Sjogren syndrome

Other Names: dacryosialoadenopathia atrophicans Gougerot-Houwer-Sjogren syndrome Gougerot-Sjogren syndrome keratoconjunctivitis sicca keratoconjunctivitis sicca-xerostomia secreto-inhibitor-xerodermostenosis sicca syndrome Sjogren-Gougerot syndrome Sjogren's syndrome

Sjögren syndrome is an autoimmune disorder in which immune cells attack and destroy the glands that produce tears and saliva. Sjögren syndrome is also associated with rheumatic disorders such as rheumatoid arthritis or systemic lupus erythematosus. Some individuals who are first diagnosed with another rheumatic disorder, such as rheumatoid arthritis or systemic lupus erythematosus, later develop the dry eyes and dry mouth characteristic of Sjögren syndrome. Other autoimmune disorders can also develop after the onset of Sjögren syndrome. In all, about half of all individuals with Sjögren syndrome also have another autoimmune disorder

Characteristic dryness appears at multiple locations like the tongue, face, and eyes.

Cause

Sjögren syndrome is thought to result from a combination of genetic and environmental factors; however, no associations between specific genetic changes and the development of Sjögren syndrome have been confirmed. Researchers believe that variations in many genes affect the risk of developing Sjögren syndrome, but that development of the condition may be triggered by something in the environment. In particular, viral or bacterial infections, which activate the immune system, may have the potential to encourage the development of Sjögren syndrome in susceptible individuals. The genetic variations that increase susceptibility may reduce the body's ability to turn off the immune response when it is no longer needed.

Inheritance

A predisposition to develop autoimmune disorders can be passed through generations in families. Relatives of people with Sjögren syndrome are at an increased risk of developing autoimmune diseases, although they are not necessarily more likely to develop Sjögren syndrome in particular. The inheritance pattern of this predisposition is unknown.

Signs and symptoms

The hallmark symptoms of Sjögren syndrome are dry mouth and dry eyes. In addition, Sjogren syndrome may cause skin, nose, and vaginal dryness. It also may affect other organs of the body including the kidneys, blood vessels, lungs, liver, pancreas, and brain. The main symptoms of Sjögren's syndrome are:

• Dry mouth. Your tongue and your throat will feel dry and chewing and swallowing may be difficult or painful.
• Dry eyes. Your eyes may burn or itch or feel as if you have a piece of sand in your eye. Vision may be blurry or you could be bothered by bright light.

Sjögren's syndrome can also affect other parts of the body, causing symptoms such as: Joint and muscle pain

• Dry skin
• Rashes on the skin of hands or feet
• Numbness or tingling in the hands or feet
• Vaginal dryness
• Dry cough that doesn't go away
• Fatigue that doesn't go away
• If you have Sjögren's syndrome you may only have mild symptoms of dry eyes and mouth, or you could experience cycles of mild symptoms followed by more severe symptoms.

Diagnosis

Doctors diagnose Sjögren’s using:

• Medical history.
• Physical exam.
• Certain eye and mouth tests.
• Blood tests.

Doctors may also order:

• A urine test.
• A chest x-ray.

Blood tests can be done to determine if a patient has high levels of antibodies that are indicative of the condition, such as antinuclear antibody (ANA) and rheumatoid factor (because SS frequently occurs secondary to rheumatoid arthritis), which are associated with autoimmune diseases. Typical SS ANA patterns are SSA/Ro and SSB/La, of which Anti-SSB/La is far more specific; Anti-SSA/Ro is associated with numerous other autoimmune conditions, but are often present in SS. However, Anti-SSA and Anti-SSB tests are frequently not positive in SS.

A radiological procedure is available as a reliable and accurate test for SS. A contrast agent is injected into the parotid duct, which opens from the cheek into the vestibule of the mouth opposite the neck of the upper second molar tooth. Histopathology studies should show focal lymphocytic sialadenitis. Objective evidence of salivary gland involvement is tested through ultrasound examinations, the level of unstimulated whole salivary flow, a parotid sialography or salivary scintigraphy, and autoantibodies against Ro (SSA) and/or La (SSB) antigens.

Treatment

Treatment differs for each person and depends on what parts of your body are affected. Treatment will focus on getting rid of symptoms and may include:

• Medicines for joint or muscle pain (such as aspirin and ibuprofen).
• Medicines that help you make more saliva.
• Medicines that suppress inflammation (such as corticosteroids).
• Medicines that suppress the immune system.

Treatment for dry eyes may include:

• Eye drops that come in different thicknesses. You may have to try a few to find the right one.
• Eye ointments. These are thicker than eye drops. They protect the eyes and keep them wet for several hours. They can blur your vision, so you may want to use them while you sleep.
• Medicines to reduce inflammation in the eye.
• A chemical that wets the surface of the eye and keeps the natural tears from drying out so fast. It comes in a small pellet that you put in your lower eyelid. When you add eye drops, the pellet melts. This forms a film over your own tears and traps the moisture.
• Surgery to shut the tear ducts that drain tears from the eye.

Treatment for dry mouth may include: Chewing gum or sucking on hard candy helps your glands make more saliva. Use sugar-free gum and candy.

• Sipping water or a sugar-free drink often to keep your mouth wet.
• Using oil or petroleum-based lip balm or lipstick to help dry, cracked lips feel better.
• Using a saliva substitute prescribed by a doctor to make the mouth feel wet.
• Using medicine to help your mouth make more saliva.

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.

• Pilocarpine HCl (Brand name: Salagen®)Treatment of dry mouth in patients with Sjogren's syndrome.

Prognosis

Most people with Sjogren syndrome have limited symptoms such as dry eyes and dry mouth. Their general health and life expectancy are largely unaffected. Increased mortality may be related to conditions associated with Sjogren syndrome, such as systemic lupus erythematous (SLE), rheumatoid arthritis (RA), or primary biliary cirrhosis. People with Sjogren syndrome who don't develop a lymphoproliferative disorder can generally expect to have a normal life span.

Epidemiology

Sjögren syndrome is a relatively common disorder; it occurs in 0.1 to 4 percent of the population. It is difficult to determine the exact prevalence because the characteristic features of this disorder, dry eyes and dry mouth, can also be caused by many other conditions. Women develop Sjögren syndrome about 10 times more often than men; the specific reason for this difference is unknown but likely involves the effects of sex hormones on immune system function.

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