Sjögren’s Syndrome and Your Body
This autoimmune condition, which primarily affects the eyes and mouth, can involve other organs and systems.
Most people, if they’re familiar with Sjögren’s syndrome at all, think of it as a disease that makes the mouth dry and the eyes gritty. That’s true, but it can affect the skin, mucous membranes, circulatory and nervous systems, as well as the heart, liver and kidneys. Here’s a look at what Sjögren’s can do to you – and what you can do to fight back.
Dry mouth can be more than uncomfortable. Without the protection saliva offers, you may experience tooth decay, mouth pain, oral infections and difficulty swallowing.
Treatment: Many people get the relief they need from artificial saliva products, which are available over the counter under various trade names including Glandosane, MoiStir, MouthKote, Optimoist, Oralube, Salivart and Xero-Lube, says Robert I. Fox, MD, a leading Sjögren's expert and practicing rheumatologist at Scripps Memorial Hospital in La Jolla, Calif.
Also helpful are toothpastes and mouth rinses developed specially for dry mouth. These include:
- Biotene Dry Mouth Toothpaste and Biotene Mouthwash with Calcium
- CloSYS Toothpaste (formerly Retardent) and CloSYS Oral Rinse (formerly RetarDEX Oral Rinse)
- Orajel Dry Mouth Moisturizing Toothpaste
- If replacing moisture doesn’t relieve dryness, your doctor may prescribe a medication to stimulate your own salivary glands to produce more saliva. Two such drugs, pilocarpine (Salagen) and cevimeline (Evoxac), are approved for dry mouth associated with Sjögren’s syndrome.
Tips for relieving a dry mouth.
- Keep a water bottle with you and sip throughout the day.
- Suck on sugar-free candy or lozenges or chew sugar-free gum.
- If possible, avoid using drugs such as antihistamines and antidepressants that cause dryness.
- Avoid mouthwashes with alcohol which can worsen dryness.
For an inexpensive option to oral lubricants that is available in almost any pharmacy or discount store, Dr. Fox recommends vaginal lubricants. Although they aren't marketed for oral use, the FDA requires safety testing for vaginal lubricants and the risk of side effects is small, he says.
Like dry mouth, dry eyes can cause more than discomfort. Untreated dryness can damage the eye's surface, increase the risk of infection and even impair vision.
Treatment: If you suffer from dry eyes, artificial tears may help restore moisture. Many are available over the counter under trade names including Artificial Tears, Bion Tears, Gonak, Isopto Tears, Lacril, Nature’s Tears, Ocucoat Refresh and Systane. If dryness is still a problem, your doctor may prescribe the immunosuppressive drug cyclosporine in the form of an eye drop (Restasis) to be applied every 12 hours or a cellulose pellet (Lacrisert) that is placed in the lower eye lid where it dissolves, adding moisture, when artificial tears are used.
Two drugs approved for dry mouth, pilocarpine (Salagen) and cevimeline (Evoxac), may also be useful for dry eyes. While they haven’t been approved for dry eyes, your doctor may prescribe them for that purpose. Also, if you take one of these drugs for dry mouth, it may help your eyes as well.
In severe cases, your doctor may consider an outpatient procedure called punctal occlusion, which involves cauterizing the puncta (small openings at the inner corners of the eyelids where tears drain from the eyes) to close them, keeping your eye's natural tears on their surface longer. Another option is to temporarily close the puncta with a collagen plug.
Tips for easing dry eyes:
- Avoid sitting next to air conditioners or heaters. Adjust the fans in your car so that air doesn't blow directly on your face.
- Use a humidifier in your bedroom at night.
- Make a conscious effort to blink throughout the day, especially if you work at computer.
- Take a break during the day to close your eyes and relax. Cover your eyes with a warm washcloth.
- Eat more fish. Research shows that omega-3 fatty acids in coldwater fish eases dry eyes.
More than helping food go down the throat, saliva helps neutralize the acidity of gastric acids once it gets there. When saliva is lacking, you may experience heartburn. Taking nonsteroidal anti-inflammatory drugs (NSAIDs) to relieve other Sjögren's symptoms can worsen the problem.
Treatment: Medications that can help include:
- sucralfate (Carafate) – an ulcer medication that coats and protects the esophagus and stomach.
- H2 blockers, also called H2-receptor antagonists – medications that reduce the production of gastric acid by blocking histamine2. H2 blockers include cimetidine (Tagamet), famotidine (Pepcid) and ranitidine (Zantac).
- Proton pump inhibitors – a group of medications that decrease the amount of acid in the stomach and intestines. They include omeprazole (Prilosec), esomeprazole (Nexium), rabeprazole (Aciphex), lansoprazole (Prevacid) and pantoprazole (Protonix).
Tips for relieving heartburn:
- Eat smaller, more frequent meals.
- Make a note of foods that tend to worsen your heartburn and avoid those when possible. Common culprits include fatty foods, citrus fruits and juices, chocolate and caffeine.
- Avoid eating for at least two hours before going to bed at night, so that the stomach has a chance to empty and acid levels decrease before you lie down.
- Elevate the head of your bed on two-inch blocks to keep gastric acid from washing back into your esophagus while you sleep.
Upper Respiratory System
In healthy people, nasal secretions keep nasal passages moist, helping to protect against infection. If your nasal passages are dry, you may feel that you’re constantly battling sinusitis and post nasal drip. While Dr. Fox says his Sjögren’s patients don’t get sinus infections more frequently than other people, he has found that their infections tend to last longer and have a higher chance of persisting with postnasal drip and cough or developing into bronchitis or pneumonia.
Treatment: Treatment for problems associated with dry nasal passages includes:
- saline nasal sprays such as Ocean spray.
- lavage of nasal passages with a saline solution.
- decongestants including loratadine (Claritin) and desloratadine (Clarinex), fexofenadine (Allegra) and cetirizine (Zyrtec).
- antibiotics, including the sulfa-containing drug sulfamethoxazole/trimethoprim (Bactrim DS, Septra); the penicillin drug amoxicillin and clavulanate (Augmentin); the macrolide antibiotics azithromycin (Zithromax) and clarithromycin (Biaxin); the cephlasporins ceftibuten (Cedax) and cephalexin (Keflex); and flouroquinolone (quinolones) antibiotics ciprofloxacin (Cipro), levofloxacin (Levoquin), moxifloxacin (Avelox) and gatifloxacin (Tequin).
- steroid sprays including Flonase, Beconase and Nasonex.
- expectorants such as alkalol (a saline solution that includes a mix of menthol, camphor, eucalyptol, thymol, and oils of wintergreen, spearmint, pine, and cinnamon) and guaifenesin (Humabid-LA).
Tips for moisturizing and protecting dry nasal passages:
- Use a humidifier or vaporizer.
- Make and use your own saltwater spray by mixing one teaspoon salt to one quart of deionized water and boiling to fully dissolve the salt.
- Drink plenty of fluids to thin your mucus.
- Avoid drinking alcohol, which can dry out your nasal passages.
- Avoid chlorinated swimming pools, because chlorine can irritate nasal passages.
- Apply warm, wet towels to your face.
Dry skin that cracks and itches is common with Sjögren's syndrome. The lips are also frequently affected. In some people, Sjögren's is associated with skin rashes. The most common appear initially as areas of dark blotches on the legs and feet that grow together.
Treatment: Treatment for dry skin typically consists of topical creams and lotions. If the corners of your lips crack, it could be due to a yeast infection. Treatment with a topical antifungal such as econazole nitrate (Spectazole) or ciclopirox (Loprox) may needed to clear the infection.
Tips for moisturizing skin:
- Limit showers to five minutes, because water removes the skin's natural oils.
- When drying the skin with a towel, pat, don’t rub.
- Apply creams and lotions after a skin or bath when your skin is still moist.
- For between-shower application, dampen your skin with a wet towel before applying lotion.
- Use a humidifier in your bedroom at night to help moisten your skin.
Vaginal dryness can make intercourse painful and interfere with sexual relationships.
Treatment: Vaginal lubricants and moisturizers can restore moisture and make intercourse easier. There are many different vaginal lubricants available over the counter as gels, sprays and inserts under a variety of trade names, including Astroglide, KY Jelly, Lubrin, Maxilube, Replens and Surgilube. Avoid petroleum-based ointments such as petroleum jelly.
Tips for moisturizing intimate areas:
- Use vaginal moisturizers and lubricants.
- Avoid scented body washes or bubble baths that can irritate the inside of the vagina.
- Prolong foreplay so that your vagina has more time for natural lubrication.
Sjögren's syndrome may be associated with joint pain and inflammation itself or it may accompany an inflammatory form of arthritis, such as rheumatoid arthritis or lupus.
Treatment: Treatment for joint inflammation typically consists of nonsteroidal anti-inflammatory drugs (NSAIDs) such as iburopfen (Motrin) or naproxen (Anaprox, Aleve). For more severe inflammation, your doctor may prescribe a corticosteroid medication such as prednisone, which mimics natural substances that control immune response, or a disease-modifying anti-rheumatic drug (DMARD) such as hydroxychloroquine (Plaquenil), methotrexate (Rheumatrex) or leflunomide (Arava) to actually inhibit the body’s immune response.
Tips for treating painful joints:
- Apply heat or cold (or alternate the two) to painful joints with warm washcloths or a bag of ice or frozen vegetables wrapped in a towel.
- Speak to your doctor or physical therapist about exercises to keep joints mobile, relieve stiffness and improve range of motion.
- Rest painful or swollen joints – use assistive devices to spare joints that are inflamed.
In some people with Sjögren's syndrome, a bout with the flu or sinus infection can leave dried mucus that obstructs the upper airways, causing a dry cough and potentially leading to pneumonia.
Sometimes the inflammatory process of Sjögren's affects the walls of tiny balloon-like airs sacs (alveoli) in the lungs, where oxygen is added to your blood and the waste product carbon dioxide is removed. This inflammation can cause the interstitium, the tissue that lines and supports the sacs, to thicken and scar – a problem known as interstitial pneumonitis or interstitial lung disease. This thickening makes it more difficult for oxygen to enter bloodstream, causing shortness of breath. Interstitial pneumonitis may not show up on a routine chest X-ray. If your doctor suspects this problem, she may order a high-resolution CT scan to look for inflammation.
Treatment: Staying well hydrated after an upper respiratory infection may help prevent the development of pneumonia as may sinus lavage, a procedure that uses salt water to remove mucus and debris from the nasal passages and sinuses. While scarring of the lungs cannot be reversed, medical treatment may help prevent it from progressing. Treatment may consist of a combination of medications, including corticosteroids; DMARDs such as azathioprine (Imuran) or cyclophosphamide (Cytoxan); the mucus-thinning medication acetylcysteine; and anti-fibrotics (anti-scarring) medications such as bosentan (Tracleer) and pirfenidone. Oxygen therapy may make breathing less difficult. Pulmonary rehabilitation may help you live better by teaching you exercised to improve breathing and how to breathe more efficiently.
Tips for managing an upper respiratory infection:
- If you are sick with an upper respiratory infection, drink plenty of fluids to stay well hydrated.
- Use a humidifier or vaporizer in your room.
- Use a salt-water solution to relieve nasal congestion.
The inflammation that accompanies Sjögren's syndrome can affect the nervous system, resulting in problems such as peripheral neuropathy, damage to the nerves in the arms in legs, causing pain, numbness and weakness; carpal tunnel syndrome, a condition in which inflamed tissue in the wrist area compresses the median nerve that runs from the arm to the hand causing pain, numbness and tingling on the thumb side of the hand; and cranial neuropathy, a condition that causes pain or loss of feeling in the face, and loss of taste and smell. In some cases, the brain itself is affected, leading to blood clots and lesions similar to those seen in multiple sclerosis.
Treatment: Corticosteroids may be used to treat nerve inflammation. Doctors may prescribe analgesics to relieve pain.
Two problems with the kidneys, interstitial nephritis or glomerulonephritis, can occur with Sjögren's syndrome. Interstitial nephritis is inflammation of the tissue around the kidney's filters (tubules). In some people with Sjögren's this problem exists in a latent form, perhaps even before they experience dry eyes and mouth. They don't experience symptoms until exposure to certain toxins or medications (including NSAIDs) causes kidney function to deteriorate. Glomerulonephritis, inflammation of the tubules themselves, is a condition in which kidneys leak protein into the urine.
Treatment: Unless these problems start to affect kidney function, your doctor may now propose treatment. When necessary, treatment may consist of alkaline agents to maintain the balance of blood chemicals normally handled by the kidneys and corticosteroids and DMARDs to control inflammation.
While it's important to be aware that Sjögren’s can affect your entire body, Dr. Fox stresses that having Sjögren’s syndrome doesn’t mean you can't – or won't – have any of the medical problems other people do. Just because you experience a symptom, you can't assume it's caused by Sjögren's.
The best course of action is to see a doctor who can help determine the cause of troublesome symptoms and the best treatment for them.