Sjogren's (Sjögren's, pronounced SHOW-grins) syndrome is an autoimmune disease in which the immune system targets moisture-producing glands and causes dryness in the mouth and eyes. Other parts of the body can be affected as well, resulting in a wide range of possible symptoms.
You might hear Sjogren's syndrome referred to as rheumatic disease. A rheumatic disease causes inflammation in joints, muscles, skin or other body tissue, and Sjogren's can do that. The many forms of arthritis, which often involve inflammation in the joints among other problems, are examples of rheumatic diseases. Sjogren's is also considered a disorder of connective tissue, which is the framework of the body that supports organs and tissues (joints, muscles and skin).
Primary versus Secondary Sjogren's Syndrome
Sjogren's syndrome is classified as either primary or secondary. Primary Sjogren's occurs by itself, and secondary Sjogren's occurs with another disease. Both are systemic disorders, although the symptoms in the primary form are more restricted.
In cases of primary Sjogren's syndrome, the doctor can trace the symptoms to problems with the tear and saliva glands. These patients are likely to have different antibodies circulating in their blood compared to people with the secondary form. These antibodies are called SS-A and SS-B; people with primary Sjogren's are also more likely to have antinuclear antibodies (ANAs) in their blood. ANAs are autoantibodies that are directed against the body.
In cases of secondary Sjogren's syndrome, the patient will have had an autoimmune disease such as rheumatoid arthritis or lupus before Sjogren's developed. People with this type tend to have more health problems because they have two diseases, and they are also less likely to have the antibodies associated with primary Sjogren's.
Parts of the Body Affected
The autoimmune response that causes dry eyes and mouth can cause inflammation throughout the body. People with Sjogren's often have skin, lung, kidney, and nerve problems, as well as disorders of the digestive system and connective tissue. Following are examples of extraglandular problems.
Skin Problems. About half of the people who have Sjogren's have dry skin. Some experience only itching, but it can be severe. Others develop cracked, split skin that can easily become infected. Infection is a risk for people with itchy skin, too, particularly if they scratch vigorously. The skin may darken in infected areas, but it returns to normal when the infection clears up and the scratching stops.
Some patients who have Sjogren's, particularly those who have lupus, are sensitive to sunlight and can get painful burns from even a little sun exposure, such as through a window. So, if you're sensitive to sunlight, you need to wear sunscreen (at least SPF 15) whenever you go outdoors and try to avoid being in the sun for long periods of time.
Vaginal Dryness. Vaginal dryness is common in women with Sjogren's syndrome. Painful intercourse is the most common complaint. A vaginal moisturizer helps retain moisture, and a vaginal lubricant can make intercourse more comfortable. Vaginal moisturizers attract liquid to the dry tissues and are designed for regular use. Vaginal lubricants should be used only for intercourse – they don't moisturize. Oil-based lubricants, such as petroleum jelly, trap moisture and can cause sores and hinder the vagina's natural cleaning process. A water-soluble lubricant is better.
Regular skin creams and ointments relieve dry skin on the outer surface of the vagina (the vulva).
Lung Problems. Dry mouth can cause lung problems. For example, aspiration pneumonia can happen when a person breathes in food instead of swallowing it (dry mouth can keep you from swallowing food properly), and the food gets stuck in the lungs. Pneumonia can also develop when bacteria in the mouth migrate into the lungs and cause infection, or when bacteria get into the lungs and coughing doesn't remove them. (Some people with Sjogren's don't produce enough mucus in the lungs to remove bacteria, and others are too weak to be able to cough.) Pneumonia is treated with various antibiotics, depending on the person and the type of infection. It is important to get treatment for pneumonia to prevent lung abscess (a hole in the lung caused by severe infection).
People with Sjogren's also tend to have lung problems caused by inflammation, such as bronchitis (affecting the bronchial tubes), tracheobronchitis (affecting the windpipe and bronchial tubes), and laryngotracheobronchitis (affecting the voice box, windpipe, and bronchial tubes). Depending on your condition, the doctor may recommend using a humidifier, taking medicines to open the bronchial tubes, or taking corticosteroids to relieve inflammation. Pleurisy is inflammation of the lining of the lungs and is treated with corticosteroids and nonsteroidal antiinflammatory drugs.
It is believed that some 1 to 4 million people have the disease; most (90%) are women. It can occur at any age, but it usually is diagnosed after age 40 and can affect people of all races and ethnic backgrounds. It is rare in children, but can occur.
The disease-fighting cells of the immune system attack the glands that produce tears and saliva (the lacrimal and salivary glands). The disease can affect other glands too, such as those in the stomach, pancreas and intestines, and can cause dryness in other places that need moisture, such as the nose, throat, airways, vagina and skin.
Researchers believe Sjogren's syndrome is caused by a combination of genetic and environmental factors. Having one of the associated genes will not cause a person to develop the disease without some sort of trigger to activate the immune system.
It is thought that the trigger may be a viral or bacterial infection, and may work like this: A person who has a Sjogren's-associated gene gets a viral infection. The virus stimulates the immune system to act, but the gene alters the attack, sending fighter cells (lymphocytes) to the eye and mouth glands. Once there, the lymphocytes attack healthy cells, causing the inflammation that damages the glands and keeps them from working properly. These fighter cells are supposed to die after their attack in a natural process called apoptosis, but in people with Sjogren's syndrome, they continue to attack, causing further damage. The possibility that the endocrine and nervous systems play a role is also under investigation.
In the autoimmune attack that causes Sjogren's, disease-fighting cells called lymphocytes target the glands that produce moisture – primarily the lacrimal (tear) and salivary (saliva) glands. Although no one knows exactly how damage occurs, damaged glands can no longer produce tears and saliva, and eye and mouth dryness result. When the skin, sinuses, airways, and vaginal tissues are affected, dryness occurs in those places, too.
The main symptoms are:
When Sjögren's affects other parts of the body, the condition is called extraglandular involvement because the problems extend beyond the tear and salivary glands.
Finally, Sjogren's can cause extreme fatigue that can seriously interfere with daily life.
The doctor will first take a detailed medical history, which includes asking questions about general health, symptoms, family medical history, alcohol consumption, smoking, or use of drugs or medications. The doctor will also do a complete physical exam to check for other signs of Sjogren's.
There may be some tests, too. First, the doctor will want to check the eyes and mouth to see whether Sjogren's is causing the symptoms and how severe the problem is. Because there are many possible causes of dry eyes and dry mouth, the doctor will take these into account.
One is generally considered to have definite Sjogren's if one has dry eyes, dry mouth, and a positive lip biopsy. The doctor may decide to perform additional tests to see whether other parts of the body are affected, and whether various antibodies are found in the blood.
Common eye and mouth tests are:
These tests may include:
Immunological tests – These blood tests check for antibodies commonly found in the blood of people with Sjogren's syndrome. For example:
General Tips for Eye Care:
General Tips for Dry Mouth
If your salivary glands still produce some saliva, you can stimulate them to make more by chewing gum or sucking on hard candy. However, gum and candy must be sugar free because dry mouth makes you extremely prone to cavities. Take sips of water or another sugar free drink often throughout the day to wet your mouth, especially when you are eating or talking. Note that you should take sips of water – drinking large amounts of liquid throughout the day will not make your mouth any less dry. It will only make you urinate more often and may strip your mouth of mucus, causing even more dryness. You can soothe dry, cracked lips by using oil- or petroleum-based lip balm or lipstick. If your mouth hurts, the doctor may give you medicine in a mouth rinse, ointment, or gel to apply to the sore areas to control pain and inflammation.
If you produce very little saliva or none at all, your doctor might recommend a saliva substitute. These products mimic some of the properties of saliva, which means they make the mouth feel wet, and if they contain fluoride, they can help prevent cavities. Gel-based saliva substitutes tend to give the longest relief, but all saliva products are limited since you eventually swallow them.
At least two drugs that stimulate the salivary glands to produce saliva are available. These are pilocarpine and cevimeline. The effects last for a few hours, and you can take them three or four times a day. However, they are not suitable for everyone, so talk to your doctor about whether they might help you.
People with dry mouth can easily get mouth infections. Candidiasis, a fungal mouth infection, is one of the most commonly seen in people with Sjogren's. It most often shows up as white patches inside the mouth that you can scrape off, or as red, burning areas in the mouth. Candidiasis is treated with antifungal drugs. Various viruses and bacteria can also cause infections; they're treated with the appropriate antiviral or antibiotic medicines.
Oral Hygiene is Important
Natural saliva contains substances that rid the mouth of the bacteria that cause cavities and mouth infections, so good oral hygiene is extremely important when you have dry mouth. Here's what you can do to prevent cavities and infections:
To treat dry skin, apply heavy moisturizing creams and ointments three or four times a day to trap moisture in the skin. Lotions, which are lighter than creams and ointments, aren't recommended because they evaporate quickly and can contribute to dry skin. Also, doctors suggest that you take only a short shower (less than 5 minutes), use a moisturizing soap, pat your skin almost dry, and then cover it with a cream or ointment. If you take baths, it's a good idea to soak for 10 to 15 minutes to give your skin time to absorb moisture. Having a humidifier in the bedroom can help hydrate your skin, too. If these steps don't help the itching, your doctor might recommend that you use a skin cream or ointment containing steroids.
Protect Your Voice. People with Sjögren's can develop hoarseness if their vocal cords become inflamed as part of the disease or become irritated from throat dryness or coughing. To prevent further strain on your vocal cords, try not to clear your throat before speaking. Instead, take a sip of water, chew gum, or suck on candy. Or else make an "h" sound, hum, or laugh to gently bring the vocal cords together so you can get sound out. Clearing your throat does the same thing, but it's hard on the vocal cords, and you want to avoid irritating them further.
Kidney Problems. The kidneys filter waste products from the blood and remove them from the body through urine. The most common kidney problem in people with Sjogren's is interstitial nephritis, or inflammation of the tissue around the kidney's filters, which can occur even before dry eyes and dry mouth. Inflammation of the filters themselves, called glomerulonephritis, is less common. Some people develop renal tubular acidosis, which means they can't get rid of certain acids through urine. The amount of potassium in their blood drops, causing an imbalance in blood chemicals that can affect the heart, muscles, and nerves.
Often, doctors do not treat these problems unless they start to affect kidney function or cause other health problems. However, they keep a close eye on the problem through regular exams, and will prescribe medicines called alkaline agents to balance blood chemicals when necessary. Corticosteroids or immunosuppressants are used to treat more severe cases.
Nerve Problems. People with Sjogren's syndrome can have nerve problems. When they do, the problem usually involves the peripheral nervous system (PNS), which contains the nerves that control sensation and movement. Involvement of the PNS is increasingly being recognized. Carpal tunnel syndrome, peripheral neuropathy, and cranial neuropathy are examples of peripheral nervous system disorders that occur in people with Sjogrens. In carpal tunnel syndrome, inflamed tissue in the forearm presses against the median nerve, causing pain, numbness, tingling, and sometimes muscle weakness in the thumb and index and middle fingers. In peripheral neuropathy, an immune attack damages nerves in the legs or arms, causing the same symptoms there. (Sometimes nerves are damaged because inflamed blood vessels cut off their blood supply.) In cranial neuropathy, nerve damage causes face pain; loss of feeling in the face, tongue, eyes, ears, or throat; and loss of taste and smell.
Digestive Problems. Inflammation in the esophagus, stomach, pancreas, and liver can cause problems like painful swallowing, heartburn, abdominal pain and swelling, loss of appetite, diarrhea, and weight loss. It can also cause hepatitis (inflammation of the liver) and cirrhosis (hardening of the liver). Sjogren's is closely linked to a liver disease called primary biliary cirrhosis (PBC), which causes itching, fatigue, and, eventually, cirrhosis. Many patients with PBC have Sjogren's.
Treatment varies, depending on the problem, but may include pain medicine, anti-inflammatory drugs, steroids, and immunosuppressants.
Connective Tissue Disorders. Connective tissue is the framework of the body that supports organs and tissues. Examples are joints, muscles, bones, skin, blood vessel walls, and the lining of internal organs. Many connective tissue disorders are autoimmune diseases, and several are common among people with Sjogren's:
In cases of Sjogren's syndrome, the body's immune system mistakenly attacks tear ducts and saliva glands, leading to chronically dry eyes and dry mouth (xerostomia).
Sjogren's Syndrome can result in painful mouth sores due to a dry mouth and/or Candida infection.
Many Celiac Disease patients report they also have Sjogren's Syndrome, and vice versa. Sjogren's Syndrome has been reported in up to 15% of patients with proven Celiac Disease.
October, 2014: Australian researchers published their research findings in the journal Immunity that chronic cytomegalovirus (CMV) infection can lead to the development of Sjogren's syndrome, the second-most common autoimmune disease. Some 50-80% of people in developed countries are infected with CMV, which is usually not a problem, but combined with a certain genetic background, chronic CMV can trigger autoimmunity.
Sjogren's is an autoimmune disease sometimes misdiagnosed as retinitis pigmentosa. [Am J Ophthalmol, 1996 Dec, 122:6, pp.903-5 Abstract]
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