- Risk Factors
What is Sjögren's syndrome?
Sjögren's syndrome is an autoimmune disease characterized by dryness of the mouth and eyes.
- Autoimmune diseases feature the abnormal production of extra antibodies in the blood that are directed against various tissues of the body.
- The misdirected immune system in autoimmunity tends to lead to inflammation of tissues.
- This particular autoimmune illness features inflammation and dysfunction in glands of the body that are responsible for producing tears and saliva.
- Inflammation of the glands that produce tears (lacrimal glands) leads to decreased water production for tears and dry eyes.
- Inflammation of the glands that produce the saliva in the mouth (salivary glands, including the parotid glands) leads to decreased saliva production and dry mouth and dry lips.
Sjögren's syndrome with gland inflammation (resulting in dry eyes and mouth, etc.) that is not associated with another connective tissue disease is referred to as primary Sjögren's syndrome. Sjögren's syndrome which is also associated with a connective tissue disease, such as rheumatoid arthritis, systemic lupus erythematosus, or scleroderma, is referred to as secondary Sjögren's syndrome. Dryness of eyes and mouth, in patients with or without Sjögren's syndrome, is sometimes referred to as sicca syndrome.
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. Most patients with Sjögren's syndrome are female.
What are risk factors for 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, certain genes that are inherited from ancestors can likely 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 the symptoms of Sjögren's syndrome?
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 symptoms such as eye irritation, decreased tear production, a "gritty" sensation, and 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 the following symptoms and signs:
- mouth dryness,
- swallowing difficulties,
- dental decay,
- gum disease,
- mouth sores and swelling,
- hoarseness or impaired voice,
- abnormality of taste or loss of taste,
- dry cough, and
- stones and/or infection of the parotid gland inside of the cheeks.
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 a woman 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, joint pain, and muscle disease with muscle pain and weakness. 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 disorder 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.
Diagnosis of Sjögren's syndrome
Doctors that treat Sjögren's syndrome include general medicine physicians including general practitioners, family medicine doctors, and internists, as well as specialists including rheumatologists, ophthalmologists, and otolaryngologists (ENT physicians).
The diagnosis of Sjögren's syndrome involves noting criteria such as dryness of the eyes and mouth. Significant dryness of the eyes can be determined in the doctor's office by testing the eye's ability to wet a small testing paper strip placed under the eyelid (Schirmer's test using Schirmer tear test strips). More sophisticated testing can be done by an eye specialist (ophthalmologist).
Sjögren's syndrome salivary glands can become larger and harden or become tender. Salivary-gland inflammation can be detected by radiologic nuclear medicine salivary scans. Also, the diminished ability of the salivary glands to produce saliva can be measured with salivary flow testing. The diagnosis is strongly supported by abnormal findings of a biopsy of salivary gland tissue.
The glands of the lower lip are occasionally used for a biopsy sample of the salivary-gland tissue in the diagnosis of Sjögren's syndrome. The lower lip salivary-gland biopsy procedure is performed under local anesthesia with the surgeon making a tiny incision on the inner part of the lower lip to expose and remove a sample of the tiny salivary glands within.
Patients with Sjögren's syndrome typically produce a variety of extra antibodies against body tissues (autoantibodies). These can be detected through blood testing and include antinuclear antibodies (ANA), which are present in nearly all patients. Typical antibodies that are found in most, but not all patients, are SS-A and SS-B antibodies (Sjögren's syndrome A and B antibodies, also known as anti-Ro and anti-La antibodies), rheumatoid factor, thyroid antibodies, and others. Low red blood count (anemia) and abnormal blood levels of markers of inflammation (sedimentation rate, C-reactive protein) are seen.
What is the treatment for Sjögren's syndrome?
The treatment of patients with Sjögren's syndrome is directed toward the particular areas of the body that are involved and the prevention of complications such as infection. There is no cure for Sjögren's syndrome.
Dryness of the eyes can be helped by artificial tears, using eye-lubricant ointments at night, and minimizing the use of hair dryers. When dryness becomes more significant, the ophthalmologist can plug the tear duct closed so that tears cover the eye longer. Cyclosporine eyedrops (Restasis) are approved medicated eyedrops that can reduce the inflammation of the tear glands, thereby improving their function. Signs of eye infection (conjunctivitis), such as pus or excessive redness or pain, should be evaluated by the doctor. The dietary addition of flaxseed oil may also benefit eye dryness. Vitamin D supplementation may be beneficial, especially in those who have insufficient vitamin D blood levels.
The dry mouth can be helped by drinking plenty of fluids, humidifying the air, and good dental care to avoid dental decay. The glands can be stimulated to produce saliva by sucking on sugarless lemon drops or glycerin swabs. Additional treatments for the symptom of dry mouth are prescription medications that are saliva stimulants, such as pilocarpine (Salagen) and cevimeline (Evoxac). These medications should be avoided by people with certain heart diseases, asthma, or glaucoma.
Artificial saliva preparations can ease many of the problems associated with dry mouth. Many of these types of agents are available as over-the-counter products, including toothpaste, gum, and mouthwash (Biotene). Numoisyn Liquid and lozenges are also available for the treatment of dry mouth. Vitamin E oil has been used with some success. Infections of the mouth and teeth should be addressed as early as possible to avoid more severe complications. Diligent dental care is very important. Moist, warm compresses can be massaged onto the parotid glands to help relieve swelling and pain.
- Saltwater (saline) nasal sprays can help dryness in the passages of the nose. A woman should consider vaginal lubricant for sexual intercourse if vaginal dryness is a problem.
- Hydroxychloroquine (Plaquenil) has been helpful for some manifestations of Sjögren's syndrome, particularly fatigue, and muscle and joint pains. Serious complications of Sjögren's syndrome, such as vasculitis, can require immune-suppression medications, including cortisone (prednisone and others) and/or azathioprine (Imuran) or cyclophosphamide (Cytoxan).
- Infections, which can complicate Sjögren's syndrome, are addressed with appropriate antibiotics. Cancer of the lymph nodes (lymphoma), a rare complication of Sjögren's syndrome, is treated independently.
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What are the complications of Sjögren's syndrome?
It is not uncommon for people with Sjögren's syndrome to have complications of infections. Infections that are common in these patients include dental infections, eye infections, sinusitis, bronchitis, and vaginitis. Close monitoring and early treatment of these infections are keys to optimal outcomes.
Some people with Sjögren's syndrome can develop inflammation of the parotid gland in the cheeks. This can lead to dryness of the ducts that drain the gland and stones can form that block these drainage tubes. This can be serious and require aggressive antibiotics and surgery.
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.
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.
Another 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.
What is the prognosis for Sjögren's syndrome?
With proper attention to eye and oral care, the outlook for patients with Sjögren's syndrome is generally excellent. Eye dryness can lead to serious injury to the eye, particularly the cornea, and this should be avoided by consultation with an eye specialist. Mouth dryness can lead to dental decay and parotid gland infection and parotid stones. Optimal oral hygiene is essential.
The serious complications of primary biliary cirrhosis and lymphoma can dramatically affect prognosis and are monitored for in routine regular office visits.
Sjögren syndrome is an area of active immunology research. Many new treatments will be available in the near future. Research has suggested that rituximab (Rituxan) may be beneficial for many features of Sjögren syndrome.
Sjögren syndrome is named after a Swedish ophthalmologist, Henrik Sjögren. In the early 1900s, Sjögren called the syndrome "keratoconjunctivitis sicca." The name sicca syndrome is technically now used only to describe the combination of dryness of the mouth and eyes, regardless of cause. The term sicca refers to the dryness of the eyes and mouth.
Is it possible to prevent Sjögren's syndrome?
Because Sjögren's syndrome is felt to be inherited, there is no particular way to prevent developing the disease. However, preventing complications of Sjögren's syndrome, such as infection, eye irritation, etc., can be achieved using the methods described above.
Koopman, William, et al., eds. Clinical Primer of Rheumatology. Philadelphia: Lippincott Williams & Wilkins, 2003.
Ruddy, Shaun, et al., eds. Kelley's Textbook of Rheumatology. 6th ed. Philadelphia: W.B. Saunders Co., 2001.
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