Answer

My diagnosis is Sjögren syndrome. Sjögren syndrome is a chronic autoimmune disorder characterized by xerostomia (dry mouth), xerophthalmia (dry eyes), and lymphocytic infiltration of the exocrine glands. This triad is also known as the sicca complex. Sjogren's syndrome is a chronic autoimmune disorder in which immune cells attack and destroy the glands that produce tears and saliva. The hallmark symptoms of the disorder are dry mouth and dry eyes. It is named after the Swedish eye doctor, Dr. Henrik Sjogren, who first described it.

#1---You complain of "severe problems with dry mouth; tonge has irritations all over and is cracked; eyes are dry and stick together; swollen salivary glands."---One suspects Sjogren's Syndrome (SS) with dryness of the eyes and mouth; joint inflammation completes the classic triad. Arthritis occurs in about 33% of patients and is similar in distribution to that seen in rheumatoid arthritis (RA); however, joint symptoms in SS tend to be milder and rarely lead to destruction. Some patients with undiagnosed SS who have rheumatic symptoms may not complain spontaneously of sicca complex; SS is then defined by laboratory evaluation.

When bilateral parotid enlargement occurs in conditions such as hyperlipoproteinemia, malnutrition, cirrhosis, or diabetes mellitus, the glands are soft and puffy, in contrast to the firm glands of SS; oral dryness is absent.

#2---You complain that "skin also appears to be quite dry; dryness vaginally; voice trailing off at the end of my sentences;." With SS desiccation may also develop in the skin and in mucous membranes of the nose, throat, larynx, bronchi, vulva, and vagina. Alopecia may occur. Dryness of the respiratory tract often leads to lung infections and sometimes to fatal pneumonia

#3---You complain that "teeth have become brittle and are crumbling away"   Dryness that promotes ductal calculi and rampant dental caries may be avoided by sipping fluids throughout the day, chewing sugarless gum, and using a 2% solution of methylcellulose as a mouthwash. Drugs that decrease salivary secretion, such as decongestants and antihistamines, should be avoided. Fastidious oral hygiene and regular dental supervision are essential. Calculi must be promptly removed, preserving viable salivary tissue. The temporary pain of suddenly enlarged salivary glands is best treated only with analgesics. Connective tissue involvement usually is mild and chronic; therefore, corticosteroids and immunosuppressive agents are indicated only occasionally, eg., in a patient with severe vasculitis or visceral involvement. Irradiation and drugs that increase the risk of lymphoproliferative disorders and infection should be avoided.

#4---You complain of "chronic lymph node swelling in the neck and have had one lymph node removed"---The lymph nodes are part of the lymphatic system that is part of the immune system. Lymph nodes often swell in response to disease. There are three set of glands that produce saliva. They're located under your tongue, in the cheeks in front of your ears, and in the back of your mouth. They may feel swollen and tender. This may occur along with a fever. This affects about one-half of people with SS.


#5---You complain of a "positve testing for Rheumatoid arthritis"---Remarkable immunologic reactivity, detected in blood serum, is characteristic of SS; most patients have elevated levels of antibodies against y-globulin, nuclear protein, and many tissue constituents. Precipitating antibodies to nuclear antigens (identified by immunodiffusion analysis), termed SS-B antibodies, are highly specific for primary SS.

Rheumatoid factor is present in >70% of cases; the LE cell preparation is positive in 15 to 20%. The VDRL test is negative. ESR is elevated in 70% of patients. One third of patients have anemia; 1/4, and eosinophilia. Urinalysis may show proteinuria, reflecting intersititial nephritis. Prognosis in SS is often related to the associated connective tissue disorder, although death may also result from pulmonary infection and, rarely, renal failure or lymphoma.

Immunologic reactivity, detected in blood serum, is characteristic of SS; most patients have elevated levels of antibodies against -globulin (RF), nuclear protein, and many tissue constituents. Precipitating antibodies to nuclear antigens (identified by immunodiffusion analysis), termed SS-B antibodies, are frequently present but are not specific for primary SS. RF is present in > 70% of cases, ESR is elevated in 70%, 33% have anemia, and 25% have leukopenia and eosinophilia. Urinalysis may show proteinuria, reflecting interstitial nephritis.

#6---You complain that you "urinate excessively and always have keytones and blood present on a urinalysis"---This is Classic Distal Renal Tubular Acidosis (RTA). To diagnose RTA, your doctor will check the acid-base balance in samples of your blood and urine. If the blood is more acidic than it should be and the urine less acidic than it should be, RTA may be the reason, but additional information is needed first to rule out other causes. If RTA is suspected, additional information about the sodium, potassium, and chloride levels in the urine and the potassium level in the blood will help identify which of the three types of RTA you have. In all cases, the first goal of therapy is to neutralize acid in the blood, but different treatments may be needed to address the different underlying causes of acidosis.

Type 1 Distal Renal Tubular Acidosis is also called classic distal RTA. "Distal," which means distant, refers to the point in the urine-forming tube where the defect occurs. It is relatively distant from the point where fluid from the blood enters the tiny tube, or tubule, that collects fluid and wastes to form urine. This disorder may be inherited as a primary disorder or may be one symptom of a disease that affects many parts of the body. Researchers have now discovered the abnormal gene responsible for the inherited form. More often, however, classic distal RTA is a complication of diseases that affect many organ systems (systemic diseases), like the autoimmune disorders Sjögren's syndrome and lupus.

Sjogren's syndrome can occur in two ways: primary and secondary. Both forms affect roughly an equal number of people.

Primary Sjogren's syndrome occurs by itself and is not associated with other diseases.

Secondary Sjogren's syndrome occurs with rheumatic diseases such as rheumatoid arthritis, systemic lupus erythematosus ("lupus"), polymyositis, and some forms of scleroderma. Rheumatic diseases are conditions that affect joints, bones, muscles, skin, and sometimes other organs.

Sjogren's syndrome is generally not life-threatening. The outlook for people with this condition is usually good. Dryness, however, may last for the rest of your life. By using artificial moisture and practicing good oral hygiene, you can help prevent serious problems.

A certain blood marker often found in women with Sjogren's syndrome can, very rarely, be associated with heart problems in newborn babies. If you're planning to become pregnant, see your doctor about testing for this marker. If it is present, ask your doctor whether pregnancy is advisable. If you do become pregnant, you and your doctor can work out the best plan to manage the situation.

Normal Range: Negative Autoantibody against acidic nuclear ribonucleoproteins that is found in patients with some connective tissue diseases, especially Sjogren's syndrome.

Positive in Primary Sjogren's syndrome (70%), SLE (40%), rheumatoid arthritis (10%).

Anti-La/SS-B is another antibody against acidic ribonucleoproteins that is less sensitive for Sjogren's (50-60%) and SLE (10-15%). Patients with antibodies to SS-A may have a negative ANA test.

Sjogren's syndrome can affect people of any race and any age. It usually affects women. It affects more than one million people in the U.S.


Symptoms include:

Dry mouth. The mouth normally contains saliva, which aids chewing and swallowing. In people with Sjogren's syndrome, the amount of saliva is much less. This makes chewing, swallowing, and speaking difficult. It may also cause a decreased sense of taste.

Dry eyes. Your eyes may feel dry, "gritty," or "sandy." They may burn and look red. A thick substance may accumulate in the inner corner of your eyes while you sleep. Your eyes may be more sensitive to sunlight. If not properly treated, Sjogren's syndrome can lead to ulcers of the cornea (the clear covering of the eyeball). On rare occasions, this can cause blindness.

Swollen salivary glands

Dental cavities. This is a common problem that results from a dry mouth. Saliva fights bacteria and defends against cavities. Because you have decreased saliva, your teeth may develop cavities more easily.

Dry nose, throat, and lungs. This may make your throat feel dry and tickly. You may have a dry cough, hoarseness, a decreased sense of smell, and nosebleeds. It can also lead to pneumonia, bronchitis, and ear problems.

Dryness of the vagina. This can cause painful intercourse for women with Sjogren's syndrome.

Fatigue is a common complaint. You may get easily exhausted and feel tired and worn out.

Sjogren's syndrome can affect other parts of the body, such as blood vessels, the nervous system, muscles, skin, and other organs. This can lead to muscle weakness, confusion and memory problems, dry skin, and feelings of numbness and tingling.

Sjogren's syndrome can also affect the liver and pancreas. When it does, there is a greater chance for developing cancer of the lymph tissue. Although this is unusual, it is one reason why medical exams and continued follow-up are important.

The causes of Sjogren's syndrome are not known. There is some evidence that viral infections, heredity, and hormones may in some way contribute to Sjogren's syndrome.

In Sjogren's syndrome, changes occur in the immune system--the body's defense against disease. In Sjogren's, the immune system lacks the usual controls. This causes white blood cells to invade glands in the body that produce moisture, such as the tear and salivary glands, and the Bartholins glands in the vagina. They can destroy the glands and cause them to stop producing moisture.

Sjogren's syndrome can also cause problems in other parts of the body, including the joints, lungs, muscles, kidneys, nerves, thyroid gland, liver, pancreas, stomach, and brain (see figure 1). 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, and lungs. Blindness is a rare complication of Sjogren's.


Tests for specific blood markers can determine if you have Sjogren's syndrome. However, not everyone with Sjogren's has these markers.

Diagnostic tests:

Schirmer test---This helps determine how dry your eyes may be. It involves placing a small piece of filter paper under the lower eyelid to measure the amount of tears your eyes produce.

Slit-lamp examination---This is a more accurate way to find out if your eyes are dry. In this test, the doctor puts a drop of dye into your eye and examines the eye with a special instrument called a slit lamp. The dye will stain dry or eroded areas of the eye. This test is often done by an ophthalmologist (eye doctor}.

Lip biopsy---In this test, the doctor removes a few salivary glands from inside your lip. The tissue is examined under a microscope. The appearance of the tissue helps determine if you have Sjogren's syndrome.

Salivary function tests---These measure the actual amount of saliva you produce, to help determine if you have Sjogren's.

Urine tests---These may be done to test your kidney function.

Chest X-ray---This can help detect changes in your lungs.

As yet, there is no cure for Sjogren's syndrome. However, proper treatment can help relieve symptoms so you can live a comfortable and productive life.

The main goal of treatment is to relieve discomfort and lessen the effects of the dryness. Since Sjogren's syndrome affects everyone differently, your treatment plan will be based on your specific needs.

See your family doctor and your dentist regularly. Since Sjogren's syndrome can affect many parts of the body, regular checkups can help detect and prevent future problems. You may also need regular check ups with your rheumatologist and an eye specialist. Make sure you follow your doctor's complete treatment program.

For dry mouth: Sip fluids throughout the day.Use sugar-free gum or candies to stimulate saliva production.Try saliva substitutes or mouth coating products. They may be useful in some people, and are available without a prescription.

To prevent dental cavities: Have frequent dental checkups.Use mouth rinses that contain fluoride.Brush and floss your teeth regularly.Use sugar-free products.

For dry eyes: Use artificial tears or eye drops to help relieve the discomfort of dry eyes. Use preservative-free products, if you apply the drops more than four times per day.
Try lubricating ointments or small, long-acting pellets for overnight or long-lasting relief.
Your ophthalmologist may recommend a simple operation that blocks tear drainage from your eye.

For dry skin: Use moisturizing lotions for sensitive skin.Avoid drafts from air conditioners, heaters, and radiators, when possible. Use a humidifier in your house and at work.

For vaginal dryness: Use lubricants made specifically to help vaginal dryness. Do not use petroleum jelly.

Exercise and therapy: Mild exercise, such as walking or swimming, can help keep joints and muscles flexible. Exercise may also protect against further joint damage.

Medications: Aspirin and nonsteroidal anti-inflammatory drugs (NSAIDs) help reduce joint swelling and stiffness, as well as muscle aches.

If you have serious complications, your doctor may recommend stronger medicines.

Contact one of the following organizations for more information.

The National Sjogren's Syndrome Association is an international, nonprofit, all-volunteer organization dedicated to providing educational information to patients and health professionals worldwide. It sponsors support groups and national and regional conferences. Publishes a national newsletter ("The Sjogren's Digest"), a quarterly collection of articles ("Patient Education Series"), and a patient guide ("Learning to Live with Sjogren's Syndrome").

National Sjogren's Syndrome Association
Toll-free: 1-800-395-NSSA (6772)

The Sjogren's Syndrome Foundation is an international organization that provides materials, educational programs, and support groups throughout the U.S. and abroad. It publishes "Sjogren's Syndrome Handbook: An Authoritative Guide for Patients" and a monthly newsletter,

Sjogren's Syndrome Foundation
XXXX XXXXXXXX XXX. Suite 530
Bethesda, MD 20814
XXX-XXX-XXXX
www.sjogrens.org