Sjögren's syndrome

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at April 13, 2016
StartSjögren's syndrome

Sjögren's syndrome may occur in its primary form by itself and with no relation to any other diseases, or as a secondary disease that evolves due to the existence of another autoimmune disease like lupus, rheumatoid arthritis, or vasculitis. It's been estimated that around 4,000,000 people in the United States have Sjögren's syndrome, and over 90% of them are women. Although the condition may develop in people of all races and ages, the average age range for symptoms to develop is the late 40s.

Contents

Definition & Facts

Sjögren's syndrome is a condition wherein the immune system destroys the moisture-producing glands of the body, like those that produce sweat, tears and saliva. The disease prevents these glands from functioning properly, which results in the most common symptoms of dry eyes and dry mouth.

The condition can also lead to other health issues, which include low energy and joint discomfort. In the most advanced cases, it may injure the nerves, lungs, and kidneys. Anybody is susceptible to developing Sjögren's, but it's most frequently observed in Caucasian women in their 40s and 50s.

Symptoms & Complaints

Sjögren’s syndrome can affect other body structures, such as the skin, nerves, joints, kidneys, lungs, arteries, and digestive system, and as a result the symptoms associated with it can be quite numerous and varied. The trademark signs of Sjögren's syndrome are extremely dry eyes and mouth lasting for more than three months that are not triggered by medications.

People may also have itchiness or burning in the eyes. The mouth will typically feel very dried out, like it is stuffed with cotton. Other frequent symptoms may include: dry skin; skin sores; persistent dry cough; thyroid and metabolism issues; joint or muscle discomfort; vaginal dryness; pins and needles in the arms or legs; and chronic fatigue.

Causes

Health care providers haven't yet pinpointed a specific cause for Sjögren's syndrome, but they have theorized that it could be the result of a combination of genes and exposure to some pathogens like a virus or harmful bacteria. A person's genetic profile may put him or her at greater risk of developing the condition, but it seems that an activation process - like a viral infection or bacterial infection - must also occur. Because Sjögren's syndrome is an autoimmune disease, it can occur in conjunction with other immune-related health issues, including rheumatoid arthritis, lupus, and scleroderma.

Diagnosis & Tests

Sjögren's syndrome may be challenging to diagnose as the symptoms often differ between people and are often very much like those brought on by other diseases. Complications from several different medications also imitate some of the symptoms of Sjögren's syndrome. A physician begins the diagnostic process with a physical examination and inquiry about a patient's symptoms and past medical history. The physical also includes questions about any medications the patient may be taking that might trigger dry eyes and mouth.

If necessary, some other tests may be performed. One of these is a Schirmer's tear test, which measures eye dryness with a tiny piece of paper that is placed beneath the patient's lower eyelid to determine the level of tear production. An ophthalmologist may also check the eye surface with a special magnifier known as a slit lamp, while placing drops into the eye that make any corneal injuries more visible.

Doctors may also run blood tests or urine tests to check for excessive levels of antibodies. A certain X-ray called a sialogram detects a colored dye that's injected directly into the salivary glands in front of the ears. This test reveals the rate of salivary flow in the mouth. Salivary scintigraphy is a nuclear medicine test that injects a radioactive isotope into the bloodstream, which is then monitored for about an hour to determine how fast it reaches all of the salivary glands.

A doctor may also perform a lip biopsy to find any groups of inflammatory cells, which may point to Sjögren's syndrome. For this examination, a tiny piece of cell tissue is surgically removed from the salivary glands found in the lip (labial glands) and analyzed under the lens of a microscope.

Treatment & Therapy

The best treatment and therapy for Sjögren's syndrome depends on how the condition affects a patient as time passes. In the majority of situations, treatment is focused on enabling patients to manage their own symptoms. A doctor will typically prescribe: artificial tears, oral lubricants, and saliva substitutes to moisten and preserve the eyes, mouth, and throat; estrogen ointments for vaginal dryness; and steroid medications to alleviate joint and muscle pain.

More aggressive medications can be prescribed if these measures fail to control symptoms. Hydroxychloroquine (or Plaquenil®) is a medication originally used for the treatment of malaria that is frequently beneficial for managing Sjögren's syndrome. Immunosuppressants, which may include drugs like ciclosporin and methotrexate, may also be recommended.

For the relief of dry eyes, a patient may wish to have a minor surgical treatment called a punctal occlusion, which seals the tear ducts that drain tears and moisture from the eyes. The procedure inserts collagen or silicone plugs into these tear ducts for short or long-term closure. The collagen plugs gradually dissolve, but silicone plugs remain in place until they come off or are taken out. As an alternative, a physician can use a laser to permanently close the tear ducts.

Prevention & Prophylaxis

While there is no known prevention for Sjögren's disease, there are many self-care protocols that patients can do at home to help control their symptoms, like quitting smoking, avoiding secondhand smoke, and drinking plenty of fluids to prevent any dehydration caused by antihistamines or other medicines.

Sugarless gum or hard candies may help to increase salivary flow in the mouth. Because Sjögren's syndrome raises the chance of developing cavities, sugarless sweets are recommended, particularly between meals. Patients should also brush and floss their teeth after each meal and have dental checkups on a regular basis, at least twice a year.