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Sjogren's Syndrome

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

Yet Sjogren's syndrome is more than just a simple set of symptoms. It's a disease that often accompanies other autoimmune disorders — rheumatoid arthritis, lupus and others. These rheumatic 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, your 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. This can lead to problems from difficulty swallowing to dental cavities to light-sensitive eyes to corneal ulcers. Sjogren's syndrome can also result in damage to tissues of your lungs, kidneys and liver.

Although you can develop Sjogren's syndrome at any age, most people are older than 40 at diagnosis. The condition is nine times as likely to occur in women as in men. There's no cure for Sjogren's syndrome, but treatments can relieve many of the symptoms.

Causes:
Sjogren's syndrome is an autoimmune disorder. This means that your 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 your nervous system. In the case of Sjogren's syndrome, white blood cells called lymphocytes target, attack and damage your moisture-producing glands. They can also damage other organs, including your 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 Factor :
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.

When to seek medical advice :
Sjogren's syndrome tends to develop slowly. But once symptoms occur they can lead to complications that require medical attention. If you notice such symptoms as unusually dry eyes or a dry mouth that lasts for more than three months, seek medical advice.

How do you know if your eyes are unusually dry? If the dryness persists or if you frequently feel as if you have grit or sand in your eyes, you're probably experiencing unusually dry eyes. Or you may find you're using artificial tears more than three times a day. Important signs and symptoms of oral dryness include daily dry mouth for more than three months or recurrent or persistent swollen salivary glands. If you drink liquids to help swallow dry foods, you may also have an unusually dry mouth.

Keep your primary doctor informed
A diagnosis of Sjogren's syndrome can be missed or delayed if you report only individual symptoms to your dentist or eye doctor. Therefore, it's important to also let your primary doctor know about a symptom or combination of signs and symptoms. If you have a rheumatic disease, such as rheumatoid arthritis or lupus, tell your doctor about any symptoms that may be related to Sjogren's.

Be aware that many medications can cause dry-mouth symptoms, including drugs used to treat high blood pressure, depression and insomnia. In addition, many drugs — such as phenothiazines, tricyclic antidepressants, antispasmodics and medications used to treat Parkinson's disease — can cause symptoms of dryness throughout your body. If your medications cause you to experience symptoms associated with dryness, ask your doctor whether alternative medications are an option for you.

Symptoms :
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 include :

  • Dry eyes
  • Dry mouth
  • Dental cavities
  • Fatigue
  • Fever
  • 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
  • Irritation and mild bleeding in your nose
  • Skin rashes or dry skin
  • Vaginal dryness
  • Dry cough that doesn't produce sputum
  • Joint pain, swelling and stiffness

Diagnosis:
To diagnose Sjogren's syndrome, your doctor will most likely ask for a history of your 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. Your doctor may ask about your diet, including the types and quantities of liquids that you drink in a day.

Beyond reviewing your medical history, your doctor can use a variety of tests to diagnose Sjogren's syndrome :

  • Blood tests. Your doctor may order blood tests to check your blood counts and sedimentation rate and to check for autoantibodies. Checking your 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. Autoantibodies can play a role in the inflammatory response, which can damage your tissues and organs.
  • Tear test. Your doctor can measure the dryness of your eyes with a Schirmer tear test, in which a small piece of filter paper is placed under your lower eyelid to measure your tears. In another version of the Schirmer test, a cotton swab is used to stimulate the tear reflex in your nose. A medical eye doctor (ophthalmologist) may also examine your eyes with a slit-lamp after placing a drop of liquid containing a dye in your 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, your doctor may order a special X-ray called a sialogram. It detects dye that is injected into your parotid glands, located behind your jaw and in front of your ears. The dye is injected through the opening of a small duct in your mouth. This procedure reveals the flow of saliva into your mouth. Your 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 your salivary gland function. Your doctor may also order a chest X-ray to check for lung inflammation.
  • Biopsy. Your 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 your 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.
  • Slit-lamp exam. Your doctor may use magnifying equipment to determine how dry your eye is and whether the outside of your eye is inflamed.

Complications :
Many of the complications of Sjogren's syndrome are more annoying than serious. But serious problems can develop, especially if you have a rheumatic disease along with Sjogren's syndrome. Common complications of Sjogren's syndrome include :

  • Difficulty swallowing. Lack of saliva may make it more difficult for you to chew and swallow.
  • Dental cavities. Because saliva helps protect the teeth from bacteria, you are more prone to developing cavities if your mouth is dry.
  • Vision problems. Dry eyes can lead to light sensitivity, blurred vision and corneal ulcers.

Less common complications include :

  • Inflammation of organs such as your lungs, kidneys or liver. Inflammation may cause pneumonia, bronchitis or other problems in your lungs; may lead to problems with kidney function; and may cause hepatitis or cirrhosis in your liver.
  • Heart problems for babies born to mothers with Sjogren's syndrome. If you're a woman with Sjogren's syndrome and you plan to become pregnant, talk with your doctor about being tested for certain autoantibodies that may be present in your blood. These antibodies, anti-Ro (anti-SS-A) and anti-La (anti-SS-B), have been associated with a neonatal lupus syndrome, which can cause a rash on your baby that will clear up within a few months. In rare cases this antibody is associated with heart problems in newborns.
  • Cancer of the lymph nodes (lymphoma). A small percentage of people with Sjogren's syndrome develop lymphoma. Symptoms include swelling of the salivary glands, fatigue, weight loss and night sweats.
  • Peripheral nervous system disorders. Peripheral neuropathies are common in people with Sjogren's syndrome. Your legs may be especially affected and you may experience symptoms of numbness, tingling and burning. Your cranial nerves — such as those relating to your eyes (optic nerve) or face (trigeminal nerve) — can be affected and may cause visual problems or facial pain.
Treatment:
You can relieve many of the symptoms of Sjogren's syndrome with a self-care plan you can develop with your doctor. He or she will likely review the medications that you're taking to be sure they're not contributing to the problem. Your doctor may also prescribe medication to help ease certain symptoms.

Medications

  • Nonsteroidal anti-inflammatory drugs (NSAIDs). This group of medications, which includes aspirin, helps relieve both pain and inflammation. Your doctor may recommend you 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 over-the-counter types of NSAIDs.
  • Corticosteroids. These medications reduce inflammation and may slow joint damage. In the short term, corticosteroids can make you feel dramatically better. But when used for many months or years, they may become less effective and also cause serious side effects. Side effects may include easy bruising, thinning of your bones, cataracts, weight gain, a round face, diabetes and high blood pressure.
  • Hydroxychloroquine (Plaquenil). This antimalarial drug may be useful if you have inflamed joints, as with rheumatoid arthritis. It's not usually effective for the dryness caused by Sjogren's syndrome.
  • Pilocarpine (Salagen). Your doctor may prescribe pilocarpine if you have dry-mouth symptoms caused by Sjogren's syndrome. It's not an option if you have poorly controlled asthma, inflammation of the iris (acute iritis), glaucoma or significant cardiovascular disease, or if you're pregnant or breast-feeding. Pilocarpine may cause increased sweating and headaches.
  • Cevimeline (Evoxac). This prescription medication also is used to relieve symptoms of a dry mouth. The medication works by causing certain mouth glands to produce more saliva. Common side effects may include excessive sweating, nausea, and a runny or stuffy nose. Less common side effects, for which you should check with your doctor immediately, may include difficulty breathing, fast heartbeat and itching.
  • 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.

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). Collagen or silicone plugs are inserted into the ducts for a temporary closure. Collagen plugs eventually dissolve, but silicone plugs will keep ducts sealed until they fall out or are removed. Your doctor may use a laser to permanently seal your ducts.

 
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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