Sjogren's syndrome-often underdiagnosed

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By Sheila O'Connor

In the early 20th century, Swedish physician Henrik Sjögren (SHOW-gren) first described a group of women whose chronic arthritis was accompanied by dry eyes and dry mouth. Today rheumatologists know more about the syndrome that is named Sjögren's syndrome.

Sjögren's syndrome is a chronic systemic inflammatory autoimmune disorder that affects the mucous membranes, causing dry mouth, decreased tear production, and other dry conditions of the body’s membranes. The syndrome occurs by itself or can and often does occur with other autoimmune diseases such as lupus, rheumatoid arthritis, scleroderma, and other lesser known rheumatic autoimmune diseases.

'Primary' Sjögren's syndrome occurs in people with no other rheumatologic disease. 'Secondary' Sjögren's occurs in people who do have another rheumatologic disease, most often systemic lupus erythematosus and rheaumatoid arthritis.

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Most of the complications of Sjögren's syndrome occur because of decreased tears and saliva. Patients with dry eyes are at increased risk for infections around the eye and may have damage to the cornea. Dry mouth may cause an increase in dental decay, gingivitis (gum inflammation), and oral yeast infections (thrush) that may cause pain and burning. Some patients have episodes of painful swelling in the saliva glands around the face.

Complications in other parts of the body occur rarely in patients with Sjögren's syndrome. Pain and stiffness in the joints with mild swelling may occur in some patients, even in those without rheumatoid arthritis or lupus. Rashes on the arms and legs related to inflammation in small blood vessels (vasculitis) and inflammation in the lungs, liver, and kidney may occur rarely and be difficult to diagnose. Neurological complications that cause symptoms such as numbness, tingling, and weakness have also been described in some patients.

There is an increased incidence lymphoma among patients with this syndrome.

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What causes Sjögren's syndrome?

The cause of Sjögren's syndrome is not known, but it is considered an autoimmune disorder.

Normally, the immune system acts to protect the body from external threats like germs. When a person has an autoimmune disease, the immune system attacks the body instead.

If you have Sjögren's syndrome, this attack typically starts with your moisture-producing glands, which are called the exocrine glands. You need healthy exocrine glands to produce saliva and tears. These glands also produce moisture needed in the vaginal area, and in the gastrointestinal tract (stomach and intestine) and respiratory tract (airway and lungs). If your exocrine glands fail to create enough moisture, you may experience a number of uncomfortable symptoms.

People with this disease have abnormal proteins in their blood suggesting that their immune system, which normally functions to protect the body against cancers and invading infections, is reacting against their own tissue. The decreased production of tears and saliva seen in Sjögren's syndrome occurs when the glands that produce these fluids are damaged by inflammation. Research suggests that genetic factors and possibly viral infections (as yet unidentified) may predispose people to developing this condition.

Researchers think Sjögren's syndrome is caused by a combination of genetic and environmental factors. Several different genes appear to be involved, but scientists are not certain exactly which ones are linked to the disease since different genes seem to play a role in different people. For example, there is one gene that predisposes Caucasians to the disease. Other genes are linked to Sjögren's in people of Japanese, Chinese and African American descent. However simply having one of these genes will not cause a person to develop the disease. Some sort of trigger must activate the immune system.

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Scientists think that the trigger may be a viral or bacterial infection. It might work like this: A person who has a Sjögren'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 Sjögren's syndrome, they continue to attack, causing further damage. Scientists think that resistance to apoptosis may be genetic.

The possibility that the endocrine and nervous systems play a role is also under investigation.

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Who is most likely to develop Sjögren's syndrome?

Between 1 and 4 million Americans, or roughly 1 to 2% of the population, have Sjögren's syndrome. This condition can affect people of any age, but symptoms usually appear between the ages of 45 and 55. It affects 10 times as many women as men. About half of affected patients also have rheumatoid arthritis or other connective tissue diseases, such as systemic lupus.

In Sjögren's syndrome, the immune system produces lymphocytes (white blood cells) that produce self-reactive proteins called autoantibodies which attack the tear, salivary, and other moisture-producing glands, thus destroying the glands and their ability to produce moisture. Many parts of the body can be affected by Sjogren's syndrome, such as the lungs, brain, nerves, joints, kidneys, thyroid, and liver. In the majority of cases, the autoimmune response is confined to the tear ducts and salivary glands.

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What are the symptoms of Sjögren's syndrome?

Symptoms may include a dry, gritty, or burning sensation in the eyes; difficulty talking, chewing, or swallowing; a sore or cracked tongue; dry or burning throat; a change in the sense of taste or smell; increased dental decay; joint pain; digestive problems; and fatigue; swelling of the glands around the face and neck are also common. Some patients experience dryness of other mucous membranes (such as the nasal passages, throat, and vagina) and skin.

No two people have the exact same set of symptoms.

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Is it easy to diagnose Sjögren's syndrome?

Sjögren's syndrome often is undiagnosed or misdiagnosed.

Early diagnosis and treatment are important for preventing complications. The symptoms of Sjögren's syndrome may overlap with or 'mimic' those of other diseases including lupus, rheumatoid arthritis, fibromyalgia, chronic fatigue syndrome, and multiple sclerosis. Furthermore, dryness can occur for other reasons, such as a side effect of medication like anti-depressants or high blood pressure medication.

Additionally, because all symptoms are not always present at the same time and because Sjögren's can involve several body systems, physicians and dentists sometimes treat each symptom individually and do not recognize that a systemic disease is present. The average time from onset of symptoms to diagnosis is over six years.

Because the symptoms of Sjögren's are similar to those of many other diseases, getting a diagnosis can take time. The average time from first symptom to diagnosis ranges from two to eight years.

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What kind of doctor treats Sjögren's?

Rheumatologists have primary responsibility for managing Sjögren's syndrome. Ophthalmologists, dentists and other specialists also can treat symptoms related to Sjögren's.

During the years prior to diagnosis, depending on the symptoms, a person might see a number of doctors, any of whom may diagnose the disease and be involved in treatment. Usually, a rheumatologist (a doctor who specializes in diseases of the joints, muscles and bones) will coordinate treatment among a number of specialists. Other doctors who may be involved include:

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Tests

Once Sjögren's syndrome is suspected, a physician will request a series of blood tests.
The physician is likely to refer the patient to an ophthalmologist for further tests and to an oral pathologist or dentist for additional procedures.

Will I die from Sjögren's syndrome?

Sjögren's syndrome is serious but generally not fatal if complications are diagnosed and treated early. In one study, the incidence of lymphoma (cancer of the lymph glands) was 44 times higher in people with Sjögren's syndrome than in the general population. Sjögren's syndrome patients must be monitored carefully for development of related autoimmune diseases, lymphoma, and other complications.

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Is there a cure?

Not yet. Sjögren's syndrome cannot be cured.

Treatment

Treatment is based on the symptoms.

In many cases proper treatment helps to alleviate symptoms. Rheumatologists are specialists in musculoskeletal disorders and therefore are more likely to make a proper diagnosis. They can also advise patients about the best treatment options available.

Most treatment is designed to lessen the most bothersome symptoms.

Dry eyes usually respond to the use of artificial tears applied regularly during the day or to gels applied at night. Other measures, such as plugging or blocking tear ducts, can be used in more severe cases. Eyedrops that reduce inflammation in the glands around the eyes may be used to increase tear production.

Dry mouth can be relieved by drinking water, chewing gum, or using saliva substitutes. Some patients benefit from using prescription medications that stimulate saliva flow, such as pilocarpine or cevimuline . If patients develop yeast infections, these can be relieved by anti-fungal therapies. The currently available treatments may help relieve some of the dryness but usually some dryness persists.

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All patients should receive regular dental care in order to prevent cavities and tooth loss that may occur as a complication of the disorder. Patients with dry eyes should see an ophthalmologist (eye doctor) regularly for signs of damage to the cornea. Patients with excessive redness and pain in the eyes should be evaluated for infections.

Arthritis symptoms are treated with anti-inflammatory medicines, such as aspirin, acetaminophen, and other NSAIDs.

Hydroxychloroquine, an antimalarial drug used in lupus and rheumatoid arthritis, may be helpful in some patients with Sjögren's syndrome by reducing joint pain and rash experienced by some patients. Patients with rare but serious systemic symptoms, such as fever, rashes, abdominal pain, or lung or kidney problems, may require treatment with corticosteroids such as prednisone and/or immunosuppressive agents methotrexate, azathioprine and mycophenolate.

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Broader health impact of Sjögren's syndrome

A vast majority of patients with Sjögren's syndrome remain very healthy without any serious complications. Patients should be aware that they do face an increased risk for infections in and around the eyes and an increased risk for dental problems, both of which are due to the long-term reduction in tears and saliva.

Rarely, patients may have complications related to inflammation in other body systems, including:

In a small number of people, Sjögren's syndrome may be associated with lymphoma, a cancer of the lymph glands.

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Living with Sjögren's syndrome

People with Sjögren's syndrome are usually able to live normal lives with very few adjustments. When a diagnosis is made, many patients must focus a great deal of attention dealing with dry eyes and dry mouth, but these symptoms tend to subside with time. Any pain or redness in the eyes should be evaluated promptly, as this may signal an infection. To reduce risk for cavities and other dental problems, patients must pay close attention to proper oral hygiene and regular dental care.

Patients should see their physician regularly for general health screening, and should pay close attention to any abnormal swelling in the glands around the face or neck, under the arms, or in the groin area, as this may be a sign of lymphoma.

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