Sjögren’s syndrome  is a chronic disease in which the body’s immune system affects glands that produce moisture in the eyes (tears), the mouth (saliva), and elsewhere in the body. The most common symptoms of Sjögren’s syndrome are dry eyes and dry mouth. Sjögren’s syndrome can also affect skin, lung, heart, kidney, and nerves.

It is called an autoimmune disease, meaning that the body’s immune system attacks its own tissues or organs. Lymphocytes are  types of blood cells that normally help to protect the body from infection. In Sjögren’s syndrome, these cells are overactive and damage the glands that produce tears and saliva.

A patient with Sjögren’s syndrome probably inherits the risk from one or both parents and is then exposed to some type of environmental trigger (eg, a viral infection), but the exact cause in not known.

Although there is no cure for Sjögren’s syndrome, a number of treatments are available.

The most prominent symptoms of Sjögren’s syndrome (eye and mouth dryness) are common and can be caused by conditions other than Sjögren’s syndrome. Therefore, it is important to identify medications or conditions that cause dryness and to determine if alternate non-drying treatments are available.

The definition of Sjögren’s syndrome requires that the person have symptoms for a prolonged time (eg, dry mouth for greater than three months) and have positive laboratory tests. One of the most important is the test for the presence of markers of this condition in the blood.

A salivary gland biopsy may be recommended to aid in the diagnosis of Sjögren’s syndrome. The biopsy is done by removing a small piece of tissue from the inner portion of the lip. Other salivary gland tests may also be recommended.

Tests are usually recommended to determine if you produce a normal amount of tears and to determine if there are areas of the eye that have been damaged as a result of dryness. An eye specialist (ophthalmologist) may perform these tests.

Other symptoms of Sjogren’s syndrome and its complications:

• Damage to the surface of the eye that can  present as a pain, redness and gravel sensation secondary to the decreased tear production.
• People with decreased saliva production are at risk of developing cavities in the teeth and infections in the mouth, including painful fungal infection (thrush).
• Diseases of the lungs (so called interstitial pneumonitis), kidneys (so called interstitial nephritis), and thyroid gland abnormalities.
• Vasculitis, an inflammation of the blood vessels. Vasculitis can cause bleeding and pain and can lead to skin, nerve, and/or internal organ damage.
• Sjögren’s also increases the risk of  non-Hodgkin lymphoma that can presents as fevers, weight loss, night sweats and enlargement of the lymph nodes. Report those symptoms to your physician if they develop.
• There is an increased risk of heart attack or stroke in patients with the Sjogren’s syndrome. It is important to know the common risk factors for the premature heart attack or stroke that you can modify and decrease the risks. Please, read the “Atherosclerosis and inflammation” section to learn more. Also talk to your Family doctor about the risk factors that you can change.

 TREATMENTS:

Moisturizing treatments and preserving natural tears: eye drops (artifical tears) are used to treat dry eyes. Sometimes, eye drop containing cyclosporine, which suppresses part of the local immune reaction, is used.

At night, an eye ointment may be used to provide moisture. It is important to use only a small amount ( 3 mm) of the ointment because overuse can block the ducts and can lead to a condition called blepharitis.

Regular, 2-3 times per year ophthalmology visits are important. Sometimes an eye doctor used a procedure that blocks tear ducks and improves symptoms.

Mouth dryness: sugarless candy or sugar-free gum can stimulate the flow of saliva. In some people, medications such as pilocarpine or cevimeline can be given to increase saliva production.

Sipping on water throughout the day or use of artificial saliva can be helpful.

People with Sjögren’s syndrome are at increased risk for dental cavities. You should brush and floss after eating meals and snacks. You should visit your dentist at every 3-6 months for a cleaning and evaluation.

Toothpaste with fluoride may help to prevent cavities.

People with Sjögren’s syndrome may have dryness in other areas, including the lips, the skin, and the vagina.

Some women with Sjögren’s syndrome have difficulty with vaginal dryness, especially after menopause. There are several products designed for vaginal dryness, including vaginal moisturizers, estrogen cream, vitamin E oil, and vaginal lubricants.

Fungal infections in the mouth: prescription medications are available to treat painful mouth lesions due to oral candidiasis (yeast infection).

Eyelid inflammation: eyelid inflammation, also called blepharitis, causes symptoms that are similar to those of dry eye (swollen lids and redness of the inside of the lids). Gently washing the skin of the eyelids and warm compresses can relieve it.

Heartburn: reatment of reflux in people with Sjögren’s syndrome is similar to treatment in other people (see Scleroderma section).

Joint and muscle pain: nonsteroidal antiinflammatory drugs (ibuprofen, naproxen)  are recommended.

Low-dose steroids are used for short-term treatment.

So called disease-modifying drugs (DMARDs) are used when arthritis is moderate to severe.

Fatigue: fatigue is common in Sjögren’s syndrome. Fatigue may be due to the disease itself or to difficulties staying asleep.

General treatment for fatigue includes adequately controlling of dry mouth, using a humidifier in the bedroom, and practicing good sleep hygiene. Sleep hygiene modifications include avoiding daytime napping, avoiding caffeine and other stimulants in the evening, observing a period of relaxation before sleep, and keeping  bedroom quiet and comfortable. Going outside for 20 minute walk before sleep can prepare you mentally to good night sleep.

Fibromyalgia: some people with Sjögren’s syndrome also have a condition called fibromyalgia. Fibromyalgia causes muscle aching and fatigue.

Vasculitis: if vasculitis occurs, it often requires treatment with drugs that suppress the immune system.

Anesthesia and Sjögren’s syndrome: if you need surgery for any reason, the anesthesiologist should be aware of your diagnosis. Medications used during the surgery can dry the airways. The anesthesiologist can take special measures to lower the risk of the complications.

Read more on importance of exercise under  the “Medical news”.