Sjogren’s Syndrome

Sjogren's syndrome classically features a combination of dry eyes, dry mouth, and another disease of the connective tissues, most commonly rheumatoid arthritis.

Sjogren's syndrome is an autoimmune disease, characterized by the abnormal production of extra antibodies in the blood that are directed against various tissues of the body.

This particular autoimmune illness is caused by inflammation in the glands of the body. Inflammation of the glands that produce tears (lacrimal glands) leads to decreased water production for tears and eye dryness. Inflammation of the glands that produce the saliva in the mouth (salivary glands, including the parotid glands) leads to mouth dryness.

Sjogren's syndrome that involves the gland inflammation (resulting in dryness of the eyes and mouth, etc.), but not associated with a connective tissue disease, is referred to as primary Sjogren's syndrome. Secondary Sjogren's syndrome involves not only gland inflammation, but is associated with a connective tissue disease, such as rheumatoid arthritis, systemic lupus erythematosus, or scleroderma.

What causes Sjogren's syndrome?

While the exact cause of Sjogren's syndrome is not known, there is growing scientific support for genetic (inherited) factors. 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, juvenile diabetes, etc. 90% of Sjogren's syndrome patients are female.

What are symptoms of Sjogren's syndrome?

Symptoms of Sjogren's syndrome can involve the glands, as above, but there are also possible affects of the illness involving other organs of the body (extraglandular manifestations).

When the tear gland (lacrimal gland) is inflamed from Sjogren's, the resulting eye dryness can progressively lead to eye irritation, decreased tear production, "gritty" sensation, infection, and serious abrasion of the dome of the eye (cornea).

Inflammation of the salivary glands can lead to mouth dryness, swallowing difficulties, dental decay, gum disease, mouth sores and swelling, stones and/or infection of parotid gland inside of the cheeks.

Other glands that can become inflamed, though less commonly, in Sjogren's syndrome include those of the lining of the breathing passages (leading to lung infections) and vagina (sometimes noted as pain during intercourse).

Extraglandular (outside of the glands) problems in Sjogren's syndrome include joint pain or inflammation (arthritis), Raynaud's phenomenon, lung inflammation, lymph node enlargement, kidney, nerve, and muscle disease.

A rare serious complication of Sjogren'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 Sjogren's syndrome is autoimmune thyroiditis (Hashimoto's thyroiditis), which can lead to abnormal hormone levels detected by thyroid blood tests.

Heartburn and difficulty swallowing can result from gastroesophageal reflux disease (GERD), another common condition associated with Sjogren's syndrome.

A rare disease that is uncommonly associated with Sjogren's syndrome is primary biliary cirrhosis, an immune disease of the liver that leads to scarring of the liver tissue.

A small percentage of patients with Sjogren's syndrome develop cancer of the lymph glands (lymphoma).

This usually develops after many years with the illness. Unusual gland swelling should be reported to the physician.

How is Sjogren's syndrome diagnosed?

The diagnosis of Sjogren's syndrome involves detecting the features of dryness of the eyes and mouth. The 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). More sophisticated eye testing can be done by an eye specialist (ophthalmologist).

Salivary glands can become larger and harden or become tender. Salivary gland inflammation can be detected by radiologic 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 the abnormal findings of a biopsy of salivary gland tissue.

The glands of the lower lip are often used to sample the salivary gland tissue in the diagnosis of Sjogren's syndrome.

The lower lip salivary gland biopsy procedure is easily 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 Sjogren's syndrome typically produce a myriad of extra antibodies against a variety of body tissues (autoantibodies).

These can be detected through blood testing and include antinuclear antibodies (ANAs), 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, rheumatoid factor, thyroid antibodies, and others. Low red blood count (anemia ) and abnormal blood testing for inflammation (sedimentation rate) are seen.

How is Sjogren's syndrome treated?

The treatment of patients with Sjogren's syndrome is directed toward the particular areas of the body that are involved and complications, such as infection. There is no cure for Sjogren's syndrome.

Dryness of the eyes can be helped by artificial tears, 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.

Cyclosporin eyedrops (Restasis) are recently approved medicated drops that can reduce the inflammation of the tear glands improving their function.

Signs of eye infection (conjunctivitis), such as pus or excessive redness or pain, should be evaluated by the doctor.

The dry mouth can be helped by drinking plenty of fluids, humidifying 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 treatment 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 persons 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).

Vitamin E oil has been used with some success. Infections of the mouth and teeth should be addressed as early as possible in order to avoid more severe complications. Diligent dental care is very important.

Salt water (saline) nasal sprays can help dryness in the passages of the nose. Vaginal lubricant should be considered for sexual intercourse.

Hydroxychloroquine (Plaquenil) has been helpful for some manifestations of Sjogren's syndrome. Serious complications, such as vasculitis, can require immune suppression medications, including cortisone (prednisone and others) and/or azathioprine (Imuran) or cyclophosphamide (Cytoxan).

Infections, which can complicate Sjogren's syndrome, are addressed with appropriate antibiotics. A rare complication of Sjogren's syndrome, cancer of the lymph glands (lymphoma) is treated independently.

Conclusion

Sjogren's syndrome is an area of active immunology research. Many new treatments will be available in the near future.

Sjogren's syndrome is named after a Swedish ophthalmologist, Henrik Sjogren. In the early 1900s, Sjogren 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).

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