Sjogren's syndrome



Sjogren's syndrome

Definition

Sjogren's (SHOW-grins) syndrome is a disorder of immune system often defined by its two most common symptoms — dry eyes and a dry mouth.

Sjogren's syndrome often accompanies other autoimmune disorders — such as rheumatoid arthritis and lupus. These diseases are marked by inflammation of your connective tissues, and it's common for people with Sjogren's syndrome to also have a connective tissue disorder.

In Sjogren's syndrome, immune system attacks healthy tissue. The mucous membranes and moisture-secreting glands of your eyes and mouth are usually affected first, resulting in decreased production of tears and saliva. The disease can damage other tissues as well.

Although occur Sjogren's syndrome at any age, most people are older than 40 at diagnosis. The condition is much more common in women. There's no cure, but treatments can relieve many symptoms

Symptoms

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|[pic] |Salivary glands |

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Sjogren's syndrome can be difficult to diagnose because the signs and symptoms are similar to those caused by other diseases and can vary from person to person. In addition, the side effects of a number of medications can mimic some signs and symptoms of Sjogren's syndrome. Still, typical signs and symptoms of Sjogren's syndrome include:

• Dry eyes

• Dry mouth

• Dental cavities

• Fatigue

• Enlarged parotid glands — one particular set of your salivary glands, located behind your jaw and in front of your ears

• Difficulty swallowing or chewing

• Change in sense of taste

• Hoarseness

• Oral yeast infections, such as candidiasis

• Skin rashes or dry skin

• Vaginal dryness

• Dry cough that doesn't produce sputum

• Joint pain, swelling and stiffness

Causes

Sjogren's syndrome is an autoimmune disorder. This means that 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 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 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.

Risk factors

Although anyone can develop Sjogren's syndrome, it typically occurs in people with one or more known risk factors. These include:

• Having a rheumatic disease. It's common for people who have Sjogren's syndrome to also have a rheumatic disease, such as rheumatoid arthritis, lupus, scleroderma or polymyositis.

• Being female. Women are nine times as likely as men are to have Sjogren's syndrome.

• Being a certain age. Sjogren's syndrome is usually diagnosed in people older than 40.

• Having a family history of Sjogren's. Sjogren's syndrome sometimes runs in families

Tests and diagnosis

To diagnose Sjogren's syndrome, doctor will likely ask for a history of signs and symptoms, including what they are and how long you've had them. He or she will also want to discuss other conditions you may have and medications you're taking..

Tests to diagnose Sjogren's syndrome:

• Blood tests. blood tests to check blood count and sedimentation rate and to check for autoantibodies, proteins formed when immune system attacks your body's own cells. Checking blood count lets your doctor know the proportion of the various types of blood cells in a given volume of your blood. Sedimentation rate refers to the speed at which the red blood cells settle to the bottom of a column of blood in a glass tube. Certain inflammatory conditions increase the sedimentation rate.

• Eye tests. doctor can measure the dryness of eyes with a test called a Schirmer tear test. In this test, a small piece of filter paper is placed under lower eyelid to measure tears. In another version of the test, a cotton swab is used to stimulate the tear reflex in nose.

A (ophthalmologist) may also examine eyes with a slit lamp after placing a drop of liquid containing a dye in eye. The dye stains areas of the cornea that have been damaged by the dryness.

• Imaging. To check on the condition of your salivary glands, doctor may order a special X-ray called a sialogram. It detects dye that's injected into parotid glands, located behind jaw and in front of ears. The dye is injected through the opening of a small duct in mouth. This procedure reveals the flow of saliva into mouth.

doctor may also perform a parotid gland flow test to determine the amount of saliva that you produce over time. Another imaging test is a salivary scintigraphy, which measures salivary gland function. doctor may also order a chest X-ray to check for lung inflammation.

• Biopsy. doctor may also want to do a lip biopsy to detect the presence of clusters of inflammatory cells, which can indicate Sjogren's syndrome. For this test, a small sliver of tissue is removed from salivary glands located in lip and examined under a microscope.

• Urine sample. Your doctor may want you to provide a urine sample that can be analyzed in the laboratory to determine whether Sjogren's syndrome has affected your kidneys.

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Treatments and drugs

Medications

• Nonsteroidal anti-inflammatory drugs (NSAIDs). This group of medications, which includes aspirin, helps relieve both pain and inflammation. doctor may recommend take these medications if you have painful or swollen joints. Side effects may include indigestion and stomach bleeding. Therefore, always take NSAIDs with food. Prescription NSAIDs can provide higher doses and more potency than do over-the-counter types of NSAIDs.

ophthalmologist may recommend topical nonsteroidal eyedrops, but they need to be used with caution because they may affect cornea. doctor will want to monitor closely while you're using these eyedrops.

• Corticosteroids. These medications reduce inflammation and may slow joint damage. In the short term, corticosteroids can make dramatically better. But when used for many months or years, they may become less effective and also cause serious side effects. ophthalmologist may recommend topical corticosteroid eyedrops for short-term use.

• Hydroxychloroquine (Plaquenil). This antimalarial drug may be useful if have inflamed joints, as with rheumatoid arthritis.

• Pilocarpine (Salagen). doctor may prescribe pilocarpine if you have dry-mouth symptoms caused by Sjogren's syndrome. It's not an option if have poorly controlled asthma, inflammation of the iris (acute iritis), glaucoma or significant cardiovascular disease, or if you're pregnant or breast-feeding.

• Cevimeline (Evoxac). This prescription medication also is used to relieve symptoms of a dry mouth. It works by causing certain mouth glands to produce more saliva.

• Cyclosporine. Your ophthalmologist may recommend you use eyedrops containing cyclosporine (Restasis) to treat symptoms of Sjogren's syndrome that affect your eyes.

• Immunosuppressants. These medications, such as cyclophosphamide (Cytoxan), methotrexate (Rheumatrex), mycophenolate (CellCept) and azathioprine (Imuran), suppress the immune system. Your doctor may prescribe them for you if you develop problems with your lungs, kidneys, blood vessels or nervous system.

• Biological agents.it include antiTNF and anti CD20

Surgery

One way to relieve dry eyes is to undergo a minor surgical procedure to seal the tear ducts that drain tears from your eyes (punctal occlusion).

Experimental therapies

• One small study found that the injected medication rituximab (Rituxan) improved Sjogren's symptoms, but more research is needed to confirm these findings.

• In other clinical studies, researchers found that etanercept (Enbrel) and dehydroepiandrosterone (DHEA) weren't effective for Sjogren's.

• Early studies using oral interferon alpha initially looked promising for the treatment of Sjogren's, but the treatment isn't recommended because subsequent trials weren't able to confirm the early results.

• Epratuzumab (anti-CD22) is currently being studied for the treatment of Sjogren's syndrome.

• Drops made from a person's own serum — a blood component — have been reported to be beneficial in patients with severe dry eyes.

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