The Lupus Group of WA

Sjogren's Syndrome

A reprint from MayoClinic.com Health Information February 2002

Sjogren's ( SHUR-grenz ) syndrome affects an estimated 1 million to 4 million people in the United States and is often defined by its two most common symptoms ‹ dry eyes and a dry mouth.
Yet Sjogren's is more than just a simple set of symptoms. It's an autoimmune disease that often accompanies rheumatoid arthritis, systemic lupus erythematosus, scleroderma or polymyositis. These rheumatic diseases are marked by inflammation of the connective tissues, and it's common for people with Sjogren's syndrome to also have a connective tissue disorder.
In Sjogren's syndrome the immune system attacks healthy tissue. The mucous membranes or moisture-secreting glands of the eyes and mouth are usually attacked first, decreasing the production of tears and saliva. This can lead to everything from difficulty swallowing and dental cavities to light-sensitive eyes and corneal ulcers. Tissues of the lungs, kidneys or liver also may be damaged.
Although you can develop Sjogren's syndrome at any age, most people receiving the diagnosis of the condition are older than 40.
The condition is also nine times more likely to occur in women than in men. Currently, there's no cure for Sjogren's syndrome, but treatments are available to relieve many of the symptoms.

Signs and symptoms
Sjogren's syndrome can be difficult to diagnose because the symptoms are similar to those caused by other diseases. The symptoms can also mimic the side effects caused by a number of medications and can vary from individual to individual. Still, watch for typical signs and symptoms, which include :

Dry eyes
Dry mouth
Dental cavities
Fatigue
Low-grade fever
Enlarged parotid glands ‹ located behind the jaw, in front of the ears
Difficulty swallowing or chewing
Oral yeast infections
Nosebleeds
Bruising
Skin rashes or dry skin
Vaginal dryness
Shortness of breath.

Causes
Sjogren's syndrome is an autoimmune disorder. This means that the body attacks its own cells and tissues. It's unknown why this happens, but researchers believe that a combination of factors cause something to go wrong with your immune system. These factors may be related to heredity, hormones or a viral infection. In the case of Sjogren's syndrome, they cause the overproduction of white blood cells called lymphocytes, which attack and damage your moisture-producing glands. They can also damage other organs, including your lungs, kidneys or liver.

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, systemic lupus erythematosus, scleroderma or polymyositis.
Being female. Women are nine times more likely than men to have Sjogren's syndrome.
Being a certain age. Sjogren's syndrome is usually diagnosed in people older than 40. It rarely occurs in children or teenagers.
Having a family history. 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 and require medical attention. If you notice such symptoms as unusually dry eyes or a dry mouth that persist for more than 3 months, seek medical advice.
How do you know if your eyes are unusually dry? If the dryness persists or if you frequently feel as though 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 daily. Important symptoms of oral dryness include daily dry mouth for more than 3 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.
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 care provider know about a symptom or combination of symptoms.
If you have a rheumatic disease such as rheumatoid arthritis or lupus, be sure to tell your doctor about any symptoms that may be related to Sjogren's. This is an important part of your ongoing disease management.

Screening and diagnosis
To diagnose Sjogren's syndrome, your doctor will most likely start by asking 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.
Your doctor may order blood tests to check your blood counts, sedimentation rate and for autoantibodies. Checking your blood count will let 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 are antibodies that attack your own tissues.
The dryness of your eyes can be measured with a Schirmer's test, in which a small piece of filter paper is placed under your lower eyelid to measure your tears. An ophthalmologist may also examine your eyes with a slit lamp after placing a drop of dye in your eyes. The dye will stain areas of the cornea that have been eroded by the dryness.
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 the parotid gland, which is located behind your jaw and in front of your ear. The dye is injected through the opening of a small duct in your mouth. This procedure reveals the flow of saliva into the mouth.
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. In addition, your doctor may want you to give a urine sample that can be analyzed in the laboratory to determine whether Sjogren's syndrome has affected your kidneys.

Complications
Most 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 and corneal ulcers.

Less common complications include :

Inflammation of organs, such as the lungs, kidneys or liver.
Heart problems for babies born to mothers with Sjogren's syndrome. If you are a woman with Sjogren's syndrome and plan to become pregnant, consult with your doctor about being tested for certain autoantibodies that may be present in your blood. These antibodies, anti-Ro ( SS-A ) and anti-La ( SS-B ), have been associated with a neonatal lupus syndrome, which can cause a rash on the baby that will clear up within a few months. In rare cases this antibody is associated with heart problems in newborns.

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 currently taking to be sure that they aren't contributing to the problem. Your doctor may also prescribe medication to help ease certain symptoms.
Medications
Medication can be helpful in treating inflammation due to Sjogren's syndrome. Anti-inflammatory medications include :

Nonsteroidal anti-inflammatory drugs ( NSAIDs ). This group of medications, which includes aspirin, helps relieve both pain and inflammation. Your doctor may recommend you take them if you have achy or swollen joints. Side effects may include indigestion and stomach bleeding. Therefore, NSAIDs should always be taken with food. Prescription NSAIDs can provide higher dosages and more potency than over-the-counter types of NSAIDs.
Corticosteroids. These medications reduce inflammation and 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. Doctors often prescribe a corticosteroid to relieve acute symptoms, with the goal of gradually tapering off the medication.
Hydroxychloroquine ( Plaquenil, Quineprox ). This is an antimalarial drug that may be useful if you have inflamed joints similar to rheumatoid arthritis. It's not usually very 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 should not be used if you have poorly controlled asthma, acute iritis, glaucoma, 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.

Self-care
Many symptoms of Sjogren's syndrome respond well to self-care. A self-care plan may include :

Artificial tears and eye drops. These can relieve the discomfort of dry eyes. Artificial tears and eyedrops are available by prescription or over the counter. Thicker drops are available that don't have to be applied as often as other drops, but they may cause visual blurring and collect along the eyelashes. Ask your doctor about whether you should select artificial tears with or without preservatives because the preservatives used to prolong the shelf life of some products can cause eye irritation in some people.
Increasing fluid intake. Drinking lots of fluids, particularly water, helps to reduce dry mouth. Sugarless gum or lemon drops also may be helpful. Because the risk of dental cavities increases with Sjogren's syndrome, it's wise to reduce sugar intake.
Moisturizers. If dry skin is a problem, avoid using very hot water to wash and avoid soaking your hands in water or cleaning solutions. Pat your skin ‹ don't rub ‹ with a towel and apply moisturizers when your skin is still damp. Use rubber gloves when doing dishes or housecleaning. Lubricants help women who experience vaginal dryness.
Smoking cessation. Avoid smoking or exposure to secondhand smoke because smoke can increase your dryness.
Increase humidity. Increasing the indoor humidity or protecting yourself in windy environments may help you reduce dryness.

In addition to developing a self-care routine, schedule regular dental appointments to protect against the increased risk of dental cavities.

Disclaimer :
The Lupus Group of WA ( Inc ) does not recommend or endorse any products, drugs, treatments, procedures, medical or health professional in this article. We suggest you discuss this information with your doctor or specialist.