Sjogren's Syndrome (cont.)
William C. Shiel Jr., MD, FACP, FACR
Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.
Catherine Burt Driver, MD
Catherine Burt Driver, MD, is board certified in internal medicine and rheumatology by the American Board of Internal Medicine. Dr. Driver is a member of the American College of Rheumatology. She currently is in active practice in the field of rheumatology in Mission Viejo, Calif., where she is a partner in Mission Internal Medical Group.
In this Article
- Sjögren's syndrome facts
- What is Sjögren's syndrome?
- What causes Sjögren's syndrome?
- What are risk factors for developing Sjögren's syndrome?
- What are Sjögren's syndrome symptoms and signs?
- How is Sjögren's syndrome diagnosed?
- What is the treatment for Sjögren's syndrome? Will dietary changes improve Sjögren's syndrome symptoms and signs?
- What are complications of Sjögren's syndrome?
- Can Sjögren's syndrome be prevented?
- What is the prognosis for patients with Sjögren's syndrome?
- Find a local Rheumatologist in your town
What causes Sjögren's syndrome?
While the exact cause of Sjögren's syndrome is not known, there is growing scientific support for genetic (inherited) factors. The genetic background of Sjögren's syndrome patients is an active area of research. The illness is sometimes found in other family members. It is also found more commonly in families that have members with other autoimmune illnesses, such as systemic lupus erythematosus, autoimmune thyroid disease, type I diabetes, etc. About 90% of patients with Sjögren's syndrome are female.
What are risk factors for developing Sjögren's syndrome?
The main risk factor for the development of Sjögren's syndrome is being a member of a family that is already characterized as having autoimmune illnesses. This does not mean that it is predictable that a member of a family with known autoimmunity will develop the disease, only that is more likely than if there were no family members with known autoimmunity. Accordingly, it is likely that certain genes that are inherited from ancestors can predispose one to the development of Sjögren's syndrome. It should also be noted that Sjögren's syndrome can also be sporadic and occur in a person from a family with no known autoimmunity.
What are Sjögren's syndrome symptoms and signs?
Symptoms of Sjögren's syndrome can involve the glands, as above, but there are also possible effects of the illness involving other organs of the body (extraglandular manifestations).
When the tear gland (lacrimal gland) is inflamed from Sjögren's, the resulting eye dryness can progressively lead to eye irritation, decreased tear production, a "gritty" sensation, eye infection, and serious abrasion of the dome of the eye (cornea). Dry eyes can lead to infections of the eyes and inflammation of the eyelids (blepharitis). The condition of having dry eyes is medically referred to as xerophthalmia. When the eyes become inflamed from dryness, it is referred to as keratoconjunctivitis sicca.
Inflammation of the salivary glands can lead to mouth dryness, swallowing difficulties, dental decay, cavities, gum disease, mouth sores and swelling, and stones and/or infection of the parotid gland inside of the cheeks. Dry lips often accompany the mouth dryness. Dry mouth is medically referred to as xerostomia.
Other glands that can become inflamed, though less commonly, in Sjögren's syndrome include those of the lining of the breathing passages (leading to lung infections) and the vagina (sometimes causing pain during intercourse or recurrent vaginal infections).
Extraglandular (outside of the glands) problems in Sjögren's syndrome include fatigue, joint pain or inflammation (arthritis), Raynaud's phenomenon, lung inflammation, lymph node enlargement, and kidney, nerve, and muscle disease. A rare serious complication of Sjögren's syndrome is inflammation of the blood vessels (vasculitis), which can damage the tissues of the body that are supplied by these vessels.
A common disease that is occasionally associated with Sjögren's syndrome is autoimmune thyroiditis (Hashimoto's thyroiditis), which can lead to abnormal thyroid hormone levels detected by thyroid blood tests. Heartburn and difficulty swallowing can result from gastroesophageal reflux disease (GERD), another common condition associated with Sjögren's syndrome. A rare and serious disease that is uncommonly associated with Sjögren's syndrome is primary biliary cirrhosis, an autoimmune disease of the liver that leads to scarring of the liver tissue. A small percentage of patients with Sjögren's syndrome develop cancer of the lymph glands (lymphoma). This usually develops only after many years with the illness. Unusual lymph node swelling should be reported to the physician.
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