An autoimmune disease is one when the healthy tissues and cells are mistakenly attacked by the body's own immune system. The immune system is designed to attack invaders of the body that do us harm - pathogens. A person with an autoimmune disease has abnormal antibodies circulating in the blood that attack healthy tissues as if they were harmful pathogens, such as infectious viruses or bacteria.
Doctors classify Sjogren's syndrome as either primary or secondary Sjogren's syndrome:
- Primary Sjogren's syndrome - the condition does not develop as a result of another condition, it is not the consequence of another condition.
- Secondary Sjogren's syndrome - the condition develops because of or alongside another condition, such as lupus or rheumatoid arthritis.
There are approximately 4 million people in the USA and half-a-million in the UK with Sjogren's syndrome.
What are the signs and symptoms of Sjogren's syndrome?A symptom is something the patient senses and describes, while a sign can be spotted by other people, such as a doctor. An example of a sign is a rash, while a symptom may be pain.
The most common symptoms associated with Sjogren's syndrome include dry eyes and dry mouth. Females may also report vaginal dryness. Dry eyes and dry mouth can be linked to other symptoms.
Signs and symptoms associated with dry mouth:
- Tooth decay, and eventual loss of teeth
- Persistent dry cough
- Problems chewing
- Problems swallowing
- Voice becomes hoarse
- Some people may have difficulty speaking
- Salivary glands become swollen
- Recurring oral thrush (fungal infection in the mouth)
- A sensation that there is something in the eye(s), such as sand or gravel
- Eyes become and look tired
- Itchy eyes
- Mucus discharge from the eyes
- Photophobia - sensitivity to light
- Stinging or burning eyes
- Swollen eyelids
- Irritated eyelids
In some cases, the patient's immune system may attack other parts of the body, causing the following signs and symptoms:
- General tiredness
- Aching muscles
- Inflammation of joints, as well as stiffness and pain
- Peripheral neuropathy - numbness and sometimes pain in the arms or legs
- Raynaud's phenomenon - hands feel painful, cold and numb
- Vasculitis - the blood vessels become inflamed
What are the causes of Sjogren's syndrome?Sjogren's syndrome is an autoimmune disease - the patient's immune system attacks healthy cells and tissues, mistaking them for dangerous pathogens, such as infectious bacteria or viruses.
Experts are not sure why Sjogren's syndrome affects some individuals, and not others. Studies have indicated that some viral or bacterial infections can trigger the disease.
Initially, with Sjogren's syndrome the salivary and tear glands are attacked and harmed by the immune system, resulting in less moisture production - less saliva in the mouth and tears in the eyes. The vaginal glands may also be affected, causing vaginal dryness.
The tear, salivary and vaginal glands are collectively known as the exocrine glands. Exocrine glands are glands of external secretion. They secrete a substance out through a duct. Examples include salivary, tear and sweat glands, as well as glands within the gastrointestinal tract.
However, other parts of the body may also be attacked and harmed, including the nerves, skin, joints, lungs, kidneys, and thyroid gland.
Primary Sjogren's syndrome - as mentioned before, experts are not sure what the exact cause of Sjogren's syndrome is. Most believe it is likely to be a combination of environmental and genetic factors. Some people are probably born with certain genes that make them more susceptible to having an abnormal immune system. Sometime during their life, an environmental factor, such as hepatitis C viral infection, or an infection with the Epstein-Barr virus may have triggered the immune system not to work properly.
As the majority of people who develop Sjogren's syndrome are female, and because the menopause is the most common time for a diagnosis to be made, doctors believe that estrogen, a female hormone, plays an important role. Possibly falling levels of estrogen might in some way disrupt the immune system, some experts theorize.
Secondary Sjogren's syndrome - the syndrome can develop as another autoimmune condition progresses, such as lupus or rheumatoid arthritis.
How is Sjogren's syndrome diagnosed?As Sjogren's signs and symptoms are similar to some other health conditions, it can sometimes be hard to diagnose. The patient may be seeing various health professionals, perhaps a dentist for dry mouth, an ob/gyn for dry vagina, and an ophthalmologist for dry eyes.
Some medications can also cause symptoms similar to those in Sjogren's syndrome.
Screening questions - the National Health Service (UK) offers these screening questions for those who are concerned they might have Sjogren's syndrome. If you answer "Yes" to most of the questions below, see your doctor. It does not mean you have the syndrome, but you may:
- Do you apply eye drops containing tears more than three times a day?
- Do you feel as if there were gravel or sand in your eyes?
- Has dry eyes bothered you persistently, every day, for over three months?
- Do the glands between your jaw and your ears (salivary glands) keep swelling?
- Do you often need to drink something when you are trying to swallow food?
- Has dry mouth bothered you continuously for over three months?
The Rose Bengal and Schirmer tests - usually done by an ophthalmologist.
- Rose Bengal test - measures the state and function of the tear glands. Rose Bengal, a non-toxic dye, is dropped onto the surface of the eyes. The dye's distinctive color helps the specialist determine how well the tear film is functioning and how long it takes for the tears to evaporate.
- The Schirmer test - strips of blotting paper are placed under the eyelid for about five minutes. They are removed and analyzed to determine how much liquid the paper absorbed.
Blood tests - when the immune system is affected by Sjogren's syndrome, there are special antibodies in the blood, known as SS-A and SS-B (or anti-Ro and anti-La). As these antibodies only show up in about 60% of Sjogren's syndrome patients, a negative result does not necessarily mean the syndrome can be ruled out.
Salivary flow rate - the patient is asked to spit as much as he/she can into a cup for about five minutes. By weighing the saliva, the doctor can determine whether the salivary glands are working properly. If not enough saliva is being produced, it could mean the patient has Sjogren's syndrome.
Sialogram - this is a special X-ray. A dye is injected into the parotid glands (behind the jaw and in front of the years). The aim is to determine how much saliva flows into the patient's mouth.
Salivary scintigraphy - a radioactive isotope is injected and tracked to measure salivary gland function.
Chest X-ray - this is to determine whether there is lung inflammation, which can be caused by Sjogren's syndrome.
Urine sample - the urine is tested to determine whether the kidneys have been affected.
What are the treatment options for Sjogren's syndrome?Artificial tears - artificial tears can usually treat moderate cases of dry eye effectively. They can be purchased at a pharmacy OTC (over the counter, no prescription required). If the eyes become severely irritated, the doctor may prescribe a short course of corticosteroid-containing eye drops. Long-term usage raises the risk of undesirable and serious side-effects, such as Cushing's syndrome.
Moisture chamber spectacles - these are special glasses that wrap around the eyes, a bit like goggles. They help keep the moisture in, and also protect the eyes from dust, particles and other irritants.
Surgery - small plugs seal the tear ducts which drain the tears away. If they are sealed tears will stay in the eye longer. This procedure is called punctual occlusion. It is generally only recommended if all else fails. Initially, the doctor will probably place temporary silicone plugs to see whether the procedure was successful. If it was, the silicone plugs are later replaced by permanent ones.
Keeping your mouth lubricated:
- Consume more liquids
- Ice cubes - sucking them helps keep the mouth lubricated
- Mouth rinses - regular mouth washing helps protect against infection and also soothes the mouth
- Oral hygiene - good oral hygiene prevents gum disease and tooth decay
- Smoking - give up. Smoking speeds up the rate at which saliva evaporates. Smoke irritates the mouth.
- Sugar free chewing gum - this stimulates the production of saliva
Pilocarpine - a medication for dry eyes and mouth. The drug stimulates the saliva and tear glands to produce more tears and saliva.
Hydroxychloroquine - a medication that slows the immune system's attack on saliva and tear glands. It may also help with symptoms of joint pain and stiffness, and muscle pain. Patients will not notice any effects for several weeks.
Vaginal dryness - lubricants, such as KY Jelly help. HRT (hormone replacement therapy) and using estrogen creams are also options.
Joint and muscle pains - OTC NSAIDs (non-steroidal anti-inflammatory drugs), such as ibuprofen are usually effective. The doctor may prescribe a stronger NSAID if needed.
Complications linked to Sjogren's syndromePatients with Sjogren's syndrome have a 44 times higher risk of developing non-Hodgkin's lymphoma than other people (about 5% of all patients).
People with Sjogren's syndrome have a higher risk of developing yeast infections, dental cavities, and having vision problems. Less commonly, there is also a risk of inflammation that may cause bronchitis, pneumonia, and other lung problems that may lead to kidney function problems, which in turn raise the risk of hepatitis or cirrhosis in the liver. In rare cases a pregnant woman with Sjogren's syndrome may give birth to a baby with heart problems. There is also a higher risk that the baby may develop lupus.
Peripheral neuropathy - Some patients develop a burning, tingling or numbing sensation in their hands and feet.
Written by: Christian Nordqvist