Sjogren's Syndrome and Lupus: What You Should Know

Sjogren's syndrome (pronounced SHOW-grins) is a chronic inflammatory disease that can affect many organs of the body. But it most commonly damages the body's moisture producing glands, causing dry eyes and dry mouth. Like lupus, Sjogren's is an autoimmune disease; this means that the body's immune system has run amok - instead of just protecting you from invaders, it is attacking parts of your body as if they were invaders. The parts of the body largely under attack in SS are its moisture-producing glands - such as the salivary glands that produce saliva in your mouth and the glands that produce tears in your eyes. So the most common symptoms are dry eyes and dry mouth (xerostomia). The dryness of Sjogren's is also called sicca.

Types of Sjogren's Syndrome (abbreviated SS)

SS can occur alone (known as primary SS). Or it can occur in conjunction with other autoimmune disorders, such as lupus, rheumatoid arthritis, scleroderma, dermatomyositis, or primary biliary cirrhosis (called secondary SS).

Who gets it?

Research suggests that four new cases of Sjogren's occur per 100,000 people in the population every year, and that 70% of cases are primary SS. But this is likely a low estimate because up to 38% of elderly people report having dry mouth and eyes. However, only 0.04 to 2.7% of elderly meet the full criteria for SS. In addition, Sjogren's is more common in women than in men.

About 20% of people with lupus also have SS. Because some of the symptoms of lupus and Sjogren's syndrome are similar, it can sometimes be difficult to discern which disease is causing a particular problem.

Criteria for SS

When doctors are conducting research on a disease, it's important that everyone in a research trial have disease that is defined in the same way. That's why researchers have developed criteria to define diseases. Criteria are a group of symptoms and signs - the standard by which admission to a research study is measured.

The Revised International Classification Criteria for SS requires the presence of four of the following six factors.

• Ocular (eye) symptoms - yes to one or more of these questions:
  • Have you had daily, persistent, troublesome dry eyes for more than three months?
  • Do you have a recurrent sensation of sand or gravel in the eyes
  • Do you use tear substitutes more than three times a day?
• Oral (mouth) symptoms - "yes" to one or more of these questions:
  • Have you had a feeling of dry mouth for more than three months?
  • Have you had recurrent or persistently swollen salivary glands as an adult?
  • Do you frequently drink liquids to help you swallow dry foods?
• Ocular (eye) tests
  • Positive results when your doctor gave you the Schirmer's test, which involves placing a small piece of absorptive paper into the edge of your eye and evaluating how much moisture was absorbed after five minutes.
  • Positive results when your doctor did the Rose Bengal staining test, which involves placing a bit of dye on the eye and assessing the condition of the cornea under a special lamp.
• Histopathology (study of cells and tissues)
  • Positive results of a biopsy of your salivary gland.
• Salivary gland involvement - abnormal results on one or more of the following tests:
  • Parotid scintigraphy - a type of photograph of the glands after a radiation emitting; substance has been injected into it;
  • Parotid sialography - an X-ray of the glands after dye has been injected;
  • Unstimulated salivary flow - the amount of saliva you produce.
• Autoantibodies (cells in the blood that attack your own tissue) - one or more of the following:
  • Antibodies to Ro/SSA or La/SSB;
  • Antinuclear antibodies;
  • Rheumatoid factor.

All criteria agree that you don't have Sjogren's if you already have lymphoma, graft-versus-host disease, AIDS, hepatitis C -- diseases that can cause similar symptoms.

However, remember that these are the criteria used in research studies. In clinical practice, your doctor may diagnose SS based solely on your symptoms, physical examination and blood tests without requiring invasive testing.

Symptoms of Sjogren's

The severity of SS can range from mild to quite debilitating. Some people only have mild to severe dry eyes and mouth. Others may have a wide range of symptoms including (listed in decreasing order of frequency):

• mucous-like strands in the eyes, especially in the morning, due to abnormalities in the tears;
• difficulty swallowing dry foods due to lack of saliva
• enlargement of the salivary glands;
• dryness in the nose and throat, leading to a dry cough;
• vaginal dryness;
• candida (yeast infection) of the mouth (thrush) or vagina;
• skin rashes, including purple spots;
• fatigue;
• pain in the joints (in approximately 50% of patients)
• muscular weakness or pain;
• color changes in the fingers or toes when exposed to cold or emotional stress (Raynaud's phenomenon) (in about 35% of patients)
• inflammation of blood vessels (vasculitis)- rare (in only 5% of patients)
• shortness of breath or other difficulty breathing;
• nausea, stomach pain, or indigestion due to gastritis;
• yellow eyes or skin (jaundice) or skin itching, when it coexists with liver disease;
• neurological symptoms, including abnormal sensations or movements, hearing loss, double vision, drooping on one side of the face (Bell's palsy), dizziness, or psychiatric symptoms.

The Importance of Diagnosis

Any such symptoms should be reported to your doctor promptly. It could be Sjogren's - or it could be something else. For example, eye symptoms could also be due to contact lens use, a dysfunction of your corneal nerve, inadequate vitamin A, or other problems. Dry mouth can also be caused by a wide range of drugs (such as antidepressants, antihistamines, and diuretics), other diseases (such as poorly controlled diabetes), as well as anxiety. Indeed, any symptom that "looks like" SS could have another cause - some mild and easily treatable and others very severe.

It's important that SS be diagnosed not only because you want treatment for the symptoms but also because your doctor will watch for problems associated with Sjogren's more carefully. For example, although not common, SS increases your risk for kidney, liver, thyroid, lung, and blood and blood vessel problems as well as, rarely, lymphoma. (A 1998 study suggested that only about 3% of people with Sjogren's develop lymphoma.). Despite all these risks, the overall lifespan of people with primary SS is similar to that of people without the disease.

Your dentist also needs to know if you have Sjogren's in order to participate in your therapy and provide preventive treatment. For example, people with SS are more likely to develop cavities and gum disease due to the shortage of saliva - and more likely to have their fillings fall out and their bondings fall off.

In addition, if you have surgery, your surgeon and anesthesiologist need to know about your Sjogren's. Because operating rooms have low humidity, you will need to have an eye lubricant before the procedure to reduce your risk of damage to the cornea of the eye; bring your own eye ointment with you. Because of dry mouth and dental problems, your anesthesiologist will take special protective steps too.

Treatment

More than 90% of Sjogren's patients can be treated with simple and effective measures, most of which involve self-help but some of which require a doctor's prescription or a procedure. The Moisture Seekers newsletter of the Sjogren's Syndrome Foundation can be particularly helpful; call (800) 475-6473 or visit their website www.sjogrens.com.

Your doctor will try to avoid prescribing drugs that may worsen your symptoms of dryness, and you should avoid non-prescription antihistamines. In addition, try these tips.

For dry mouth: Stimulate your existing saliva by sucking on sugarless candies or dried fruit slices, chewing sugar-free gums, or sucking citrus-flavored sugarless tablets (such as Salix SST). Take frequent small sips of water. Try using artificial saliva preparations, such as Oral Balance, Moi-Stir, Mouthkote, Optimoist, Salivart, Orex, and Salix lozenges. If you don't see them on your pharmacist's shelves, ask for a special order. If medication is necessary, your doctor may prescribe pilocarpine (brand named Salagen) although it may cause increased sweating and urination and flushing or abdominal pain in some people, or cevimeline (Evozac), although it can't be used by people with uncontrolled asthma or narrow angle glaucoma.

Whatever your dry mouth treatment, you also need meticulous dental care. Try Biotene toothpaste to help prevent mouth irritation. Ask about fluoride solutions and gels; if you develop rapidly progressing dental disease, fluoride can be applied directly to the teeth from plastic trays worn at night. Consider using an electric toothbrush, such as Sonicare, especially if you have gum disease or have joint pains that make regular brushing uncomfortable. Floss daily. Dentures should be disinfected overnight with chlorhexidine solution 0.2% to help avoid infection with candida.

For dry eyes: Use artificial tears throughout the day and an opthalmic ointment at night. To prevent air from drying your eyes, try wrap-around sunglasses or side shields can be fitted to your glasses; ask your eye doctor about Moist Eye Moisture Panels. (You can see them at http://www.eaglevis.com/products.htm.) Or try ski or swim goggles. If none of this works, your eye doctor can do a procedure called punctal occlusion in which tiny (removable) plugs are placed in the tear ducts of your eyes to slow the drainage of tears.

Try to avoid low humidity situations (air conditioning, central heating, airplanes, windy locations) or use extra eye drops in these situations. If the air in your home is dry, use of a humidifier may be helpful. Also try to avoid irritants such as dust and cigarette smoke.

For dry skin: Dry skin can be treated with moisturizing lotions and bath additives.

For vaginal dryness: Lubricants, such as K-Y Jelly, add moisture, while Replens helps retain your own moisture longer.

For systemic disease: If your symptoms go beyond your eyes and mouth, your doctor may recommend an oral drug. Depending on the severity of your SS, one or more of the following might be prescribed: hydroxychloroquine (brand named Plaquenil and particularly useful for joint pain), prednisone, methotrexate, azathioprine, cyclosporine or cyclophosphamide. Current research is also exploring investigational therapies, including: oral interferon-alpha lozenges and an intravenous drug used to treat rheumatoid arthritis called infliximab (Remicade). However, infliximab cannot be given to people who have lupus, because it might worsen their disease.

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