go wrong with your immune system. These factors may be related to heredity, hormones, a viral or bacterial infection, or your nervous system.
In the case of Sjogren's syndrome, white blood cells called lymphocytes mistakenly target, attack and damage your moisture-producing glands. They can also damage other organs, including your lungs, kidneys and liver.
Sjogren's syndrome that results from a rheumatic condition is classified as secondary Sjogren's syndrome. Primary Sjogren's syndrome occurs by itself.
Primary Vs. Secondary Sjogren's Syndrome
When only gland inflammation and resulting dry eyes and mouth are involved the disease is known as primary Sjogren's syndrome. There can also be extraglandular problems associated with Sjogren's syndrome which may include:
The disease is known as secondary Sjogren's syndrome when the gland inflammation exists in combination with another connective tissue disease or autoimmune disease such as: