Sjogren’s Syndrome

Sjogren’s syndrome is an autoimmune disease where the immune system abnormally targets tear and saliva-producing glands leading to eye and mouth dryness. Other parts of the body can be affected as well, resulting in a wide range of possible symptoms, which will be addressed in detail below.

Experts believe 1 to 4 million people have the disease. Most–90 percent—are women. It can occur at any age, but it usually is diagnosed after age 40 and can affect people of all races and ethnic backgrounds. It’s rare in children, but it can occur.

Sjogren’s syndrome is classified as primary or secondary, meaning it occurs with another disease such as rheumatoid arthritis or lupus. Both subtypes are associated with specific blood test abnormalities, antibodies to SSA and SSB. These can be used to help with diagnosis.

Symptoms

The primary symptoms are:

  • Dry eyes – With progressive dryness, one may develop redness, burning and itching of the eyes. There may be a gritty sensation experienced. Vision may become blurry, and bright light may irritate the eyes..
  • Dry mouth – Dry mouth feels like a mouth full of cotton. It’s difficult to swallow, speak, and taste. Your sense of smell can change, and you may develop a dry cough. There is a greatly increased risk for dental cavities and oral infections.

Both primary and secondary Sjogren’s syndrome can affect other parts of the body as well, including the skin, joints, lungs, kidneys, blood vessels, gastrointestinal tract and nervous system, and cause symptoms such as

  • Dry skin – particularly dry, itchy skin. Skin may crack and become infected.
  • Skin rashes – due to dry skin
  • Joint and muscle pain – May develop inflammatory like arthritis and muscle pain
  • Pneumonia – May develop infectious or inflammatory lung disease.
  • Vaginal dryness – May result in vaginal itching, burning and painful intercourse.
  • Kidney problems – Risk for interstitial nephritis or glomerulonephritis (inflammation of the tissue around the kidney’s filters or the filter’s themselves, respectively)
  • Numbness and tingling in the extremities – Examples include carpal tunnel syndrome, peripheral neuropathy, cranial neuropathy.
  • Digestive Tract – Inflammation of the esophagus, stomach, pancreas, and liver can cause painful swallowing, heartburn, abdominal pain and swelling, loss of appetite, diarrhea and weight loss.

When Sjogren’s affects other parts of the body, the condition is called extraglandular involvement because the problems extend beyond the tear and salivary glands. Finally, Sjogren’s can cause extreme fatigue that can seriously interfere with daily life.

Causes

Researchers believe Sjogren’s syndrome is likely caused by a combination of genetic and environmental factors. Several different genes appear to be involved, but scientists are not certain which ones are specifically linked to the disease since different genes seem to play a role in different people.

Diagnosis

A thorough medical history and physical exam is necessary to make a diagnosis of Sjogren’s syndrome and to rule out other similarly presenting conditions. Frequently specialized physical and laboratory testing will be performed to help confirm the diagnosis.

Because the symptoms of Sjogren’s are similar to 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.

Common eye and mouth tests are

  • Schirmer test –This test measures tear production from the lacrimal glands. It is done by placing a thin strip of paper under the lower eyelid and measuring the amount of wetness on the paper after 5 minutes. Sjogren’s patients with have decreased tear production.
  • Staining with vital dyes (rose bengal or lissamine green) –The tests show how much damage dryness has done to the surface of the eye.
  • Slit lamp examination–This test, done by an ophthalmologist, shows how severe the dryness is and whether the outside of the eye is inflamed.
  • Mouth exam – The mouth will be examined for signs of dryness or for swelling of the salivary glands. Signs of dryness include a dry, sticky mouth, dental cavities, lack of salivary pooling or thick saliva, dry or cracked lips, and sores at the corners of the mouth.
  • Salivary gland biopsy of the lip – The doctor removes tiny minor salivary glands from the inside of the lower lip and examines them under the microscope for signs of Sjogren’s syndrome.

Because there are many causes of eye and mouth dryness, the doctor will take other possible causes into account. A definite diagnosis of Sjogren’s syndrome can be made with 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

  • Routine blood tests– Check blood count, blood sugar levels and liver and kidney function.
  • Immunological tests – Laboratory blood tests to check for antibodies commonly found in the blood of people with Sjogren’s syndrome. For example:SS-A (or SS-Ro) and SS-B (or SS-La), are specific antinuclear antibodies (ANAs) common in people with Sjogren’s syndrome. However, you can have Sjogren’s without having these ANAs. Other autoimmune blood testing may also be done.

Treatment

Treatment will vary on the individual’s symptoms and affected bodily organs. For the most prominent symptoms of eye and mouth dryness, the treatment is usually symptomatic. Detailed recommendations are detailed below.

For those with extraglandular involvement, your doctor – or the appropriate specialist – will also treat those problems. Treatment may include nonsteroidal anti-inflammatory drugs for joint or muscle pain, saliva- and mucus-stimulating drugs for nose and throat dryness, and corticosteroids or drugs that suppress the immune system for lung, kidney, blood vessel, or nervous system problems. Hydroxychloroquine, methotrexate, and cyclophosphamide are examples of such immunosuppressants (drugs that suppress the immune system).

Eye dryness

  • They first step is the use of artificial tear drops. Those not containing preservative are typically tolerated better. They come in different thicknesses, so one may need to experiment to find the right one.
  • Eye ointments, thicker than tear drops, provide increased moisture in the eyes and are typically used overnight.
  • Other eye drop options include Cyclosporine drops (Restasis) for more severe symptoms not improved with artificial tears. This is a prescription eye drop.
  • If conservative measures do not work, consultation with an ophthalmologist may be necessary for consideration for moisturizing implants or tear duct surgery.
  • Avoidance of drafts, breezes, and wind is helpful. Some recommend a humidifier in the rooms where you spend most of your time. Avoid smoking and smoky rooms.

Dry mouth

  • If your salivary glands still produce some saliva, you can stimulate them to make more by chewing gum or sucking on hard candy. Note that gum and candy must be sugar free due to the already increased risk for cavities. Take sips of water or another sugar free drink often throughout the day to wet your mouth, especially when you are eating or talking.
  • You can soothe dry, cracked lips by using oil- or petroleum-based lip balm or lipstick. If your mouth hurts, the doctor may give you medicine in a mouth rinse, ointment, or gel to apply to the sore areas to control pain and inflammation.
  • If you produce very little saliva or none at all, your doctor might recommend a saliva substitute.
  • There are at least two drugs that stimulate the salivary glands to produce saliva. These are pilocarpine and cevimeline. The effects last for a few hours, and you can take them three or four times a day. However, they are not suitable for everyone, so talk to your doctor about whether they might help you.
  • Be aware of mouth infections, specifically thrush, a fungal mouth infection, which appears as white patches inside the mouth that you can scrape off, or as red, burning areas in the mouth. It is treated with antifungal drugs.

Prevention of eye and mouth dryness

Certain drugs can contribute to eye and mouth dryness. However, don’t stop taking them without asking your doctor.

Drugs that can cause dryness include:

  • Antihistamines
  • Decongestants
  • Diuretics
  • Some anti-diarrheal drugs
  • Some antipsychotic drugs
  • Tranquilizers
  • Some blood pressure medicines
  • Antidepressants

Lymphoma Risk

About 5 percent of people with Sjogren’s syndrome develop cancer of the lymph nodes, or lymphoma. The most common symptom of lymphoma is a painless swelling of the lymph nodes in the neck, underarm, or groin. When lymphoma develops it often involves the salivary glands. Persistent enlargement of the salivary glands should be investigated further.

Other symptoms may include:

  • Unexplained fevers
  • Night sweats
  • Constant fatigue
  • Unexplained weight loss
  • Itchy skin
  • Reddened patches on the skin

These symptoms are not sure signs of lymphoma. They may be caused by other, less serious conditions, such as the flu or an infection. If you have these symptoms, see a doctor so that any illness can be diagnosed and treated as early as possible.

If you’re worried that you might develop lymphoma, talk to your doctor to learn more about the disease, symptoms to watch for, any special medical care you might need, and what you can do to relieve your worry.