What is it?
Sjögren’s (SHOW-grens) syndrome is an autoimmune disease that primarily affects moisture-producing glands. The damage caused to these glands leaves you with dry eyes and mouth. Other parts of the body (such as the skin, respiratory system, nerves, thyroid glands and digestive tract) can be affected by the inflammation as well.
In this disease, the body’s immune system mistakenly sends its disease-fighting cells to attack the lacrimal and salivary glands, which produce tears and saliva, respectively. Rather than protecting the body as it is intended to do, the immune system damages these glands causing dry eyes (called keratoconjunctivitis sicca) and dry mouth (xerostomia).
Sjögren’s syndrome can occur in two forms:
- Primary occurs alone and is not associated with any other disease.
- Secondary occurs with rheumatic diseases such as polymyositis, rheumatoid arthritis, scleroderma and systemic lupus erythematosus.
Scientists think that Sjögren’s syndrome is caused by a combination of genetic and environmental factors. Several different genes seem to be involved, but simply having one of these genes will not cause you to develop the disease. Some environmental trigger must activate the immune system. Researchers think that the trigger may be a viral or bacterial infection. Because most people with Sjögren’s syndrome are women, it is thought that hormonal factors play some role in the disease’s initiation.
What are the effects?
Along with the main symptoms of dry eye and dry mouth, several other parts of the body can be affected by Sjögren’s syndrome.
- Eyes: Your eyes may burn and feel dry, gritty and itchy. They may appear red and can be sensitive to bright lights. Having dry eyes makes you more prone to getting eye infections.
- Mouth: Your mouth will feel dry, and you may have difficulty swallowing and speaking. Your sense of taste and smell may be altered. Because you are lacking natural saliva, which contains substances that fight bacteria and cleanse the mouth, you are susceptible to cavities and mouth infections. The fungal infection candidiasis (oral thrush) is particularly common among people with Sjögren’s syndrome and causes redness, burning and a white film in the mouth.
- Energy: Sjögren’s syndrome can cause extreme fatigue that can interfere with your daily life.
- Salivary glands: The saliva-producing glands located under your tongue, in front of your ears and near the jawline may become swollen and sometimes tender.
- Nose and throat: A dry nose and throat can cause a tickly feeling and persistent cough, leading to irritation and hoarseness.
- Skin: About half of people with Sjögren’s have dry skin. The skin may only itch, or it may also become so dry that it cracks and splits. Skin that is split or has been vigorously scratched is prone to infection. Some people with Sjögren’s (particularly when it is secondary to lupus) are sensitive to sunlight and can get sunburn from minor exposure.
- Vagina: Vaginal dryness is common in women with Sjögren’s syndrome. Painful intercourse is the most common complaint.
- Lungs: Pneumonia can be a complication of dry mouth when food becomes aspirated when swallowing is difficult or bacteria from the dry mouth can migrate to the lungs. The disease itself can cause inflammation of the bronchial tubes, windpipe and voice box.
- Kidneys: Some people with Sjögren’s syndrome have a diminished ability to concentrate urine. People may have distal renal tubular acidosis, which gives people acidic urine and can lead to calcium loss from bones. Parts of the kidney’s filtering system can be affected by this autoimmune disease, causing inflammation around the kidney’s filters (interstitial nephritis) or in the filters themselves (glomerulonephritis).
- Nerves: Peripheral nervous system involvement can cause carpal tunnel syndrome, peripheral neuropathy or cranial neuropathy. Carpal tunnel syndrome causes pain, numbness and tingling in the thumb and index and middle fingers. Peripheral neuropathy causes similar symptoms in the legs or arms. Cranial neuropathy causes face pain; loss of feeling in the face, tongue, eyes, ears or throat; and loss of taste and smell. Rarely, Sjögren’s syndrome can affect the brain and cause stroke-like symptoms.
- Digestive system: Inflammation in the esophagus, stomach, pancreas, and liver can cause painful swallowing, heartburn, abdominal pain and swelling, loss of appetite, diarrhea and weight loss. It can also cause hepatitis (inflammation of the liver) or cirrhosis (hardening of the liver).
- Connective tissues: The connective tissues of the body include the joints, muscles, bones, skin, blood vessel walls and lining of internal organs. Sjögren’s syndrome could affect any one of these on its own. In addition, several autoimmune disorders that effect the connective tissues are associated with Sjögren’s syndrome, including:
- Raynaud’s phenomenon
- Rheumatoid arthritis
- Systemic lupus erythematosus
- Thyroid disorders: Grave’s disease (overactive thyroid) or Hashimoto’s thyroiditis (underactive thyroid)
- Lymph nodes: About five percent of people with Sjögren’s develop lymphoma (cancer of the lymph nodes). Lymphoma occurring in patients with Sjögren’s syndrome often involves the salivary glands.
The main goals of treatment for Sjögren’s syndrome are to alleviate the symptoms of dry eyes and dry mouth. If other parts of your body are affected, your doctor will add other therapies as needed. One general tip for anyone with Sjögren’s syndrome is to check with your doctor to be sure that none of the medications you take for any other problems could be contributing to your dryness. You may not be able to stop taking the medication, but perhaps some other formulation will be available.
Dry eye treatments
- Artificial tears: These are available in different thicknesses, some with preservatives and some without. Preservatives may irritate the eyes.
- Eye ointments: These are thicker and longer lasting than artificial tears. Most people use them only at night as they may blur your vision.
- Hydroxypropyl methylcellulose (Lacriserts): You place these small pellets in your lower eyelid; when artificial tears are added, a film covers the eye that traps in moisture.
- Cyclosporine emulsion drops (Restasis): These drops deliver the immunosuppressant cyclosporine directly to the eye. It is thought that using this drug causes the immune reaction in the tear gland to be suppressed, resulting in increased tear production.
- Punctal plugs: Either collagen or silicone plugs are inserted into the tear ducts to prevent what tears you have from being drained away.
- Punctal occlusion: If punctal plugs work for you, the tear ducts can be surgically closed permanently.
Dry mouth treatments
- Sugar-free gum and candy: If your salivary glands are still making some saliva, you can stimulate production by chewing gum or sucking on hard candies. Be sure they are sugar-free so that you don’t contribute to cavities.
- Sips of water: Take small sips of water or other sugar-free drink throughout the day.
- Lip balm: Oil- or petroleum-based balm or lipstick can soothe dry, chapped lips.
- Saliva substitutes: Gel-based substitutes give the longest relief. Some contain fluoride, which will help prevent cavities.
- Salivary gland stimulants: Pilocarpine (Salagen) and cevimeline (Evoxac) are two medications that can stimulate the glands to produce saliva.
- Antifungals: Candidiasis (oral thrush) is a common infection in people with Sjögren’s. Antifungal medications, such as nystatin or clotrimazole, are available to treat the infection.
- Moisturizing creams: Heavy moisturizing creams and ointments should be applied three or four times per day to trap moisture in the skin.
- Sunscreen: If you are sensitive to sunlight, wear a minimum of SPF 15 sunscreen every day.
- Moisturizer: Vaginal moisturizers attract liquid to dry tissues and can be used every day.
- Lubricant: Vaginal lubricants are used only to make intercourse more comfortable, they do not moisturize.
- Skin creams: Regular skin creams and ointments can be used on the vulva (outer surfaces of the genital region).
- Estrogen: Vaginal estrogen creams may be beneficial for postmenopausal women.
- Antibiotics: Pneumonia is treated with antibiotics; your doctor will choose the appropriate one.
- Humidifier: Placing a humidifier in the rooms you use the most can alleviate bronchial irritation and inflammation.
- Corticosteroids: Steroid medications (such as prednisone) may be needed to relieve inflammation within the lungs.
Most kidney problems will not need treatment unless kidney function is affected.
- Alakaline preparations: Renal tubular acidosis is treated with oral alkaline medications that contain sodium and potassium to correct the body’s acid-base balance.
- Corticosteroids: Steroid medications (such as prednisone) may be needed to treat inflammation within the kidneys.
- Immunosuppressants: Immunosuppressants (such as hydroxychloroquine [Plaquenil], methotrexate [Rheumatrex], and cyclophosphamide [Cytoxan]) may be needed to inhibit the immune reaction occurring within the kidneys.
Nervous system treatments
- Pain medication: Pain relievers, such as acetaminophen (Tylenol, etc.) and nonsteroidal anti-inflammatory drugs (aspirin, ibuprofen, etc.) can be used to control the pain caused by carpal tunnel syndrome, peripheral neuropathy or cranial neuropathy.
- Corticosteroids: Steroid medications (such as prednisone) may be needed to treat the inflamed tissues.
- Immunosuppressants: Immunosuppressants (such as hydroxychloroquine [Plaquenil], methotrexate [Rheumatrex] and cyclophosphamide [Cytoxan]) may be needed to inhibit the immune reaction occurring within the body.
Digestive system treatments
Treatment varies depending on the digestive problems you have, but may include the same as those used to treat the nervous system.
Connective tissue treatments
The connective tissue diseases [link] associated with Sjögren’s syndrome are treated as they would be if they were occurring by themselves.