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
response that causes dry eyes and mouth can cause inflammation throughout the body. People with Sjogren's often have skin, lung
, 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
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
Incidence; Causes and Development
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
) 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.
Signs and Symptoms
The main symptoms are:
- Dry eyes – Your eyes may be red and burn and itch. People say it feels like they have sand in their eyes. Also, your vision may be blurry, and bright light, especially fluorescent lighting, might bother you.
- Dry mouth – Dry mouth feels like a mouth full of cotton. It's difficult to swallow, speak, and taste. Your sense of smell can change, and you may develop a dry cough. Also, because you lack the protective effects of saliva, dry mouth increases your chances of developing cavities and mouth infections.
Both primary and secondary Sjogren's syndrome can affect other parts of the body as well, including the skin, joints, lungs
, blood vessels, and nervous system
, and cause symptoms such as:
- Dry skin
- Skin rashes
- Thyroid problems
- Joint and muscle pain
- Vaginal dryness
- Numbness and tingling in the extremities
When Sjögren's affects other parts of the body, the condition is called extraglandular involvement because the problems extend beyond the tear and salivary
Finally, Sjogren's can cause extreme fatigue
that can seriously interfere with daily life.
Diagnosis and Tests
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:
- Schirmer test – This test measures tears to see how the lacrimal gland is working. It can be done in two ways: In Schirmer I, the doctor puts thin paper strips under the lower eyelids and measures the amount of wetness on the paper after 5 minutes. People with Sjogren's usually produce less than 8 millimeters of tears. The Schirmer II test is similar, but the doctor uses a cotton swab to stimulate a tear reflex inside the nose.
- Staining with vital dyes (rose bengal or lissamine green) – The tests show how much damage dryness has done to the surface of the eye. The doctor puts a drop of a liquid containing a dye into the lower eye lid. These drops stain on the surface of the eye, highlighting any areas of injury.
- Slit lamp examination – This test shows how severe the dryness is and whether the outside of the eye is inflamed. An ophthalmologist (eye specialist) uses equipment that magnifies to carefully examine the eye.
- Mouth exam – The doctor will look in the mouth for signs of dryness and to see whether any of the major salivary glands are swollen. Signs of dryness include a dry, sticky mouth; cavities; thick saliva, or none at all; a smooth look to the tongue; redness in the mouth; dry, cracked lips; and sores at the corners of the mouth. The doctor might also try to get a sample of saliva to see how much the glands are producing and to check its quality.
- Salivary gland biopsy of the lip – This test is the best way to find out whether dry mouth is caused by Sjogren's syndrome. The doctor removes tiny minor salivary glands from the inside of the lower lip and examines them under the microscope. If the glands contain lymphocytes in a particular pattern, the test is positive for Sjogren's syndrome.
These tests may include:
- Routine blood tests – The doctor will take blood samples to check blood count and blood sugar level, and to see how the liver and kidneys are working.
- Immunological tests – These blood tests check for antibodies commonly found in the blood of people with Sjogren's syndrome. For example:
- Antithyroid antibodies are created when antibodies migrate out of the salivary glands into the thyroid gland. Antithyroid antibodies cause thyroiditis (inflammation of the thyroid), a common problem in people with Sjogren's.
- Immunoglobulins and gamma globulins are antibodies that everyone has in their blood, but people with Sjogren's usually have too many of them.
- Rheumatoid factors (RFs) are found in the blood of people with rheumatoid arthritis, as well as in people with Sjogren's. Substances known as cryoglobulins may be detected; these indicate risk of lymphoma.
- Similarly, the presence of antinuclear antibodies (ANAs) can indicate an autoimmune disorder, including Sjogren's.
- Sjogren's antibodies, called SS-A (or SS-Ro) and SS-B (or SS-La), are specific antinuclear antibodies common in people with Sjogren's. However, you can have Sjogren's without having these ANAs.
- Chest X-ray – Sjogren's can cause inflammation in the lungs, so the doctor may want to take an X-ray to check them.
- Urinalysis – The doctor will probably test a sample of your urine to see how well the kidneys are working.
Treatment and Prevention
General Tips for Eye Care:
General Tips for Dry Mouth
- Don't use artificial tears that irritate your eyes--try another brand or preparation.
- Nonpreserved drops may be more comfortable.
- Blink several times a minute while reading or working on the computer.
- Protect your eyes from drafts, breezes, and wind.
- Put a humidifier in the rooms where you spend the most time, including the bedroom, or install a humidifier in your heating and air conditioning unit.
- Don't smoke and stay out of smoky rooms.
- Apply mascara only to the tips of your lashes so it doesn't get in your eyes. If you use eyeliner or eye shadow, put it only on the skin above your lashes, not on the sensitive skin under your lashes, close to your eyes.
- Ask your doctor whether any of your medications contribute to dryness and, if so, how to reduce that effect.
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:
- Visit a dentist at least three times a year to have your teeth examined and cleaned.
- Rinse your mouth with water several times a day. Don't use mouthwash that contains alcohol because alcohol is drying.
- Gently brush your teeth, gums, and tongue after each meal and before bedtime. Nonfoaming toothpaste is less drying.
- Floss your teeth every day.
- Avoid sugar. That means choosing sugar-free gum, candy, and soda. If you do eat or drink sugary foods, brush your teeth immediately afterward.
- Look at your mouth every day to check for redness or sores. See a dentist right away if you notice anything unusual or have any mouth pain or bleeding.
- Ask your dentist whether you need to have a protective varnish put on your teeth to protect the enamel.
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
ComplicationsProtect 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.
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
, 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.
Nerve problems are treated with medicines to control pain and, if necessary, with steroids
or other drugs to control inflammation
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:
- Polymyositis is an inflammation of the muscles that causes weakness and pain, difficulty moving, and, in some cases, problems breathing and swallowing. If the skin is inflamed too, it's called dermatomyositis. The disease is treated with corticosteroids and immunosuppressants.
- In Raynaud's phenomenon, blood vessels in the hands, arms, feet, and legs constrict (narrow) when exposed to cold. The result is pain, tingling, and numbness. When vessels constrict, fingers turn white. Shortly after that, they turn blue because of blood that remained in the tissue pools. When new blood rushes in, the fingers turn red. The problem is treated with medicines that dilate blood vessels. Raynaud's phenomenon usually occurs before dryness of the eyes or mouth.
- Rheumatoid arthritis (RA) is severe inflammation of the joints that can eventually deform the surrounding bones (fingers, hands, knees, etc.). RA can also damage muscles, blood vessels, and major organs. Treatment depends on the severity of the pain and swelling and which body parts are involved. It may include physical therapy, aspirin, rest, nonsteroidal anti-inflammatory agents, steroids, or immunosuppressants.
- Scleroderma causes the body to accumulate too much collagen, a protein commonly found in the skin. The result is thick, tight skin and damage to muscles, joints, and internal organs such as the esophagus, intestines, lungs, heart, kidneys, and blood vessels. Treatment is aimed at relieving pain and includes drugs, skin softeners, and physical therapy.
- Systemic lupus erythematosus (SLE) causes joint and muscle pain, weakness, skin rashes, and, in more severe cases, heart, lung, kidney, and nervous system problems. As with RA, treatment for SLE depends on the symptoms and may include aspirin, rest, steroids, and anti-inflammatory and other drugs, as well as dialysis and high blood pressure medicine.
- Vasculitis is an inflammation of the blood vessels, which then become scarred and too narrow for blood to get through to reach the organs. In people with Sjogren's, vasculitis tends to occur in those who also have Raynaud's phenomenon and lung and liver problems.
- Autoimmune thyroid disorders are common with Sjogren's. They can appear as either the overactive thyroid of Graves' disease or the underactive thyroid of Hashimoto's. Nearly half of the people with autoimmune thyroid disorder also have Sjogren's, and many people with Sjogren's show evidence of thyroid disease.