Sjogren's Syndrome

Definition
Sjögren's syndrome is an autoimmune disorder in which immune cells attack and destroy the glands that produce tears and saliva. Sjögren's syndrome is also associated with rheumatic disorders such as rheumatoid arthritis. The hallmark symptoms of the disorder are dry mouth and dry eyes. In addition, Sjogren's syndrome may cause skin, nose, and vaginal dryness, and may affect other organs of the body including the kidneys, blood vessels, lungs, liver, pancreas, and brain.
It was found by Henrik Samuel Conrad Sjogren n 1933.

Etiology
Researchers think Sjogren'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 Sjogren's in people of Japanese, Chinese, and African American descent. Simply having one of these genes will not cause a person to develop the disease, however. Some sort of trigger must activate the immune system.
Scientists think that the trigger may be a viral or bacterial infection. It might 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. Scientists think that resistance to apoptosis may be genetic.
The possibility that the endocrine and nervous systems play a role is also under investigation.

Types of Sjogren's Syndrome
SS comes in two types, primary and secondary.
The main difference between the two is that Primary SS occurs by itself while Secondary SS occurs when it's accompanied by a rheumatic condition such as Lupus, Scleroderma and Arthritis.
The rheumatic diseases cause inflammation and pain in the joints, muscles and skin.
If you have Primary SS you most likely have types of antibodies in your blood called SSA and SSB. Additionally, you may have another group of antibodies called ANA (Anti-Nuclear Antibody) that are a group of antibodies that react against normal components of a cell nucleus, meaning they attack normal and healthy cells. If you have ANA's it doesn't necessarily mean you have Sjogren’s as these antibodies are also present in other autoimmune diseases.
Another indication that you have Sjogren's syndrome is the presence of a high Immunoglobulin (IG) count in your blood; IG's are blood proteins usually elevated in both types of SS.
Although a person can develop Sjogren's syndrome at any age, it is very rare in children and most often occurs in people older than 40. It is estimated that 1 to 4 million Americans have SS and 90% are women. It is estimated that 50% suffer from Primary SS and 50% with Secondary SS.


Signs and Symptoms
•    Dry Eyes - this is a very typical symptom of Sjogren's syndrome, however, not everyone with SS has dry eyes. What do dry eyes feel like? You may feel like you have "sand" in your eyes that causes them to be red and itchy with blurred vision. Fluorescent light could also be an irritant.
•    Dry Mouth - you feel like you need to drink water all day long and may experience difficulty swallowing or chewing.
•    Achy Joints - your hips and knees hurt and are often swollen and stiff. For some people, it gets very painful to kneel down.
•    Sensory Changes – SS can affect your sense of smell and taste.
•    Dry Skin - this is another common symptom that can vary in severity with the extreme being scaly skin.
•    Glands – there can be pain in the parotid glands and with some enlargement.
•    Fatigue – this is a common symptom that is sometimes overlooked by doctors.
•    Low-grade fever, numbness in arms and legs, vaginal dryness, bruising and dry cough.

Symptoms vary and can remain the same, worsen or go into remission. Some people experience mild symptoms while others suffer debilitating ones that can affect their quality of life.
Although Sjogren's syndrome does not represent a life threatening condition there can be serious complications especially for those who suffer from a rheumatic disease as well.


Complications
1.    Peripheral neuropathies in the legs.
2.    Symptoms of numbness, tingling and burning.
3.    Dental cavities.
4.    Dry eyes that can lead to corneal ulcers.
5.    Malabsorption of nutrients due to damage to the mucus of the stomach lining.
Other complications that are less common include: pneumonia, bronchitis, and problems with kidney function, hepatitis or cirrhosis of the liver. Additionally, a small percentage of people with Sjogren's syndrome develop cancer of the lymph nodes (lymphoma).

Diagnosis
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.
You may have some tests, too. First, the doctor will want to check your eyes and mouth to see whether Sjogren's is causing your symptoms and how severe the problem is. Then, the doctor may do other tests to see whether the disease is elsewhere in the body as well.
Common eye and mouth tests are
1.    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.
2.    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.
3.    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.
4.    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.
5.    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.
6.    Because there are many causes of dry eyes and dry mouth, the doctor will take other possible causes into account. Generally, you are considered to have definite Sjogren's if you have dry eyes, dry mouth, and a positive lip biopsy. But the doctor may decide to do additional tests to see whether other parts of the body are affected. These tests may include
7.    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.
8.    Immunological tests--These blood tests check for antibodies commonly found in the blood of people with Sjogren's syndrome. For example:
a.    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.
b.    Immunoglobulins and gamma globulins are antibodies that everyone has in their blood, but people with Sjogren's usually have too many of them.
c.    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.
d.    Similarly, the presence of antinuclear antibodies (ANAs) can indicate an autoimmune disorder, including Sjogren's.
e.    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.
9.    Chest x ray--Sjogren's can cause inflammation in the lungs, so the doctor may want to take an x ray to check them.
10.    Urinalysis--The doctor will probably test a sample of your urine to see how well the kidneys are working.

What Type of Doctor Diagnoses and Treats Sjogren's Syndrome?
Because the symptoms of Sjogren's are similar to those of many other diseases, getting a diagnosis can take time--in fact, the average time from first symptom to diagnosis ranges from 2 to 8 years. During those years, 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
a.    Allergist
b.    Dentist
c.    Dermatologist (skin specialist)
d.    Gastroenterologist (digestive disease specialist)
e.    Gynaecologist (women's reproductive health specialist)
f.    Neurologist (nerve and brain specialist)
g.    Ophthalmologist (eye specialist)
h.    Otolaryngologist (ear, nose, and throat specialist)
i.    Pulmonologist (lung specialist)
j.    Urologist

Medical treatment
There is no known cure for Sjögren's syndrome nor is there a specific treatment to restore gland secretion. Treatment is generally symptomatic and supportive. Moisture replacement therapies may ease the symptoms of dryness. Nonsteroidal anti-inflammatory drugs may be used to treat musculoskeletal symptoms. For individuals with severe complications, corticosteroids or immunosuppressive drugs may be prescribed.

What is the treatment for Sjogren's syndrome?
The treatment of patients with Sjogren's syndrome is directed toward the particular areas of the body that are involved and prevention of complications such as infection. There is no cure for Sjogren's syndrome.
Dryness of the eyes can be helped by artificial tears, using eye-lubricant ointments at night, and minimizing the use of hair dryers. When dryness becomes more significant, the ophthalmologist can plug the tear duct closed so that tears cover the eye longer. Cyclosporine eyedrops (Restasis) are approved medicated eyedrops that can reduce the inflammation of the tear glands, thereby improving their function. Signs of eye infection (conjunctivitis), such as pus or excessive redness or pain, should be evaluated by the doctor. Dietary addition of flaxseed oil may also benefit eye dryness.
The dry mouth can be helped by drinking plenty of fluids, humidifying air, and good dental care to avoid dental decay. The glands can be stimulated to produce saliva by sucking on sugarless lemon drops or glycerin swabs. Additional treatments for the symptom of dry mouth are prescription medications that are saliva stimulants, such as pilocarpine (Salagen) and cevimeline (Evoxac). These medications should be avoided by people with certain heart diseases, asthma, or glaucoma. Artificial saliva preparations can ease many of the problems associated with dry mouth. Many of these types of agents are available as over-the-counter products, including toothpaste, gum, and mouthwash (Biotene). Numoisyn liquid and lozenges are also available for the treatment of dry mouth. Vitamin E oil has been used with some success. Infections of the mouth and teeth should be addressed as early as possible in order to avoid more severe complications. Diligent dental care is very important.
Saltwater (saline) nasal sprays can help dryness in the passages of the nose. Vaginal lubricant should be considered for sexual intercourse if vaginal dryness if a problem.
Hydroxychloroquine (Plaquenil) has been helpful for some manifestations of Sjogren's syndrome. Serious complications, such as vasculitis, can require immune-suppression medications, including cortisone (prednisone and others) and/or azathioprine (Imuran) or cyclophosphamide (Cytoxan).


Prognosis
Sjögren's syndrome can damage vital organs of the body with symptoms that may remain stable, worsen, or go into remission. Some people may experience only the mild symptoms of dry eyes and mouth, while others go through cycles of good health followed by severe disease. Many patients are able to treat problems symptomatically. Others are forced to cope with blurred vision, constant eye discomfort, recurrent mouth infections, swollen parotid glands, hoarseness, and difficulty in swallowing and eating. Debilitating fatigue and joint pain can seriously impair quality of life.

References

1. http://www.ninds.nih.gov/disorders/sjogrens/sjogrens.htm

2. http://www.medicinenet.com/sjogrens_syndrome/article.htm

3. http://www.sjogrens.org/home/about-sjogrens-syndrome/symptoms

4. http://www.medic8.com/skin-disorders/sjogrens-syndrome.htm